Coombs-positive giant cell hepatitis of infancy: effect of steroids and azathioprine therapy.

Abstract:

:An 8-month-old boy and a 7-month-old girl presented with an acute, Coombs-positive auto-immune haemolytic anaemia and severe hepatitis. The clinical manifestations were pallor, jaundice and hepatomegaly. The liver histology revealed diffuse giant cell transformation and extensive necrosis with central-portal bridging. Combined immunosuppressive regimen with steroids and azathioprine led to prolonged clinical and biological remission with a respective 2 years and 7 months follow up. The girl, however, after 7 months developed a progressive encephalopathy of unknown aetiology, while liver and haematological disease were still under control. She died subsequently from severe recurrent seizures. We conclude that acute Coombs-positive giant cell hepatitis of infancy can be improved by sustained immunosuppressive therapy.

journal_name

Eur J Pediatr

authors

Brichard B,Sokal E,Gosseye S,Buts JP,Gadisseux JF,Cornu G

doi

10.1007/BF01955929

subject

Has Abstract

pub_date

1991-03-01 00:00:00

pages

314-7

issue

5

eissn

0340-6199

issn

1432-1076

journal_volume

150

pub_type

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