Growth and body proportions in congenital adrenal hyperplasia.

Abstract

Total height, sitting height, and subischial leg length were measured in 27 patients (19 girls and eight boys aged 4.3-21.1 years) with congenital adrenal hyperplasia to determine the influence of chronic hyperandrogenaemia on body proportions. Proportions were normal in 24 patients with classical congenital adrenal hyperplasia who had received steroid treatment since birth, but one of three patients with non-classical (late onset) congenital adrenal hyperplasia had a disproportionately large trunk. Eleven patients with classical congenital adrenal hyperplasia had completed growth, of whom seven had height standard deviation (SD) scores for chronological age less than zero, and one had extremely short stature (SD score -3.25). In 13 patients who were still growing, nine had height SD scores for chronological age of less than zero despite having mean (SD) advances in bone age over chronological age of 1.64 (1.68) years. Height SD scores for bone age were less than 0 in all 13 patients, indicating a loss of height despite advanced skeletal maturation. Doses of glucocorticoid that permit mild chronic or intermittent hyperandrogenaemia also seem to be associated with mild growth retardation. An adult height below average may be an inevitable consequence for many patients with congenital adrenal hyperplasia receiving conventional glucocorticoid treatment.