A Summary of the Endocrine Society Clinical Practice Guidelines on Congenital Adrenal Hyperplasia due to Steroid 21-Hydroxylase Deficiency

The Endocrine Society, along with 6 sister medical societies in North America and Europe, as well as a patient education and support organization, recently sponsored the development of an updated set of guidelines for clinicians treating child and adult patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency [1]. The reader is referred to brief [2] and comprehensive [3] reviews of this disease for background. A "Consensus Statement" had been published jointly by representatives of pediatric endocrine societies in 2002 [4, 5], and the present Task Force made an effort to include more information regarding adult patients. In addition, the 2010 Endocrine Society Guideline had the benefit of panel members who are experts in evidence based medicine, specifically, systematic literature review and meta-analysis. As readers may be aware, there are very few high quality randomized, controlled clinical trials dealing with CAH treatment. Notwithstanding this obstacle, the Task Force combined the results of meta-analyses in two topical and controversial areas of CAH treatment, with expert's opinions, tempered with valuable judgments to arrive at the recommendations. The guidelines were reviewed and approved sequentially by The Endocrine Society's Clinical Guidelines Subcommittee and Clinical Affairs Core Committee, members responding to a web posting, The Endocrine Society Council, and finally by representatives of each of the cosponsoring sister medical societies. At each stage the Task Force incorporated changes in response to written comments. Thus, the document represents the collective wisdom of a large and diverse group of practitioners.The following is a summary of recommendations presented in each of ten areas of interest. A description of the GRADE system for ranking the quality of medical evidence is reviewed in [6]. In brief, "recommendations" are relatively firmly held and graded "1," whereas less well-supported "suggestions" are graded "