Liliane Loridan

Studies of Liver Glycogenoses, with Particular Reference to the Metabolism of Intravenously Administered Glycerol

Abstract To investigate the pathophysiology of glycogenoses of the liver, glycerol, an endogenous precursor of glucose, was administered intravenously to eight patients and to 22 control subjects. ...

Cushing's syndrome in infancy.

Three infants and one 3-year-old child with Cushings syndrome are reported, and the published cases of Cushings syndrome in infancy are reviewed. One infant had a congenital form of Cushings syndrome, in another the disorder was associated with hemihypertrophy, and the 2 remaining patients had similar multiple congenital anomalies. Three of the patients had hyperplasia of the adrenal glands; the fourth had bilateral carcinoma. The clinical presentation of marked obesity, growth failure, and hypertension was uniform in the 4 patients. None was virilized. The urinary excretion of 17-hydroxycorticosteroids and the concentrations of cortisol in the plasma were increased. In the older patient the normal circadian pattern of adrenal secretion was absent, and the administration of dexamethasone failed to decrease the level of cortisol in the plasma.

Gluconeogenesis and insulin in the ketotic variety of childhood hypoglycemia and in control children.

Following deprivation of carbohydrate and of calories, children with ketotic hypglycemia and control children exhibited equally low levels of glucose and of insulin. Gluconeogenesis from glycerol was unimpaired. In comparison with adults, children readily develop hypoglycemia when deprived of food, possibly due to the relatively greater mass of the brain and its greater need for fuel.

Functional differentiation of glycogenoses of the liver with respect to the use of glycerol.

Abstract In seven patients with glycogenoses of the liver the effects of glycerol administered by mouth on levels of glucose and of lactate, together with the response to epinephrine or glucagon, w...

Hypersecretion of insulin after the administration of l-leucine to obese children

The administration of l -leucine by mouth to 8 markedly obese children resulted in elevations in the concentrations of insulin in the blood which were strikingly greater than those observed in control subjects. Such changes were not dependent on differences in the absolute doses of leucine. Despite the marked elevations of insulin after administration of leucine, the fall in concentrations of glucose differed little from that in the control subjects, reflecting a relative insensitivity to insulin in obese subjects. The increased secretion of insulin after administration of leucine to obese subjects correlates with an increased basal secretory capability of the islets and appears to be in keeping with a hyperresponsiveness of the islets of Langerhans to a variety of stimuli.

An effect of ketones on the concentrations of glucose and of free fatty acids in man independent of the release of insulin

Ketones were infused into dogs, obese hyperinsulinemic patients, children whose weights were in the normal range, and subjects with long-standing, insulin-dependent, juvenile diabetes mellitus. Six of the obese group also had a second infusion two hours after the first one. A significant rise in the serum concentration of insulin was seen in the dogs and in the obese children but only with the first infusion. No increase in the levels of insulin was seen in the control children nor in the obese group with the second infusion. Bv contrast, the blood levels of glucose and of free fatty acids (FFA) declined consistently, even in the absence of a significant increase in the serum concentrations of insulin, and also in the diabetic children who were unable to secrete insulin. It appears that the effect of ketones in lowering levels of glucose and of FFA in the blood may be accompanied by, but need not depend upon, the release of insulin.

Studies of factors affecting gluconeogenesis and glycolysis in glycogenoses of the liver

The activities of fructose-1,6-diphosphatase and of phosphofructokinase, and the concentrations of insulin, growth hormone, cortisol, and free fatty acids, were measured in patients with the three major varieties of glycogenoses of the liver and in control children. In the patients the activity of fructose-1,6-diphosphatase was increased; significant phosphofructokinase activity was present; the concentrations of cortisol and of growth hormone were normal, those of insulin were low, and those of free fatty acids were high. The results were interpreted as indicative of increased gluconeogenesis accompanied by concurrent glycolytic activity; they further suggested: (a) that a primary disorder of the liver may cause modification of the secretion of insulin, and (b) more speculatively, that two functionally distinct Embden-Meyerhof pathways may coexist.

Fat cell function and insulin in a patient with generalized lipodystrophy.

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Effects of infusion of ketones in children with ketotic hypoglycemia

Four children with ketotic hypoglycemia and 2 control children were given infusions of β-hydroxybutyrate (15 Gm. per square meter) after an overnight fast and following the induction of gluconeogenesis by a diet low in calories and carbohydrate. After the overnight fast the concentrations of glucose decreased markedly, to a greater degree than we had previously observed in adults, and lipolysis was inhibited. After the induction of gluconeogenesis the decrease in the concentration of glucose was no longer seen, although the inhibitory effect on lipolysis was still evident. In neither study did the levels of insulin change significantly nor were significant differences of response apparent between the patients and the control children.

The roentgenographic manifestations of Cushing's syndrome in infancy.

Three cases of Cushings syndrome in infancy are described, and the x-ray changes found in previous case reports are reviewed. Atrophy of the thymus associated with excessive deposits of fat and atrophy of muscles in the extremities appear to be characteristic of this age group. When seen in combination with osteoporosis and retarded bone maturation, Cushings syndrome should be suggested. Definitive diagnosis requires an endocrinological evaluation combined with investigation of the adrenal glands by excretory urography, selective angiography, and/or retroperitoneal air insufflation.