[Familial Budd Chiari syndrome and glomerular involvement]

We report a case of a child with familial Budd-Chiari syndrome and proteinuria. Renal biopsy disclosed predominant arteriolo-capillary endothelial and mesangial lesions suggesting a microangiopathy. This may be due to hypoxemic damage with polycythemia and high venous pressure resulting from precapillary pulmonary arteriovenous shunts.