Abstract

Introduction: Congenital portosystemic shunts (CPSS) are vascular abnormalities between portal and systemic veins with an incidence of 1/30,000-1/50,000 in children. Clinically, shunts are pathologic because they allow the splanchnic venous circulation to bypass the liver and deposit into the systemic circulation. Thus, various biochemical metabolites including ammonia, galactose, and bile acids do not undergo first pass metabolism through the liver and instead accumulate in the blood. Case Report: A 4 week-old male infant delivered via C-section at 36 weeks of gestation with a birth weight of 1, 521 grams (small for gestational age) presented with direct hyperbilirubinemia, …

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