Spontaneous remission in Cushing's disease.

Four patients with proved Cushing's disease underwent spontaneous clinical and biochemical remission. They were part of an 11-patient group treated with methods that allowed spontaneous remission to be observed. Each patient was treated differently, one each with metyrapone tartrate, ketoconazole, bilateral adrenalectomy and adrenal autotransplantation, and no treatment. Spontaneous remission occurred 9 months to 5 years after diagnosis as evidenced by restoration of normal adrenal function occurring symptomatically in two patients and advent to hypoadrenalism with addisonian crisis in the two others. We conclude that spontaneous remission in Cushing's disease may not be a rare entity, although its actual incidence has yet to be established.

Isolated ACTH deficiency with transitory GH deficiency.

A nine-year-old girl, who presented with a severe hypoglycemic coma, proved to have isolated ACTH deficiency, a finding previously reported in only two children. On the initial evaluation, before any treatment, GH did not respond to provocative stimuli. On replacement therapy with hydrocortisone, normal linear growth was observed. Repeated testing while on glucocorticoids replacement four years after the initial attack revealed normal GH response to stimulation test. It is suggested that cortisol deficiency was responsible for the severe hypoglycemic coma and subnormal GH response. A similar mechanism is speculated for the normal growth observed in some patients with apparent deficiency of anterior pituitary hormones, including GH. The possibility of permanent ACTH deficiency and transitory GH deficiency following hypophysitis is discussed.

Leucine-induced hypoglycemia in a family: effect of diphenylhydantoin, oxprenolol, and diazoxide.

The present report describes a mother and 2 children with leucine-induced hypoglycemia (LIH). Hypoglycemic episodes following high-protein meals first appeared at age 4-7 months. Leucine-stimulation tests triggered marked hyperinsulinism and hypoglycemia in the children and a milder but abnormal response in the mother. To evaluate the therapeutic effects and to study the mechanism of hyperinsulinism in LIH, the leucine test was repeated under treatment with diphenylhydantoin, oxprenolol (a beta-blocker), and diazoxide. Diazoxide abolished hyperinsulinism; diphenylhydantoin did not affect the response to leucine; and oxprenolol, tested in the mother only, increased hyperinsulinism and hypoglycemia. Our results indicate that LIH is an autosomal dominant disorder; LIH may persist into adulthood with milder clinical symptoms and chemical response to leucine; diazoxide is the treatment of choice in LIH. Considering the effects of the three agents on stimulated release of insulin, it is concluded that leucine triggers hyperinsulinism by a mechanism different from that of glucose and beta-adrenergic receptors.

Hypopituitarism after aseptic cavernous sinus thrombosis.

A 44-year-old man developed aseptic thrombosis of the cavernous sinuses. He recovered but 3 months later presented a clinical and laboratory picture of pituitary insufficiency. After 3 years of hormonal replacement therapy, he was in good physical condition.

The effect of postural changes on plasma renin activity during normal and pathologic pregnancies.

A study of the effect of posture on plasma renin activity (PRA) in the third trimester in 27 gravidas revealed a significantly greater increase in PRA in the supine position, compared to the left lateral. The women were classified into 3 groups: normal pregnancy, preeclampsia, intrauterine fetal death. There was no statistical difference in PRA among the 3 groups. It is assumed that the increase of PRA in the supine position was due mainly to mechanical pressure by the gravid uterus on the great vessels (regardless of whether the fetus was dead or alive) and that effective circulatory volume was thus reduced. However, low PRA in the left lateral position in women with preeclampsia seemed to correlate with more severe disease in these women.

Further investigation on the predictive value of human placental lactogen in high-risk pregnancies.

Blood samples were taken from 254 women with pregnancies with various complications and 119 completely normal pregnant women for measurement of serum human placental lactogen (hPL) during the third trimester. The value of this test in the management of these pregnancies was retrospectively evaluated through details of outcome. Serum hPL and urinary estriol were compared as tools for assessment of fetal condition. Serum hPL was found to be very efficient in the prenatal diagnosis of intrauterine growth retardation associated with maternal hypertension. Low hPL levels were recorded in all severely hypertensive patients who were delivered of small-for-dates infants (a 100 per cent prediction rate), while 30 per cent of these patients had normal estriol values. Prediction rate of postmature infants by serum hPL level was 70 per cent as compared to 50 per cent by urinary estriol level. The diagnostic significance of low hPL levels is emphasized, with stress upon its value in early detection of unfavorable intrauterine environment. The importance of preterm deliveries in pregnancies involving intrauterine growth retardation and low hPL levels is discussed and demonstration cases are presented.