Abstract

A girl and 5 boys 10 → 16 yr old with severe idiopathic short stature were studied. The 2 older boys had also thalassemia major. All were prepubertal. None had received a previous hormonal therapy. Endocrine studies were normal except for extremely-low-for-age levels of DHAS and DHA. 5 mg per day of DHAS was given per os for 6-12 months. Velocity of growth was unchanged. Bone maturation was not accelerated. DHAS, DHA, testosterone (T), Δ4-androstenedione (Δ4), 17OH progesterone (OHP) and cortisol (F) levels and their diurnal variations were measured before and under therapy : DHAS rose 5 → 10 fold, DHA rose 2 → 8 fold during treatment, but they only reached levels normal or subnormal for age. T and Δ4 increased slightly or not. OHP and F levels and their diurnal rhythms were unchanged. In conclusion 1) several months of DHAS substitutive therapy even restituting normal or subnormal levels of DHAS and DHA were ineffective to change the velocity of growth in short patients with delayed adrenarche. 2) A more prolonged therapy might be of interest ; higher doses seem needed in some cases to reach physiological levels of adrenal androgens.

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