The Endocrine Society has published a new clinical practice guideline on the diagnosis, treatment and management of patients with congenital adrenal hyperplasia (CAH) due to steroid 21-hydroxylase deficiency.
In developing the guidelines, the writing committee commissioned two systematic reviews including a review on genital surgery for females with CAH. The review could not identify enough scientifically rigorous studies delineating a favourable benefit-to-risk ratio for either early or late elective genital reconstructive surgery for females with CAH. The authors said the option to forgo surgery should be considered.
The second review explored the cardiac and metabolic morbidities associated with CAH. The investigators found no direct well-controlled evidence of cardiovascular or metabolic morbidity and mortality associated with CAH and subsequently recommended that individuals with CAH should be monitored according to conventional guidelines for unaffected children, adolescents and adults.
The new guidelines, which update the Endocrine Society’s 2010 guidelines, also provide more detailed protocols for adults. It recommends more moderate glucocorticoid dosing during minor illness or minor surgery. The guidelines recommend prenatal therapy for CAH be avoided due to incompletely defined postnatal risks.
The new guidelines are published in the Journal of Clinical Endocrinology & Metabolism (JCEM).