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Biomedical subjects

J P Frank

Publications and source records attributed to J P Frank.

5 recordsLinked to original sources

Syncope with prolonged QT interval.

Four children with syncope had a prolonged QT interval on the electrocardiogram. Neurologic studies were negative. One patient had associated deaf mutism, one had a family history of sudden death and prolonged QT interval, and two had ventricular arrhythmias while being monitored in the hospital. Treatment with propranolol hydrochloride eliminated the syncope in all patients, although the ECGs remained abnormal.

Adolescent

Atypical adrenal insufficiency with failure of the pituitary feedback receptor. A case with associated diabetes mellitus and selective IgA deficiency with steatorrhea.

A 59 year old woman with insulin-dependent diabetes mellitus and chronic diarrhea was found to have mild steatorrhea, selective plasma IgA deficiency and adrenal insufficiency. Significant adrenal secretion of corticosteroids resulted only after prolonged stimulation with large doses of exogenous ACTH. Plasma ACTH levels were not elevated during clinical adrenal insufficiency or after metyrapone administration but did respond normally to vasopressin and insulin-induced hypoglycemia. These studies were interpreted as showing both primary adrenal insufficiency and impaired pituitary reserve for ACTH secretion in response to the feedback stimulus. No deficiency was found in secretion of other pituitary tropic hormones. Jejunal biopsy showed a lack of IgA-containing plasma cells. With cortisone replacement, diarrhea subsided and a malabsorption pattern on a film of the small bowel was no longer seen. IgA deficiency has been noted frequently with steatorrhea but rarely with diabetes and only once previously with adrenal insufficiency.

17-Hydroxycorticosteroids