Membranoproliferative glomerulonephritis in a child with congenital portosystemic shunt

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...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2024-12, Vol.39 (12), p.3459-3462
Main Authors: Goyal, Divya, Tyagi, Vernika, Mantan, Mukta, Batra, Vineeta Vijay
Format: Article
Language:English
Subjects:
Abdomen
Biopsy
Clinical Insights
Cognition
Creatinine
Edema
Encephalopathy
Glomerulonephritis
Hepatic encephalopathy
Hepatocellular carcinoma
Hyperammonemia
Hyperplasia
Hypertension
Immunosuppressive agents
Kidney diseases
Kidneys
Liver
Medicine
Medicine & Public Health
Microscopy
Nephrology
Nephrotic syndrome
Nodules
Pediatrics
Portal vein
Proteins
Shunts
Steroids
Tomography
Ultrasonic imaging
Urine
Urology
Veins & arteries
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Summary: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.
ISSN:0931-041X
1432-198X
1432-198X
DOI:10.1007/s00467-024-06448-9