AI Article Synopsis
- * Complications from CPSS, which may include brain and liver issues due to excess ammonia, can lead to serious health problems like recurrent encephalopathy and liver cancer.
- * A case study discusses an 11-year-old girl with an intrahepatic CPSS who had liver lesions and nephrotic syndrome, which was treated with immunosuppressants after being diagnosed with a specific type of kidney disease.
Article Abstract
Congenital portosystemic shunts (CPSS) are rare congenital vascular anomalies characterized by abnormal connections between the portal vein and systemic circulation, bypassing the liver. They can lead to complications such as recurrent encephalopathy, liver nodules, portopulmonary hypertension, and neurocognitive issues due to hyperammonemia and rarely kidney involvement. Hepatic hemodynamic changes can lead to liver nodules and hepatocellular carcinoma, particularly in extrahepatic shunts. We describe here an 11-year-old girl with type 1 intrahepatic portosystemic shunt with focal nodular hyperplasia in the liver, presenting with nephrotic syndrome that was diagnosed as membranoproliferative glomerulonephritis on kidney biopsy and that responded partially to therapy with immunosuppressants.
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| http://dx.doi.org/10.1007/s00467-024-06448-9 | DOI Listing |
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