ANNUAL REPORT  
2023  
Orphazyme A/S  
Company registration no.: 32266355  
Lyskær 8A, DK-2730  
Herlev, Denmark  
 
Contents  
Management Review ........................................................................................................... 3  
State of Business.............................................................................................................................. 4  
Key Figures & Ratios......................................................................................................................... 5  
2023 Financial Review....................................................................................................................... 6  
Outlook........................................................................................................................................... 6  
Shareholder Information ................................................................................................................... 7  
Corporate Governance ...................................................................................................................... 8  
Risk Management............................................................................................................................10  
Corporate Social Responsibility..........................................................................................................10  
Data Ethics.....................................................................................................................................12  
Diversity in Management Positions.....................................................................................................12  
Board of Directors ...........................................................................................................................13  
Corporate information......................................................................................................................14  
Financial Statements......................................................................................................... 15  
2023 Financial Statements................................................................................................................16  
Notes to Financial Statements...........................................................................................................20  
Statements by Board of Directors and Executive Management...............................................................44  
Independent Auditors’ Report............................................................................................................45  
2
 
Management Review  
3
 
State of Business  
In February 2022, Orphazyme announced that it had been notified by the Committee for Medicinal Products for  
Human Use (CHMP) of the European Medicines Agency (EMA) of a negative trend vote as part of the ongoing  
review of the Marketing Authorisation Application (MAA) for its investigational product candidate, arimoclomol,  
for the treatment of Niemann-Pick disease type C (NPC).  
In May 2022, the Company announced that it had signed an agreement to sell substantially all of the  
Company’s assets and business activities, including those relating to the development and approval of  
arimoclomol and the full claw back liability related to the French early access program, to Zevra Denmark A/S,  
a wholly owned subsidiary of Zevra Inc. now known as Zevra Therapeutics (Zevra) for a total of USD 12.8  
million in cash and assumed liabilities estimated to equal approximately USD 5.2 million (the Sale of Assets).  
Under the terms of the agreement, Zevra agreed to acquire substantially all of Orphazyme’s assets and  
business activities, including those relating to the development and approval of arimoclomol, retain a majority  
of Orphazyme’s remaining Danish employees, continue the early access programs with arimoclomol and pursue  
the potential approval of arimoclomol as a treatment option for NPC.  
Since the Sale of Assets, the focus has been to ensure the smooth transition of assets to Zevra. Further, the  
Company has also continued to assist its legal representatives with a putative class action lawsuit in the United  
States.  
In 2022, the business operations and activities that were part of the Sale of Assets agreement with Zevra were  
reclassified as discontinued operations, but in 2023 all activities and financials are considered related to  
continuing operations.  
At the Annual General Meeting in May 2023, the Board of Directors withdraw their positions and was replaced  
by Michael Hove (Chairman), Jakob Have and Jakob Bendtsen as board members. In May 2023, it was  
announced that the board of directors and CEO/CFO Anders Fink Vadsholt had entered into a severance  
agreement pursuant to which Mr. Vadsholt would step down as CEO/CFO of the Company end of September  
2023, at which point it was announced that the Company had signed an CEO agreement with Jakob Bendtsen  
as the new CEO of the company. Jakob Bendtsen is also continuing in his role as board member.  
On July 9, 2021, a putative class action lawsuit was filed against the Company and certain of its current and  
former directors and officers in the United States District Court for the Northern District of Illinois. The lawsuit  
alleged that certain representations about arimoclomol in the Company’s U.S. IPO offering documents and in  
subsequent public statements were false and misleading, in violations of U.S. securities. While the  
Management at the time did not believe the class action lawsuit claims had any merit, the Company decided,  
for cost control reasons and because of the risks inherent in any litigation, to engage in settlement discussions.  
In May 2023, Orphazyme agreed with the plaintiffs on the US Security class action case to settle the case by  
Orphazyme paying a settlement amount. A written settlement document was filed by the parties to the Court  
which was approved in October 2023. Following the approval there has been a general 100-day objection  
period, where the consortia behind the class action could object to the settlement amount. The court did not  
receive any objections and the case was finally closed early February 2024. The settlement amount was USD  
2,5 million of which USD 0,5 million was covered by insurance leaving a net amount of USD 2 million which  
was paid in November 2023.  
The Company is in process to liquidate its subsidiaries in US and Switzerland and expect that the liquidation  
will be finalized in H1 2024. Following these liquidations, the Company will only consist of the Danish parent  
company.  
The final parts of the remaining activities have been handed over to Zevra, however the Company believes it is  
entitled to receive additional compensation for expenses incurred in the level of DKK 4.5 million. The company  
is currently investigating options to recover the amount from Zevra.  
The Company has carried out a number of initiatives to strengthen the capital structure and liquidity of the  
Company which includes a reverse stock split and capital reductions during November 2023 until January 2024.  
4
 
Please see Company Announcement 2/2024 for more information. The Company is still considering further  
initiatives to strengthen the capital structure including raising new capital.  
The Board has evaluated the Company's position, cash holdings and purpose and, as an extension of this, has  
decided that the primary strategy going forward for the company will be to successively raise capital to become  
a strategic investor in one or more partnerships and/or enter into a collaboration with either unlisted or listed  
biotech companies, where the company in partnership with the management will use its skills to develop the  
values in the companies.  
Throughout 2023, the board held meetings with various companies with a view to carrying out investments in  
biotech activities, including potential to entering into partnerships or collaboration with other companies where  
the Company could become a strategic co-owner. In February 2024 the Company announced the first  
investment in the Swedish listed company CombiGene AB via the purchase of 10 % of the shares in the  
company.  
The strategy will continuously require capital, and it may also be possible to issue new shares in Orphazyme as  
part of the payment that must be invested to become an active co-owner/partner in the selected companies.  
It is the board's preliminary assessment that this will best enable us to create value for the company's  
shareholders.  
Key Figures & Ratios  
TDKK  
2023  
2022  
2021  
2020  
2019  
Operating Loss  
(27,041)  
(41,241)  
(83,472)  
(608,534)  
(335,954)  
Net Financial income/expense  
Net Loss from continuing operations  
Net result from discontinued operations  
Net result for the period  
Total comprehensive profit/ loss  
Total profit/loss per share, basic (DKK)  
Total non-current assets  
Cash  
993  
(26,048)  
-
193  
(38,312)  
64,382  
26,070  
25,165  
713  
37  
(78,495)  
(548,044)  
(626,539)  
(626,841)  
(17,934)  
14,285  
(26,627)  
-
(7,043)  
-
-
-
(26,048)  
(26,048)  
(738)  
(633,246)  
(632,641)  
(22,187)  
38,829  
726,929  
56,735  
822,493  
34,698  
620,525  
166,627  
(337,497)  
(337,430)  
(16,884)  
32,529  
123,588  
19,137  
180,754  
19,984  
52,969  
65,988  
-
-
11,269  
8,043  
42,464  
15,658  
58,122  
35,312  
41,667  
16,357  
102,255  
56,689  
Other current assets  
Total assets  
19,312  
12,283  
14,242  
5,070  
173,229  
34,952  
Share capital  
Total equity  
9,339  
Total current liabilities  
129,092  
Cash flow from operating activities  
Cash flow from investing activities  
Cash flow from financing activities  
Net cash flow from discontinued operations  
(23,870)  
(6,944)  
(685)  
-
(117,945)  
90,347  
(602,571)  
46  
(539,076)  
(5,101)  
1,159,422  
-
(326,818)  
(3,285)  
58,939  
-
(35,078)  
32,862  
(30,344)  
(549,447)  
Share Price (DKK)1  
1,330  
35,312  
47.0  
74%  
403  
888  
35,312  
31.1  
68%  
1,180  
21  
17,160  
34,952  
600  
67,100  
28,514  
1,913  
75%  
72,400  
19,985  
1,447  
29%  
2,650  
74  
Total outstanding shares1  
Market capitalization (MDKK)2  
Equity ratio3  
5%  
Equity per share (DKK)4  
Average number of employees  
Number employees at year end  
267  
21,762  
117  
1
130  
1
1
62  
141  
86  
(1) Comparison figures for total outstanding shares and share price for 2019-2022 is adjusted for the reverse stock split in November 2023. (2)  
Market cap is calculated as the share price multiplied with the total outstanding shares as of the balance sheet date; (3) Equity ratio is calculated as  
the equity divided by the total assets as of the balance sheet date; (4) Equity per share is calculated as the total equity divided by the total  
outstanding shares as of the balance sheet date.  
Key figures & ratios for 2022 and 2021 are presented with a split of continuing and discontinued operations for P&L and cash flow to reflect  
restructuring and the Zevra transaction. 2020 and 2019 figures have not been restated since they represent a significantly different stage of the  
business and such presentation is not meaningful to an understanding of Orphazyme’s current business.  
5
 
2023 Financial Review  
Income statement  
The net result for the full year ended December 31, 2023 was an operating loss of DKK 26.0 million compared  
to a net operating loss of DKK 38.3 million for the same period in 2022 from continuing operations.  
The operational loss for 2023 was realized at DKK 27.0 million which is marginally better than our previously  
communicated outlook of a loss of DKK 30 35 million for the period.  
General and administrative (G&A) expenses totaled DKK 27.0 million in 2023, a reduction of approx. DKK 14.2  
million compared to the prior year (DKK 41.2 million in 2022). G&A expenses include costs associated with  
employees and Board of Directors, service providers and external assistance, legal and technology expenses.  
The difference primarily relates to lower costs in 2023 following the Sale of Assets to Zevra. G&A expenses  
from discontinued operations were DKK 59.5 million for the year ended December 31, 2022.  
Net financial income for the financial period ended December 31, 2023, was DKK 690 thousand compared to  
DKK 185 thousand for the same period in 2022. Net financial items from discontinued operations were an  
expense of DKK 5.0 million in 2022.  
Income tax was DKK 0 million compared to DKK 2.8 million in the same period in 2022 which included income  
tax benefits related to tax credits for R&D expenses at the applicable tax rate under the Danish Corporate  
Income Tax Act, which is no longer relevant for the Company due to the limited activities.  
Statement of financial position  
As of December 31, 2023, Orphazyme held cash of DKK 11.3 million as compared to DKK 38.9 million as of  
December 31, 2022. The cash position is marginally better than our previously communicated outlook for cash  
of DKK 6 10 million.  
As of December 31, 2023, total equity amounted to DKK 14.2 million compared to DKK 41.0 million as of  
December 31, 2022.  
Cash flows  
Net cash flow from operating activities amounted to an outflow of DKK 23.9 million for the full year ended  
December 31, 2023, compared to an outflow of DKK 102.6 million for the same period in 2022. Net cash flow  
from discontinued operating activities was an outflow of DKK 43.0 million in 2022.  
Net cash flow from investing activities amounted to an outflow of DKK 6.9 million in 2023 (cash inflow of DKK  
90.3 million in 2022).  
Net cash flow from financing activities amounted to an outflow of DKK 0.7 million in 2023 compared to an  
outflow of DKK 35.1 million in 2022.  
Outlook  
For the full-year 2024 we anticipate an operating loss in the range of DKK 3 4 million. There are inherent  
risks and uncertainties in our Outlook for 2024 including the limited nature of our business activities, potential  
investments and our future prospects.  
6
 
Shareholder Information  
Orphazyme’s shares are listed on Nasdaq Copenhagen (since November 16, 2017) under the ticker symbol  
ORPHA.  
We conduct our communications in accordance with the applicable rules and regulations required under Danish,  
and EU laws, including as set forth by the Danish Financial Supervisory Authority.  
As of December 31, 2023, Orphazyme has a total share capital of nominally DKK 12,283,333 divided into  
35,312 shares and representing a total of 35,312 voting rights.  
At the company’s extraordinary general assembly on November 2, 2023, it was decided to implement a reverse  
share split at a consolidation ratio of 1.000:1. The reverse stock split reduced the number of shares in the  
company so 1.000 current shares at a nominal value of 1 DKK was consolidated to 1 new share at a nominal  
value of 1.000 DKK.  
At an extraordinary general meeting on 30 November, 2023, it was adopted to lower the nominal value per  
share from DKK 1,000 to DKK 150. A nominal value per share below the share price after the reverse stock  
split takes effect will enable the board of directors with various options to optimize the capital structure of the  
company under its current authorizations.  
At the same extraordinary general it was also adopted to reduce the share capital with a total nominal value of  
DKK 30,015,200, which included a reduction of nominal DKK 23,028,667 for the purpose of covering losses and  
a reduction of nominal DKK 6,986,533 for the purpose of transfers to a special reserve.  
The reduction of DKK 6,986,533 did not take effect before January 9, 2024, after a four week proclamation  
period.  
As of December 31, 2023, the Company had approximately 1,984 registered shareholders. The Company has  
limited ongoing operational business activities and, as of the date of this annual report, does not expect to  
make dividend payments within the foreseeable future.  
As of the publication of this Annual Report the Company has the below major shareholders:  
-
-
-
-
MH Investment ApS, Dyrehavevej 47, 2930 Klampenborg owns 5,482 shares representing 15.52% of  
total share capital.  
Nordic Compound Invest A/S, Annexstræde 6, 2500 Valby owns 5,482 shares representing 15.52% of  
total share capital.  
Færch B Holding ApS, Stengårds Alle 243, 2860 Søborg owns 3,012 shares representing 8.53% of  
total share capital.  
LSP V Cooperatieve U.A., Johannes Vermeer, Plein 9, 1071 DV Amsterdam, Netherlands owns 2,426  
shares representing 6.87% of total share capital.  
7
 
Corporate Governance  
Orphazyme is committed to ensuring transparent and good corporate governance. As a Danish company listed  
on Nasdaq Copenhagen, Orphazyme is subject to the Danish Recommendations on Corporate Governance. The  
Recommendations on Corporate Governance are best practice guidelines for the management of companies  
admitted to trading on a regulated market.  
Orphazyme complies with the Recommendations on Corporate Governance where deemed relevant given  
Orphazyme’s current situation and focus.  
Orphazyme’s corporate governance statement includes Orphazyme’s position on the Recommendation on  
Corporate Governance as well as a complete list of the Company’s comments to recommendations that the  
Company opted to deviate from.The corporate governance statement is available under Corporate  
Governancein the Investors & Media section of our website: investors.orphazyme.com/corporate-governance.  
Board of Directors  
The Board of Directors is responsible for the overall management and strategic direction of Orphazyme’s  
business and operations and it supervises the Company’s activities, management, and organization. The Board  
of Directors appoints and dismisses the members of the Executive Management, who are responsible for the  
day-to-day management of the Company.  
The Board has chosen that the functions of the Audit Committee are exercised by the entire board of directors  
with Jakob Have as Chairman of the Audit Committee.  
Meetings  
The Board of Directors normally holds at least five regular meetings annually, including a strategy review, plus  
ad-hoc meetings as required. Extraordinary board meetings are convened by the Chairman when necessary or  
when requested by a member of the Board of Directors, a member of the Executive Management, or by the  
Company‘s auditor. There was a high frequency of meetings in 2023 due to the change in management,  
initiatives to strengthen the capital structure of the Company and US lawsuit. The Board of Directors forms a  
quorum when more than half of its members are represented, including the Chairman. Resolutions of the Board  
of Directors are passed by a simple majority of the votes present at the meeting. In the event of equal votes,  
the Chairman shall have the casting vote.  
The members of the Board of Directors elected by the general meeting are elected for a term of one year.  
Members of the Board of Directors may be re-elected.  
Orphazyme Board of Directors  
(1)  
Name  
Position  
Independent  
Year of first  
appointment  
2023  
Expiration of  
term  
Michael Hove  
Jakob Have  
Jakob Bendtsen  
Chairman  
Member  
Member  
Independent  
Independent  
2024  
2024  
2024  
2023  
2023  
Not Independent  
(1) According to the Danish Recommendations on Corporate Governance at least half of the members of the  
Board of Directors should be independent.  
8
 
Internal controls and financial reporting procedures  
The Board of Directors and the Executive Management are responsible for risk management and internal  
controls over its financial reporting and approve general policies in that regard. The Board of Directors is  
overseeing the reporting process and the most important risks involved in this respect. The Executive  
Management is responsible for the effectiveness of the internal controls and risk management and for the  
implementation of such controls aimed at mitigating the risk associated with the financial reporting.  
The Board of Directors and Executive Management assess risks on an on-going basis, including risks related to  
financial reporting, and assess measures to manage, reduce, or eliminate identified risks.  
Orphazyme has adopted and defined an internal control framework that identifies key processes, inherent  
risks, and control procedures in order to secure appropriate accounting processes. The control procedures  
include a variety of processes in order to prevent any misrepresentation, significant errors, omissions, or  
fraudulent behavior.  
Orphazyme’s independent auditors are appointed for a term of one year by the shareholders at the Company’s  
annual general meeting. The Board of Directors assesses the independence and competencies and other  
matters pertaining to the auditors. The framework for the auditors’ compensation and duties, including audit  
and non-audit tasks, is agreed annually between the Board of Directors and the auditors.  
At the company’s extraordinary general assembly on November 2, 2023, KPMG P/S was elected as new  
auditors replacing EY Godkendt Revisionspartnerselskab.  
9
 
Risk Management  
Our financial situation and risks are assessed on an ongoing basis and reported to the Board of Directors. The  
Company has currently only identified currency risks as relevant for the Company due to the limited ongoing  
business operations of Orphazyme.  
Financial risks may arise from changes in exchange rates. The Company are mostly exposed to foreign  
exchange movements relating to SEK, USD and EUR.  
Corporate Social Responsibility  
This section constitutes the Company’s statutory reporting according to Section 99a of the Danish Financial  
Statements Act.  
In May 2022, substantially all Orphazyme’s assets and business activities were sold to Zevra and we now have  
limited ongoing operational business activities and employees. Our headquarter is in Copenhagen, and we have  
non-operating subsidiaries in the U.S. and Switzerland which are expected to be liquidated in 2024.  
In light of such limited business operations, Orphazyme’s Corporate and Social Responsibility (CSR) risks are  
considered very limited. Our CSR activities for 2023 were commensurate with the size and limited operations of  
the Company, though we continued to strive to uphold our values and responsibilities towards society,  
employees, and our stakeholders. For 2024 we will continue, where possible, to fulfill our CSR obligations as  
we execute our strategy.  
CSR reporting areas  
Human Rights  
Risk: Very Limited: Orphazyme has limited operations, employees and suppliers.  
Actions: Continued to respect internationally declared human rights and did not employ child labor.  
Policies in place: Orphazyme acknowledges and supports the maintenance of internationally declared human  
rights and bases its work on the UN Universal Declaration of Human Rights and the interpretation that it is the  
responsibility of the State to protect, and the companies’ responsibility to respect, these rights. Orphazyme  
interprets human rights to comprise respect for diversity.  
Diversity policy.  
Whistleblower policy.  
Results:  
No diversity related incidents or human rights violations reported in 2023.  
Employee composition: Not meaningful given company structure (One employee as of December 31,  
2023; male).  
Leadership: Not meaningful given company structure (One employee as of December 31, 2023; male).  
The Company has set a target of increasing the representation of women on the Board of Directors to  
40% by 2026 in accordance with the guidelines from the Danish Business Authority. As at year end  
2023 the Board of Directors was comprised of three members, none of which were women.  
FuturePlans:  
Continue to support and respect internationally declared human rights and will not employ child labor.  
Aim to improve Board diversity in the future, subject to expansion of current business operations.  
10  
 
Anti-Corruption & Bribery  
Risk: Very Limited: We do not tolerate the use of bribery or corruption to achieve business objectives. Given  
that we have limited operations, employees and suppliers our anti-corruption and bribery risk is very limited.  
Actions: The Company is committed to maintaining the highest standards of conduct and will not tolerate the  
use of bribery or corruption to achieve its business objectives.  
Anti-corruption and bribery training conducted when employees start.  
Legal & Compliance training refreshers, including anti-corruption and bribery  
Policies in place: Our policies on bribery and corruption are clearly set out in our anti-corruption policy and  
our employee handbook.  
Results: No bribery and corruption violations identified or reported in 2023.  
Future Plans: Continue to maintain the highest standards of conduct and not tolerate the use of bribery or  
corruption to achieve business objectives.  
Environment & Climate  
Risk: Very limited: We have a very limited number of employees, minimal physical office presence and use  
external suppliers for certain activities such as administration, finance and legal activities which we believe  
have a low potential risk for impact on the environment & climate.  
Actions: Followed established procedures both during use and at disposal of hazardous substances.  
Policies in place: Considering the business of the Company, and its limited operations and employees,  
Orphazyme’s general potential impact on the environment and climate and the impact of the climate on  
Orphazyme’s business is viewed as minimal. Applicable rules and procedures were followed regarding the use  
of hazardous substances (no longer relevant due to closure of our laboratory space early in 2022) and we  
continue to endeavor to protect the environment and climate through mindful business practices such as, e.g.  
careful use of office materials and energy consumption.  
Results:  
Continued to keep records of all accidents in 2023.  
Recorded no records of spill of hazardous substances  
Continued to focus on efficient energy use and management of office materials in 2023.  
Future Plans: Orphazyme is no longer active in research and development activities and further with only  
limited operating activities, employees and suppliers focusing on administration, finance and legal activities,  
the general potential impact on the environment and climate and the impact of the environment and climate on  
Orphazyme’s business is viewed as minimal. We will continue to endeavor to protect the environment and  
climate through mindful business practices such as, e.g. careful use of office materials and energy  
consumption.  
Social / Employees  
Risk: Very Limited: As of December 31, 2023, Orphazyme had only one employee. The company continues to  
value diversity in gender, age, ethnicity, nationality, religion, education, sexual orientation, work history,  
perspectives, opinions, and skills at all levels of our business however given its limited employees it currently  
does not have a diverse workforce. Further, the Company’s limited operations, such as office space and  
support network, could impact the working conditions of remaining employees.  
Actions: The health and safety of our employees is of utmost importance and Orphazyme continually works to  
ensure that all systems and processes meet strict international standards. Continued to foster an open,  
trusting and inclusive workplace committed to freedom from discrimination, harassment, and bullying.  
Policies in place: Diversity Policy as well as Health and Safety Policies.  
11  
 
Results: Established a resilient culture centered on trust and collaboration.  
Future Plans: As of the date of this annual report, the Company has limited ongoing business activities and  
only one employee. Our future social / employee activities will be commensurate with the size and limited  
operations of the Company. We will continue to strive to uphold our values and responsibilities and promote a  
healthy, diverse and inclusive workplace, as we execute our strategy.  
Data Ethics  
We currently do not have a data ethics policy. Given the Company has limited operational business activities, it  
is no longer an integrated part of the Company’s business strategy or activities to process data or use  
algorithms for data analysis in connection with clinical trials, etc. However, our practices will be evaluated on  
an ongoing basis in order to ensure they align with the statutory requirements set forth in Section 99d of the  
Danish Financial Statements Act.  
Diversity in Management Positions  
Given the size of our workforce with only one employee, it is not meaningful to set out diversity figures nor is it  
required in accordance with the Danish Companies Act. This is also the case for the information requirements  
in section 107 d in the Danish Companies Act.  
Due to the evolution of the business, there were only three Board members at the end of 2023, all of which  
were men. The company remains committed to promoting a diverse and inclusive workplace and has therefore  
set a target of increasing the representation of women on the Board of Directors to 40% by 2026 in accordance  
with the guidelines from the Danish Business Authority.  
2023  
Board of directors  
Total number of members  
3
0%  
Underrepresented gender in %  
Target figure in % Net result from discontinued operations  
Year of target fulfilment  
40%  
2026  
Other Management levels  
Total number of members (all male)  
1
12  
 
Board of Directors  
Michael Hove, Chairman of the Board  
Member since:  
Born in:  
Nationality:  
2023  
1971  
Danish  
Special competencies: Strategy, Capital structure, MA-competences and turn-around of companies.  
Michael Hove holds a bachelor at CBS from 1995 and several degrees in management.  
Current positions: CEO MH Investment ApS, CEO Scandinavian Investment Company A/S, Chairman Antique  
89 Invest A/S. Large investor/advisor in a number of listed companies.  
Jakob Have  
Member since:  
Born in:  
Nationality:  
Committees:  
2023  
1981  
Danish  
Audit (Chair)  
Special competencies: Capital markets, accounting and financial management, tax and M&A.  
Jakob Have has previous experience from positions at various listed companies and holds a cand.merc.aud  
from 2007.  
Current positions: Jakob Have is currently member of the executive management of Nordic Compound Invest  
A/S, Nordic Compound Management A/S and Nordic Compound A/S and a board member of Scandinavian  
Medical Solutions A/S, Scandinavian Investment Company A/S, Nordic Compound A/S and Nordic Compound  
Management A/S.  
Jakob Bendtsen  
Member since:  
Born in:  
Nationality:  
2023  
1978  
Danish  
Special competencies: Compliance, accounting, financial management, tax and M&A.  
Jakob Bendtsen has previous experience from positions at various listed companies and as external consultant  
and advisor and holds a cand.merc.aud from 2004.  
Current positions: Jakob Bendtsen is currently member of the executive management of Færch Family Invest  
and Consulting ApS, Cewijo ApS, Færch B Holding ApS, Færch Ejendomsservice ApS and Accounti ApS.  
Further, Jakob Færch Bendtsen is a member of the board of directors of Nordic Compound Invest A/S.  
13  
 
Executive Management  
Jakob Bendtsen, Chief Executive Officer  
Jakob Bendtsen took the position of Chief Executive Officer, in addition to his role as Board Member, October 1,  
2023.  
Corporate information  
Annual Report  
This annual report will be available on www.orphazyme.com and printed copies are available upon request.  
Annual General Meeting  
Information about our Annual General Meeting can be found in the section for Investors & Media at  
www.orphazyme.com under Investors and General Meetings.  
14  
 
Financial Statements  
15  
 
2023 Financial Statements  
STATEMENTS OF PROFIT OR LOSS AND OTHER COMPREHENSIVE INCOME  
For the years ended December 31  
DKK 000, except per share and share data  
General and administrative expenses  
Note  
2.3  
2023  
(27,041)  
2022  
(41,241)  
Operating loss  
(27,041)  
993  
(41,241)  
185  
Financial income  
2.6  
2.6  
Financial expenses  
Loss before tax  
(26,048)  
(41,056)  
2,750  
Income tax benefit  
2.7  
1.7  
Loss from continuing operations  
Net result from discontinued operations  
(26,048)  
(38,306)  
73,378  
Net result for the year  
(26,048)  
35,072  
Items that will be reclassified subsequently to the Statement of  
Profit or Loss:  
Exchange difference from translation of foreign operations  
-
-
(141)  
(141)  
Other comprehensive profit/loss  
Total comprehensive profit/loss  
(26,048)  
34,931  
Weighted-average shares outstanding  
Weighted-average shares outstanding, dilutive  
35,312  
35,518  
35,269  
35,269  
Profit/ loss per share from continuing operations, (DKK)  
Profit/ loss per share from discontinuing operations, (DKK)  
Profit/ loss per share, basic (DKK)  
(738)  
(1,086)  
2,081  
995  
4.3  
4.3  
(738)  
(733)  
Profit/ loss per share, diluted (DKK)  
995  
16  
 
STATEMENTS OF FINANCIAL POSITION  
As of December 31,  
DKK 000  
ASSETS  
Current assets  
Note  
2023  
2022  
5,500  
Corporation tax receivable  
Trade receivables  
2.8  
3.5  
3.4  
3.7  
3.7  
4,103  
8,305  
Prepayments and other receivables  
Securities  
1,099  
6,944  
Cash  
11,269  
19,312  
38,918  
56,826  
Total current assets  
Total assets  
19,312  
56,826  
EQUITY AND LIABILITIES  
Equity  
Note  
2023  
2022  
Share capital  
Share premium  
Other reserves  
Retained earnings  
4.2  
3.6  
12,283  
1,959  
14,242  
35,312  
2,087,437  
(303)  
(2,081,472)  
40,974  
Total equity  
Non-current liabilities  
Other non-current liabilities  
Total non-current liabilities  
Current liabilities  
Trade payables and accruals  
Discount and rebate liabilities  
Other liabilities  
98  
98  
3.6  
3.6  
3.6  
5,070  
9,206  
4,457  
2,090  
Total current liabilities  
Total equity and liabilities  
5,070  
19,312  
15,753  
56,826  
17  
 
STATEMENTS OF CHANGES IN SHAREHOLDERS’ EQUITY  
DKK 000  
Share-based  
compensation  
acquisition  
of intangible  
assets  
Foreign  
currency  
transla.  
reserve  
Share  
capital  
Accumulated  
Deficit  
Share premium  
2,082,487  
Total  
(1,121)  
Balance as of December 31, 2021  
34,952  
(167)  
2,486  
(2,120,884)  
Net loss for the year  
(141)  
(141)  
35,072  
35,072  
(141)  
Other comprehensive income  
Total other comprehensive income (loss)  
Transactions with owners:  
Capital increase  
35,072  
34,931  
360  
5,091  
5,451  
(141)  
Transaction cost related to capital increase  
Reserve for bonus shares reversed  
Share-based compensation expense  
Total transactions with owners  
Balance as of December 31, 2022  
Total other comprehensive income (loss)  
Reclassification  
(141)  
(2,486)  
2,486  
4,950  
2,087,437  
1,853  
1,853  
7,163  
40,974  
(26,048)  
360  
35,312  
(2,486)  
4,339  
(303)  
(2,081,472)  
(26,048)  
(303)  
303  
Transactions with owners:  
Purchase and sale of shares  
Capital reduction  
(23,029)  
(684)  
23,029  
(684)  
Reclassification  
(2,087,437)  
(2,087,437)  
2,087,437  
2,109,782  
1,959  
Total transactions with owners  
Balance as of December 31, 2023  
(23,029)  
12,283  
(684)  
14,242  
18  
 
STATEMENTS OF CASH FLOWS  
For the years ended December 31,  
DKK 000  
Note  
2023  
(27,041)  
2022  
(41,241)  
Operating result from continuing operations  
Operating result from discontinuing operations  
(67,278)  
Adjustments to reconcile operating result to cash  
flows from operating activities:  
Equity-settled share-based compensation expense  
Depreciation and amortization  
2.5  
1,773  
782  
2.2,2.3  
3.4, 3.5  
3.6  
Change in prepayments, deposits, and other receivables  
Change in trade payables, accruals, and other liabilities  
Change in intercompany receivables  
7,125  
(10,124)  
41,393  
(37,889)  
4,183  
(657)  
1,117  
(7,794)  
5,500  
Change in intercompany payables  
Corporation taxes received  
Interest received  
5,500  
993  
185  
Interest paid  
(1,064)  
(117,945)  
Cash flow from operating activities  
(20,021)  
Investing activities  
Proceeds from sale of property, plant and equipment  
1,460  
Proceeds from sale of activity  
Purchase of securities  
1.7  
3.2  
88,887  
(6,944)  
Cash flow from investing activities  
(6,944)  
90,347  
Financing activities  
Repayment of borrowings  
(39,155)  
(1,233)  
5,451  
Repayment of lease obligations  
Proceeds from issuance of shares  
Purchase of shares in connection with reverse stock split  
Sale of shares  
(3,020)  
2,335  
Transaction cost related to issuance of shares  
(141)  
Cash flow from financing activities  
(685)  
(35,078)  
Net change in cash and cash equivalents  
Effects of changes in exchange rates  
Cash at the beginning of the year  
Cash at the end of the year  
(27,650)  
1
(47,298)  
523  
38,918  
11,269  
85,692  
38,918  
19  
 
Notes to Financial Statements  
SECTION 1  
Basis of preparation and significant accounting policies  
1.1 CORPORATE INFORMATION  
Orphazyme A/S (the “Company”) is headquartered in Copenhagen, Denmark and is publicly traded on Nasdaq  
Copenhagen.  
In April 2018, a wholly-owned subsidiary, Orphazyme U.S., Inc., was incorporated in Delaware, USA and in  
March 2020, a wholly-owned subsidiary, Orphazyme Schweiz GmbH, was incorporated in Zug, Switzerland  
(together with Orphazyme A/S, “Orphazyme” or “the Company”). These companies are currently in the process  
of being dissolved by solvent liquidations.  
Orphazyme was previously involved in the research and development of novel therapeutics for the treatment of  
neurodegenerative rare diseases. In May 2022, Orphazyme sold substantially all of its assets and business  
activities to Zevra who retained all of Orphazyme’s remaining Danish employees. The disposed assets and  
business activities were presented as discontinued operations in 2022.  
As of the date of publication of this annual report, Orphazyme has defined a new strategy and has currently  
entered into a strategic partnership with the Swedish listed company CombiGene AB.  
These financial statements were approved and authorized for issuance by the Board of Directors on March 27,  
2024.  
1.2  
BASIS OF PREPARATION  
The financial statements have been prepared in accordance with International Financial Reporting Standards  
(IFRS) as adopted by the EU and additional requirements of the Danish Financial Statements Act.  
The financial statements have been prepared on a going concern basis and are presented in Danish Kroner, or  
DKK, which is both the functional and presentation currency of the Company. Where indicated, amounts are  
rounded to the nearest thousand.  
The financial statements are prepared based on the concept of materiality, which considers both quantitative  
and qualitative factors. Items that are considered individually significant or are required under the minimum  
presentation requirements of IFRS are presented separately. If items are individually immaterial, they are  
aggregated with other items of similar nature in the financial statements or in the notes.  
1.3  
SIGNIFICANT ACCOUNTING POLICIES  
A detailed description of accounting policies and significant accounting estimates and judgements related to  
specific financial statement line items is presented in each note to the relevant line item. The financial  
statements have been prepared on a historical cost basis except for share-based compensation and securities,  
which is measured at fair value.  
Principles of consolidation  
The financial statements include the financial statements of the parent company, Orphazyme A/S (the “Parent  
Company”), Orphazyme US, Inc. and Orphazyme Schweiz GmbH, fully-owned subsidiaries over which the  
Parent Company has control.  
Pursuant to the exemption clause for the Group in section 110 of the Danish Financial Statements Act, the  
annual report of Orphazyme A/S does not comprise consolidated financial statements.  
Management determined that the Group falls into the reporting Class B in the financial year 2023 and 2022  
applying the size limits per the Danish Financial Statements Act.  
Translation of foreign currencies  
On initial recognition, transactions denominated in foreign currencies are recorded using the foreign exchange  
spot rate at the transaction date. For monetary assets and liabilities, differences arising between the foreign  
exchange spot rates at the transaction date and the date of settlement or period-end exchange rates are  
recognized in the Statement of Profit or Loss as financial income or financial expenses.  
Statement of cash flows  
The statement of cash flows is presented using the indirect method and shows cash flows resulting from  
operating activities, investing activities, financing activities, and the cash at the beginning and end of the year,  
including any effects of exchange rate changes.  
20  
 
Cash flows used in operating activities converts items in the Statement of Profit or Loss from the accrual basis  
of accounting to the cash basis of accounting. Non-cash items such as foreign exchange gains and losses,  
depreciation, amortization, and changes in working capital are reversed from the net result for the year and  
actual cash receipts and payments are included.  
Cash flows from investing activities shows payments related primarily to the purchase of licenses and property,  
plant, and equipment and sale of activity.  
Cash flows from financing activities shows proceeds from share issuance, borrowings, net of transaction costs,  
repayment of debt, and lease payments.  
Discontinued operations  
Discontinued operations in 2022 are excluded from the results of continuing operations and are presented as a  
single amount as profit or loss after tax from discontinued operations in the statement of profit or loss.  
Additional disclosures are provided in Note 1.7. All other notes to the financial statements include amounts for  
continuing operations, unless indicated otherwise.  
Segment information  
The Company is managed and operated as one business unit that is reflected in the internal reporting. No  
separate lines of business or separate business entities have been identified with respect to any product  
candidate or geographical market and no segment information is currently disclosed in the internal reporting.  
1.4 SIGNIFICANT ACCOUNTING ESTIMATES AND JUDGEMENTS  
The use of reasonable estimates and judgements is an essential part of the preparation of the financial  
statements. Given the uncertainties inherent in the Company’s business activities, Management must make  
certain significant accounting estimates and judgements, which affect the application of accounting policies and  
therefore the reported amounts of assets, liabilities, expenses, and disclosures in the consolidated financial  
statements and parent company financial statements. The significant accounting estimates and judgements  
identified are those that have a significant risk of resulting in a material adjustment to the financial statements.  
Management bases its estimates on historical experience, assumptions, and information currently available and  
deemed to be reasonable at the time the financial statements are prepared. However, actual amounts may  
differ from the estimated amounts as more detailed information becomes available. Estimates and assumptions  
are reviewed on an ongoing basis and, if necessary, changes are recognized in the period in which the estimate  
is revised. Management has made significant accounting estimates and judgements in the following areas,  
which are further presented in each note to the relevant financial statement line items:  
Estimate of inputs and assumptions used in share-based compensation valuation models (Note  
2.5)  
Judgement regarding the recognition of deferred tax assets related to taxable losses to be carried  
forward (Note 2.7)  
Please refer to the specific referenced notes for further information on the significant accounting estimates and  
judgements as well as assumptions applied.  
1.5 NEW IFRS STANDARDS APPLICABLE TO THE COMPANY  
The Company has implemented the standards and amendments that are effective for the financial year 2023.  
The new standards and amendments have not affected the Companys recognition or measurement for 2023,  
nor are they expected to have significant future impact.  
The IASB has issued a number of new standards and updated some existing standards, which are effective for  
accounting periods beginning January 1, 2024 or later. Therefore, they are not incorporated in these financial  
statements. There are no standards presently known that are not yet effective and that would be expected to  
have a material impact on our current or future reporting periods.  
1.6 SIGNIFICANT EVENTS AFTER THE REPORTING PERIOD  
In May 2023, Orphazyme agreed with the plaintiffs on the US Security class action case to settle the case by  
Orphazyme paying a settlement amount. A written settlement document was filed by the parties to the Court  
which was approved in October 2023. Following the approval there has been a general 100-day objection  
period, where the consortia behind the class action could object to the settlement amount. The court did not  
receive any objections and the case was finally closed early February 2024. The settlement amount was USD  
2,5 million of which USD 0,5 million was covered by insurance leaving a net amount of USD 2 million which  
was already paid in November 2023.  
No other significant events after the reporting period to disclose.  
21  
 
1.7 DISCONTINUED OPERATIONS AND DISPOSALS COMPANY HELD FOR SALE  
In May 2022, Orphazyme announced that it had signed an agreement to sell substantially all of the Company’s  
assets and business activities, including those relating to the development and approval of arimoclomol and the  
full claw back liability related to the French early access program, to Zevra.  
The Sale of Assets agreement for a total of USD 12.8 million in cash and assumed liabilities estimated to equal  
approximately USD 5.2 million (the Sale of Assets). Under the terms of the agreement, Zevra agreed to also  
retain a majority of Orphazyme’s employees.  
The business operations and activities that were part of the Sale of Assets agreement with Zevra have been  
reclassified as discontinued operations in 2022 -, which is presented below-. The net result related to the  
above-mentioned operations held for sale are therefore presented separately in the income statement and the  
statement of cash flows.  
Judgement regarding management’s accounting for discontinued operations  
Significant judgment is required to determine the presentation of discontinued operations in profit and loss and  
cash flow, for both the current and prior year. Management has done the judgement based on the Purchase  
agreement from the sale of assets to Zevra.  
Substantially all of Orphazyme’s assets and business activities was sold to Zevra (the “Sale of Assets”) on an  
“as-is” basis and there have not been given any substantial representations or warranties in favor of Zevra.  
While the management are not aware of any outstanding matters that would reasonably form a basis for a  
claim related to the Sale of Assets, if the Company become subject to liability based upon our contractual  
obligations to Zevra or otherwise, it could have a material adverse effect on our financial position.  
Net result and net cash flow from operations held for sale and assets and liabilities in disposal Company held  
for sale are specified below:  
For the years ended December 31,  
DKK 000  
Net revenue  
Note  
2.1  
2023  
2022  
17,867  
-
-
-
-
-
-
-
-
Research and development expenses  
General and administrative expenses  
Operating loss  
2.2  
2.3  
(36,660)  
(48,485)  
(67,278)  
4,106  
Financial income  
2.6  
2.6  
Financial expenses  
(9,116)  
(72,288)  
Loss before tax  
Income tax benefit  
2.7  
Net result for the period  
-
-
-
(72,288)  
145,666  
73,378  
Gain from disposal of assets and liabilities held for sale  
Net result from discontinued operations  
DKK 000  
Note  
2023  
2022  
Cash flow from operating activities  
Cash flow from investing activities  
Cash flow from financing activities  
Net cash flow from operations held for sale  
-
-
-
-
(6,973)  
90,347  
(40,388)  
42,986  
22  
 
Gain on disposal/carrying amount of disposed assets  
DKK 000  
Carrying values of intangible assets  
Note  
2023  
2022  
1,603  
-
-
-
-
-
-
-
Carrying values of property, plan and equipment  
Carrying values of liabilities  
134  
(58,370)  
(56,633)  
145,520  
145,520  
88,887  
Carrying values of assets and liabilities  
Gain on disposal net  
Gain on disposal  
Net cash inflow from disposal of business  
2.1  
NET REVENUE  
§ ACCOUNTING POLICIES  
Orphazyme recognizes revenue when fulfilling its performance obligation by transferring control of promised  
goods or services to its customer, in an amount that reflects the consideration that the entity expects to  
receive in exchange for those goods or services.  
Due to the sale of activities to Zevra in 2022, the Company did not have any revenue in 2023. Net revenue in  
2022 comprises revenue from the sale of arimoclomol for the treatment of NPC under the remunerated early  
access compassionate use program (“nATU”) in France. An early access compassionate use program is a  
program giving specific patients access to a drug, which is not yet approved for commercial sale. Only drugs  
targeting serious or rare indications and for which there is currently no appropriate treatment are considered  
for early access compassionate use programs. Further, to be considered for the early access compassionate  
use program, the drug must have proven efficacy and safety and must either be undergoing price negotiations  
or seeking marketing approval.  
As of June 2022, the remunerated early access compassionate use program (“nATU”) in France, was  
transferred to Zevra as part of the sale of substantially all of the Company’s assets and business activities. All  
revenue is therefore presented as discontinued operations (Note 1.7).  
The following table presents net revenue for the years ended December 31:  
DKK 000  
Revenue by type  
2023  
2022  
17,867  
Revenue from sale of goods  
Revenue by partner  
Clinigen Health Limited  
Geographical areas  
France  
17,867  
17,867  
2.2  
RESEARCH AND DEVELOPMENT EXPENSES  
§ ACCOUNTING POLICIES  
Research expenses comprise of costs incurred during the very early stages of the drug development cycle from  
initial drug discovery until the drug is ready for administration to humans.  
Research and development expenses include costs arising from research and clinical development activities  
including employee costs for research and development personnel (i.e. salaries, bonuses, employer  
contributions to pension schemes, share-based compensation), legal expenses related to the protection,  
defense and enforcement of the Company’s intellectual property, depreciation of right-of-use assets associated  
with facilities and equipment used for research and development purposes, as well as close down and  
restructuring costs for clinical trial close-out costs and employee redundancies. The following table presents  
research and development expenses recognized for the years ended December 31:  
DKK 000  
External costs  
Employee costs (Note 2.4)  
2023  
2022  
18,385  
17,926  
Depreciation, amortization and impairment (Notes 3.1, 3.2,  
3.3)  
Total research and development expenses  
349  
36,660  
23  
 
External costs comprise mainly expenses related to third party vendors providing services related to our  
research and development activities and facility costs. Research and development expenses include costs  
relating to products sold under the French early access compassionate use program.  
As of June 2022, all research and development activity were transferred to Zevra as part of the sale of  
substantially all of the Company’s assets and business activities. All research and development expenses are  
therefore presented as discontinued operations (Note 1.7) in 2022.  
As a result of the Sale of Assets in 2022, there are no research and development expenses in 2023.  
2.3  
GENERAL AND ADMINISTRATIVE EXPENSES  
§ ACCOUNTING POLICIES  
General and administrative expenses include salaries for our employees working on pre-launch preparation  
activities as well as administrative employees and Executive Management; remuneration to the Board of  
Directors; share-based compensation costs related to such employees and the Board; depreciation of right-of-  
use assets associated with facilities not used for research and development purposes, investor relations, and  
accounting and legal fees. In addition, we include costs incurred in pre-launch preparation activities such as  
market access, marketing, and medical affairs in general and administrative expenses, including the costs  
associated with the Early Access Program for NPC in the U.S., tradename costs, market and pricing studies and  
related costs.  
The following table presents general and administrative expenses for the years ended December 31:  
DKK 000  
Intercompany expense  
2023  
2022  
8,944  
External costs  
5,057  
7,827  
14,157  
59,650  
20,699  
433  
89,726  
Employee costs (Note 2.5)  
Settlement of U.S. lawsuit  
Depreciation (Notes 3.2 and 3.3)  
Total general and administrative expenses  
27,041  
As of June 2022, general and administrative activity was partly transferred to Zevra as part of the sale of  
substantially all of the Company’s assets and business activities. General and administrative expenses were  
therefore in 2022 presented as both continued operations, DKK 41.2 million and discontinued operations (Note  
1.7), DKK 59.5 million.  
2.4  
EMPLOYEE COSTS  
§ ACCOUNTING POLICIES  
Employee costs primarily comprise salaries, bonuses, social security contributions, share-based compensation,  
vacation and sick leave as well as the employer portion of pension contributions. In addition, severance  
payments or termination benefits are also included under Employee Costs. The cost of these benefits is  
recognized as an expense as services are received. All employee pension plans are defined contribution plans  
and not defined benefit plans.  
Employees are eligible to receive a discretionary bonus subject to certain predefined and individual goals as  
determined by the Board of Directors. Employees are also eligible to receive an extraordinary bonus at the  
discretion of the Board of Directors.  
The following table presents Employee Costs, including remuneration to the Board of Directors and Executive  
Management, for the years ended December 31, 2023. Refer to note 4.5 for more discussion on remuneration  
of Board of Directors and Executive Management.  
24  
 
DKK 000  
Employee costs  
Salaries  
Cash bonus  
Share-based compensation  
Pension  
Other social security contributions  
Other staff costs  
Total employee costs excluding board remuneration  
Board remuneration (Note 4.5)  
Board share-based compensation (Note 4.5)  
Total employee costs  
2023  
2022  
21,488  
5,623  
9,046  
1,853  
1,588  
108  
1,474  
35,557  
3,148  
(80)  
(310)  
400  
7
258  
5,978  
1,724  
125  
7,827  
38,625  
Recognized as follows in the statement of Profit or Loss  
Research and development expenses (Note 2.2.)  
General and administrative expenses  
17,926  
20,699  
38,625  
7,827  
7,827  
Total employee costs  
Average number of full-time employees  
Year-end number of full-time employees  
1
1
19  
1
As of June 2022, substantially all of the Company’s assets and business activities were transferred to Zevra as  
part of the sale. Employee costs were in 2022 therefore presented as both continued operations, DKK 10.5  
million and discontinued operations (Note 1.7), DKK 36.0 million.  
2.5  
SHARE-BASED COMPENSATION COSTS  
§ ACCOUNTING POLICIES  
Equity-settled awards  
Shares awarded under the long-term incentive program (“LTIP”) are equity-settled awards. The fair value of  
these awards is determined at the date of grant, resulting in a fixed fair value at grant date that is not adjusted  
for future changes in the fair value of the awards that may occur over the service period. The fair value of the  
LTIP awards has been determined using the Black-Scholes or Monte-Carlo model depending on the terms and  
conditions of the respective award. Further details of the valuation models are presented below.  
The fair value of equity-settled awards with service conditions and non-market performance conditions is  
recognized as compensation expense pro rata over the service period to the extent such awards are estimated  
to vest. The compensation expense is recognized together with a corresponding increase in equity over the  
period in which the performance and/or service conditions are fulfilled. The cumulative expense for the  
Company’s share-based compensation awards recognized at each reporting date until the vesting date reflects  
the extent to which the vesting period has expired and Management’s best estimate of the number of  
instruments that will ultimately vest. The expense or credit in the Statement of Profit or Loss for a period  
represents the movement in cumulative expense recognized as at the beginning and end of that period.  
In the event that equity instruments are granted conditionally upon an equal number of equity instruments  
granted in prior periods not being exercised, they are treated as a new grant for the current period and a  
modification of the equity instruments granted in the prior period.  
When the terms of an equity-settled award are modified, the minimum expense recognized is the grant date  
fair value of the unmodified award, provided that the original terms of the award are met. An additional  
expense, measured as at the date of modification, is recognized for any modification that increases the total  
fair value of the share-based payment transaction, or is otherwise beneficial to the employee. Where an award  
is cancelled by the entity or by the counterparty, any remaining fair value of the award is expensed  
immediately in the Statement of Profit or Loss.  
Cash-settled awards  
The phantom share-based incentive programs established by the Company are settled in cash and are treated  
as cash-settled awards. Similarly, as the Restricted Share Units (RSU) awards to the board of directors may be  
settled in cash or in shares at the choice of the participant, they are also treated as cash-settled awards. If the  
RSUs are ultimately exercised by the holder and settled in equity, the amount accrued as a liability is settled  
by reversing it into equity.  
A liability is recognized for the fair value of cash-settled awards, measured initially and at each reporting date  
up to and including the settlement date, with changes recognized through profit or loss at each reporting date.  
The fair value is expensed over the period until vesting date with recognition of a corresponding liability. The  
fair value is determined using the Monte-Carlo model, further details of which are presented below. The fair  
value of the cash-settled awards, which vest subject to obtaining a specified share price (i.e. market  
25  
 
condition), is reported as compensation expense regardless of whether the share price condition is met if all  
other vesting conditions are met. For these awards, fair value is determined taking into account the probability  
of meeting the share price target. No expense is recognized for awards that do not ultimately vest. If the RSUs  
are finally exercised, the related liability is reclassified as equity.  
Summary of share-based compensation 2023  
In October 2023, the Company initiated a new share-based incentive program for the Board of Directors. The  
program comprised Restricted Share Units (“RSUs”) which entitle the participants, subject to vesting occurring,  
to be allocated a number of shares in the Company, equivalent to the number of vested RSUs, against  
payment of the nominal value of each share. The RSUs will have a vesting period from the date of grant and  
until approval of the annual report at the annual general meeting in the following calendar year and is  
therefore aligned with the one-year election period. Vesting of the RSUs is not conditional on any financial  
performance criteria, however vesting will be conditional upon the Participant’s continued membership of the  
Board of Directors during the entire Vesting Period. The vested RSUs can only be exercised within twelve  
months after the expiration of the total vesting period. However, the delivery period may be extended to the  
next open trading window in certain circumstances. The program comprises up to 825 shares in total.  
The program in 2023 has a fair value per unit of 304.56, expected volatility of 115.4%, risk-free interest rate  
of 2.5% and share price of 979 on the grant date.  
The fair value of all RSUs was calculated using a Black-Scholes valuation model with the inputs shown in the  
following table. As the RSUs may be settled in cash, we have re-valued them as of year-end with updated  
inputs and recognized a cumulative share-based compensation income in the amount of DKK 0.1 million (2022:  
0.3 million) and a corresponding short-term liability as of December 31, 2023. The Exercise Period for all 2023  
RSUs is one year following full vesting and for valuation purposes we have assumed exercise three months  
upon full vesting.  
The following amounts were recognized as share-based compensation for the years ended December 31  
DKK 000  
2023  
2022  
Share-based compensation included in R&D  
Share-based compensation included in G&A  
Total share-based compensation expense recognized  
126  
126  
1,773  
1,773  
All descriptions below in the remaining part of this note (2.5 SHARE-BASED COMPENSATION COSTS) relates to  
prior years programs, which are all lapsed at December 31, 2023. Part of the programs described below were  
still valid by the end of the comparison year 2022, and hence we have kept the description.  
Estimate of inputs and assumptions used in share-based compensation valuation models  
All references to share price relate to the Company’s share price on Nasdaq Copenhagen.  
a) Long-term incentive program (equity-settled)  
In connection with the completion of the Company’s initial public offering (IPO) on Nasdaq Copenhagen in  
November 2017, the Executive Management and Key Employees were offered to subscribe for Offer Shares  
(“Investment Shares”).  
Under the post-IPO long-term incentive program (2017 LTIP), the Executive Management as well as certain  
Key Employees of Orphazyme had subscribed to 14,875 ordinary shares (Investment Shares). In 2018, a Key  
Employee subscribed to 4,300 Investment Shares.  
The participants in the 2017 LTIP had the opportunity to be allocated a number of shares in Orphazyme  
(“Performance Shares”) at a price per Performance Share of DKK 1 at the end of a vesting period of four years  
from Orphazyme’s first day of trading and official listing on Nasdaq Copenhagen. The number of Performance  
Shares should be proportional to the potential increase in the price of Orphazyme’s shares at the time of  
exercise compared to the offer price. Performance Shares was allocated on a linear scale with maximum  
allocation triggered by an 80% increase in share price, whereas no Performance Shares would be allocated if  
the price of Orphazyme’s shares has increased 20% or less at the end of the vesting period. The vesting period  
ended in November 2021 with no performance shares granted as the minimum increase of 20% over the  
vesting period was not met.  
In July 2019, the Company initiated a 2019 long-term investment program (2019 LTIP) for the Executive  
Management and certain Key Employees with the same terms and conditions as the 2017 LTIP, i.e. Matching  
Shares vesting over one year and Performance Shares vesting over four years, respectively, and vesting  
among other things also being subject to the participants having maintained ownership of their Investment  
Shares and continued employment. The maximum number of Performance Shares that can vest in July 2023 as  
part of the 2019 LTIP is 125,000. The vesting period ended in July 2023 with no performance shares granted  
as the minimum increase of 20% over the vesting period was not met.  
26  
 
In July 2020, the Company initiated a 2020 long-term investment program (2020 LTIP) for the Executive  
Management and certain other employees with the same terms and conditions as the 2017 LTIP and the 2019  
LTIP. However, in case of termination of a participant’s employment and designation as a Good Leaver, the  
right to receive Matching Shares and Performance Shares will be prorated and calculated through the date of  
notice of termination. During 2020, awards were granted on four different grant dates shown in the table  
below. Matching Shares for all awards granted under the 2020 LTIP was fully vested on January 1, 2021. The  
maximum number of Performance Shares that can vest in January 2024 as part of the 2020 LTIP is 489,757.  
In April 2021, the Company initiated a 2021 new long-term share-based incentive program (original 2021  
LTIP) for the Executive Management and other employees. The LTIP grants comprise Restricted Share Units  
(“RSUs”) and Performance Share Units (“PSUs”) which entitle the participants, subject to vesting occurring, to  
be allocated a number of shares in the Company, equivalent to the number of vested RSUs and/or PSUs,  
against payment of the par value of each share. The RSUs will have a total vesting period of three years  
(beginning on January 1 or July 1 in 2021) and with one third of the granted RSUs vesting on each January 1  
or July 1 in the following three financial years. The PSUs will have a total vesting period of three years  
(beginning on January 1 or July 1 in 2021) and with the granted PSUs vesting, in whole or in part, on January  
1 or July 1 in the third year. Vesting of RSUs is not conditional upon achieving any financial or non-financial  
targets, whereas vesting of PSUs is conditional upon an increase in the quoted share price of the Company’s  
shares, while vesting of both RSUs and PSUs is conditional upon the participant remaining employed  
throughout the total vesting period. However, in case of termination of a participant’s employment and  
designation as a Good Leaver, the right to receive vested RSUs or PSUs will be prorated and calculated through  
the date of release of the Participant’s work obligations. The vested RSUs and PSUs can only be exercised  
within four months after the expiration of the total vesting period. However, the delivery period may be  
extended to the next open trading window in certain circumstances. The original LTIP were expected to  
comprise up to 607,460 shares in total.  
In October 2021, the Company initiated a modified 2021 long-term share-based incentive program (modified  
2021 LTIP) for the Executive Management and other employees. The terms of the modified LTIP are the same  
as the LTIP that was implemented in April 2021, however, the number of RSUs and PSUs and the applicable  
performance target for the PSUs were reset, calculated based on a share price equal to DKK 31.94 per share,  
corresponding to the volume weighted average share price of the Company’s shares as quoted on Nasdaq  
Copenhagen during the ten (10) trading days from September 1, 2021. The exercise of the RSUs and PSUs to  
be granted under the modified LTIP is conditional upon the participant not exercising his or her RSUs or PSUs  
granted in April 2021, which will subsequently lapse and no longer be exercisable, and are therefore considered  
replacement equity instruments for the cancelled equity instruments. The fair value of the originally granted  
RSUs and PSUs at the date of the modification was determined to be DKK 24.72 and DKK 7.70, respectively.  
The incremental fair value, calculated based on the number of modified awards granted multiplied with the  
modified unit fair value less the fair value of the original LTIP granted remeasured at the modification date, will  
be recognised as an expense over the period from the modification date to the end of the vesting period. The  
expense for the original LTIP grant will continue to be recognised as if the terms had not been modified. In  
connection with the modified LTIP, the members of Executive Management received an extraordinary grant of  
RSUs and PSUs corresponding to 100% of the grant under the modified LTIP and on the same terms as the  
modified LTIP, and the sign-on RSUs granted to the CEO in April 2021 were also reset after the same principles  
as the modified LTIP but with immediate vesting upon grant. The modified LTIP including the other share-  
based retention grants to the Executive Management are expected to comprise up to 595,916 shares in total.  
No long-term share-based incentive program was announced in 2022 because of the restructuring of the  
Company and subsequent sale of substantially all of its assets and business activities.  
The fair value of RSU awards was estimated using a Black Scholes option valuation model, whereas all other  
LTIP awards were estimated using a Monte-Carlo simulation model at the respective grant dates, considering  
the terms and conditions on which the awards were granted.  
The risk-free interest rate has been estimated based on Danish government bonds with similar maturities.  
Since November 2020, expected volatility has been determined based on the Company’s own historical  
volatility, as the Company has been publicly traded for three years. Before November 2020, expected volatility  
was determined based on the historical volatility of comparable listed companies. The Company does not plan  
to pay out dividends in the foreseeable future.  
The following table presents the fair value of the shares granted in 2021 under each program and the inputs  
used in the valuation models at the respective grant dates:  
2021  
RSU  
Apr 2021  
(original)  
58,040  
-
2021  
PSU  
Apr 2021  
(original)  
20,020  
-
Program  
2021 RSU  
Oct 2021  
(modified)  
24,720  
2021 PSU  
Oct 2021  
(modified)  
12,560  
Grant date  
Fair value at the measurement date (DKK 000)  
Dividend yield (%)  
-
-
Expected volatility (%)  
98.6%  
(0.61%)  
0.23-2.23  
25,700  
98.6%  
(0.61%)  
2.23  
55.6%  
(0.53%)  
0.69-2.69  
59,050  
55.6%  
(0.53%)  
2.69  
Risk-free interest rate (%)  
Expected life of awards (years)  
Weighted average share price (DKK)  
25,700  
59,050  
27  
 
Matching Shares under all of the LTIP programs were fully vested as of January 1, 2021.  
The following table presents the weighted average remaining contractual life in years of the Performance  
Shares of the LTIP awards outstanding at December 31 for the respective year presented. Matching Shares  
under all of the LTIP programs were fully vested as of January 1, 2021:  
2023  
2022  
Program  
2020 LTIP  
2019 LTIP  
0
0
1.0  
0.7  
The exercise price for each LTIP award outstanding as of December 31, 2022 was DKK 1,000 (2021: DKK  
1,000).  
The table below summarizes the activity related to the LTIP awards for the years ended December 31:  
Executive  
Key  
Total  
Awards  
DKK 000  
Management  
Employees  
Awards  
exercisable  
172,488  
Outstanding at December 31, 2020  
Granted  
52,865  
118,686  
172,488  
Exercised  
(52,865)  
(118,686)  
(172,488)  
Expired  
Forfeited  
Outstanding at December 31, 2021  
Granted  
Exercised  
Expired  
Forfeited  
Outstanding at December 31, 2022  
For the year ended December 31, 2023, DKK 0.0 million (2022: DKK 1.9 million) was recognized as  
compensation expense related to the LTIP awards. Of the total expense, DKK 0.0 million (2022: DKK 1.4  
million) is attributed to the Executive Management.  
b) Phantom share-based incentive program (cash-settled)  
In June 2018, Orphazyme introduced a four-year phantom share-based incentive program (the “2018 Phantom  
Shares Program”) for all employees other than the Executive Management and Key Employees under the LTIP.  
Programs with similar terms and conditions were initiated in August 2019 (2019 Phantom Shares Program) and  
December 2020 (2020 Phantom Shares Program), respectively.  
The Phantom Shares Programs are based on the share price of the Company and entitles the participants to a  
cash bonus if there has been an increase of at least 20% in Orphazyme’s share price compared to the entry  
price at the grant date. The Phantom Shares Programs will not have any dilutive effect on the shareholders of  
Orphazyme as the phantom shares do not constitute or qualify for actual shares in Orphazyme.  
The overall objectives of the Phantom Shares Programs are (i) to retain qualified employees, (ii) to create  
long-term incentive for the participants, and (iii) to align the interests of the employees with those of  
Orphazyme’s shareholders. Each employee participating in the program earns the right to a certain number of  
phantom shares per month, depending on the employee’s position. Subject to any adjustments to the Phantom  
Shares Programs made by the Board of Directors due to, for example, changes in Orphazyme’s share capital  
structure or other significant events, each employee will be eligible to receive up to a total of 144 or 288  
phantom shares under the program. By the end of each calendar year of the four-year program, the  
participants will have earned phantom shares free of charge.  
The entry price per phantom share for the 2018 and 2019 Phantom Programs was DKK 61,000 and for the  
2020 Phantom Program was DKK 71,200. The entry prices were calculated on the basis of the volume-  
weighted average closing price of Orphazyme’s share on Nasdaq Copenhagen during a period of 10 trading  
days prior to the introduction of the respective Phantom Shares Program. The phantom shares will  
automatically be settled in cash at the end of January 2023 for the 2018 Phantom Shares Program, at the end  
of January 2024 for the 2019 Phantom Shares Program and at the end of January 2024 for the 2020 Phantom  
Shares Program by subtracting the entry price per share from the market price per share and multiplying the  
change by the total number of granted phantom shares, presuming the market-based condition (share price  
increase by 20%) is met . The market price per share will be based on the volume-weighted average closing  
price of Orphazyme’s shares on Nasdaq Copenhagen during a period of 10 trading days prior to the settlement  
of the phantom shares. Leavers are entitled to keep already granted phantom shares and will receive a pro-  
rata grant compared to the time of employment in the applicable granting year.  
28  
 
None of the programs resulted in a payment to the employees due to a large decrease in the share price. As  
the Phantom Shares Programs are cash-settled, the fair value of the phantom shares granted as part of the  
program is estimated at each reporting date. For the year ended December 31, 2023, an aggregate amount of  
DKK 0.0 million (2022: DKK 0.1 million) was recognized as compensation income related to the Phantom  
Shares Programs, with a corresponding amount recognized as a non-current liability. As per December 31,  
2022, the Company had accrued DKK 0.1 million for liabilities related to the 2018-2020 Phantom Shares  
Programs. The liability was reversed in 2023 corresponding in a DKK 0.1 million income.  
c) Restricted Share Units (cash-settled)  
According to the terms and conditions of the Restricted Share Units program (RSU), directors may annually be  
granted a number of RSUs with a value corresponding to up to 50% of the participant’s fixed annual base fee  
as member of the Board of Directors, not including committee membership fees. The value is calculated on the  
basis of the volume-weighted average share price of Orphazyme’s shares as quoted on Nasdaq Copenhagen  
during the ten trading days preceding the grant date. The RSUs vest from the grant date to the date of the  
next annual general meeting. Upon vesting, RSUs may be exercised within a period of twelve months from  
vesting (Exercise Period) at a price corresponding to the volume-weighted average share price during the ten  
trading days preceding the grant date (Exercise Price). In the event of a participant’s resignation from the  
Board of Directors, any unvested RSUs will lapse without any rights of compensation. A decision not to be re-  
elected is not a resignation from the Board of Directors.  
The RSUs are classified as a cash-settled program, as the Board of Directors may choose to settle any vested  
RSUs in cash. In such event, the cash settlement amount is based on the difference between the Exercise Price  
and the volume-weighted average share price as quoted on Nasdaq Copenhagen during the ten trading days  
preceding the first day of the Exercise Period.  
In August 2019, Restricted Share Units (2019 RSUs) were granted to members of the Board of Directors.  
During 2021 certain board members exercised their RSUs. As these RSUs were not cash-settled, the  
corresponding liability of DKK 35 thousand was reversed into equity and treated as equity-settled. The  
remaining 1,927 RSUs expired in March 2021 resulting a positive impact on the Statement of Profit or Loss of  
DKK 38 thousand.  
In March 2020, the 2020 RSU program was announced, granting the Board of Directors an aggregate of 15,177  
RSUs under similar terms and conditions as the 2019 RSUs. 13,525 RSUs lapsed in 2021 in connection with the  
exercise of grants under the new 2020-2 RSU program, while 1,652 RSUs expired in 2022. Neither the lapses  
in 2021 nor the expiry in 2022 had any impact on the Statement of Profit or Loss as Management had assessed  
that none of the options under the 2020 RSU program would ultimately vest as the grants under the 2020-2  
RSU program were more beneficial to exercise for the participants.  
In September 2020, a new RSU incentive program was announced (2020-2 RSU program), which comprised  
22,993 RSUs in total, including an on-boarding grant to a new board member in accordance with the  
Company’s remuneration policy. The 2020-2 RSU program runs in parallel with the 2020 RSU program and  
board members can only exercise RSUs under one of the programs.  
In December 2020, 4,351 RSUs (2020-3 RSU program) were granted to the Chairman of the Board as part of a  
consultancy agreement (see Note 4.6). The RSUs fully vested on the date of the general meeting in 2021. All  
RSUs except for 1,927 were exercised in 2021. The remaining 1,927 RSUs expired in March 2022 resulting a  
positive impact on the Statement of Profit or Loss of DKK 31 thousand.  
In May 2021, the 2021 RSU program was announced, granting the Board of Directors an aggregate of 30,450  
RSUs under similar terms and conditions as the 2020-1 RSUs. 10,211 and RSUs 8,168 RSUs lapsed during  
2022 and 2021, respectively, as the participants resigned from the Board of Directors forfeiting the vesting  
condition resulting a positive impact on the Statement of Profit or Loss of DKK 180 thousand and 96 thousand,  
respectively.  
No RSU program was announced in 2022 because of the restructuring of the Company and subsequent sale of  
substantially all of its assets and business activities.  
d) Sign-on bonus shares to previous CEO  
As part of the former CEO service agreement, Kim Stratton was granted 58,000 ordinary shares, which would  
vest if the Company’s share price increased to DKK 125,000 per share within three years from the date of  
employment. The total award consisted of (i) 6,000 shares provided that the share price increased to DKK  
75,000 per share, (ii) 12,000 shares provided that the share price increased to DKK 100,000 per share, and  
(iii) 40,000 shares provided that the share price increased to DKK 125,000 per share. The target prices were  
achieved and the 58,000 ordinary shares were issued to Ms. Stratton in February 2021 (see Note 4.8). The  
expense was recognized in 2020 when the target price was achieved.  
29  
 
e) Sign-on bonus shares to former CEO  
As part of the CEO service agreement made in 2021 with the former CEO, Christophe Bourdon was granted  
34,941 RSUs in connection with the on-boarding, which had a total vesting period of three years (beginning on  
January 1, 2021) and with one third of the granted RSUs vesting on each January 1 in the following three  
financial years. Vesting was not conditional upon achieving any financial or non-financial targets. However, in  
case of termination of employment and designation as a Good Leaver, the right to receive vested RSUs would  
be prorated and calculated through the date of release of the Participant’s work obligations. The vested RSUs  
could only be exercised after the expiration of the total vesting period. The RSUs were valued at grant date,  
April 2021, using a Black Scholes option valuation model similar to the original 2021 LTIP. The valuation of the  
award at grant date was DKK 2.0 million. The share-based compensation expense was classified as  
administrative and with recognition from January 2021.  
In October 2021, the grant of onboarding RSUs to was modified similar to the other long-term incentive  
programs for 2021. The terms of the modified grant are unchanged except for the immediate vesting upon  
grant and number of RSUs calculated based on a share price equal to DKK 31,940 per share, corresponding to  
the volume weighted average share price of the Company’s shares as quoted on Nasdaq Copenhagen during  
the ten (10) trading days from September 1, 2021. The exercise of the RSUs to be granted under the modified  
LTIP was conditional upon the participant not exercising the RSUs granted in April 2021, which will  
subsequently lapse and no longer be exercisable, and are therefore considered replacement equity instruments  
for the cancelled equity instruments. The fair value of the originally granted RSUs at the date of the  
modification was determined to be DKK 24,720. The incremental fair value, calculated as the number of  
modified awards granted multiplied with the modified unit fair value less the fair value of the original LTIP  
granted was remeasured at the modification date. The remaining expenses related to the original LTIP grant  
were similar recognised at the modification date. The incremental fair value of the modified award was DKK 0.9  
million was classified as general and administrative expenses recognised in October 2021.  
2.6  
FINANCIAL INCOME AND FINANCIAL EXPENSES  
§ ACCOUNTING POLICIES  
Financial income and expenses include interest income and expense, gains and losses due to changes in  
foreign exchange rates, interest expense related to the right-of-use assets, interest expense related to the  
Loan Agreement and other immaterial miscellaneous items.  
The following table presents the various items of financial income and expense recognized for the years end  
December 31:  
DKK 000  
2023  
786  
2022  
Interest income on cash balances  
Foreign currency exchange gains  
Total financial income  
185  
207  
993  
4,106  
4,291  
Interest expense on Loan Agreement (Note 3.7)  
Interest expense on lease liabilities (Note 3.2)  
Interest expense on cash balances  
Foreign currency exchange loss  
Bank fees and other charges  
Total financial expenses  
5,959  
96  
158  
2,843  
60  
9,116  
As of June 2022, the Company transferred substantially all of the Company’s assets and business activities to  
Zevra. Financial income and financial expenses are in 2022 therefore presented as both continued operations,  
net income DKK 0.2 million and discontinued operations (Note 1.7), net expense DKK 2.8 million.  
2.7  
INCOME TAXES  
§ ACCOUNTING POLICIES  
Income tax benefit includes the current benefit due from the current period’s taxable loss and deferred tax  
adjustments. The benefit is comprised primarily of refundable tax credits for costs incurred in connection with  
research and development activities under the Danish Tax Credit Regime.  
Corporation tax receivable is recognized in the balance sheet as the tax benefit computed on the taxable loss  
for the year, adjusted for any changes to the prior year benefit due to changes in the taxable loss of prior  
years and for any taxes already paid or refunded.  
Deferred tax is measured using the balance sheet liability method on all temporary differences between the  
carrying amount and the tax value of assets and liabilities, with the exception of temporary differences  
30  
 
occurring at the time of acquisition and liabilities neither affecting the result of operation nor the taxable  
income.  
As of December 31, 2023 and 2022, there were no tax audits in process nor has management been notified of  
any pending tax audit.  
Judgement regarding the recognition of the deferred tax assets related to taxable losses to be  
carried forward  
Orphazyme is subject to income taxes in Denmark. The Company recognizes deferred income tax assets if it is  
probable that sufficient taxable income will be available in the future against which the temporary differences  
and unused tax losses can be utilized. Significant judgment is required to determine the amount of deferred  
tax assets that may be recognized, based upon the likely timing and the level of future taxable profits together  
with future tax planning strategies. This judgment is made periodically after considering current facts and  
circumstances, budgets and business plans as well as the risks and uncertainty associated with the Company’s  
ability to successfully commercialize and defend its intellectual property. After consideration of these factors,  
Management has concluded in lack of significant activity in the Company, the deferred income tax assets  
related to taxable losses carried forward in Denmark do not meet the criteria for being recognized as assets in  
the Statement of Financial Position.  
The Company’s tax losses can be carried forward infinitely subject to the general rules on limited deductibility  
due to ownership changes. In Denmark, the Company’s ability to use tax loss carry forwards in any one year is  
limited to 100% of the first DKK 9.1 million of taxable income plus 60% of taxable income above DKK 9.1  
million.  
For the years ended December 31, 2023 and 2022, the Company has unrecognized net tax loss carry-forwards  
in the Danish entity in the amount of DKK 2,212 million and DKK 2,186 million, respectively.  
Management periodically evaluates positions taken in tax returns with respect to situations in which applicable  
tax regulations are subject to interpretation or uncertainty and establishes provisions, where appropriate. To  
date, there have not been any provisions established for uncertain tax positions.  
The following table presents the total income tax benefit for the years ended December 31:  
DKK 000  
2023  
2022  
Current tax benefit on net result  
Tax credit research and development expenses  
Change in unrecognized deferred tax before tax credit  
7,111  
2,750  
(25,129)  
32,240  
2,750  
Permanent differences  
Total income tax benefit for the year  
The following table presents the reconciliation of the effective tax rate to the statutory corporate income tax  
rate in Denmark.  
DKK 000  
2023  
(26,048)  
2022  
(41,056)  
73,378  
22%  
Net result before tax from continuing operations  
Net result before tax from discontinuing operations  
Corporate income tax rate in Denmark  
Computed income tax benefit  
22%  
5,731  
(7,111)  
Tax effect of:  
Other non-deductible expenses, including US listing-  
related costs and share-based compensation  
Deferred tax asset not recognized after tax credit  
Total income tax benefit for the year  
(5,731)  
32,240  
(22,379)  
2,750  
The following table presents the carrying amount of deferred tax in the Statement of Financial Position:  
DKK 000  
2023  
488,392  
2022  
480,554  
Tax deductible losses  
Deferred tax on intangible assets  
Other temporary differences  
44  
488,392  
488,392  
480,588  
480,588  
Deferred tax asset not recognized  
Carrying amount included in the Statement of  
Financial Position  
31  
 
2.8  
INVESTMENT IN GROUP COMPANIES  
§ ACCOUNTING POLICIES  
Investments in subsidiaries are measured at the lower of cost or recoverable amount. Any distributed  
dividends are recognized in the income statement.  
DKK 000  
Cost at January 1  
2023  
3,942  
2022  
3,942  
Cost end of year December 31  
Adjustment January 1  
Adjustment end of year December 31  
Carrying amount of investment  
3,942  
(3,942)  
(3,942)  
-
3,942  
(3,942)  
(3,942)  
-
DKK 000  
Ownership  
interest  
(%)  
Registered  
office  
Share  
capital  
Equity  
Net result  
Orphazyme US, Inc.  
Delaware,  
USA  
Zug,  
Switzerland  
USD 1  
100 %  
100 %  
(USD 000)  
CHF 20,000  
(CHF 000)  
1,403  
355  
0
0
Orphazyme GmhH (CH)  
SECTION 3  
Assets and liabilities  
3.1  
INTANGIBLE ASSETS  
§ ACCOUNTING POLICIES  
Intangible assets comprise software development costs and license rights to develop and commercialize  
products and are acquired separately and measured on initial recognition at cost. Software assets consist of  
implementation costs to get cloud computing arrangements ready for use, as long as they meet the  
requirements of IAS 38, Intangible Assets. These cloud computing arrangements begin to be amortized when  
they are ready for intended use and are amortized over seven years.  
For acquisition of intangible rights involving equity-settled share-based payment transactions, Management  
measures the fair value of the rights received and the corresponding increase in equity by reference to the fair  
value of the rights received, unless that fair value cannot be estimated reliably. If Management cannot  
estimate reliably the fair value of the rights received, it measures the fair value and the corresponding increase  
in equity by reference to the fair value of the equity instruments granted.  
Following initial recognition, intangible assets are carried at cost less any accumulated amortization and  
accumulated impairment losses. The useful lives of intangible assets are assessed as either finite or indefinite.  
Intangible assets with finite lives such as software and license rights to develop and commercialize products  
are amortized over the useful economic life and assessed for impairment whenever there is an indication that  
the intangible asset may be impaired.  
The amortization period and the amortization method for an intangible asset with a finite useful life are  
reviewed at least at the end of each reporting period. Changes in the expected useful life or the expected  
pattern of consumption of future economic benefits embodied in the asset are considered to modify the  
amortization period or method, as appropriate, and are treated as changes in accounting estimates. The  
amortization expense on intangible assets with finite lives is recognized in the Statement of Profit or Loss in the  
expense category that is consistent with the function of the intangible assets.  
Assets with finite useful lifetime are assessed for impairment indicators. Each year, the assets are reviewed in  
order to assess whether there are indications of impairment. If such indications exist, the recoverable amount,  
determined as the higher amount of the fair value of the asset adjusted for expected costs to sell and the value  
in use of the asset, is calculated. The impairment expense on intangible assets with finite lives is recognized in  
the Statement of Profit or Loss in the expense category that is consistent with the function of the intangible  
assets.  
32  
 
As of June 2022, all software and licenses were transferred to Zevra as part of the sale of substantially all of  
the Company’s assets and business activities.  
The following table presents the cost and respective amortization of software and licenses held by Orphazyme.  
The foreign currency effect is immaterial:  
DKK 000  
Cost at December 31, 2021  
Disposals  
Cost at December 31, 2022  
Cost at December 31, 2023  
Software  
981  
Licenses  
12,083  
(12,083)  
Total  
13,064  
(13,064)  
(981)  
Accumulated amortization at December 31, 2021  
Amortization expense  
Disposal accumulated amortization  
Accumulated amortization at December 31, 2022  
Accumulated amortization at December 31, 2023  
433  
463  
(896)  
10,480  
10,912  
463  
(10,912)  
(10,480)  
Net carrying value at  
December 31, 2022  
December 31, 2023  
3.2  
LEASES  
§ ACCOUNTING POLICIES  
On January 1, 2019, Orphazyme adopted IFRS 16, Leases, using the modified retrospective method. At  
contract inception, the Company assesses whether a contract is, or contains, a lease. A contract is, or contains,  
a lease if the contract conveys the right to control the use of an identified asset for a period of time in  
exchange for consideration. The Company is party to lease agreements only in which it is a lessee and not a  
lessor.  
As a lessee, the Company applies a single recognition and measurement approach for all leases, except for  
short-term leases and leases of low-value assets. At inception or on reassessment of a contract that contains a  
lease component, the Company allocates the consideration in the contract to each lease component on the  
basis of their relative stand-alone prices.  
The Company recognizes lease liabilities to make lease payments and right-of-use assets representing the right  
to use the underlying assets.  
Right-of-use assets  
The Company recognizes right-of-use assets at the commencement date of the lease (i.e., the date the  
underlying asset is available for use). Right-of-use assets are measured at cost, less any accumulated  
depreciation and impairment losses, and adjusted for any remeasurement of lease liabilities. The cost of right-  
of-use assets includes the amount of lease liabilities recognized, initial direct costs incurred, and lease  
payments made at or before the commencement date less any lease incentives received.  
Right-of-use assets are depreciated on a straight-line basis over the shorter of the lease term and the  
estimated useful life of the underlying asset. If ownership of the leased asset transfers to the Company at the  
end of the lease term or the cost reflects the exercise of a purchase option, depreciation is calculated using the  
estimated useful life of the asset, which for the operating equipment under lease is ten years. The right-of-use  
assets are also subject to impairment.  
Lease liabilities  
At the commencement date of the lease, the Company recognizes lease liabilities measured at the present  
value of lease payments to be made over the lease term. The lease payments include fixed payments less any  
lease incentives receivable, variable lease payments that depend on an index or a rate, and amounts expected  
to be paid under residual value guarantees. The lease payments also include the exercise price of a purchase  
option reasonably certain to be exercised by the Company and payments of penalties for terminating the lease,  
if the lease term reflects the Company exercising the option to terminate. Variable lease payments that do not  
depend on an index or a rate are recognized as expenses in the period in which the event or condition that  
triggers the payment occurs. In calculating the present value of lease payments, the Company uses its  
incremental borrowing rate at the lease commencement date because the interest rate implicit in the lease is  
not readily determinable. After the commencement date, the amount of lease liabilities is increased to reflect  
the accretion of interest and reduced for the lease payments made. In addition, the carrying amount of lease  
liabilities is remeasured if there is a modification, a change in the lease term, a change in the lease payments  
(e.g., changes to future payments resulting from a change in an index or rate used to determine such lease  
33  
 
payments) or a change in the assessment of an option to purchase the underlying asset. The Company’s non-  
current lease liabilities are included as a separate line item on the Company’s consolidated balance sheet and  
the current portion of lease liabilities is included in Other current liabilities.  
Short-term leases and leases of low-value assets  
The Company applies the short-term lease recognition exemption to its short-term leases of machinery and  
equipment (i.e., those leases that have a lease term of 12 months or less from the commencement date and  
do not contain a purchase option). It also applies the lease of low-value assets recognition exemption to leases  
of office equipment that are considered to be low value. Lease payments on short-term leases and leases of  
low-value assets are recognized as expense on a straight-line basis over the lease term.  
Lease modifications  
Lease modifications are accounted for at the effective date of modification, which is the date when both parties  
agree to the lease modification. Modifications are accounted for either as a separate lease or as a  
remeasurement of the initial lease. A modification is accounted for as a separate lease if both of the following  
conditions are met: (a) the modification increases the scope of the lease by adding the right to use one or  
more underlying assets; and (b) the consideration for the lease increases by an amount equivalent to the  
stand-alone price for the underlying asset. For a modification that is not a separate lease, the lease liability is  
remeasured using a discount rate determined at the effective date of the modification.  
As of June 2022 the lease contracts were transferred to Zevra as part of the sale of substantially all of the  
Company’s assets and business activities.  
The following table presents the carrying amounts of right-of-use assets recognized and the movements during  
the period:  
Office  
DKK 000  
At December 31, 2021  
Disposals  
Depreciation expense  
At December 31, 2022  
At December 31, 2023  
buildings  
5,726  
(2,405)  
(1,321)  
The following table presents the carrying amounts of lease liabilities and the movements during the period:  
DKK 000  
At January 1  
Disposals  
2023  
2022  
6,503  
(6,503)  
At December 31  
The maturity analysis of lease liabilities is disclosed in Note 3.6. The following amounts are recognized in the  
Statement of Profit or Loss:  
DKK 000  
2023  
2022  
1,057  
Depreciation and impairment expense of right-of-use assets (R&D)  
Depreciation and impairment expense of right-of-use assets (G&A)  
Interest expense on lease liabilities  
264  
96  
Total amount recognized in the Statement of Profit or Loss within  
discontinued operations  
1,417  
3.3  
PROPERTY, PLANT, AND EQUIPMENT  
§ ACCOUNTING POLICIES  
Property, plant, and equipment includes IT, lab and other equipment, furniture and leasehold improvements  
that are measured at cost less accumulated depreciation and impairment losses. Cost includes the acquisition  
price and costs directly related to the acquisition until the time the asset is ready for use. The residual value of  
equipment is not material. Depreciation is calculated on a straight-line basis over the expected useful life of the  
asset, being 3-5 for equipment and furniture. Leasehold improvements are depreciated over the shorter of the  
useful life of the improvement or the remaining lease term. The useful life of assets and method of depreciation  
are reviewed by management at least each year-end or more often based on changes in facts and  
circumstances. Changes in useful lives or residual values are adjusted prospectively as changes in accounting  
estimates. In addition, the Company has fully depreciated equipment still in use.  
Property, plant, and equipment is required to be tested for impairment when there are impairment indicators  
present. Impairment tests are conducted at the individual asset level, or at the lowest level for which  
34  
 
separately identifiable cash flows for the groups of assets exist. Impaired assets or asset groups are written  
down to their recoverable amount, which is the higher of the value in use and the net realizable value of the  
asset or asset Company, with impairment charges allocated proportionately to the assets within the impaired  
asset Company.  
Gross carrying amount of any fully depreciated property, plant and equipment that is still in use is DKK 0.0  
million.  
As of June 2022 the all property, plant and equipment was transferred to Zevra as part of the sale of  
substantially all of the Company’s assets and business activities.  
The following table presents the Company’s Property, plant and equipment as of the years presented:  
DKK 000  
Furniture  
and  
Lease  
equipment  
improvements  
Total  
8,260  
Cost at December 31, 2021  
Additions  
Disposals  
Cost at December 31, 2022  
Cost at December 31, 2023  
6,194  
2,066  
(6,194)  
(2,066)  
(8,260)  
Accumulated depreciation at December 31, 2021  
Depreciation expense  
5,121  
266  
934  
123  
6,055  
389  
Disposals  
(5,387)  
(1,057)  
(6,444)  
Accumulated depreciation at December 31, 2022  
Accumulated depreciation at December 31, 2023  
Net carrying value at  
December 31, 2022  
December 31, 2023  
There has been no impairment of property, plant and equipment for the years ended December 31, 2023 and  
2022. Depreciation expense is included within discontinued operations as follows:  
DKK 000  
2023  
2022  
Research and development expenses  
143  
246  
General and administrative expenses  
Total depreciation expense included within discontinued  
operations  
389  
3.4  
§ ACCOUNTING POLICIES  
Prepayments  
PREPAYMENTS AND OTHER RECEIVABLES  
Prepayments include advance payments made to vendors that will be incurred and expensed in subsequent  
financial reporting periods. When the period for full expense recognition is longer than one year from the  
balance sheet date, the portion to be expensed subsequent to one year is classified as non-current.  
Deposits  
Deposits include advance payments made to vendors to be settled upon completion of the underlying contract.  
When the contract term is longer than one year from the balance sheet date, the deposit is classified as non-  
current.  
Other receivables  
Other receivables include current and non-current amounts due to the Company.  
Estimate of prepayments related to clinical trial development costs  
As explained in Note 2.2, Orphazyme incurs substantial costs associated with clinical trials related to its  
development programs and there is a high degree of estimation involved in accounting for clinical trial  
development costs. In particular, certain CROs and vendors are paid upfront in connection with clinical  
35  
 
activities and Management is required to estimate the timing of the prepayment release to expense. This  
expense for the year is estimated by using an expense model, as described in Note 2.2.  
Current prepayments and other receivables are specified below:  
DKK 000  
Receivables from group companies  
Prepayments to vendors  
VAT receivable, net  
Foreign VAT receivable  
2023  
2022  
4,183  
216  
1,706  
662  
1,275  
479  
Other current receivables  
Total current prepayments and other receivables  
883  
1,099  
8,305  
Current prepayments to vendors include prepayments made to CROs for clinical trial costs of DKK 0.0 million  
(2022: DKK 1.7 million).  
3.5 TRADE RECEIVABLES  
§ ACCOUNTING POLICIES  
Trade receivables are recognized and derecognized on a settlement date basis. They are measured at nominal  
value less expected credit losses based on historical experience. Orphazyme applies the simplified approach for  
determining expected credit losses.  
At December 31, 2023 trade receivables in the amount of DKK 0.0 million (2022: DKK 4.1 million) are  
recognized in the balance sheet at the total invoiced amount less any expected credit losses. Due to the nature  
of the revenue transactions, expected credit losses are very limited.  
There are no overdue receivables and the write-down for expected credit losses is not material, due to all  
receivables at December 31, 2023 have been received at the reporting date.  
3.6 FINANCIAL ASSETS AND LIABILITIES  
§ ACCOUNTING POLICIES  
Financial assets  
Initial recognition and measurement  
Financial assets that meet certain criteria are classified at initial recognition as subsequently measured at  
amortized cost, fair value through other comprehensive income (OCI), or fair value through profit or loss. The  
Company does not hold any financial assets meeting these classification criteria except cash and securities  
valued at fair value and certain types of other receivables, which are valued at amortized cost. Generally, the  
Company’s financial assets are available to support current operations and amounts expected to be realized  
within the next twelve months are classified in the Statement of Financial Position as current assets.  
The Company’s financial assets are recognized initially at fair value plus, in the case of financial assets not  
carried at fair value through profit and loss, transaction costs that are attributable to the acquisition of the  
financial asset, if any. Financial instruments recognized at fair value are allocated to one of the following  
valuation hierarchy levels:  
Level 1: Quoted (unadjusted) prices in active markets for identical assets or liabilities.  
Level 2: Other techniques for which all inputs that have a significant effect on the recorded fair  
value are observable, either directly or indirectly.  
Level 3: Techniques that use inputs that have a significant effect on the recorded fair value that  
are not based on observable market data.  
Subsequent measurement  
Historically, the Company’s receivables are due within a twelve-month period and therefore the impact of using  
the effective interest rate method on the Company’s financial statements has been immaterial. Securities are  
measured using level 1 methods above. In 2023 fair value adjustments of securities was realized at DKK 42  
thousand which was included in Financial Income (2022: DKK 0 thousand).  
Financial asset impairment  
Trade receivables  
The Company’s receivables are due within twelve months and these do not contain a significant financing  
component which means that measuring the loss allowance as a lifetime ECLs (expected credit losses)  
36  
 
generally does not differ from measuring it as 12-month ECLs. The receivables are measured on initial  
recognition at the transaction price and do not have a contractual interest rate. This implies that the effective  
interest rate for these receivables is zero or immaterial. Accordingly, the discounting of cash shortfalls to  
reflect the time value of money when measuring ECLs is generally not required.  
Securities  
The securities are measured at fair value through profit or loss and all decreases in value are reflected in the  
statement of profit or loss, eliminating the need for an impairment assessment.  
Financial liabilities  
Borrowings  
Financial liabilities, including borrowings, are initially measured at fair value less transaction costs incurred.  
Subsequently, borrowings are measured at amortized cost. Amortized cost is calculated as original cost less  
instalments plus/less the accumulated amortization of the difference between cost and nominal value, so that  
the effective interest rate is recognized in the income statement over the loan period. Financial liabilities are  
derecognized when settled.  
The Facilitation Fee in our Loan Agreement, defined below, is accounted for as an embedded derivative. The  
variability arising from the change in Orphazyme’s share price is not closely related to the host debt instrument  
characterized mainly by interest rate and credit risk. Therefore, the embedded equity-linked amount is  
separated from the host debt instrument and accounted for as an embedded written call option at fair value  
through profit and loss.  
The portion of the debt maturing after one year is presented as non-current debt and the remainder as current  
debt.  
Trade payables and accruals  
Trade payables and accruals relate to the Company’s purchase of products and services from various vendors  
in the normal course of business.  
Other liabilities  
Other payables are measured at amortized cost. The amount payable to employees for the Phantom Shares  
Program (Note 2.5) is classified as non-current and is measured at fair value, at Level 2 in the fair value  
hierarchy.  
Discount and rebate liabilities  
Discount and rebate liabilities is classified as both current and non-current liabilities based on an existing legal  
or constructive obligation as a result of events occurring prior to or on the balance sheet date, and it is  
probable that the utilization of economic resources will be required to settle the obligation and is measured at  
management’s best estimate of the expenses required to settle the obligation.  
The Company’s financial assets include mainly cash and securities. The Company has no derivative financial  
assets nor has there been a change in classification of a financial asset after initial recognition and  
measurements as discussed herein. The Company has not placed any financial assets as security for loans at  
either December 31, 2023 or 2022.  
The Company’s financial liabilities comprise the following as of the years ended December 31:  
DKK 000  
Trade payables  
2023  
2022  
4,357  
Accruals  
5,070  
4,849  
Total liabilities measured at amortized cost  
5,070  
9,206  
Maturities of financial liabilities  
The table below presents the Company’s financial liabilities by relevant maturity groupings based on their  
contractual maturities for all non-derivative financial liabilities and derivative financial instruments for which  
the contractual maturities are essential for an understanding of the timing of the cash flows.  
37  
 
The amounts disclosed in the table are the contractual undiscounted cash flows. Balances due within 12  
months equal their carrying balances as the impact of discounting is not significant.  
Less  
than  
12 months  
Between  
1 and 2  
years  
Between  
2 and 5  
years  
Total  
contractual  
cash flows  
Carrying  
amount  
DKK 000  
Non-derivatives  
Accruals  
5,070  
5,070  
5,070  
Total non-derivatives  
5,070  
5,070  
5,070  
Total derivatives  
Liabilities from accrued discount and rebates are calculated based on specific terms in the individual  
agreements. Please refer to note 2.1 further information on the accrued discount and rebates and  
managements estimates and judgements.  
December 31, 2022 accrued discount and rebates relates to rebates granted to co-financing healthcare  
authorities for 2021 and 2022.  
Total current other liabilities are comprised of the following as of the years ended December 31:  
DKK 000  
2023  
2022  
Payables to group entities  
Remuneration to the Board of Directors  
Payroll and employee-related costs  
Total current other liabilities  
657  
213  
1,220  
2,090  
In addition, the Company has the following total other non-current liabilities as of the years ended December  
31:  
DKK 000  
2023  
2022  
Phantom shares liability to employees  
Total non-current other liabilities  
98  
98  
3.7  
§ ACCOUNTING POLICIES  
Securities are recognized and measured on the trading day at fair value on initial recognition.  
SECURITIES AND CASH  
Subsequently, the company's securities are measured at fair value. The return on the securities is included in  
the financial income. The fair value of listed securities is calculated on the basis of the stock market price at  
the time of the balance sheet. Cash includes cash on hand and in banks. Please see Financial Risks discussed in  
Note 4.4. The Company’s cash balance denominated in foreign currencies were as follows as of the years  
ended December 31:  
DKK 000  
DKK  
2023  
3,011  
2022  
14,794  
USD  
EUR  
GBP  
1,274  
6,801  
183  
10,609  
13,209  
306  
Total cash  
11,269  
38,918  
SECTION 4  
Other disclosures  
4.1  
CAPITAL MANAGEMENT  
For the purpose of the Company’s capital management, capital includes issued capital, share premium and all  
other equity reserves attributable to the equity holders of the Company. The primary objective of the  
Company’s capital management is to maximize shareholder value while limiting the financial risk. The Board of  
Directors’ policy is to maintain needed capital base in order to maintain investor, creditor and market  
confidence.  
As of December 31, 2023, the Company held securities and cash significantly exceeding expected costs to be  
incurred over the following 12 months. Management therefore considers it appropriate to prepare these  
financial statements on a going concern basis.  
38  
 
4.2  
EQUITY  
The following table summarizes the Company’s share activity:  
Ordinary shares  
34,952,241  
360,000  
December 31, 2021  
Capital increase, U.S. At-the-Market Offering Program  
December 31, 2022  
Reverse stock split 1:1,000  
December 31, 2023  
35,312,241  
(35,276,929)  
35,312  
At the Company’s extraordinary general assembly November 2, 2023 it was decided to implement a reverse  
share split at a consolidation ratio of 1.000:1. The reverse stock split reduced the number of shares in the  
company so 1,000 current shares at a nominal value of 1 DKK was consolidated to 1 new share at a nominal  
value of 1,000 DKK. The above share overview is adjusted to reflect the reverse stock split.  
The Company has never declared or paid any cash dividends on its ordinary shares and does not anticipate  
doing so in the foreseeable future. The Company intends to use all available financial resources as well as  
revenue, if any, for purposes of the Company’s current and future business.  
In February 2022, the Company completed a capital increase of 360,000 shares as a result of the utilization of  
the U.S. At-the-Market Offering Program.  
At an extraordinary general meeting on 30 November, 2023, it was adopted to lower the nominal value per  
share from DKK 1,000 to DKK 150. The reduction to DKK 150 did not take effect before January 9, 2024, after  
a four-week proclamation period.  
At the same extraordinary general meeting it was also adopted to reduce the share capital with a total nominal  
value of DKK 30,015,200, which included a reduction of nominal DKK 23,028,667 for the purpose of covering  
losses and a reduction of nominal DKK 6,986,533 for the purpose of transfers to a special reserve.  
The reduction of DKK 6,986,533 did not take effect before January 9, 2024, after a four-week proclamation  
period.  
As a result of the above transactions, the total nominal share capital of the Company as of December 31, 2023  
was DKK 12,283,333, divided into 35,312 ordinary shares each with a nominal value of DKK 347,85.  
4.3  
PROFIT/LOSS PER SHARE  
Basic profit/loss per share for the year is calculated by dividing the net result for the year by the weighted  
average number of ordinary shares outstanding during the year. The diluted profit/loss per share is calculated  
by dividing the net result for the year by the weighted average number of ordinary shares outstanding during  
the period increased by the dilutive effect of the assumed issuance of outstanding share-based awards. The  
potential shares issuable related to outstanding share-based awards have been excluded from the calculation  
of diluted per share amounts, as the effect of such shares is anti-dilutive.  
The following reflects the net loss attributable to shareholders and share data used in the basic and diluted  
earnings/(loss) per share computations for the years ended December 31:  
2023  
(26,048)  
-
2022  
(38,306)  
Net result for the year from continuing operations (DKK 000)  
Net result for the year from discontinuing operations (DKK 000)  
73,378  
Weighted-average shares outstanding  
Weighted-average shares outstanding, dilutive  
35,312  
35,518  
35,269  
35,269  
Profit/loss per share from continuing operations  
Profit/loss per share from discontinuing operations  
Profit/loss per share, basic (DKK)  
(738)  
-
(738)  
(733)  
(1,086)  
2,081  
995  
Profit/loss per share, dilutive (DKK)  
995  
39  
 
4.4  
FINANCIAL RISKS  
The Company’s activities expose it to a number of financial risks whereby future events, which can be outside  
the control of the Company, could have a material effect on its financial position and results of operations. The  
known risks include foreign currency, interest and credit risk and there could be other risks currently unknown  
to Management. The Company has not historically hedged its financial risks.  
Liquidity Risk  
At December 31, 2023, the Company’s liquidity risk was assessed to be low. Management continuously  
assesses the Company’s capital structure in order to evaluate whether its liquidity reserves allow it to achieve  
its business objectives. At December 31, 2023, the available liquidity reserves, including funded capital in  
subsequent period, were assessed to be sufficient for the Company to meet its planned operating activities in  
the normal course of business for at least the next twelve months.  
Foreign Currency Risk  
The Company’s foreign currency risk is assessed to be low. Accordingly, future changes in the exchange rates  
is only of the DKK against the SEK or USD exposure for the Company to currency gains or losses that will  
impact the reported amounts of assets, liabilities, income and expenses.  
The Company has prepared a sensitivity analysis in order to assess the potential impact on the Company’s net  
loss for possible fluctuations in the SEK, EUR and USD exchange rates against the DKK and the impact for the  
possible fluctuations in the interest rate on bank deposits in Denmark. The methods and assumptions used are  
consistent with prior year and consider increases and decreases in the Company’s main currencies, as well as  
reasonable fluctuations in the interest rate on its bank deposits.  
Based on the company's positions per 31 December 2023, a change of +/-10% in share prices would result in  
a gain/loss for the company of DKK 1 million.  
A change of +/-10% in the rate of the currencies the company is exposed to would result in the following  
gains/losses:  
SEK +/- DKK 1 million  
EUR +/- DKK 1 million  
USD +/- DKK 0 million  
Interest Rate Risk  
The Company’s interest rate risk is assessed to be low. The Company has no borrowing of December 31, 2023.  
Credit Risk  
The Company’s credit risk is assessed to be medium. The Company’s credit risk is associated with securities  
and cash held in banks. The Company’s cash is held primarily in one Danish bank with Moody’s long-term  
credit ratings exceeding of A1.  
40  
 
4.5  
REMUNERATION OF BOARD OF DIRECTORS AND EXECUTIVE MANAGEMENT  
Executive Management consists of the Company’s Chief Executive Officer, who is also the registered  
management of the Company.  
In February 2022, Orphazyme announced that the Board of Directors appointed Chief Financial Officer Anders  
Vadsholt as the new Chief Executive Officer in addition to his existing position as Chief Financial Officer,  
succeeding Christophe Bourdon on March 1, 2022.  
In May 2023, it was announced that the board of directors and CEO/CFO Anders Fink Vadsholt had entered into  
a severance agreement pursuant to which Mr. Vadsholt would step down as CEO/CFO of the Company end of  
September 2023, at which point it was announced that the Company had signed an CEO agreement with Jakob  
Bendtsen as the new CEO of the company. Jakob Bendtsen is also continuing in his role as board member.  
Mr. Bourdon resigned from his position at Orphazyme on February 28, 2022. The resignation entails a  
forfeiture of the vesting conditions attached to the RSUs and PSUs under the modified and extraordinary  
granted in October 2021 except for the modified sign-on bonus shares that immediately vested when granted  
in March 2021. For any ongoing RSU vesting period, Mr. Bourdon was entitled to receive a pro rata allocation of  
RSUs until the date of release of his work obligations.  
A cash bonus received under the short-term incentive program may not exceed 100% of the annual fixed  
salary of the participants. The Executive Management is also eligible to receive an extraordinary bonus at the  
discretion of the Board of Directors.  
The following table presents remuneration to the Executive Management for the years ended December 31,  
2023 and 2022.  
REMUNERATION TO INDIVIDUAL  
MEMBERS OF EXECUTIVE MANAGEMENT (DKK 000)  
2023  
2022  
Jakob Bendtsen (CEO from October 1, 2023)  
Salary  
75  
Total  
75  
Anders Vadsholt (CEO from March 1, 2022 and CFO since 2016 until  
September 30, 2023)  
Salary  
Bonus  
4,252  
400  
4,652  
2,908  
4,134  
1,657  
294  
Share-based compensation (1)  
Other employee benefits (3)  
Total  
8,993  
Christophe Bourdon (CEO from April 1, 2021 until February 2022)  
Salary  
Bonus  
650  
231  
Share-based compensation (1,2)  
Other employee benefits  
186  
Total  
4,727  
1,067  
10,060  
Total remuneration to the Executive Management  
(1) Investing expense on grants in prior years measured at fair value at grant date  
(2) includes two times share based compensation. Both sign-on bonus and LTIP 2021 program, both described in  
note 2.6  
(3) Board fee shown of DKK 231 thousand not included  
Remuneration paid to members of the Board of Directors is made up of board and committee fees, a travel  
allowance, ad-hoc fees for additional services provided and share-based compensation related to the Restricted  
Share Units (RSUs) as described in Note 2.5. Board remuneration is recognized as general and administrative  
expenses in the Statement of Profit or Loss. The following table lists Board of Directors remuneration for the  
years ended December 31:  
41  
 
REMUNERATION TO INDIVIDUAL MEMBERS OF THE  
OF THE BOARD OF DIRECTORS (DKK 000)  
Michael Hove (elected in May 2023)  
Board and committee fees  
Share-based compensation  
Total  
2023  
465  
2022  
57  
522  
Jakob Have (elected in May 2023)  
Board and committee fees  
Share-based compensation  
Total  
278  
34  
312  
Jakob Bendtsen (elected in May 2023)  
Board and committee fees  
Share-based compensation  
Total  
278  
34  
312  
Bo Jesper Hansen (resigned in May 2023)  
Board and committee fees  
Ad hoc board fees  
282  
606  
Travel allowance  
83  
33  
Share-based compensation  
Total  
365  
(50)  
589  
John Sommer Schmidt (resigned in May 2023)  
Board and committee fees  
Total  
169  
169  
231  
231  
Anders Fink Vadsholt (resigned in May 2023)  
Board and committee fees  
Total  
169  
169  
231  
231  
Georges Gemayel (Chairman until June 2022)  
Board and committee fees  
Share-based compensation  
Total  
370  
(161)  
209  
Martin Bonde (resigned in May 2022)  
Board and committee fees  
Travel allowance  
Share-based compensation  
Total  
176  
(24)  
152  
Carrolee Barlow (resigned in May 2022)  
Board and committee fees  
Travel allowance  
Share-based compensation  
Total  
176  
(24)  
152  
Stephanie Okey (resigned in May 2022)  
Board and committee fees  
Ad hoc board fees  
Share-based compensation  
Total  
176  
(72)  
104  
Andrew Mercieca (resigned in June 2022)  
Board and committee fees  
Total  
212  
212  
Total remuneration to the Board of Directors  
1,849  
3,834  
4.6  
RELATED PARTIES  
Orphazyme A/S, incorporated in Denmark, wholly owns Orphazyme US, Inc and Orphazyme Switzerland  
GmbH. These three entities are considered related parties. Orphazyme A/S is not ultimately controlled by any  
of its investors.  
For the years ended December 31, 2023 and 2022, the following related party transactions were identified:  
Remuneration to Executive Management (Note 4.5)  
Remuneration to the Board of Directors (Note 4.5)  
Participation of the Board members in the RSU programs (Note 2.5)  
As of December 31, 2023 and 2022, the Company did not have any amounts receivable from related parties  
and therefore recorded no related impairment. The Company has not granted any loans, guarantees, or other  
commitments to or on behalf of any of the members of the Board of Directors or Executive Management.  
42  
 
Executive Management and members of the Board of Directors had the following shareholding in Orphazyme  
A/S for the year ended December 31, 2023. They had no shares in 2022. All shares owned by the member are  
owned through controlled companies.  
December 31,  
2023  
Number of  
MEMBERS OF THE  
Number of  
shares  
owned  
5,482  
Unvested  
BOARD OF DIRECTORS:  
Michael Hove  
RSUs 2023  
375  
Jakob Have  
5,482  
225  
Jakob Bendtsen  
3,012  
225  
Orphazyme A/S’ related parties are the parent company’s Board of Directors, Executive Management and close  
members of the family of these persons.  
Transactions with subsidiaries  
Orphazyme US, Inc. and Orphazyme GmbH (CH) are 100% owned subsidiaries of Orphazyme A/S and are  
included in the financial statements. They have not had any activity in 2023 and are in the process of  
liquidation.  
4.7  
FEES TO AUDITORS  
The following table presents the fees to our independent registered public accounting firms KPMG P/S newly  
elected in 2023 and EY Godkendt Revisionspartnerselskab for 2022, recognized in general and administrative  
expenses in the Statement of Profit or Loss for the years ended December 31. This note was prepared in  
accordance with the requirements of the Danish Financial Statements Act:  
KPMG  
DKK 000  
2023  
2022  
Audit services  
115  
Audit-related services  
Other assistance  
Total fees to auditors  
115  
EY  
DKK 000  
2023  
2022  
Audit services  
Audit-related services  
Other assistance  
Total fees to auditors  
79  
692  
493  
79  
1,185  
Audit services  
Audit services consist of fees billed for professional services rendered for the audit of our annual consolidated  
financial statements or services that are normally provided by the accountant in connection with statutory and  
regulatory filings or engagements for those fiscal years.  
Audit-Related services  
Audit-related services consist of assurance and related services performed that are reasonably related to the  
performance of the audit or review of our consolidated financial statements and are not reported under "Audit  
services".  
43  
 
Statements by Board of Directors and  
Executive Management  
The Board of Directors and Executive Management have today considered and approved the annual report of  
Orphazyme A/S for the financial year January 1-December 31, 2023.  
The financial statements have been prepared in accordance with International Financial Reporting Standards  
(IFRS) as issued by the International Accounting Standards Board (IASB) and in accordance with IFRS as  
endorsed by the EU as well as additional disclosure requirements under the Danish Financial Statements Act.  
In our opinion, Management’s Review provides a fair presentation of the development in the operations and  
financial circumstances, the results of the year, and the overall financial position of the Company as well as a  
description of the most significant risks and elements of uncertainty facing the Company.  
In our opinion, the financial statements provide a fair presentation of the assets, liabilities, and financial  
position and the results of the operations and cash flows for the financial year. In our opinion, the Annual  
Report of Ambu A/S for the financial year January 1December 31, 2023 identified as OZ-2023-12-31-en.zip  
has been prepared, in all material respects, in compliance with the ESEF Regulation.  
We recommend that the annual report be adopted at the Annual General Meeting scheduled to be held on April  
18, 2024.  
Copenhagen, March 27, 2024  
BOARD OF DIRECTORS  
Michael Hove  
Jakob Have  
Chairman of the Board  
Jakob Bendtsen  
EXECUTIVE MANAGEMENT  
Jakob Bendtsen  
Chief Executive Officer  
44  
 
Independent Auditors’ Report  
To the shareholders of Orphazyme A/S  
Report on the audit of the Company's Financial Statements  
Opinion  
In our opinion, the Company’s financial statements give a true and fair view of the Company's assets, liabilities  
and financial position at 31 December 2023 and of the results of the Company's operations and cash flows for  
the financial year 1 January 31 December 2023 in accordance with the IFRS Accounting Standards as  
adopted by the EU and additional requirements in the Danish Financial Statements Act.  
Our opinion is consistent with our long-form audit report to the Board of Directors and the Audit Committee.  
Audited financial statements  
Orphazyme A/Sfinancial statements for the financial year 1 January 31 December 2023 comprise the  
income statement, statement of comprehensive income, balance sheet, statement of changes in equity,  
statement of cash flows and notes, including summary of material accounting policy information. The financial  
statements are prepared in accordance with the IFRS Accounting Standards as adopted by the EU and  
additional requirements in the Danish Financial Statements Act.  
Basis for opinion  
We conducted our audit in accordance with International Standards on Auditing (ISAs) and the additional  
requirements applicable in Denmark.  
Our responsibilities under those standards and requirements are further described in the "Auditor's  
responsibilities for the audit of the financial statements" section of our report.  
We believe that the audit evidence we have obtained is sufficient and appropriate to provide a basis for our  
opinion.  
Independence  
We are independent of the Company in accordance with the International Ethics Standards Board for  
Accountants' International Code of Ethics for Professional Accountants (IESBA Code) and the additional ethical  
requirements applicable in Denmark, and we have fulfilled our other ethical responsibilities in accordance with  
these requirements and the IESBA Code.  
We declare, to the best of our knowledge and belief, that we have not provided any prohibited non-audit  
services, as referred to in Article 5(1) of the Regulation (EU) 537/2014 and that we remained independent in  
conducting the audit.  
We were appointed auditors of Orphazyme A/S for the first time on November 2, 2023 for the financial year  
2023.  
45  
 
Key audit matters  
Key audit matters are those matters that, in our professional judgement, were of most significance in our audit  
of the financial statements for the 2023 financial year. These matters were addressed in the context of our  
audit of the financial statements as a whole, and in the forming of our opinion thereon. We do not provide a  
separate opinion on these matters.  
How our audit addressed the key audit  
Key audit matters  
matter  
Accounting for Settlement of U.S. Lawsuit  
For the purpose of our audit, the procedures we  
carried out included the following:  
The US class action lawsuit has been closed in  
early February 2024 and the settlement amount  
was USD 2,5 million of which USD 0,5 million was  
covered by insurance leaving a net amount of  
USD 2 million which was paid in November 2023.  
Inquired of management and evaluated their  
assessment of the recognition of expenses in  
2023 when the decision was made by the  
court.  
Refer to note 2.3 and page 4 of the financial  
statements for more details.  
Obtained  
payment  
notification  
from  
management to check to whom the payment  
has been made and the amount is correct per  
the written settlement document. We noted  
that the amount had been transferred to an  
escrow account named after the plaintiffs in  
November 2023.  
Inquired of predecessor auditors to obtain the  
background of the issue as well as evaluation  
of the accounting treatment and disclosure  
thereof in the financial statements.  
Checked if the transaction is classified correctly  
and presented appropriately in the financial  
statements.  
Performed checks of after the year-end bank  
payments to ensure all expenses related to this  
settlement had been accrued for in the correct  
accounting period.  
Statement on the Managements review  
Management is responsible for the Management's review.  
Our opinion on the financial statements does not cover the Management's review, and we do not express any  
form of assurance conclusion thereon.  
In connection with our audit of the financial statements, our responsibility is to read the Management's review  
and, in doing so, consider whether the Management's review is materially inconsistent with the financial  
statements or our knowledge obtained during the audit, or otherwise appears to be materially misstated.  
Moreover, it is our responsibility to consider whether the Management's review provides the information  
required by relevant law and regulations.  
Based on the work we have performed, we conclude that the Management's review is in accordance with the  
financial statements and has been prepared in accordance with relevant law and regulations. We did not  
identify any material misstatement of the Management's review.  
46  
 
Management's responsibility for the financial statements  
Management is responsible for the preparation of financial statements that give a true and fair view in  
accordance with the IFRS Accounting Standards as adopted by the EU and additional requirements in the  
Danish Financial Statements Act and for such internal control that Management determines is necessary to  
enable the preparation of financial statements that are free from material misstatement, whether due to fraud  
or error.  
In preparing the financial statements, Management is responsible for assessing the Company's ability to  
continue as a going concern, disclosing, as applicable, matters related to going concern and using the going  
concern basis of accounting unless Management either intends to liquidate the Company or to cease  
operations, or has no realistic alternative but to do so.  
Auditor's responsibilities for the audit of the financial statements  
Our objectives are to obtain reasonable assurance as to whether the financial statements as a whole are free  
from material misstatement, whether due to fraud or error, and to issue an auditor's report that includes our  
opinion. Reasonable assurance is a high level of assurance but is not a guarantee that an audit conducted in  
accordance with ISAs and the additional requirements applicable in Denmark will always detect a material  
misstatement when it exists. Misstatements may arise from fraud or error and are considered material if,  
individually or in the aggregate, they could reasonably be expected to influence the economic decisions of  
users taken on the basis of these financial statements.  
As part of an audit conducted in accordance with ISAs and the additional requirements applicable in Denmark,  
we exercise professional judgement and maintain professional scepticism throughout the audit. We also:  
-
identify and assess the risks of material misstatement of the financial statements, whether due to  
fraud or error, design and perform audit procedures responsive to those risks, and obtain audit  
evidence that is sufficient and appropriate to provide a basis for our opinion. The risk of not detecting  
a material misstatement resulting from fraud is higher than for one resulting from error as fraud may  
involve collusion, forgery, intentional omissions, misrepresentations or the override of internal control.  
obtain an understanding of internal control relevant to the audit in order to design audit procedures  
that are appropriate in the circumstances, but not for the purpose of expressing an opinion on the  
effectiveness of the Company's internal control.  
-
-
-
evaluate the appropriateness of accounting policies used and the reasonableness of accounting  
estimates and related disclosures made by Management.  
conclude on the appropriateness of Management's use of the going concern basis of accounting in  
preparing the financial statements and, based on the audit evidence obtained, whether a material  
uncertainty exists related to events or conditions that may cast significant doubt on the Company's  
ability to continue as a going concern. If we conclude that a material uncertainty exists, we are  
required to draw attention in our auditor's report to the related disclosures in the financial statements  
or, if such disclosures are inadequate, to modify our opinion. Our conclusions are based on the audit  
evidence obtained up to the date of our auditor's report. However, future events or conditions may  
cause the Company to cease to continue as a going concern.  
-
-
evaluate the overall presentation, structure and contents of the financial statements, including the  
disclosures, and whether the financial statements represent the underlying transactions and events in  
a manner that gives a true and fair view.  
obtain sufficient appropriate audit evidence regarding the financial information of the entities or  
business activities within the Company to express an opinion on the financial statements. We are  
responsible for the direction, supervision and performance of the audit. We remain solely responsible  
for our audit opinion.  
We communicate with those charged with governance regarding, among other matters, the planned scope and  
timing of the audit and significant audit findings, including any significant deficiencies in internal control that  
we identify during our audit.  
We also provide those charged with governance with a statement that we have complied with relevant ethical  
requirements regarding independence, and to communicate with them all relationships and other matters that  
may reasonably be thought to bear on our independence, and where applicable, actions taken to eliminate  
threats or safeguards applied.  
From the matters communicated to those charged with governance, we determine those matters that were of  
most significance in the audit of the financial statements of the current period and therefore the key audit  
matters. We describe these matters in our auditor's report unless law or regulation precludes public disclosure  
about the matter or when, in extremely rare circumstances, we determined that a matter should not be  
communicated in our report because the adverse consequences of doing so would reasonably be expected to  
outweigh the public interest benefits of such communication.  
47  
 
Report on compliance with the ESEF Regulation  
As part of our audit of the Financial Statements of Orphazyme A/S we performed procedures to express an  
opinion on whether the annual report of Orphazyme A/S for the financial year 1 January 31 December 2023  
with the file name OZ-2023-12-31-en.zip is prepared, in all material respects, in compliance with the  
Commission Delegated Regulation (EU) 2019/815 on the European Single Electronic Format (ESEF Regulation)  
which includes requirements related to the preparation of the annual report in XHTML format of the Financial  
Statements.  
Management is responsible for preparing an annual report that complies with the ESEF Regulation. This  
responsibility includes:  
-
-
The preparing of the annual report in XHTML format; and  
For such internal control as Management determines necessary to enable the preparation of an annual  
report that is compliant with the ESEF Regulation.  
Our responsibility is to obtain reasonable assurance on whether the annual report is prepared, in all material  
respects, in compliance with the ESEF Regulation based on the evidence we have obtained, and to issue a  
report that includes our opinion. The nature, timing and extent of procedures selected depend on the auditor’s  
judgement, including the assessment of the risks of material departures from the requirements set out in the  
ESEF Regulation, whether due to fraud or error. The procedures include:  
-
Testing whether the annual report is prepared in XHTML format;  
In our opinion, the annual report of Orphazyme A/S for the financial year 1 January 31 December 2023 with  
the file name OZ-2023-12-31-en.zip is prepared, in all material respects, in compliance with the ESEF  
Regulation.  
Copenhagen, March 27, 2024  
KPMG  
Statsautoriseret Revisionspartnerselskab  
CVR no. 25 57 81 98  
Sara Carstensen  
State Authorised  
Public Accountant  
mne34191  
48