No abstract

A Negro girl has been followed from age 11/2 to 6 years for a severe musculoskeletal disorder with many of the clinical and roentgenological characteristics of Camurati-Engelmann disease. Atypical features, however, have included a geographic pattern of sclerosis of the long bones, a markedly increased density of the neural arches of the vertebral column, and a sharply demarcated sclerosis in the calvaria. The findings in the long bones and spine have become somewhat less pronounced over a five-year period, but the sclerosis of the skull has increased in both extent and thickness. Initially thought to have Camurati-Engelmann disease, the patient now is considered, on the basis of skeletal changes during this time, to have a diagnosis of craniodiaphyseal dysplasia.

The effect of pituitary stalk section on growth hormone secretion, insulin secretion and carbohydrate tolerance was studied in 13 women with advanced metastatic breast cancer. Plasma immunoreactive growth hormone concentrations were measured during standard insulin tolerance tests in 8 women preoperatively and 12 women postoperatively. Glucose disappearance rates (K 8 ), plasma growth hormone and insulin concentrations were measured after the rapid intravenous administration of glucose in 5 women preoperatively and 9 women postoperatively. In addition, the acute effect of stalk section on thyroidal discharge rate was studied in one patient. Measurable growth hormone levels in the fasting state were found on one or more occasions in 4 out of 8 patients studied during the early postoperative period. None of the 5 patients studied 8 months or longer after surgery had detectable levels of growth hormone under basal conditions. Peak preoperative growth hormone responses to insulin were between 22 and 115 ng/ml, with 2 patients showing exaggerated and prolonged responses. Postoperatively, the responses to insulin were either abolished or markedly attenuated. The responses which did occur had peak values which coincided in time with those of the preoperative tests. These responses progressively diminished in the 3 patients who were tested at successive time intervals after stalk section. Preoperative growth hormone responses to rapid iv glucose were about | the magnitude of the insulin responses. Both early and late peak values were observed.Five of the 8 patients tested postoperatively in this manner had growth hormone responses during the early hyperglycemic phase of the test but no late responses were observed. The mean fasting blood sugar of the group as a whole postoperatively was significantly lower than preoperatively (0.025

ALTHOUGH elaborate classifications have been proposed, most children who develop hypoglycemia after the newborn period still fall into the group vaguely termed "idiopathic hypoglycemia of infancy and childhood." This study was undertaken to determine whether the clinical features and the responses to standard tests of blood sugar regulation are sufficiently distinctive to discriminate "idiopathic" cases from those of known etiology or provide any clues to possible etiologic mechanisms within the idiopathic group. This was done by analyzing the findings in a group of infants and children with idiopathic hypoglycemia and contrasting them with those in selected cases of hypoglycemia caused by hypopituitarism or insulinoma.A further objective of this study was to resolve some of the conflicting recommendations for the management of these patients. This report is based on the records of selected children admitted with the diagnosis of hypoglycemia to the North Carolina Memorial Hospital and Vanderbilt University Hospital. Much of these data were obtained under the ordinary pediatrie ward conditions of the two teaching hospitals. These retrospective analyses are reported together with the results of controlled therapeutic trials performed at the Clinical Research Unit of the North Carolina Memorial Hospital. Materials and Methods Clinical Data.-The charts of children admitted for hypoglycémie convulsions at North Carolina Memorial Hospital and Vanderbilt UniversityHospital were reviewed. Only those patients whose convulsions were proven on one or more occasions to be due to hypoglycemia at a time when no other illness was evident were included in the analysis. Patients were excluded if their hypoglycémie episodes were limited to the neo¬ natal period, or if, on further study, they were found to have glycogen storage disease, galac¬ tosemia, hypopituitarism, liver disease, episodic ketosis, or some other systemic illness. Other patients were not included because their data were insufficient to rule out these disorders or to contribute meaningful information. Thirteen infants and children met these criteria and were selected for analysis. Their findings were con¬ trasted with those of five other patients who were first admitted because of hypoglycémie seizures, but in whom the cause of the hypo¬ glycemia was eventually proven to be hypo¬ pituitarism or an insulinoma. The patients were grouped as follows: Group 1.-This group contained seven infants with hypoglycemia of undetermined cause with the onset of symptoms before 1 year of age. Group 2.-This group contained six children with hypoglycemia of undetermined cause with the onset of symptoms after 1 year of age. Group 3.-Three children with documented