Robert C. Franks

Neonatal Hyperbilirubinemia and Hypoglycemia in Congenital Hypopituitarism

Christian syndrome in infancy. Br J Dermatol 98:175, 1978 2. Vestermark S: Weber-Christian syndrome in a newborn. Acta Paediatr Scand 55:432, 1966 3. Larkin V, De Sanctis AG, Margulis AE: Relapsing febrile nodular non-suppurative panniculitis (WeberChristian disease); review of the literature with report of a case. Am J Dis Child 67:120, 1944 4. Sanford HN, Eubank DF, Stenn F: Chronic panniculitis with leukopenia (Weber-Christian syndrome). Am J Dis Child 83:156, 1952 5. Forstrom L, Winkelmann RK: Acute panniculitis. A clinical and histopathologic study of 34 cases. Arch Dermatol 113:909, 1977

Maternal and fetal renin activity and renin and big renin concentrations in second-trimester pregnancy

Plasma renin activity (PRA) and the concentrations of renin (PRC) and big renin (PBRC) have been determined in maternal and fetal blood, and renin and big renin have been measured in amniotic fluid, at 16 to 20 weeks of gestation. Gradients between peripheral arterial and venous and uterine venous maternal circulation were not apparent for PRA, PRC, or PBRC. PRC and PBRC but not PRA were consistently higher in fetal cord blood than in the maternal compartment. The concentrations of big renin and of renin were tenfold higher in amniotic fluid than in maternal plasma and were significantly correlated in amniotic fluid but not maternal or fetal plasma.

Aldosterone-producing Adenoma Presenting with Hypokalemic Myopathy Case Report and Review

A 9-year-old boy who complained of fatigue, myalgias, and progressive weakness was found to have a markedly elevated serum creatine phosphokinase (CPK). He developed polyuria with polydipsia and was noted to be hypertensive and severely hypokalemic. Treatment with potassium and spironolactone alleviated his signs and symptoms and normalized the blood pressure and CPK. Initial studies revealed low plasma renin activity that did not increase with change from supine to upright position. Plasma aldosterone was consistently elevated in the supine position, decreased with upright posture, and was not suppressed by administration of dexamethasone. Plasma 18-hydroxycorticosterone also was elevated. Enhanced computerized tomography (CT) revealed a mass in the left adrenal that had not been seen on the initial unenhanced scan. Adrenal vein catheterization confirmed elevated plasma aldosterone on that side. Adrenalectomy was performed, and a well-encapsulated adenoma was found at examination of the surgical speciman. Postoperatively, suppression of plasma renin activity continued for many months without signs of aldosterone deficiency.

Cushing's disease in a child with lymphosarcoma and acute leukemia

A 6‐year‐old girl with lymphosarcoma developed Cushings syndrome. Suppression‐stimulation studies verified adrenal hyperfunction secondary to bilateral adrenocortical hyperplasia, and the course suggested the possibility of “ectopic ACTH” production by the neoplasm as the etiology.

Kinetic Comparisons of Amniotic Fluid Inactive Renin and Renal Renin Using Synthetic and Human Renin Substrates

Inactive renin has been isolated from pooled amniotic fluid and purified approximately 642-fold. Prior to activation the isolates had approximately 4% of the activity found after activation. The observation is similar to that reported for inactive renin from chorionic cell culture and suggests a placental origin of amniotic fluid inactive renin. Using plasma from an estrogen-treated woman, renin substrate was recovered free of renin and inactive renin and a portion was separated into NMW and HMW components. The NMW form constituted approximately 93% and the HMW form approximately 7% of the renin substrate. Amniotic fluid inactive renin was used for determinations of enzyme-substrate kinetics with the pooled, NMW, and HMW plasma substrate and tetradecapeptide synthetic substrate, and the results were compared to similar determinations using standard renal renin. Using synthetic substrate, the kinetics of renal renin and amniotic fluid inactive renin before and after activation were similar. The kinetics of renal renin with pooled, NMW, and HMW plasma substrate were also similar. Amniotic fluid inactive renin had a lower Km with pooled than with NMW substrate, however, which resulted from a significantly smaller Km with HMW component. Although the affinity constants with pooled substrate were not different for renin and inactive renin, the Km of inactive renin was significantly less with the HMW component of plasma renin substrate. The observations are compatible with a role for placental inactive renin in normal pregnancy and suggest the possibility of a further role in hypertensive pregnancy.