[Difficulties in the diagnosis of congenital hyperplasia of adrenal glands].
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Biomedical subjects
Publications and source records attributed to R Bernheim.
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Systematic studies of blood glucose regulation have been performed in 103 cases of adult acquired hypothyroïdism. The results were the following: 11 cases of overt diabetes were discovered. The diagnostic was made when fasting blood glucose was superior to 120 mg/100 ml and glucosuria was present. 22 cases of asymptomatic (chemical) diabetes. The diagnosis was based on the abnormalities of the oral glucose tolerance test (OGTT). The criteria were those of Fajans and Conn. In 70 cases the results were normal. The comparison of these results with these obtained in a matched population of normal subjects did not show significative differences. Particularly the proportion of "flat curves" during the OGTT were not higher in cases of hypothyroïdism. Plasma insulin levels were determined in 20 cases of hypothyroïdism during OGTT. The results were the same as in non-hypothyroïd subjects. The abnormalities of glucose regulation observed in hypothyroïdism were not related to sex, age, overweight or cause of the disease. The number of diabetic patients is not significatively more important in cases of hypothyroïdism of immunological origin (especially Hashimoto's disease.
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Six women aged from 17 to 30 years (mean: 21 years) were referred on account disorders which had begun at puberty and had been present for 3 to 15 years. The reasons for consulting were hirsutism in 5 cases and sterility in one. The patients' height (1.61 to 1.70 m; mean: 1.64 m) and weight (54 to 70 kg; mean: 59 kg) were normal. Three women menstruated regularly and 3 had anovular spaniomenorrhaea. Hirsutism with enlargement of the clitoris (Prader's stage I) was apparent in all six cases. Three patients had permanent, though moderate hypertension. The biochemical changes essential to the diagnosis were as follows: 1) in 2 women with 21-hydroxalyse deficiency there was a rise in cortisol precursors (plasma 17-OH progesterone and its urinary metabolite, pregnanetriol). In 4 women with 11-hydroxylase deficiency urinary 17-OH corticosteroids were increased; two had high desoxycorticol levels. A rise in plasma desoxycortisol/cortisol ration under tetracosactrin stimulation is of considerable diagnostic value; 2) plasma androgens (testosterone, delta 4 androstenedione) and their urinary metabolites (17-ketosteroids) were increased; 3) all abnormalities disappeared when the adrenal function was suppressed. Under dexamethasone treatment hirsutism became stabilized or even regressed, blood pressure values rapidly returned to normal, menstrual disorders disappeared and ovular cycles were established. Three women became pregnant and delivered on term.
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