432 LOW PLASMA DHEA AND DHEA SULFATE IN PROGERIA. DIMINISHED RESPONSE TO CORTROSYN

Abstract

Progeria (Hutchinson-GiIford syndrome) manifests with apparent accelerated aging during youth. We studied a 21 mo. old female with marked growth retardation (Ht. 76cm, <2SD, Wt. 6.5 Kg <2SD), alopecia, small face, micrognathia, pinched nose and sclerodermic like changes of the skin. Osteoporotic changes were measured by the radiographic photodensitometry of Colbert et al, and compared to age and sex norms (25.7 vs 30-40 nl range). Pituitary and thyroid hormones were within normal limits (hGH 1.8 ng/ml; FSH 3.8 and LH 2.6 mIU/ml; Prolactin 8.0 ng/dl; TSH lμU/ml; T₄ 9.1 and T₃ 180 ng/dl). Insulin2.0μU/ml, Estrogens 1.7ng/dl. DHEA and DHEA sulfate have been reported to markedly decrease with normal aging. To study them in progeria we stimulated the patient's adrenals with 0.125 mg of cortrosyn I.M.(Organon Labs.) and 3 normal controls (Ages 5-9 yr.). Twenty-four hour urinary 17KS before were 0.4 and after 0.5 mg/24 hr. (controls 0.9 and 3.3); DHEA 0: 35 vs 190±72; 1 hr. 70 vs 256±70; 2 hr 35 vs 279±41 and 12 hr. 35 vs 226±186 ng/dl. DHEA-S O: undetectable = 0 vs 28+14; 1 hr 0 vs 29+21; 2 hr 0 vs 32±14; 12 hr 0 vs 26±20 mcg/dl. We concluded that there is a marked decrease in basal and post ACTH stimulation of DHEA and DHEA sulfate in our progeria patient. A deficienty of adrenal androgens might explain the growth retardation and osteoporosis seen in progeria. DHEA has been reported to have antiaging effects It's deficiency in progeria might not only be important as a biochemical marked for the disease but might also contribute to the premature aging.

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