Related Experiment Videos
Hepatocellular enzyme patterns and hepatitis B virus exposure in multitransfused young and very young hemophilia
Insights
Children with severe hemophilia A often experience liver function abnormalities and hepatitis B virus exposure from factor VIII transfusions. Early screening and monitoring are crucial for managing these risks in young patients.
Area of Science:
- Pediatric Hematology
- Hepatology
- Transfusion Medicine
Background:
- Children with severe hemophilia A receive factor VIII transfusions, increasing exposure risks.
- Liver function and viral hepatitis are significant concerns in this patient population.
Purpose of the Study:
- To evaluate liver function tests (LFTs) in young hemophilia A patients.
- To assess the relationship between transfusion therapy and liver health.
- To determine the prevalence of hepatitis B virus (HBV) and non-A, non-B hepatitis.
Main Methods:
- Longitudinal study of 38 children (≤11 years) with severe hemophilia A.
- Initial and follow-up LFTs, including serum enzyme levels (AST, ALT, LDH).
- Serological testing for hepatitis B surface antigen (HBsAg) and antibody.
Main Results:
- 39-51% of patients had abnormal LFTs at baseline.
- 44% of initially seronegative patients developed HBV antibodies.
- Two children developed HBsAg-positive hepatitis.
- 55% of a subgroup receiving screened products had elevated ALTs, suggesting non-A, non-B hepatitis.
Conclusions:
- Young hemophilia A patients frequently exhibit abnormal LFTs and HBV exposure.
- Non-A, non-B hepatitis is prevalent even with screened transfusion products.
- Ongoing monitoring of liver function and viral status is essential.
Abstract:
Thirty-eight children with severe hemophilia A, 11 years of age and under, were evaluated by initial and follow-up liver function tests (LFTs) in relation to age of onset of transfusion therapy. Each child had at least two complete evaluations within one year for a follow-up period of at least one year. The mean number of exposure days was 36 with a mean of 275 units of factor VIII per exposure day prior to initial LFTs. At initial testing, 30% of patients demonstrated antibody to HBsAg and 39--51% at least one abnormal serum enzyme level (AST, ALT, LDH). During an average follow-up period of 34.8 months, two children developed HBsAg-positive icteric hepatitis. Of those initially serologically negative for HBsAg or antibody, 44% became antibody-positive. Intermittent abnormalities of at least one serum enzyme were observed in 79% of the patient group, with 13% and 8% being persistently normal and abnormal. Eleven children born after January 1976, receiving only third-generation RIA-tested products for HBsAg, constituted a subgroup. Although only one child at first assessment had evidence of hepatitis B virus exposure, 55% had elevated ALTs, indicating considerable frequency of non-A, non-B hepatitis in this very young group.