DNL.L Regulatory News (DNL)

29 June 2021

Diurnal Group plc

("Diurnal" or the "Company")

Distribution agreement with Er-Kim for Bulgaria and Romania

Extends relationship with Er-Kim following Turkey agreement for Alkindi® and Efmody®

Diurnal Group plc (AIM: DNL), the specialty pharmaceutical company targeting patient needs in chronic endocrine (hormonal) diseases, announces that it has extended its distribution arrangements with Er-Kim, a leading partner for global biotech and pharma companies seeking to commercialise its products in Turkey, Europe and the Middle East, to include the distribution and marketing of Alkindi® (hydrocortisone granules in capsules for opening) and Efmody® (hydrocortisone modified-release hard capsules) in Bulgaria and Romania.

Under the terms of the agreement, Er-Kim will receive the exclusive rights to distribute and market Alkindi® and Efmody® in Bulgaria and Romania. The combined market in both countries is estimated to be more than $13m in paediatric adrenal insufficiency (AI) and adult congenital adrenal hyperplasia (CAH).

Alkindi® already has marketing authorisation in the European Union and is the first preparation of hydrocortisone (the synthetic version of cortisol) specifically designed for use in children suffering from AI, including the related condition CAH.

Efmody® is a modified-release preparation of hydrocortisone for the treatment of patients with CAH, a rare condition caused by a genetic deficiency of adrenal enzymes. In May 2021, Efmody® was granted marketing authorisation in the European Union and, to facilitate timely commercial availability, the Company has commenced market access activities with commercial launches in European countries anticipated to commence from Q3 2021.

In line with the Company's other global distribution agreements, Diurnal will provide Alkindi® and Efmody® from its established European supply chain for sale by Er-Kim. This agreement adds to the Company's ongoing strategy for commercialisation of its lead products by optimising market access outside of the larger European markets, where Diurnal directly markets its products, through entering marketing and distribution agreements with companies focused on niche and orphan conditions.

Martin Whitaker, Chief Executive Officer of Diurnal, commented:

"We are pleased to announce the extension of our relationship with Er-Kim to make our products, Alkindi® and Efmody®, available to patients with diseases of cortisol deficiency in Bulgaria and Romania. As Efmody® becomes available for patients in Europe from Q3 2021, this agreement is in line with our strategy to optimise global market access for our products. Since signing our initial agreement with

Er-Kim in January 2021 for Turkey, we have been impressed with Er-Kim's in-market expertise and with a strong track record of commercialisation, we look forward to strengthening this relationship going forward."

Cem Zorlular, Chairman and Chief Executive Officer of Er-Kim, commented:

"We are incredibly motivated by the prospect of enabling access for patients in Bulgaria and Romania to Diurnal's much needed pediatric and adult cortisol deficiency treatments. Furthermore, we feel privileged to expand our collaboration with Diurnal who share our passion in addressing patients' unmet needs in rare and chronic endocrine conditions."

This is a business press release containing financial information and/or data for the benefit of shareholders and potential investors. Data are included to allow informed investment decisions.

This announcement contains inside information for the purposes of Article 7 of Regulation (EU) 596/2014 (MAR).

Notes to Editors

About paediatric adrenal insufficiency

Paediatric AI, including the genetic condition CAH is a condition characterised by deficiency in cortisol, an essential hormone in regulating metabolism and the response to stress. The primary symptoms of AI are chronic fatigue and patients are at risk of adrenal crisis and death if they do not have adequate cortisol replacement. AI is either primary or secondary, with primary AI resulting from diseases intrinsic to the adrenal gland and secondary AI resulting from pituitary diseases where there is a failure of stimulation of the adrenal by the pituitary of the signalling hormone ACTH (adrenocorticotropic hormone).

About Alkindi® (hydrocortisone granules in capsules for opening)

Alkindi® is the first preparation of hydrocortisone specifically designed for use in children suffering from paediatric adrenal insufficiency (AI), including congenital adrenal hyperplasia (CAH). Alkindi® is a patented, oral, immediate-release paediatric formulation of hydrocortisone granules in capsules for opening that allows for accurate age-appropriate dosing in children. This therapeutic approach has the potential to help young patients less than eighteen years of age suffering from diseases due to cortisol deficiency including paediatric AI and CAH. AI requires life-long treatment and Diurnal's novel approach to product development has the potential to significantly improve these young patients' lives. The European Commission has granted a paediatric use marketing authorisation (PUMA) for Alkindi® as replacement therapy of AI in infants, children and adolescents (from birth to ® is approved and known as Alkindi® Sprinkle in the US where it is licensed and marketed by Eton Pharmaceuticals. Alkindi® is also approved in Israel and Australia. The human medicine European public assessment report (EPAR) for Alkindi® can be viewed here and the Summary of Product Characteristics (SmPC) here.

About congenital adrenal hyperplasia

Congenital adrenal hyperplasia (CAH) is an orphan condition caused by deficiency of adrenal enzymes, most commonly 21-hydroxylase. This enzyme is required to produce the adrenal steroid hormone, cortisol. The block in the cortisol production pathway causes the over-production of male steroid hormones (androgens), which are precursors to cortisol. The condition is congenital (inherited at birth) and affects both sexes. The cortisol deficiency and over-production of male sex hormones can lead to increased mortality, infertility and issues during sexual development including ambiguous genitalia, premature (precocious) sexual development and short stature. Sufferers, even if treated, remain at risk of death through an adrenal crisis.

Current therapy for CAH uses a variety of generic glucocorticoid (steroids including hydrocortisone, dexamethasone, prednisolone and prednisone in the US) with no standard treatment regimen. Approximately two-thirds of CAH patients are estimated to have poor disease control, leading to elevated androgen levels. The condition is estimated to affect a total of approximately 400,000 patients worldwide.

About Efmody® (hydrocortisone modified-release hard capsules)

Efmody® is a preparation of hydrocortisone that has been specifically designed to mimic the circadian rhythm of cortisol when given in a twice-a-day "toothbrush" regimen (administered last thing at night before sleep and first thing in the morning on waking) to control androgen excess and chronic fatigue in patients with diseases of cortisol deficiency. The first indication for Efmody® is Congenital Adrenal Hyperplasia (CAH) in adults and adolescents (children older than 12 years of age). Efmody® has been extensively studied in 239 human subjects including 138 CAH patients who have taken part in clinical trials in Europe and the US.

The European Commission market authorisation of Efmody® is based on a Phase 3 study conducted in a total of 122 patients enrolled across 11 clinical sites, the largest ever interventional clinical trial completed in CAH. The Phase 3 data was supported by detailed analysis of data from an open-label safety extension study for patients completing treatment in the Phase 3 study, which is assessing the impact of treatment with Efmody® over an extended period, with a number of patients on this trial having been treated for over 54 months. Patient retention rates in this study have been high and patients on this trial have, to date, shown sustained benefit from extended Efmody® treatment. The human medicine European public assessment report (EPAR) for Efmody® can be viewed here and the Summary of Product Characteristics (SmPC) here.

About Diurnal Group plc

Diurnal Group plc is a European, UK-headquartered, specialty pharmaceutical company dedicated to developing hormone therapeutics to aid lifelong treatment for rare and chronic endocrine conditions, including congenital adrenal hyperplasia, adrenal insufficiency, hypogonadism and hypothyroidism. Its expertise and innovative research activities focus on circadian-based endocrinology to yield novel product candidates in the rare and chronic endocrine disease arena.

For further information about Diurnal, please visit www.diurnal.co.uk

About Er-Kim

Er-Kim is a leading commercial partner for global biotech and pharma companies seeking to commercialise its products in Turkey, Europe and Middle East. In its 40-year of presence, Er-Kim has commercialised over 150 original therapies from more than 50 leading global pharmaceutical companies. Additionally, Er-Kim operates in drug discovery for unmet needs in rare blood diseases and R&D providing cost-effective manufacturing of advanced medicinal products easing the economic burden of the healthcare system.

For further information about Er-Kim, please visit www.erkim-ilac.com.tr/en/ 

Date of Preparation: June 2021 Code: CORP-GB-0133