Treatment of adult men with congenital adrenal hyperplasia syndrome due to 21-hydroxylase deficiency

• Published in: Deutsche medizinische Wochenschrift (1946) Vol. 133; no. 19; p. 1025
• Main Authors: Knape, P, Reisch, N, Dörr, H-G, Reincke, M, Quinkler, M
• Format: Journal Article
• Language: German
• Published: Germany 01.05.2008
• Subjects: Addison Disease - etiology; Addison Disease - prevention & control; Adrenal Hyperplasia, Congenital - complications; Adrenal Hyperplasia, Congenital - etiology; Adrenal Hyperplasia, Congenital - therapy; Adrenal Medulla - physiopathology; Adrenal Rest Tumor - etiology; Adrenal Rest Tumor - prevention & control; Adrenogenital Syndrome - complications; Adrenogenital Syndrome - etiology; Adrenogenital Syndrome - therapy; Adult; Continuity of Patient Care; Glucocorticoids - therapeutic use; Humans; Hypertension - etiology; Hypertension - prevention & control; Infertility, Male - etiology; Infertility, Male - prevention & control; Male; Mineralocorticoids - therapeutic use; Steroid 21-Hydroxylase - metabolism; Testicular Neoplasms - etiology; Testicular Neoplasms - prevention & control
• ISSN: 1439-4413

Information about the treatment of males with congenital adrenal hyperplasia (CAH) is scarce and there are no therapeutical guidelines. The aim of this review is to provide a survey of the current data. An extensive literature research was performed in PubMed for relevant articles published in the last ten years. The aim in the treatment of adult male CAH patients is preservation of fertility, prevention of an addisonian crisis, blood pressure management, prevention of testicular adrenal rest tumors (TART), maintaining well-being and good quality of life, satisfactory sexual function and prevention of long-term side effects of gluco- and mineralocorticoid therapy. The change from paediatric to adult medicine should be handled in a transition outpatient clinic organized by paediatric and adult endocrinologists. Most studies have included only small numbers of patients. The steroid therapy is usually orientated on an individual basis; but, general guidelines are lacking. It is reported that fertility is often impaired and related to the occurrence of TART. Some of these tumors are responsive to altered glucocorticoid therapy. However, glucocorticoid-resistant TART have been described, and testis-sparing surgery seems to be a treatment option. A future system of regular follow-up visits and standardized therapy guidelines are essential to provide a better medical care and a higher quality of life for male patients with CAH.