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Portosystemic shunt as a cause of congenital hyperinsulinemic hypoglycemia
Keisuke Yoshii1, Masahiro Noda1, Yasuhiro Naiki1
1Division of Endocrinology and Metabolism, National Center for Child Health and Development, Tokyo, Japan.
Insights
Congenital hyperinsulinemic hypoglycemia (CHH) can be caused by a congenital portosystemic shunt (CPSS). This rare condition bypasses liver metabolism, leading to excess insulin and hypoglycemia.
Area of Science:
- Endocrinology
- Pediatrics
- Gastroenterology
Background:
- Congenital hyperinsulinemic hypoglycemia (CHH) involves excessive insulin secretion from pancreatic beta cells.
- Normally, the liver metabolizes insulin, reducing its systemic concentration via first-pass elimination.
Observation:
- A 5-month-old boy presented with CHH attributed to a congenital portosystemic shunt (CPSS).
Findings:
- CPSS allows significant insulin to bypass hepatic metabolism, leading to elevated systemic insulin levels.
- This bypass results in recurrent hypoglycemia, particularly post-prandial hyperinsulinemic hypoglycemia.
Implications:
- CPSS is a rare but critical differential diagnosis for CHH, especially in infants.
- Recognizing CPSS is vital for timely diagnosis and management of 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.