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Portosystemic shunt as a cause of congenital hyperinsulinemic hypoglycemia
Author(s) -
Yoshii Keisuke,
Noda Masahiro,
Naiki Yasuhiro,
Horikawa Reiko
Publication year - 2017
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.13245
Subject(s) - hyperinsulinemic hypoglycemia , medicine , hypoglycemia , insulin , endocrinology , portosystemic shunt , portal vein , shunt (medical) , insulin delivery , portal hypertension , diabetes mellitus , type 1 diabetes , cirrhosis
Congenital hyperinsulinemic hypoglycemia ( CHH ) is characterized by the inappropriate secretion of insulin from pancreatic beta cells in the presence of hypoglycemia. We herein describe the case of a 5‐month‐old boy with CHH due to congenital portosystemic shunt ( CPSS ). Insulin secreted from pancreatic beta cells flows into the portal vein and is first metabolized in the liver. First‐pass elimination of insulin in the liver leads to great decrease in insulin concentration by approximately 40–80% in humans. CPSS accounts for a large quantity of insulin delivery into the systemic circulation due to the lack of hepatic first‐pass elimination. Hypoglycemia can result from consistently high levels of insulin after reaching normal glucose level. CPSS therefore should be considered as a rare cause of CHH , especially in the case of post‐prandial hyperinsulinemic hypoglycemia.