Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders

Hyperinsulinemic hypoglycemia 4

Congenital hyperinsulinism, nesidioblastosis is the most common cause of persistent hypoglycemia in infancy which is caused by increased insulin secretion despite of low blood glycose levels. The most important complication is cerebral damage as a result of prolonged hypoglycemia. Type 4 of the disease is caused by HADH mutations.

Systematic

Hyperinsulinemic hypoglycemia
HNF1A
HNF4A
Hyperinsulinemic hypoglycemia 1
Hyperinsulinemic hypoglycemia 2
Hyperinsulinemic hypoglycemia 3
Hyperinsulinemic hypoglycemia 4
HADH
Hyperinsulinemic hypoglycemia 5
Hyperinsulinemic hypoglycemia 6
Hyperinsulinemic hypoglycemia 7
Polycystic kidney disease with hyperinsulinemic hypoglycemia

References:

1.

Clayton PT et al. (2001) Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion.

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2.

Molven A et al. (2004) Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation.

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3.

Vidnes J et al. (1977) Glucagon deficiency causing severe neonatal hypoglycemia in a patient with normal insulin secretion.

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4.

Molven A et al. (2002) Hunting for a hypoglycemia gene: severe neonatal hypoglycemia in a consanguineous family.

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5.

Gotlin RW et al. (1970) Neonatal hypoglycaemia, hyperinsulinism, and absence of pancreatic alpha-cells.

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6.

OMIM.ORG article

Omim 609975 [^]
Update: April 29, 2019