Coombs-positive giant cell hepatitis of infancy: effect of steroids and azathioprine therapy.
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.
Brichard
B
B
Department of Paediatrics, University of Louvain, Brussels, Belgium.
Sokal
E
E
Gosseye
S
S
Buts
J P
JP
Gadisseux
J F
JF
Cornu
G
G
D000208:Acute Disease; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000188:drug therapy*; D001379:Azathioprine / Q000627:therapeutic use*; D003298:Coombs Test; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006505:Hepatitis / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D009336:Necrosis
D000305:Adrenal Cortex Hormones; D001379:Azathioprine