46 XX male with dysgenetic testes.

A 46 XX male with dysgenetic gonad is described. The patient was brought up as a girl and reported with primary amenorrhoea and hirsutism. The pathogenesis and management strategy of this condition are briefly discussed.

Ketoconazole in the treatment of Cushing's disease.

Six patients with Cushing's disease received ketoconazole for 7-10 weeks following ketoconazole challenge test. Clinical and hormonal alterations in circulating cortisol, 17 OH progesterone, androstenedione and testosterone were assessed during the therapy. There was significant clinical improvement which corresponded to a fall in cortisol, androstenedione and testosterone. The rise in 17 OH progesterone and the fall in cortisol suggest a blockade at 17,20 desmolase and 11 B-hydroxylase in the cortisol bio-synthetic pathway.

Effect of sulphonylurea therapy on insulin sensitivity in non insulin dependent diabetics.

Insulin tolerance test was done to assess the insulin sensitivity in a group of untreated non insulin dependent diabetic subjects before and after sulfonylurea (tolbutamide, glibenclamide, glipizide) therapy. The parameters studied were KITT, slope of the blood glucose fall, summation values, and area under the curve of glucose response. All the three commonly used sulphonylurea drugs improved insulin sensitivity after 4 weeks of therapy.

Circulating cortisol & related steroids following ketoconazole challenge in Cushing's disease.

Circulating levels of cortisol, 17 alpha hydroxy progesterone and delta 4 androstenedione were analysed by specific radioimmunoassays in 11 patients with Cushing's disease before and at 2, 4, 6, 12 and 24# h following an oral dose of 400 mg ketoconazole. A significant fall in cortisol (26 - 79.6%, 59.8 +/- 18.7 SEM), delta 4 androstenedione (21.5 - 63.3%, 47.8 +/- 4.87) with a concomitant rise in 17 alpha hydroxy progesterone (113 - 218%, 116 +/- 11.43) were noted, suggesting inhibition of 17, 20 desmolase and 11 beta-hydroxylase enzymes in cortisol biosynthetic pathway.

Persistent Cushing's disease in a patient with invasive pituitary adenoma.

A patient with invasive basophilic pituitary adenoma is described, in whom the hypercortisolaemia and clinical features persisted despite two attempts to reduce the tumour mass by transfrontal pituitary surgery and external beam radiotherapy. The patient responded to oral ketoconazole with clinical and biochemical improvement.

Clinical and hormonal studies in precocious puberty.

Data from 31 children, 23 females (74.2%) and eight males (25.8%) with precocious puberty were analysed. They included 19 cases with central precocious puberty, 5 with premature thelarche, 5 with premature adrenarche and 1 each with adrenal adenoma and virilizing hepatoblastoma. Their clinical and hormonal profile and the treatment outcome are briefly discussed.

Screening for late onset congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Basal and post-ACTH levels of 17 alpha hydroxy-progesterone (17 OHP) were determined in 53 subjects with hirsutism. Late onset congenital adrenal hyperplasia (LOCAH) was detected in five (10.6%) on the basis of elevated basal and/or ACTH stimulated levels of 17 OHP. Of the five patients, two were considered to have a heterogygous state on account of a small rise in stimulated 17 alpha OHP. Screening tests for LOCAH are essential as the clinical diagnosis is not otherwise possible for this treatable and often familial disorder.

Circulating androgens in hirsutism.

The androgen status in fifty consecutive women with hirsutism in the reproductive age group referred to the endocrine clinic was studied. Mean BMI was 24.3 +/- 5.6 kg/M2, 42% had menstrual disturbances and 30%, had severe hirsutism. The hirsute patients had significantly higher estimates of testosterone (48%), dihydrotestosterone (38%) and androstenedione (86%). Based on clinical and hormonal data, polycystic ovarian disease was diagnosed in 20 patients, nonclassical 21 hydroxylase deficiency in one and idiopathic hirsutism in 29.