Serum aminotransferase activities in sickle cell children during crises
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Published on: March 14, 2017
Insights
Sickle cell disease (HbSS) patients experience elevated liver enzymes and reduced protein during painful crises. These levels normalize during steady states, indicating liver injury linked to crisis frequency.
Area of Science:
- Pediatric Hematology
- Hepatology
- Biochemistry
Background:
- Sickle cell disease (HbSS) is a genetic blood disorder.
- Hepatic involvement is a known complication of sickle cell disease.
- Understanding liver function during disease states is crucial for patient management.
Purpose of the Study:
- To investigate serum liver enzyme and protein levels in children with sickle cell disease.
- To compare these levels during sickle cell crises versus steady states.
- To correlate liver function markers with disease activity and potential hepatic damage.
Main Methods:
- Assayed serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels.
- Measured total protein and albumin concentrations in serum.
- Compared biochemical data between HbSS children (crises and steady state) and healthy controls (HbAA).
Main Results:
- Children with HbSS exhibited significantly higher ALT and AST levels during crises compared to steady states.
- Serum total protein and albumin were significantly lower in HbSS children during crises.
- No significant differences in these markers were observed between HbSS children in steady state and healthy controls.
Conclusions:
- Sickle cell crises are associated with acute hepatic degenerative changes.
- Elevated aminotransferases and reduced proteins during crises suggest liver cell injury.
- Frequent painful crises may lead to persistent hepatic injury and potential cell death in children with HbSS.
Abstract:
A total of 78 children with diagnosis of sickle cell disease (HbSS) aged 1-12 years were involved in this study; while 60 normal children (HbAA) of the same age range served as controls. Serum levels of alanine aminotransferase (EC 2.6.1.2), aspartate aminotransferase (EC 2.6.1.1), total protein and albumin were assayed in all the sickle cell children both in crises and steady state and in all the normal children. During crises, most of the sickle cell children had significantly raised levels of serum aminotransferases together with reduced levels of serum total protein and albumin. When these levels are compared with the ones observed during the steady state, the differences are highly significant. On the other hand, when the results obtained during the steady state are compared with those of the control group, the differences are not significant. These results are discussed in relation to the hepatic degenerative changes observed in sickle cell crises. It is clear from this study that the more clinical painful crises the children experience, the hepatic cells would be exposed to persistent injury which may eventually result in cell death.

