Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty children with sickle cell anaemia had their serum alanine aminotransferase, alkaline phosphatase, total protein, albumin and bilirubin, assayed during vaso-occlusive crisis and at recovery. Alanine aminotransferase, alkaline phosphatase and bilirubin levels were significantly higher during crisis than at recovery, (p < 0.005) especially in the young patient. However, the total protein and albumin levels were not significantly different in crisis and at recovery. A transient hepatic functional derangement during vaso-occlusive crisis is a probable explanation for the reported changes.
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PMID:Hepatic function tests in children with sickle cell anaemia during vaso occlusive crisis. 788 14

The liver is one of the organs involved in the multiorgan failure that occurs in sickle cell disease, the pathophysiology of liver disease in this condition is complex because of the interrelated multifactorial causes. Liver dysfunction was assessed in both paediatric and adult sickle cell disease patients in the steady state. The transaminases and alkaline phosphatase were analysed by automation while coagulation studies were done manually. The mean (range) of Alanine transaminase (ALT), Aspartate transaminase (AST) and alkaline phosphatase (ALP) were 23.0 (2-77) IU, 48.5 (15-120) IU, 227.5 (37-1200) IU respectively. ALT and AST levels were less than 100 IU in over 95% of the patients. The gender or age of the patients did not significantly affect the level of these three enzymes. There was close association between the liver size and elevation of the liver enzymes except for alkaline phosphatase (ALT=.017, AST=.009, ALP=.056). Twenty-five percent of the patients had normal enzymes while 13% had derangement of the three enzymes, 19%, 50% and 74% had abnormal ALT, AST and ALP respectively. Only 22% and 5% had deranged PT and APTT respectively. In conclusion minimal elevation of the tramsaminases which is not gender or age dependent were observed in steady state sickle cell disease, higher levels of alkaline phosphatase may be due to associated vasoocclussive crises involving the bones rather than a pathology of the liver.
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PMID:Liver dysfunction in steady state sickle cell disease. 1643 91