In:
European Journal of Haematology, Wiley, Vol. 98, No. 3 ( 2017-03), p. 296-301
Abstract:
Because of the increased hemolytic rate, a significant proportion of patients with sickle cell disease ( SCD ) are prone to develop cholelithiasis. The present study investigated the role of several genetic factors ( UGT 1A1 promoter ( TA ) n repeat polymorphism, alpha‐globin status), hematological parameters, clinical severity, and hydroxyurea ( HU ) therapy on the occurrence of cholelithiasis in SCD . Methods One hundred and fifty‐eight children (2–18 yr old) regularly followed at the University Hospital of Lyon (France) were included. A multivariate Cox model was used to test the associations between cholelithiasis and the different parameters analyzed. Results We confirmed that alpha‐thalassemia and low basal reticulocyte ( RET ) count were independent protective factors for cholelithiasis while 7/7, 8/8 and 7/8 UGT 1A1 ( TA ) n genotypes were independent predisposing factors for this complication. We also showed for the first time that HU treatment decreased the risk for cholelithiasis while frequent vaso‐occlusive crises and acute chest syndrome events increased that risk. Conclusions Our findings demonstrate that UGT 1A1 ( TA ) n polymorphism is not the only factor triggering gallstone formation in SCD . Cholelithiasis is also modulated by RET count, the number of deleted alpha‐genes, HU therapy and the frequency of vaso‐occlusive events.
Type of Medium:
Online Resource
ISSN:
0902-4441
,
1600-0609
DOI:
10.1111/ejh.2017.98.issue-3
Language:
English
Publisher:
Wiley
Publication Date:
2017
detail.hit.zdb_id:
2027114-1