Neonate, hypoglycemia

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. 

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... 

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. 

B23. Brans, Y. W., Huff, R. W., Shannon, D. L., and Hunter, M. A., Maternal diabetes and neonatal macrosomia. 1. Postpartum maternal hemoglobin Aj. levels and neonatal hypoglycemia. Pediatrics 70, 576-581 (1982). 

Sosenko, J. M., Kitzmiller, J. L., Fliickiger, R., Loo, S. W. H., Younger, D. M., and Gabbay, K. H., Umbilical cord glycosylated hemoglobin in infants of diabetic mothers Relationships to neonatal hypoglycemia, macrosomia, and cord serum C-peptide. Diabetes Care 5, 566-570 (1982). 

Sulfonylureas are the oldest and most widely used oral hypoglycemic agents. Data on older preparations show transplacental crossing of these compounds. Prolonged neonatal hypoglycemia was more frequently described in infants exposed to sulfonylureas during pregnancy [12]. 

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