title: Portosystemic shunt as a cause of congenital hyperinsulinemic hypoglycemia.
abstract: 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.
authors.affiliation: Division of Endocrinology and Metabolism, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Yoshii
authors.affiliation: Division of Endocrinology and Metabolism, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Noda
authors.affiliation: Division of Endocrinology and Metabolism, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Naiki
authors.affiliation: Division of Endocrinology and Metabolism, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Reiko
authors.initials: R
authors.lastname: Horikawa
mesh_terms: D044903:Congenital Hyperinsulinism / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007223:Infant; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*; D054079:Vascular Malformations / Q000150:complications* / Q000175:diagnosis
keywords: congenital hyperinsulinism; hyperinsulinemic hypoglycemia; insulin clearance; portosystemic shunt; post-prandial hypoglycemia