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abernethy syndrome in Slovenian children: Five case reports and review of literature

World Journal of Gastroenterology 2020年第37期26卷 5731-5744页

作者： Jerneja Pecek Petja Fister Matjaz Homan Division of Paediatrics University Medical Centre LjubljanaLjubljana 1000Slovenia Department of Neonatology Division of PaediatricsUniversity Medical Centre LjubljanaFaculty of medicineUniversity of LjubljanaLjubljana 1000Slovenia Department of Gastroenterology Hepatology and NutritionDivision of PaediatricsUniversity Medical Centre LjubljanaFaculty of MedicineUniversity of LjubljanaLjubljana 1000Slovenia

BACKGROUND abernethy syndrome is a congenital vascular anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic *** the number of recognized and reported cases is gradually increasing,abernethy syndrome is still a rare disease entity,with an estimated prevalence between 1 per 30000 to 1 per 50000 *** this case series,we aimed to contribute to the growing knowledge of potential clinical presentations,course and complications of congenital portosystemic shunts(CPSS)in *** SUMMARY Five children are presented in this case series:One female and four males,two with an intrahepatic CPSS and three with an extrahepatic *** first patient,who was diagnosed with an intrahepatic CPSS,presented with gastrointestinal bleeding,abdominal pain and hyperammonaemia at six years of *** underwent a percutaneous embolization of his shunt and has remained asymptomatic ever *** second patient presented with direct hyperbilirubinemia in the neonatal period and his intrahepatic CPSS later spontaneously *** third patient had pulmonary hypertension and hyperammonaemia due to complete portal vein agenesis and underwent liver transplantation at five years of *** fourth patient was diagnosed immediately after birth,when evaluated due to another congenital vascular anomaly,and the last patient presented as a teenager with recurrent bone fractures associated with severe *** addition,the last two patients are characterised by benign liver nodules;however,they are clinically stable on symptomatic *** abernethy syndrome is a rare anomaly with diverse clinical features,affecting almost all organ systems and presenting at any age.

关键词： abernethy syndrome abernethy malformation Congenital portosystemic shunt Liver vascular malformation Children Infants Case report

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