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Biomedical subjects

E Colle

Publications and source records attributed to E Colle.

At least 73 records · Page 4Linked to original sources

Depressed T cells following neonatal steroid treatment.

Fourty-four patients received two doses of 12.5 mg/kg of hydrocortisone or placebo on the first day of life in attempted therapy for respiratory distress syndrome. Follow-up studies were performed on survivors at 5 years of age in ten steroid-treated and seven placebo-treated respiratory distress syndrome subjects. There were no significant differences in growth, intelligence tests, or neurologic examinations in the patients assessed. Abnormal EEGs are present in both groups. Immunologic tests showed no differences in lymphocyte counts, immunoglobulin levels, diphtheria and tetanus antibody titers, or complement components. Diminished percentages of T lymphocytes were found in steroid patients (53%) compared to control subjects (69%). There were also increased percentages of lymphocytes with C3 receptors in steroid patients (20.1%) compared to control patients (13.8%). Episodes of otitis and/or pneumonia were documented in eight of 11 steroid-treated patients between the ages of 1 and 5 years, compared to two of seven patients in the placebo group in the same time period. It is concluded that large doses of steroids on the first day of life may induce lasting immunologic abnormalities and may predispose to an increased incidence of infections.

Anthropometry

Metabolic abnormalities in the idiopathic Fanconi syndrome: studies of carbohydrate metabolism in two patients.

Two patients with idiopathic Fanconi syndrome and glucose intolerance were studied from a metabolic perspective. They had fasting hyperglycemia, massive glucosuria, insulinopenia, ketosis, and elevated serum free fatty acids. There was a markedly blunted insulin secretory response to glucagon, tolbutamide, glucose, and arginine. One patient had the findings of diabetic retinopathy and a sensory neuropathy. Neither patient could convert galactose to glucose, but they did not have galactosemia. As a result of these studies, and previous reports in which similar changes were noted, we conclude that diabetes mellitus may occur in patients who have had idiopathic Fanconi syndrome for many years.

Adolescent

Incidence of respiratory distress syndrome following antenatal betamethasone: role of sex, type of delivery, and prolonged rupture of membranes.

Premature male infants have a higher incidence of respiratory distress syndrome (RDS) than female infants (male/female = 1.7:1.) With the demonstration that antenatal use of betamethasone significantly reduces the incidence of RDS, a proportional reduction or possibly a complete elimination of the discrepancy between the two sexes might be expected. The role that sex, type of delivery, and prolonged rupture of membranes (PROM) may play in the development of RDS was tested in a prospective study of RDS prevention with betamethasone. A full course of betamethasone, ie, 12 mg, 24 hours apart, was given antenatally to 94 infants of 27 to 34 weeks gestation. The incidence of RDS in male infants was 29.1% vs 8.6% in female infants (P less than .02). The incidence of RDS among male babies delivered by cesarean section (30.3%) or vaginally (29.7%) was not statistically different, nor was there a difference between those with PROM greater than 24 hours (26.9%) and those without PROM (31.8%). The same was true of the incidence of RDS among female infants: cesarean section (14.2%) vs vaginal delivery (6.2%) and PROM greater than 24 hours (8%) vs (9.5%) without PROM. We conclude that: (1) Prevention of RDS with betamethasone is far more effective in female infants. The natural ratio of male/female of 1.7:1 becomes 3.4:1. (2) Once lung maturity has been achieved with betamethasone, the type of delivery plays no role in the development of RDS. (3) PROM does not add extra protection from RDS in the 27- to 34-week gestational age range.

Betamethasone

Profiles in altered metabolism. II--(omega -- 1)-hydroxyacid excretion in a case of episodic hypoglycemia.

A patient with recurrent severe hypoglycemia resembling Reye's syndrome was found to have large accumulations of omega -- 1 hydroxy and keto acids in serum and urine that persisted following clinical recovery. A deficiency of mitochondrial medium chain acyl CoA dehydrogenase activity is proposed on the basis of evidence obtained using gas chromatographic mass spectrometric techniques. Analytical data is presented that will allow the recognition of ths variant presenting in other patients.

Acute Disease

Insulin dependent diabetes: a comparison of families with single and multiple affected siblings.

Families (n = 14) with more than 1 sibling with insulin dependent diabetes were matched with families of similar size and age distribution containing only 1 affected child. The distribution of HLA haplotypes, age of onset of disease, and seasonal onset of disease were compared in the two groups. The data are not consistent with the hypothesis of a single autosomal recessive gene linked to the HLA region. The data do not permit a choice between other current hypotheses although they are compatible with the theory of 2 genes linked to the HLA region, acting additively, and requiring interaction either with environmental factors or other disease susceptibility genes. Diabetic children in the multiplex and simplex families did not differ in the month of onset of symptoms nor in the age at diagnosis although three multiplex pedigrees in which diabetes developed in all affected children before the age of 6 years were identified.

Adolescent

Inappropriate growth hormone release in the diencephalic syndrome of childhood: case report and 4 year endocrinological follow-up.

A boy with an astrocytoma in the anterior hypothalamic area and typical features of the diencephalic syndrome of childhood was followed with yearly endocrinological evaluation from 10 months of age until he was 5 1/2-years-old. Radiation therapy resulted in temporary clinical improvement. At the time of diagnosis height and bone age were advanced. Basal concentrations of growth hormone (GH) were markedly elevated and could only be partially suppressed by an oral glucose load. There was a paradoxical decline of GH levels following arginine-insulin infusion and an abnormal GH release with TRH administration. Abnormal GH response to the various stimuli persisted for 2 years after radiation therapy. Pituitary secretion of LH, FSH, TSH, prolactin and ACTH remained intact. The characteristic clinical features as well as the endocrinological findings remain unexplained.

Astrocytoma

Ineffectiveness of protamine zinc somatostatin as a long-acting inhibitor of insulin and growth hormone secretion.

The present studies examined the duration of inhibitory action of a preparation of cyclic somatostatin (SRIF) in suspension with protamine zinc (PZ) on (1) physiologic growth hormone (GH) and immunoreactive insulin (IRI) secretion during feeding in the rat and (2) plasma IRI levels in a 10-year-old boy with idiopathic hyperinsulinemic hypoglycemia. Chronically cannulated adult male rats were administered 250 microgram PZ-SRIF subcutaneously twice daily at 12-h intervals for 7 days. Control animals received the PZ vehicle on the same schedule. On the test day, blood samples were obtained every 15 min for periods of 6 h following a single PZ-SRIF injection 1.5 h prior to sampling. Prominent GH secretory bursts were still evident in all PZ-SRIF-treated rats and these pulses occurred at the predictable time in relation to the light-dark cycle. Plasma IRI levels were typically elevated in response to feeding and no significant differences were observed in the mean 6-h GH, IRI, and glucose levels of PZ-SRIF-treated rats when compared with PZ-treated controls. In the boy with idiopathic hyperinsulinemic hypoglycemia, subcutaneous injection of 200 microgram PZ-SRIF depressed plasma IRI levels for only 30 min. These results suggest that PZ is not the agent of choice for significant prolongation of the inhibitory action of SRIF on IRI and GH secretion.

Animals

Abnormalities of carbohydrate metabolism in idiopathic Fanconi syndrome.

Various metabolic studies were performed in a patient with the idiopathic Fanconi syndrome in whom constant ketonuria suggested that organic acidemia might contribute to the metabolic acidosis. Glucose intolerance with a diminished insulin release was found after PO or IV glucose loads and after glucagon administratio. An insulinopenic "diabetes-like" state has not previously been described in such patients. The patient had impaired galactose-glucose interconversion, elevated blood lactate levels, reduced pyruvate levels, and an increased lactate:pyruvate ratio. Hepatomegaly and hypoglycemia were not present, and liver and muscle biopsies revealed no enzymatic evidence of glycogenosis. The erythrocyte UDP galactose transferase activity was normal. The patient failed to convert fructose to glucose and had a rise in blood lactate after ethanol administration. Further studies revealed no production of glucose after alanine or glycerol administraion, each test being associated with elevated blood lactate levels and, after alanine, an increased lactate:pyruvate ratio. The lactate:pyruvate ratio was elevated after glucagon administration with increased lactate and reduced pyruvate concentrations.

Carbohydrate Metabolism

Partial short arm deletions of the X chromosome and spontaneous pubertal development in girls with short stature.

Five additional examples of partial deletion of the short arm of the X chromosome are reported. All of the patients had short stature. The presence of the other stigmata of Turner syndrome, including ovarian dysfunction, appeared to depend on the location of the deletion. Chromosomal analysis of girls with short stature (less than 140 cm), normal pubertal development, and regular menses may reveal that minor deletions of the short arm of the X chromosome are more frequent than has been previously reported.

Adolescent

Hyperbilirubinaemia and idiopathic hypopituitarism in the newborn period.

Two infants with idiopathic panhypopituitarism presented with severe neonatal hypoglycaemia, hepatomegaly and hyperbilirubinaemia (direct and indirect). Abnormal liver function tests returned to normal over a 5--8 month period. The growth rate in the absence of detectable growth hormone was 50% of normal during the first 6 months. The effect of growth hormone on somatomedin levels and growth rate during the first year of life in one of the infants is described.

Adult

Epidemiologic survey of juvenile-onset diabetes in Montreal.

Active search of hospital records was used to survey insulin-dependent juvenile-onset diabetics younger than 17 years resident in General Montreal at the time of onset of symptoms during a seven-year period (1971-1977). A mean annual incidence of 8.8/100,000 was found with variation from year to year (5.8 to 10.3). Eighty percent were five years of age or more at time of diagnosis, and the increase with advancing age was similar to that seen in other studies including the somewhat earlier increase in incidence among females. Seasonal peaks were noted in some but not all years and were more marked in years of high incidence and among males. More cases occurred in areas of high socioeconomic level as measured by average family income. The estimated incidence among siblings of diabetics is 15 times the incidence in the general population. Ten percent of diabetics have a first degree relative who is insulin dependent.

Adolescent

The antenatal use of betamethasone in the prevention of respiratory distress syndrome: a controlled double-blind study.

One hundred forty-six pregnant women were enrolled in a prospective double-blind study to assess the effectiveness and side-effects of antenatal administration of betamethasone in the prevention of respiratory distress syndrome (RDS) in potentially premature infants. On admission to the study, the women were given, at random, either 12 mg of betamethasone or placebo. The same dose was repeated 24 hours later and then weekly up to 34 weeks of gestation. Gestational age of the infants ranged from 25 to 34 weeks, and birth weights ranged between 730 and 2,650 gm. Statistically significant differences in favor of the infants in the betamethasone group were found in the incidence of RDS, 20.7% in the betamethasone group compared with 59.5% in the control group (P less than .005); in the severity of RDS (P less than .05); and in the death rate (P less than .05). A higher incidence of hypoglycemia was found among infants in the betamethasone group (P less than .05). Prolonged rupture of the membranes played no protective role against RDS, and the incidence of infection was similar in both groups.

Betamethasone

Peripheral total parenteral nutrition for premature infants with the respiratory distress syndrome: a controlled study.

In 40 premature infants, in whom severe respiratory distress precluded oral feeding, peripheral total parenteral nutrition consisting of casein hydrosylate, dextrose, and soybean emulsion was compared to nutrition with dextrose and electrolytes. The TPN group received more calories, and the total serum protein in them increased significantly. Metabolic complications did not occur. Transient thrombocytosis occurred in six infants who received TPN and eosinophilia occurred in nine. The case fatality rate in the TPN group (three of 20) was not significantly less than in the glucose group (six of 20). The differences suggested that infants who weigh less than 1,500 gm may receive the greater benefit from TPN.

Blood Proteins

Antiserum to somatostatin reverses starvation-induced inhibition of growth hormone but not insulin secretion.

The role of SRIF in starvation-induced inhibition of GH and insulin secretion was assessed by passive immunization with anti-SRIF serum. Six-hour secretory profiles obtained from chronically cannulated male rats deprived of food for 72 h showed marked suppression of GH secretory bursts and significant depression of plasma insulin levels. Administration of 1 ml SRIF antiserum (SRIF AS) iv to starved rats resulted in rapid (within 15 min) restoration of high amplitude GH pulses (600-800 ng/ml) and sighificant elevation of GH trough values. The mean 6-h GH level of starved SRIF, AS-treated rats (189.2 +/- 23.9 ng/ml) was significantly higher than that of starved, normal sheep serum-treated control animals (62.8 +/- 5.8 ng/ml) (P less than 0.005). In contrast to the effects on GH, plasma insulin levels in starved rats administered SRIF AS remained low. No significant difference was observed in the mean 6-h plasma insulin level of starved-SRIF, AS-treated rats when compared to starved, normal sheep serum-treated controls. These findings suggest that circulating SRIF is a physiological regulator of starvation-induced GH suppression but is not involved in mediating the inhibition of insulin.

Animals

Total parenteral nutrition in malnourished infants with intractable diarrhea.

Thirty-four infants (25 Inuit and 9 Caucasians) with protein-energy malnutrition and intractable diarrhea were treated with total parenteral nutrition (TNP) consisting of a casein hydrolysate, a soybean emulsion and dextrose. Initially peripheral veins were used in all the infants, and 22 were treated successfully without resort to a central venous catheter. The mean duration of treatment by the peripheral route was 29 days. Although mean energy intake and protein intake were high, weight gain was poor and growth continued at the prehospitalization percentiles. There were two deaths, both from complications of the use of central lines. Specific diagnoses were established for 7 of the 9 Caucasian infants but only 2 of the 25 Inuit infants. Concentrations of serum glutamic oxaloacetic transaminase (SGOT) were elevated in 80% of the patients at the time of admission, increased further in 82% when TPN was begun, but decreased towards normal before discharge in all patients. Eosinophilia was common during TPN. Liver biopsy in seven patients with elevated SGOT values showed eosinophilia, increased pigment in the Kupffer cells and slight lymphocytosis in the portal tract. Intercurrent infections occurred frequently and were often preceded by a short period of lipid intolerance or neutropenia, or both. Tolerance to lipids returned after the infections resolved. Thus, peripheral TPN is a safe and relatively simple method of providing adequate nutrition during episodes of diarrhea in malnourished infants.

Catheterization