Arch Dis Child 1990 Nov;65(11):1217-21

Severe hypertension after liver transplantation in alpha 1 antitrypsi
deficiency

Noble-Jamieson G, Barnes ND, Thiru S, Mowat AP
Department of Paediatrics, Addenbrooke's Hospital, Cambridge

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