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 6 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 recurrentseizures. We conclude that acute Coombs-positive giant cell hepatitis of infancy can be improved by sustained immunosuppressive therapy.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Abbreviations
- AIHA:
-
auto-immune haemolytic anaemia
- γGT:
-
gammaglutamyl transpeptidase
- LDH:
-
lactate dehydrogenase
- SGOT:
-
serum glutamate oxalate transaminase
- SGPT:
-
serum glutamate pyruvate transaminase
References
Alvarez F, Bernard O, Alagille D (1989) Hepatites auto-immunes de l'enfant. Journées Parisiennes de Pédiatrie. Flammarion Médecine-Sciences, Paris, pp 43–46
Arasu TS, Willye B, Hatch TF, Fitzgerald JF (1979) Management of chronic aggressive hepatitis in chiidren and adolescents. J Pediatr 95:514–522
Bernard O, Hadchouel M, Scotto J, Odievre M, Alagille D (1981) Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood. J Pediatr 99:704–711
Harris RC, Andersen DH, Day RL (1954) Obstructive jaundice in infants with normal biliary tree. Pediatrics 13:293
Maddrey WC (1987) Subdivisions of idiopathic autoimmune chronic active hepatitis. Hepatology 7:1372–1375
Maggiore G, Bernard O, Hadchouel M, Hadchouel P, Odievre M, Alagille D (1984) Treatment of autoimmune chronic active hepatitis in childhood. J Pediatr 104:839–844
Maggiore G, Bernard O, Homberg JC, Hadchouel M, Alvarez F, Hadchouel P, Odievre M, Alagille D (1986) Liver disease associated with anti-liver-Kidney microsome antibody in children. J Pediatr 108:399–404
Panush RS, Wilkinson LS, Fagin RR (1973) Chronic active hepatitis associated with eosinophilia and Coombs-positive hemolytic anemia. Gastroenterology 64:1015–1019
Richey J, Rogers S, Thiel DH van, Lester R (1977) Giant multinucleated hepatocytes in an adult with chronic active hepatitis. Gastroenterology 73:570–574
Thirunavukkarasu S, Fitzgerald JF (1979) Giant multinucleated hepatocytes in children with chronic aggresive hepatitis (CAH). Gastroenterology 76:1261
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Brichard, B., Sokal, E., Gosseye, S. et al. Coombs-positive giant cell hepatitis of infancy: effect of steroids and azathioprine therapy. Eur J Pediatr 150, 314–317 (1991). https://doi.org/10.1007/BF01955929
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01955929