Hyperinsulinism Of Infancy Research Articles

The surgical treatment of hyperinsulinism in infancy and childhood: M. H. Gough. Br J Surg 71:75–78 (January), 1984

The surgical treatment of hyperinsulinism in infancy and childhood: M. H. Gough. Br J Surg 71:75–78 (January), 1984

Hyperinsulinism in infancy and childhood.

Hypoglycaemia is the most common metabolic abnormality in childhood. In addition to the high prevalence of the disorder, hypoglycaemia is of particular importance for paediatricians since it is a potent cause of severe neurological damage when it is persistent or recurrent.

Hyperinsulinism in Infants and Children: Response to Diazoxlde

Early diagnosis and treatment of hypoglycemia in children of any age is important. Prompt treatment with diazoxide of infants or children with documented fasting hypoglycemia is effective. In addition, the blood glucose response to diazoxide is an important diagnostic test for hyperinsulinism along with confirmatory metabolic and hormonal studies. Longitudinal growth, neurologic and metabolic data of 17 infants and children (< 2 years at the time of diagnosis) with hyperinsulinism treated with diazoxide will be presented. The blood glucose of 13 of the 17 patients responded to diazoxide (5-10 mg/kg) within 48 hours. Ten of these children have continued to require low dose (< 5 mg/kg) treatment for symptomatic or laboratory evidence of hypoglycemia (mean duration 4 years, 1-11 years). Hyperinsulinism, although asymptomatic, is still present in the 3 children who have been able to discontinue treatment. The pathological diagnosis of the 4 infants whose blood glucose failed to respond to diazoxide were islet cell adenoma (2) and nesidioblastoma (2). Two of these infants died; the 2 who survived required total pancreatectomies and subsequent treatment with insulin and pancreatic enzymes. Hypertrichosis was evident in all children treated with diazoxide and was dose related. One child developed reversible leukopenia. Neurologic sequellae in most children surviving are surprisingly minimal and unrelated to the age of onset, the degree or duration of hypoglycemia.

Serum branched-chain amino acids in the diagnosis of hyperinsulinism in infancy

Fasting values of branched-chain amino acids (valine, leucine, and isoleucine) were measured by column chromatography in the sera of 27 normal infants and children, 15 days to 9 years of age, 14 children with documented ketotic hypoglycemia one to 7 years of age, and in 14 sera from six infants, 15 days to 2 years of age, with documented hyperinsulinism. In normal children and those with ketotic hypoglycemia, each individual branched-chain amino acid and their sum were significantly negatively correlated with blood sugar values ranging between 11 and 92 mg/dl (P < 0.001). In infants with hyperinsulinism, branched-chain amino acid concentrations were significantly lower (P < 0.001) without correlation with blood sugar values ranging between 13 and 51 mg/dl, and plasma insulin concentrations (9 to 85 microU/ML). In all the children the sum of branched-chain amino acids was positively correlated with blood beta OH butyrate concentrations measured at the same time (r = 0.75, P < 0.001). The association of low blood sugar and low branched-chain amino acid concentrations during fasting seems characteristic of hyperinsulinism, and the measurement of branched-chain amino acids in these infants offers a physiologic indicator of the diagnosis of hyperinsulinism.

Interest of the measure of serum branched-chain amino-acids (BCA) in the diagnosis of hyperinsulinism in infancy: 13

Interest of the measure of serum branched-chain amino-acids (BCA) in the diagnosis of hyperinsulinism in infancy: 13

Neonatal hyperinsulinism—Surgical and pathologic considerations

Hyperinsulinism in infancy is most often associated with a diffuse pancreatic lesion designated islet-cell dysmaturation syndrome. This disease is commonly associated with persistent hypoglycemia which usually results from inappropriate secretion of insulin. Urgent medical therapy consisting of hypertonic glucose infusion, frequent feeding, and diazoxide is mandatory in order to prevent central nervous system damage. Where medical means of therapy are not effective, an early 85% subtotal pancreatectomy with preservation of the spleen, is indicated. Nine infants who suffered from hyperinsulinism are reported. In two, medical measures were sufficient to control the disease. One of these patients in whom treatment was started late, remained slightly mentally retarded. Seven patients underwent 85% pancreatectomy. In one of these, an additional 7.5% of the pancreas had to be removed and in a second patient total pancreatectomy was performed in order to control the disease. One patient died on the eighth postoperative day after acute gastric perforation. There was no evidence of residual brain damage in the patients who underwent subtotal pancreatectomy.

Hyperinsulinism in Infancy: Diagnosis by Demonstration of Abnormal Response to Fasting Hypoglycemia

The metabolic adaptation to fasting in infants with hyperinsulinism was examined to see whether a characteristic abnormality could be found that would aid in the diagnosis of this disorder. Seven infants under 1 year of age with hyperinsulinism were studied; 7 control infants of similar age and 12 children with ketotic hypoglycemia served as contrast groups. At the time of hypoglycemia, four of the seven infants with hyperinsulinism did not have elevated levels of insulin. However, levels of beta-hydroxybutyrate were significantly lower in the infants with hyperinsulinism than in the control and ketotic hypoglycemic groups. Levels of free fatty acids were also lower in the infants with hyperinsulinism. Expected levels and normal limits for beta-hydroxybutyrate, insulin, and free fatty acids when plasma glucose is below 40 mg/100 ml were estimated by combining the control and ketotic hypoglycemic groups. Using these values as standards, the diagnosis of hyperinsulinism can be made by evaluation of the response to fasting hypoglycemia. The application of this approach is illustrated by three case examples.

Hyperinsulinism in Infants and Children: Diagnosis and Therapy*

Hyperinsulinism in Infants and Children: Diagnosis and Therapy*

HUMAN FETAL INSULIN RESPONSE TO SUSTAINED MATERNAL HYPERGLYCEMIA

OBENSHAIN, S. C.; ADAM, P. A. J.; KING, K. C.; TERAMO, K.; RAIVIO, K. O.; RÄIHÄ, N.; SCHWARTZ, R. Author Information

Hypoglycemia due to organic hyperinsulinism in infancy.

HYPOGLYCEMIA in the neonate or young infant is recognized to be a metabolic error with potentially devastating effects on the brain. Neurologic sequelae may occur in the adult, but susceptibility to hypoglycemia is much greater in the immature, poorly myelinated brain of the infant. In addition, the general metabolic immaturity of the neonate allows disturbances of carbohydrate metabolism in a variety of circumstances, such as cold exposure, 1 congestive heart failure, 2 or idiopathic respiratory distress of the newborn. 3 Hypoglycemia in the young infant may be defined as true blood glucose level less than 20 mg/100 ml in the premature infant; less than 30 mg/100 ml in the full-term infant in the first 48 hours of life; and less than 40 mg/100 ml thereafter. 4 Manifestations suggestive of hypoglycemia include pallor, lethargy, limpness, absent Moro reflex, rolling of the eyes or the abrupt appearance of internal strabismus,

Human fetal insulin response to sustained maternal hyperglycemia.

Hyperglycemia was maintained in normal mothers before delivery both at term and early in gestation to study the fetal pancreatic response to glucose. Normal controls and gestationally diabetic mothers were infused with saline. At term the human fetal pancreas responded to elevated blood glucose levels by increasing plasma insulin, and there was a direct correlation between blood glucose and plasma insulin concentrations (r equal to 0.75, p less than 0.001). In early gestation there was an attenuated plasma insulin response. Infants of gestationally diabetic mothers had increased umbilical plasma insulin levels — 23.3 ± 6.9 μU per milliliter (mean ± S.E.M.) — as compared to those of normal controls - 6.4 ± 1.3 μU per milliliter (mean ± S.E.M.) — even though the umbilical blood glucose levels in these two groups were similar. The data support the concept that hyperglycemia leads to hyperinsulinism in infants of diabetic mothers.

Hypoglycemia and hyperinsulinism in infants with erythroblastosis fetalis.

Abstract Hypoglycemia was observed in five of 16 (31 per cent) consecutively studied infants with moderate to severe erythroblastosis fetalis. Hyperinsulinemia was documented in each of four infants on whom measurements were made. All infants with erythroblastosis fetalis should have measurements of blood glucose levels in the first hours of life and after exchange transfusion. If hypoglycemia develops in an infant with erythroblastosis fetalis he should be treated with glucose given intravenously.