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[Familial Budd Chiari syndrome and glomerular involvement]
Nephrologie
|January 1, 1985
Summary
Familial Budd-Chiari syndrome in a child presented with proteinuria. Renal biopsy revealed microangiopathy, possibly from hypoxemia due to pulmonary arteriovenous shunts.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Vascular Biology
Background:
- Familial Budd-Chiari syndrome is a rare hepatic vascular condition.
- Proteinuria in pediatric patients requires thorough investigation.
- Pulmonary arteriovenous shunts can lead to systemic hypoxemia.
Observation:
- A pediatric case of familial Budd-Chiari syndrome with proteinuria was studied.
- Renal biopsy was performed to investigate the cause of proteinuria.
- Histopathology revealed arteriolo-capillary endothelial and mesangial lesions.
Findings:
- The renal lesions suggest a microangiopathy.
- Hypoxemic damage secondary to polycythemia may contribute.
- High venous pressure from precapillary pulmonary arteriovenous shunts is implicated.
Implications:
- This case highlights a potential link between hepatic and renal microvascular disease.
- Understanding the pathophysiology may guide future diagnostic and therapeutic strategies.
- Further research into the genetic and vascular underpinnings of familial Budd-Chiari syndrome is warranted.