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.

• Authors: B Brichard, E Sokal, S Gosseye, J P Buts, J F Gadisseux, G Cornu
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 150 Issue 5 Pg. 314-7 (Mar 1991) ISSN: 0340-6199 [Print] Germany
• PMID: 2044600 (Publication Type: Case Reports, Journal Article)

Chemical References

• Adrenal Cortex Hormones
• Azathioprine

Topics

• Acute Disease
• Adrenal Cortex Hormones (therapeutic use)
• Anemia, Hemolytic, Autoimmune (complications, drug therapy)
• Azathioprine (therapeutic use)
• Coombs Test
• Drug Therapy, Combination
• Female
• Follow-Up Studies
• Hepatitis (complications, drug therapy, pathology)
• Humans
• Infant
• Male
• Necrosis