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Severe hypertension after liver transplantation in alpha 1 antitrypsin deficiency
G Noble-Jamieson1, N D Barnes, S Thiru
1Department of Paediatrics, Addenbrooke's Hospital, Cambridge.
Insights
Children with alpha 1 antitrypsin deficiency and liver disease often develop severe hypertension after liver transplants, with potential renal complications impacting long-term outcomes.
Area of Science:
- Pediatric Hepatology
- Nephrology
- Transplantation Immunology
Background:
- Alpha 1 antitrypsin deficiency (AATD) is a genetic disorder that can lead to severe liver disease in children.
- Pre-existing renal disease is noted in children with AATD and end-stage liver disease.
- Liver transplantation is a potential treatment for end-stage liver disease caused by AATD.
Abstract:
Five children with alpha 1 antitrypsin deficiency and terminal liver disease received liver grafts; all five became hypertensive and four developed hypertensive encephalopathy. There was evidence of renal disease preoperatively and renal biopsy specimens showed variable glomerulonephritic histology with IgA nephropathy in one, mesangial-proliferative changes in two, and mesangio-capillary glomerulonephritis type I in two. Four hypertensive episodes were preceded by a fall in creatinine clearance. The association of glomerulonephritis with alpha 1 antitrypsin deficiency in children is more common than has been recognised. Affected patients are prone to severe hypertension of probable renal origin after liver transplantation and the renal lesion may affect long term prognosis.