The Prospect for a Reasonable Adult Height in Patients with 21-hydroxylase Deficiency Is Dismal...or Is it?

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Abstract 504 Poster Session II, Sunday, 5/2 (poster 206)

Adrenalectomy for the management of congenital adrenal hyperplasia (CAH) has recently been proposed as an alternative to traditional medical therapy with glucocorticoids and mineralocorticoids. This has been based, in part, on the widely-held assumption that adult height in patients with CAH is unavoidably poor, regardless of the control of hyperandrogenism achieved. Such claims continue to be generally accepted, despite historical U.S. (Klingensmith et. al., 1997) and recent European data that suggest otherwise. We conducted a retrospective analysis of growth in all patients with 21-hydroxylase deficiency treated at Indiana University between 1973-1998. Data from 60 patients with 21-hydroxylase deficiency were analyzed. This group consisted of 28 males and 32 females, 41 of whom were diagnosed before one year of age (early). Of the 22 patients who have achieved final height, 13 (59%) are within one SD of target height. Of the 38 patients still growing, 19 (50%) currently have a height for BA that predicts an adult height within one SD of target height. Physicians were asked to rate their patients as having their "good" or "bad" compliance. Patients rated as having good compliance were more likely to have a predicted/absolute final height within one SD of target. There was no difference in outcome between males and females. (Table) These data suggest that at least half of children with CAH may be expected to achieve an adult height within one SD of target height using conventional therapy. The results underscore the importance of compliance with treatment and suggest that increased efforts to improve control and compliance may be more effective than adrenalectomy or the addition of anti-androgens and aromatase inhibitors. Further, meta-analysis of existing studies to confirm these findings as well as controlled prospective trials employing GnRH analogs and/or GH to assess the potential benefit of these adjunctive therapies in optimizing adult height are needed.

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