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Biomedical subjects

A W Steinbeck

Publications and source records attributed to A W Steinbeck.

8 recordsLinked to original sources

Male pseudohermaphrodite reared as a female.

A 31-year-old male pseudohermaphrodite has been brought up as a female. The parents had told their child not to discuss the problem of ambiguous genitalia with anyone. As a teenager masculinization had occurred. Chromosome analysis of both peripheral blood and gonadal tissue was 46XY. The internal gonads were testes surrounded by a rim of ovarian stroma. A rudimentary cervix opened into a small vagina, as did the urethra in a valve-like fashion. The phallus was amputated during surgical reconstruction of the external genitalia to create a phenotypic female and the vagina was enlarged. Oestrogen therapy resulted in breast development.

Child Rearing

The urinary excretion of cortisol and cortisone glucosiduronates in pregnancy.

The urinary excretions of cortisol and cortisone glucosiduronates were measured at monthly intervals in three normal pregnancies by a double isotope dilution derivative method. The excretions were mostly within the normal range, but some were above the normal range suggesting that these excretions may increase in some pregnancies.

Adult

An evaluation of laboratory tests for the detection and differential diagnosis of Cushing's syndrome.

1. Results of tests for the diagnosis of Cushings syndrome of varoius aetiologies are discussed for twenty-five patients in whom the pathology was established by operation or autopsy. 2. Control values for the urinary excretion of free cortisol, 17-OHCS, Porter-Silber chromogens (P-SC) and 17-OS and plasma levels of P-SC are compared with those for normal subjects. 3. The results indicated that urinary values are within the normal range for some patients with Cushing's syndrome. 4. Plasma levels of P-SC in the morning were within the normal range for the majority and elevated for the rest. 5. Some patients showed day-night variation of plasma P-SC but evening values were above the normal range. 6. The expected response for low dosage dexamethasone was found in all patients tested but unexpected responses followed high dosage in some. 7. Plasma 11-OHCS in the five patients tested failed to respond to insulin induced hypoglycaemia. 8. Metyrapone administration and corticotrophin infusion tests had limited usefulness in establishing the aetiology of the disease. The 17-OHCS excretion became raised in the response to corticotrophin and the evaluation was prolonged beyond normal responsiveness.

17-Hydroxycorticosteroids

Total body water content in normal and grossly obese women.

Total body water content was estimated for 27 normal and 27 obese women by an isotope dilution technique using tritiated water. The mean total body water content expressed in absolute amounts was significantly raised in the obese group (P less than 0-001), but was significantly lower (P less than 0-001) when the values were expressed as a percentage of body weight. Significant correlations were found between weight and total body water content (P less than 0-001 and P less than 0-05) for the normal and obese subjects respectively. Separate regression equations based on height and weight were calculated for the normal and obese subjects, and good agreement was found in most instances between the measured and calculated total body water contents. The overweight women, with two exceptions, had total body water values in the expected range, which indicated that they were accumulating fat, not water. Although excess water was found in 2 women, this contributed only about 50% to their overweight.

Adolescent

Pregnanetriolone, a normal steroid metabolite: its excretion by normal, Cushing's syndrome and congenital adrenal hyperplasia subjects.

Synthesis of 3H-pregnanetriolone permitted the estimation of pregnanetriolone in urine with a sensitivity in excess of most previous claims. A good correlation (r = +0.97) was obtained between the values from gas liquid chromatography and those of a double isotope derivative method. In contrast to previous reports, these methods indicated that pregnanetriolone is excreted by normal adults. Urinary pregnanetriolone levels were 18-59 mug/24hr for normal subjects, 35-290mug/24hr in Cushing's syndrome and 250-7000 mug/24hr with congenital adrenal hyperplasia. It is concluded that pregnanetriolone is a normal steroid metabolite and its occurrence in Cushing's syndrome does not necessary indicate an abnormal steroid biosynthetic pathway.

Adrenal Gland Diseases

The urinary excretion of glucosiduronates of cortisol and cortisone.

Cortisol and cortisone glucosiduronic acids were synthesised in a 14C-labelled from and utilised in a double-isotope derivative procedure for the analysis of cortisol glucosiduronate (FG) and cortisone glucosiduronate (EG) in human urine. Normal adults were found to excrete between 16 and 100 mug/24 h of FG (n = 14) and between 55 and 120 mug/24 h of EG (n = 15). Elevated values were observed in subjects with Cushing's syndrome and following ACTH stimulation. Abnormal excretion was noted in one patient with hepatic cirrhosis and in one case of cholestatic jaundice. The ratio FG/EG was markedly increased after ACTH stimulation and, in the normal group, was positively correlated to a highly significant degree (P less than 0.001) with FG excretion. These two observations suggest that EG excretion is less sensitive than FG excretion to variations in cortisol production.

17-Hydroxycorticosteroids