2024 Volume 37 Pages 1-7
We report a case of an eight-year-old boy who developed immune complex-mediated membranoproliferative glomerulonephritis (MPGN), seven years after liver transplantation. He underwent Kasai operation at the age of three months, followed by liver transplantation at the age of 19 months for congenital biliary atresia. Since then, he has experienced protein-losing enteropathy due to hepatic vein stenosis and portal hypertension. At the age of eight years, he was referred to our department due to sudden onset of severe proteinuria and hematuria. Renal biopsy revealed mesangial proliferative lesion with double contours of the glomerular basement membrane under light microscopy. Immunofluorescence demonstrated a full house pattern with positive staining for IgG, IgA, IgM, C3c, C4, and C1q. Additionally, electron microscopy revealed subendothelial and mesangial deposits. Considering the absence of clinical signs such as negative antinuclear antibodies and any symptoms indicative of systemic lupus erythematosus, we diagnosed him with immune complex-mediated MPGN, resulting from hepatic vein stenosis and subsequent portal hypertension. After initiating induction therapy with prednisolone, lisinopril hydrate proved to be effective for maintenance. This case underscores the significant concern about the development of immune complex-mediated glomerulonephritis in children, several years after liver transplantation.
