[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.
D006502:Budd-Chiari Syndrome / Q000150:complications / Q000235:genetics*; D002648:Child; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology* / Q000473:pathology; D007678:Kidney Glomerulus / Q000473:pathology*
Syndrome de Budd Chiari familial et atteinte glomérulaire.