Walter R. Eberlein

Studies of the secretion and metabolic effects of human growth hormone in children with glucocorticoid-induced growth retardation

Plasma growth hormone responses to insulin-induced hypoglycemia, orally administered glucose, and the intravenous infusion of arginine were normal in 4 children with growth retardation secondary to prolonged treatment with glucocorticoids. The short-term administration of growth hormone to 3 of these subjects resulted in nitrogen retention comparable to that observed in a patient with hypopituitarism who was treated similarly. Transient acceleration of linear growth rate was observed when somatotropin was administered to each patient for 6 months. Two of the four patients developed serum gamma globulins capable of binding growth hormone. It has been concluded that glucocorticoids do not suppress growth by inhibition of growth hormone release, but rather by peripheral antagonism to the long-term growth-promoting activities of somatotropin.

Turner's syndrome in the male***

Summary 1. Five patients with the symptom complex described as Turners syndrome in the male are presented. Sixteen additional cases have been collected from the literature. The associated anomalies are summarized. 2. Chromosomal counts in 2 of our patients showed the diploid number of 46 without recognizable abnormalities. This is in accord with other reports. 3. It is suggested that the so-called Turners syndrome in the male is not a well-defined entity supported by objective chromosomal aberrations. Rather, it may represent several loosely related clinical conditions of unknown etiology. The marked clinical similarity, in some instances, to gonadal dysgenesis may signify that the somatic anomalies in the latter are not clearly attributable to the observed chromosomal defect.

Inhibition of thyroidal radioiodine uptake by human growth hormone

Thyroidal 24 hour uptake of 131 I declined by 25 per cent or more of the control value following the short-term administration of human growth hormone in 8 of 15 children with hypopituitarism and in 1 of 5 subjects with growth retardation of other etiology, an effect which was reversed by giving exogenous thyrotropin. The plasma concentration of thyrotropin decreased 20 to 40 per cent in 2 boys with acquired hypothyroidism following administration of human growth hormone. It is hypothesized that growth hormone inhibits the pituitary synthesis and/or release of thyrotropin.

The relationship of juvenile hypothyroidism to chronic lymphocytic thyroiditis

Eighteen cases of acquired juvenile hypothyroidism are described. Significant titers of antithyroid antibodies in sixteen of them suggest an underlying autoimmune thyroiditis. Comparison of these patients with 33 children who have Hashimotos struma indicates that chronic lymphocytic thyroiditis may produce a spectrum of disease in childhood ranging from euthyroid goiter to nongoitrous myxedema.

The effect of arginine infusion on plasmagrowth hormone and insulin in children

The plasma growth hormone (GH) and insulin responses to the intravenousadministration of arginine (0.5 Gm. per kilogram) have been determined in 25 children with intact anterior pituitary function and in 17 children with hypopituitarism. Arginine increased the plasma GH concentrations in 21 of the 25 control subjects, but failed to do so in 4 boys. After pretreatment with diethylstilbestrol, two of these patients had increased GH concentrations in control specimens, while in the other two subjects the GH response to arginine improved. The GH concentration was low in control specimens and did not increase following the administration of arginine in 16 of 17 patients with hypopituitarism. A low normal GH response was recorded in one such patient. The mean plasma insulin responses of the two groups were similar. It is concluded that arginine-induced GH release is a reasonable screening test of pituitary GH reserve, but that abnormal responses or results that do not agree with ones clinical impression should be confirmed by other studies.

Noonan syndrome with intestinal lymphangiectasis: A metabolic and anatomic study

A child with Noonan syndrome presented with failure to thrive, edema of the extremities, and a seemingly mild cardiac lesion at age four months. The edema progressed with the development of anasarca at six years, accompanied by severe hypoproteinemia. Investigation revealed a protein-losing enteropathy (intestinal lymphangiectasis) which responded to medium-chain triglycerides and a low-fat diet. The improvement was transient, and a compartmental shift of fluids further stressed an already compromised myocardium. The pulmonary valvular stenosis was relieved surgically, but the patient died postoperatively. It is suggested that this intestinal abnormality may be another manifestation of a generalized defect in cardiovascular-lymphatic organogenesis in this syndrome. Perhaps the cardiovascular lesion should be corrected before attempting to relieve the malabsorption.

The measurement of low levels of estrone & estradiol-17β in urine, employing ion-exchange, thin-layer, and gas-liquid chromatography

Abstract A method is described for the measurement of estrone and estradiol-17β in low titre urine. It involves ion-exchange column chromatography, borohydride reduction of estrone to estradiol-17β, thin-layer chromatography, and gas-liquid chromatography of the acetylated extract on OV-Non-radioactive estrone is used as an internal standard. The method is demonstrated to be sufficiently reliable for routine endocrine laboratory use.

Erythrocyte glucose-6-phosphate dehydrogenase activity in children with hypothyroidism and hypopituitarism.

Erythrocyte glucose-6-phosphate dehydrogenase activity was measured in 9 children with hypothyroidism, and in 7 children with hypopituitarism. Pretreatment enzyme activities were well below normal in all children with hypothyroidism, and in 6 children with hypopituitarism. The administration of thyroid hormone was followed by increased enzyme activity in 5 of 5 subjects studied. The administration of human growth hormone was associated with increased enzyme activity in 4 of 6 patients after short-term administration, and in 2 of 2 subjects after long-term administration. Red cell G-6-PD activity was a sensitive index of the metabolic status of the patient.

Idiopathic anterior hypopituitarism in one of monozygous twins

Summary Anterior hypopituitarism has been observed in one of monozygous twin females. Clinical evidence suggests that the defect in this instance was acquired postnatally.

Gonadal function in mosaic XO/XY or XX/XY Turner's syndrome

The gonadal function in four children with XO/XY or XX/XY mosaicism was studied. All were raised as females. Three patients, one prepubertal, were surgically explored and testicular tissue was noted in all three. Two of three patients of adolescent age had elevated plasma testosterone values. The third adolescent patient had a normal basal excretion of urinary testosterone glucuronide which increased 10-fold following stimulation with human chorionic gonadotropin. Prophylactic gonadectomy in this syndrome is recommended because of the increased incidence of malignant change in dysgenetic gonads. Temporization of castration appears to be unwarranted because if the gonads are to function at all, they are more likely to produce androgens.