George W. Clayton

Hyperthyroidism attributable to a hyperfunctioning thyroid carcinoma

The historical, clinical, and laboratory data of a 6-year-old girl with unilateral goiterand hyperthyroidism are presented. Both the goiter and the hyperthyroid status were attributed to a hyperfunctioning carcinoma.

FAMILIAL GOITER WITH DEFECT IN INTRINSIC METABOLISM OF THYROXINE WITHOUT HYPOTHYROIDISM

Summary A family of six Negro children,four of whom had goiters, has been described. The goitrous children were found to have a defect in organic binding of iodine, as shown by the release of thyroidal radioiodine after the administration of potassium thiocyanate. Of great interest was the fact that these children have remained euthyroid as determined by clinical and laboratory observations. Studies of thyroid metabolism have been presented and the histopathology of a thyroid biopsy from one of the children has been described. It was concluded that this familyof goitrous children clearly demonstrated examples of “simple” nontoxic goiter due to an intrinsic metabolic defect in thyroxine synthesis.

Struma lymphomatosa in children: Report of 12 cases

Summary The clinical, laboratory, and pathologic findings in 12 children with struma lymphomatosa have been presented. Laboratory studies showed an abnormally low response to thyrotropic hormone. The difference between the levels of protein-bound iodine and of butanol-extractable iodine was greater than normal in all patients but one. The pathologic studies obtained by needle biopsy and in one instance by an operative technique were described. The biopsies revealed the “hyperplastic type” of struma lymphomatosa in all instances. Possible explanations of the laboratory and pathologic findings were discussed, and the therapeutic results of treatment with desiccated thyroid described.

Effect of prior ACTH administration on ACTH release in man

Alterations in levels of plasma unconjugated17-OHCS in response to intravenous synthetic lysine-vasopressin, a corticotropin releasing factor, were utilized as an indicator of ACTH release. A reduced response was noted in subjects who had received prior ACTH as compared with controls and was considered indicative of impaired ACTH release. In view of the adrenal hyperresponsiveness simultaneously induced by prior ACTH administration, this reduction is of even greater magnitude. Application of these findings to the clinical situation of pituitary-adrenal axis suppression encountered after long-term corticosteroid therapy is cautioned against because all factors existent in such instances were not simulated by the conditions of this study.

Stimulation of ACTH-Release in Humans by Non-Pressor Fraction from Commercial Extracts of Posterior Pituitary.

Summary A non-pressor material, extracted from Protopituitrin, known to stimulate release of ACTH in vitro was shown to stimulate release of ACTH in human subjects as evidenced by measurements of plasma 17-hydroxycorticoids. This increased secretion of ACTH was obtained in absence of significant side effects.

Diagnosis and management of individuals with hermaphroditic external genitals.

Abstract An operative procedure is presented for revision of the external genitals of individuals with hermaphroditic external genitals when the gender selected is female and the infant has large labioscrotal folds. The selection of gender for infants born with ambiguous external genitals can and should be made within 3 to 5 days after birth. Female pseudohermaphrodites have normal female internal genitals and should be raised as females. Male pseudohermaphrodites and true hermaphrodites may be raised as either males or females and this decision is dependent entirely on the appearance of the external genitals. Surgical procedures are performed on these infants between the ages of 18 months and 2 years.

Congenital adrenal hyperplasia with defect in electrolyte metabolism; observations before the onset of symptoms.

Five infants with “salt losing” congenital adrenal hyperplasia were treated prior to the onset of symptoms of electrolyte imbalance. All were females with genital defects and all had elevate excretion of both 17-ketosteroids and pregnanetriol. There were observed from birth and the other two were first seen at 4 and 9 days of age, respectively. Hyperkalemia was found in all patients before the onset of symptoms and was the first evidence that an abnormality of electrolyte homeostasis existed. Maximum serum potassium values ranged from 6.8 to 12.5 mEq. per liter prior to therapy. Serum sodium determinations and urinary sodium—potassium ratios were not helpful in establishing an early diagnosis. The signs and symptoms of adrenal insufficiency were prevented by early therapy. Serum potassium concentration fell from the very high initial values; however, mild to moderate hyperkalemia persisted for 2 to 3 months despite therapy with cortisone, desoxycorticosterone, and added salt.