Last month, The Journal of Clinical Endocrinology and Metabolism published a classic congenital adrenal hyperplasia (CAH)-focused supplement, titled “Challenges and Opportunities in the Management of Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency Throughout the Lifetime.” The supplement contains eight review articles that provide a comprehensive look at the clinical, psychosocial, treatment-related and day-to-day challenges faced by individuals with classic CAH. Neurocrine Biosciences sponsored the publication.
The JCEM supplement explores various aspects of classic CAH management, including pathophysiology, clinical manifestations, treatment challenges, barriers to care, psychosocial impact and advances in treatment, including Neurocrine’s CRENESSITY™ (crinecerfont), a potent and selective oral corticotropin-releasing factor type 1 receptor (CRF1) antagonist. CRENESSITY directly reduces excess adrenocorticotropic hormone and downstream adrenal androgen production, allowing for glucocorticoid dose reduction. The articles aim to provide healthcare professionals with up-to-date insights and evidence-based approaches that can enhance clinical practice and patient care.
The eight reviews include:
- Genetics and Pathophysiology of Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency (Yang M and White PC)
- Clinical Manifestations and Treatment Challenges in Infants and Children with Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency (Nokoff NJ, Buchanan C and Barker JM)
- Challenges in Adolescent and Adult Males with Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency (Claahsen-van der Grinten HL, Adriaansen BPH and Falhammar H)
- Clinical Manifestations and Challenges in Adolescent and Adult Females with Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency (Engberg H, Nordenstrӧm A and Hirschberg AL)
- Mental Health Issues Associated with Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency, (Sandberg DE, Gardner M and Lapham ZK)
- Life With Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency: Challenges and Burdens (Witchel SF, Miller T, McCann E and Gupta A)
- Barriers to the Management of Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency (Eitel KB and Fechner PY)
- Future Directions in the Management of Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency (Sarafoglou K and Auchus RJ)
“Our understanding of the genetics, pathophysiology, and complications of CAH has exploded over the last 20 years,” says Richard Auchus, MD, PhD, professor of Pharmacology and Internal Medicine, Division of Metabolism, Endocrinology, and Diabetes at the University of Michigan. “These reviews capture the many dimensions of this complex condition and its management. The limitations of traditional glucocorticoid treatment, particularly in under-resourced areas, create a large disease burden, multiple co-morbidities, and poor outcomes for many patients. The authors are optimistic that this evolving knowledge and emerging treatments, such as corticotropin-releasing factor type 1 receptor antagonists, hold great promise for more personalized care, with improved androgen control and less glucocorticoid-related health consequences in patients.”
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