Hypoglycemia neonatal

Fructose-1,6-bisphosphatase deficiency, first describ ed by Baker and Winegrad in 1970, has now been reported in approximately 30 cases. It is more common in women and is inherited as an autosomal recessive disorder. Initial manifestations are not strikingly dissimilar from those of glucose-6-phosphatase deficiency. Neonatal hypoglycemia is a common presenting feature, associated with profound metabolic acidosis, irritability or coma, apneic spells, dyspnea, tachycardia, hypotonia and moderate hepatomegaly. Lactate, alanine, uric acid and ketone bodies are elevated in the blood and urine [11]. The enzyme is deficient in liver, kidney, jejunum and leukocytes. Muscle fructose-1,6-bisphosphatase activity is normal. 

Glutaric aciduria type II, which is a defect of P-oxida-tion, may affect muscle exclusively or in conjunction with other tissues. Glutaric aciduria type II, also termed multiple acyl-CoA dehydrogenase deficiency (Fig. 42-2), usually causes respiratory distress, hypoglycemia, hyperammonemia, systemic carnitine deficiency, nonketotic metabolic acidosis in the neonatal period and death within the first week. A few patients with onset in childhood or adult life showed lipid-storage myopathy, with weakness or premature fatigue [4]. Short-chain acyl-CoA deficiency (Fig. 42-2) was described in one woman with proximal limb weakness and exercise intolerance. Muscle biopsy showed marked accumulation of lipid droplets. Although... 

Glucagon, used to treat persistent hyperinsulinemic hypoglycemia of infancy, caused erythema necrolyticum migrans in two neonates (17). 

Sulfonylureas (n = 68) and metformin (n = 50) have been compared retrospectively with insulin (n = 42) in pregnancy (131). There were no severe attacks of hypoglycemia, no jaundice, and no differences in neonatal morbidity. However, in those who took metformin, preeclampsia and perinatal deaths were more common. Since metformin was given to obese women, and since obesity contributes to pre-eclampsia and perinatal mortality, this may have been an effect of obesity. 

Hypoglycemia has been reported in a neonate whose mother had taken tolbutamide (139). 

Because all the myriad consequences to the infant of a diabetic mother arise from maternal hyperglycemia, the therapy of hyperglycemia for the infant should begin before birth. Careful management of the maternal diabetes to prevent both hypoglycemia and hyperglycemia will lessen the likelihood to fetal death or neonatal hypoglycemia. In the present case, economic hardship (the husband s layoff and... 

It is not surprising that infant L. suffered diffuse encephalopathy (brain disorder), a cerebral infarction, and seizures during the neonatal period (Yager, 2002). Both asphyxia and hypoglycemia are injurious to the brain. The treatment for seizures consists of providing normal metabolic substrates (e.g., glucose) and appropriate anticonvulsant therapy (phenobarbital), as was done in the present case. The long-term treatment for the child s developmental disabilities is complex and involves the skills of many members of the health care team. 

Comblath M, Ichord R Hypoglycemia in the neonate. Semin Perinatol 24 136-149,2000. 

The second section of the book is Fuel Metabolism and Energetics. Important pathways and enzymes involved in fuel utilization are discussed in the chapters Pyruvate Dehydrogenase Complex Deficiency Mitochondrial En-cephalomyopathy, and Systemic Carnitine Deficiency. The role of gluconeogenesis in glucose homeostasis is illustrated by a discussion in the chapter Neonatal Hypoglycemia. 

Intrauterine fetal death and prolonged symptomatic neonatal hypoglycemia have been reported after treatment of the mother with sulfonylurea drugs (13,14). 

P Zucker, G Simon. Prolonged symptomatic neonatal hypoglycemia associated with maternal chlorpropamide therapy. Pediatrics 42 824, 1968. 

Withdrawal effects of SSRI treatment may be apparent in neonates shortly after delivery. These include jitteriness, hypoglycemia, hypothermia, and respiratory distress (83). 

Hypoglycemia, requiring temporary glucagon and glucose supplementation, was noted in a neonate whose mother had taken lithium throughout pregnancy (cord lithium concentration 1.73 mmol/1) (472). 

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