{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T03:28:51Z","timestamp":1789874931196,"version":"4.0.1"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,1,9]],"date-time":"2017-01-09T00:00:00Z","timestamp":1483920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2017,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Because of the increased hemolytic rate, a significant proportion of patients with sickle cell disease (<jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>) are prone to develop cholelithiasis. The present study investigated the role of several genetic factors (<jats:italic><jats:styled-content style=\"fixed-case\">UGT<\/jats:styled-content>1A1<\/jats:italic> promoter (<jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>)<jats:sub>n<\/jats:sub> repeat polymorphism, alpha\u2010globin status), hematological parameters, clinical severity, and hydroxyurea (<jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content>) therapy on the occurrence of cholelithiasis in <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>One hundred and fifty\u2010eight children (2\u201318 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We confirmed that alpha\u2010thalassemia and low basal reticulocyte (<jats:styled-content style=\"fixed-case\">RET<\/jats:styled-content>) count were independent protective factors for cholelithiasis while 7\/7, 8\/8 and 7\/8 <jats:italic><jats:styled-content style=\"fixed-case\">UGT<\/jats:styled-content>1A1<\/jats:italic> (<jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>)<jats:sub>n<\/jats:sub> genotypes were independent predisposing factors for this complication. We also showed for the first time that <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content> treatment decreased the risk for cholelithiasis while frequent vaso\u2010occlusive crises and acute chest syndrome events increased that risk.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our findings demonstrate that <jats:italic><jats:styled-content style=\"fixed-case\">UGT<\/jats:styled-content>1A1<\/jats:italic> (<jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>)<jats:sub>n<\/jats:sub> polymorphism is not the only factor triggering gallstone formation in <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>. Cholelithiasis is also modulated by <jats:styled-content style=\"fixed-case\">RET<\/jats:styled-content> count, the number of deleted alpha\u2010genes, <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content> therapy and the frequency of vaso\u2010occlusive events.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.12838","type":"journal-article","created":{"date-parts":[[2016,12,17]],"date-time":"2016-12-17T13:40:03Z","timestamp":1481982003000},"page":"296-301","source":"Crossref","is-referenced-by-count":9,"title":["<scp>UGT<\/scp>1A1 (<scp>TA<\/scp>)<sub>n<\/sub> genotype is not the major risk factor of cholelithiasis in sickle cell disease children"],"prefix":"10.1111","volume":"98","author":[{"given":"Philippe","family":"Joly","sequence":"first","affiliation":[{"name":"Univ Lyon \u2010 University Claude Bernard Lyon 1 \u2010 EA 7424 Inter\u2010university Laboratory of Human Movement Science Villeurbanne  France"},{"name":"Labex GR\u2010Ex Institut Universitaire de France Paris  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