Over a 3-year period, the diagnosis of persistent neonatal hyperinsulinism (PNH) was made in seven infants, from an unselected cohort of 18,726 births, all of Saudi Arabian origin. Thus the incidence of PNH was one in 2,675 births. The high incidence, associated consanguinity, and occurrence in siblings suggest that PNH may be inherited as an autosomal recessive disorder.
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References
1.
Stanley CA, Baker L.Hyperinsulinism in infants and children: Diagnosis and therapy. Adv Pediatr1976;23:315-55.
2.
Aynsley-Green A., Soltesz G.Hypoglycaemia due to hyperinsulinism. In: Hypoglycaemia in infancy and childhood. Edinburgh: Churchill Livingstone, 1985;54-102.
3.
Laidlaw GFNesidioblastoma: Islet tumor of pancreas. Am J Pathol1938;14:125-34.
4.
Brown RE, Young RBA possible role for the exocrine pancreas in the pathogenesis of neonatal leucine sensitive hypoglycaemia. Am J Dig Dis1970;15:65-72.
5.
Jaffe R., Hashida Y., Yunis E.Pancreatic pathology in hyperinsulinaemic hypoglycaemia of infancy . Lab Invest1980;42:356-65.
6.
Goudswaard WB , Houlhoft HJ, Koudstaal J., Zwierstra RPNesidioblastosis and endocrine hyperplasia of the pancreas: A secondary phenomenon. Hum Pathol1986;17:46-53.
7.
Yakovac WC, Baker L., Hummeler K.Beta cell nesidioblastosis in idiopathic hypoglycemia of infancy. J Pediatr1971;79:226-31.
8.
Heitz PU, Kloppel G., Hacki W., et al. Nesidioblastosis: The pathologic basis of persistent hyperinsulinemic hypoglycemia in infants. Diabetes1977;26:632-42.
9.
Dahms BB, Landing BH, Blaskovics M., Roc TFNesidioblastosis and other islet cell abnormalities in hyperinsulinemic hypoglycemia of childhood. Hum Pathol1980;11:641-9.
10.
Aynsley-Green A., Polak JM, Bloom SR, et al. Nesidioblastosis of the pancreas: Definition of the syndrome and the management of the severe neonatal hyperinsulinaemic hypoglycaemia. Arch Dis Child1981;56:496-508.
11.
Cardell BSHypertrophy and hyperplasia of the pancreatic islets in newborn infants . J Pathol1953;66:335-41.
12.
Steinke J., Driscoll SGThe extractable insulin content of pancreas from fetuses and infants of diabetic and control mothers. Diabetes1965;14:573-88.
13.
Thomas CG Jr, Underwood LE, Carney CN, et al. Neonatal and infantile hypoglycaemia due to insulin excess: New aspects of diagnosis and surgical management. Ann Surg1977;185:505-17.
14.
Martin LW, Ryckman RC, Sheldon CAExperience with 95 percent pancreatectomy and splenic salvage for neonatal nesidioblastosis. Ann Surg1984;200:355-62.
15.
Misugi K., Misugi N., Sotos J., Smith B.The pancreatic islets of infants with severe hypoglycaemia. Arch Pathol Lab Med1970;89:208-20.
16.
Woo D., Scopes JW, Polack JMIdiopathic hypoglycaemia in sibs with morphological evidence of nesidioblastosis of the pancreas. Arch Dis Child1976;51:528-31.
17.
Schwartz SS , Rich BH, Lucky AW, et al. Familial nesidioblastosis: severe neonatal hypoglycemia in two families. J Pediatr1979;95:44-53.
18.
Stanley CA, Baker L.Hyperinsulinism in infancy: diagnosis by demonstration of abnormal response to fasting hypoglycemia. Pediatrics1976;57:702-11.
19.
Hocking MD, Newell SJ, Rayner Phw.Use of growth hormone in treatment of nesidioblastosis in a neonate . Arch Dis Child1986;61:706-7.
20.
Kisten HF, Crossin RB, Jimenez M., et al. Somatostatin treatment of insulin excess due to β cell adenoma in a neonate. J Pediatr1980;96:145-8.
21.
Bloomgarden ZT, Sundell H., Rogers LW, et al. Treatment of intractable neonatal hypoglycemia with somatostatin plus glucagon. J Pediatr1980;96:148-51.
22.
Bougneres P. , Landier F., Garnier P., Job J., Chaussin J.Treatment of insulin excess by continuous subcutaneous infusion of somatostatin and glucagon in an infant. J Pediatr1985;106:792-4.
23.
Greene SA, Aynsley-Green A., Soltesz G., Baum JDManagement of secondary diabetes mellitus after total pancreatectomy in infancy . Arch Dis Child1985;59:356-9.
