Factitious hyperinsulinemic hypoglycemia in infancy: diagnostic pitfalls.
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Biomedical subjects
Publications and source records attributed to D V Edidin.
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To determine whether a continuous insulin infusion improves glucose tolerance in extremely low birth weight infants, we conducted a prospective, randomized trial in 24 neonates 4 to 14 days old (mean birth weight 772.9 +/- 128 gm; mean gestational age 26.3 +/- 1.6 weeks). Infants who had glucose intolerance were randomly assigned to receive either intravenous glucose and total parenteral nutrition with insulin through a microliter-sensitive pump or standard intravenous therapy alone. One infant assigned to receive insulin never required it. The groups were similar in birth weight, gestational age, race, gender, medical condition, and energy intake before the study. The mean duration of therapy was 14.6 days (range 7 to 21 days). During the study, the 11 insulin-treated infants tolerated higher glucose infusion rates (20.1 +/- 2.5 vs 13.2 +/- 3.2 mg/kg/min (1.1 +/- 0.1 vs 0.7 +/- 0.2 mmol/L); p less than 0.01), had greater nonprotein energy intake (124.7 +/- 18 vs 86.0 +/- 6 kcal/kg/day; p less than 0.01), and had better weight gain (20.1 +/- 12.1 vs 7.8 +/- 5.1 gm/kg/day; p less than 0.01) than the 12 control infants. The incidence of hypoglycemia, electrolyte imbalance, chronic lung disease, and death did not differ between groups. We conclude that a controlled insulin infusion improves and sustains glucose tolerance, facilitates provision of calories, and enhances weight gain in glucose-intolerant premature infants.
In 6 growth hormone-deficient children, we have demonstrated that 3-N-demethylation of caffeine as measured by the 13CO2 caffeine breath test is decreased following 1 month of growth hormone therapy (8.4 +/- 1.0 vs. 6.8 +/- 1.2% 13C/2 h after treatment). These findings could be of clinical importance. The initiation of growth hormone therapy in growth hormone-deficient children could alter the clearance of drugs metabolized through the mixed-function oxidase pathway given for concurrent conditions, and necessitate adjustment of the therapeutic regimen.
Renal and hepatic levels of the rate-limiting gluconeogenic enzyme phosphoenolpyruvate carboxykinase (PEPCK) were determined in control and intrauterine growth-retarded rate neonates and dams. Dexamethasone and surgically induced intrauterine growth retardation (IUGR) resulted in an elevation of neonatal renal PEPCK activity, but did not affect hepatic PEPCK levels. In the dam, glucocorticoid administration resulted in an increase in renal PEPCK, but did not affect the hepatic enzyme. We have demonstrated that, unlike the hepatic enzyme, renal PEPCK could potentially contribute to gluconeogenesis in utero, since dexamethasone administration significantly increased renal PEPCK at birth. This study confirms the hypothesis that hepatic and renal PEPCK activities are regulated independently in both the adult and neonatal animal.
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This article reviews skin changes occurring in diabetic children. Skin changes may be related to metabolic alterations associated with diabetes. Others may be manifestations of macro or microvascular disease. Insulin injections, required for management of most diabetes in the pediatric age group, may also cause cutaneous changes. There remain several cutaneous abnormalities which are observed with increased frequency among diabetics, the significance of which is unknown. The skin is a potentially invaluable tool for understanding certain diabetic complications; however, the value of observations and experimental data relating to cutaneous changes in diabetics depends upon documentation of the type of diabetes mellitus employing the currently accepted classification system.
Gynecomastia associated with high plasma estrogen levels was observed in a prepubertal boy. The family initially denied ingestion or application of any medications despite careful review of the history, but endocrinologic findings were not consistent with endogenous estrogen production. Persistent inquiry discovered the use of a hair cream containing substantial amounts of estrogen. After discontinuation of its use, breast tissue regressed and estrogen levels returned to normal. To our knowledge, scalp inunction has not hitherto been reported as a cause of prepubertal gynecomastia.
Thirty-three children with type I diabetes mellitus and 51 normal children underwent M-mode echocardiography. Abnormalities of myocardial performance were present in many of the diabetic children. The mean end-systolic volume of the left ventricle was greater in diabetics compared to control subjects. Mean ejection fraction, minor axis shortening, and velocity of circumferential fiber shortening were decreased in the diabetics. There was no evidence of increased myocardial mass in these diabetic children. There was no correlation between myocardial dysfunction, clinical assessment of control, or glycohemoglobin in the diabetic children.
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Ten cases of nutritional rickets seen over a 10-month period are reviewed. The salient clinical features are summarized, and the striking association with unsupplemented breast-feeding, fad diets, and lack of immunizations is discussed. The importance of recognizing such associated practices for purposes of early diagnosis and intervention is stressed.
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A 10-day-old girl was examined because of a peculiar, patterned hyperkeratosis that was noted at birth. The lesions were distributed over the trunk and limbs and consisted of whorls and streaks of thick, yellow, adherent scales. The salient histologic feature was marked hyperkeratosis with deep invagination of the dilated pilosebaceous ostia nearly to the level of the hair bulb. Additional physical abnormalities included patchy alopecia, bilateral zonular cataracts, a depressed nasal bridge, small stature, and multiple skeletal deformities. Roentgenograms demonstrated widespread stippled epiphyses characteristic of Conradi-Hünermann syndrome.
Psychosomatic research findings correlating psychologic stress with diabetic control fail as yet to provide valid conclusions. Investigators have presented many contradictory findings. The two major pathways by which stress could affect control, a) changes in compliance behavior, and b) a neurohumoral axis, have not been clearly distinguished from each other. The study of adolescent cohorts is associated with the problem of heterogeneity, limiting the application of results to other diabetic populations. Methods of determining diabetic control have been incomplete and the definition and measurement of stress have major inadequacies for the analysis of such a complex psychosomatic problem. Existing evidence is comprehensively reviewed and evaluated. The authors use a modified definition of stress to construct a stress scale specific to pregnant diabetics.