Clinical and metabolic data of twenty patients with hyperosmolar nonketotic coma (HNC) and ten patients in ketoacidosis (DA) are compared. HNC patients were older; fewer were previously known diabetics; more had multiple chronic diseases. Common precipitating factors in HNC included infection, dehydration and administration of diabetogenic drugs. Blood glucose and urea nitrogen, plasma sodium, bicarbonate and osmolarity were significantly higher in HNC. Plasma potassium and chloride levels were similar in both groups. Patients with HNC had significantly lower plasma levels of free fatty acids, cortisol and growth hormone.Plasma insulin levels in HNC were low and not significantly different from those observed in KA. Patients with HNC required more fluids and less insulin therapy. Mortality was 20 per cent in HNC, lower than that generally observed in this condition, but higher than that of KA, 0 per cent.On the basis of the above findings, it is suggested that dehydration and hyperosmolarity may play significant roles in the etiology of HNC, and that therapy should, therefore, be directed at restoration of normal osmolarity and correction of water deficits with 0.45 per cent saline and moderate amounts of insulin. DIABETES 20: 228-38, April, 1971. Hyperosmolar nonketotic coma is a syndrome characterized by severe hyperglycemia, hyperosmolarity and dehydration in the absence of ketoacidosis. Although first described by Frerichs 1 more than eighty years ago, this condition received little attention until the report of Sament and Schwartz 2 in 1957. Soon afterward additional reports appeared and by 1965 Schwartz and Apfelbaum 3 were able to review sixty-three cases collected from the world literature. Over two hundred case reports have now been published and available data 4 indicate that this syndrome is approximately one sixth as frequent a cause of coma as ketoacidosis. Despite increased recognition, the published mortality rate remains in the order of 40 per cent

No abstract

No abstract

The outcome of treatment in 3 groups of boys with constitutional delay in growth and development given monthly intramuscular injections of testosterone enanthate 200 mg (22 subjects) 100 mg (10 subjects) and 50 mg (12 subjects) was compared with the outcome in a control group (14 subjects) without treatment. The 4 groups were similar in chronological age, height, height age, bone age, height age/bone age ratio, pubertal development and had similar predicted adult heights. All treated subjects achieved an excellent growth response with growth velocities reflecting androgen dose. Bone age advanced commensurate with height age in all the treated groups and \g=D\height age/\g=D\ bone age ratios at the end of therapy did not differ significantly. Nor was there a significant difference in the height prediction by the RWT method before and at the end of treatment. However, the year following treatment, growth velocities reversed so that those who received the largest steroid dose and had grown the fastest, decelerated the most and eventually ended up significantly shorter than their predicted adult height. In contrast the control group and those treated with smaller doses of testosterone & Hintz R L (1982): A prospective randomized study of testosterone treatment of constitutional delay in growth and development in male adolescents. Pediatrics 69:681-687. Sobel , Raymond C S, Quinn V & Talbot (1956): The use of methyl testosterone to stimulate growth: Relative influence on skeletal maturation and linear growth.

No abstract

No abstract

A white man who had been diagnosed, 35 years previously at the age of 27 months, to have precocious puberty, was later determined to have familial male-limited precocious puberty (FMPP), on the basis of his family history, increased serum testosterone, prepubertal concentrations of follicle stimulating hormone and luteinizing hormone, and Leydig cell hyperplasia. Recently, this diagnosis was confirmed by molecular genetic analysis that demonstrated the presence of a heterozygous constitutive activating mutation of the luteinizing hormone/chorionic gonadotropin receptor. This dominant gain-of-function Asp578Gly mutation has been shown constitutively to activate the receptor in the absence of the agonist, leading to enhanced synthesis of cAMP and, in turn, to increased, sustained production of testosterone. In 1994, this patient was found to have a testicular seminoma. He represents the first case of a testicular germ cell tumor described in an FMPP patient, raising the possibility of a potentially harmful effect of prolonged increased concentrations of sex hormones, with onset early in life, upon the cellular components of the testes.

No abstract