Protector effect of α-thalassaemia on cholecystitis and cholecystectomy in sickle cell disease.
作者信息
Pontes Robéria M, Costa Elaine S, Siqueira Patrícia F R, Medeiros Jussara F F, Soares Andréa, de Mello Fabiana V, Maioli Maria C, Filho Isaac L S, Alves Liliane R, Land Marcelo G P, Fleury Marcos K
机构信息
a Clinical Medicine Postgraduate Program, Faculty of Medicine , Rio de Janeiro Federal University (UFRJ) , Rio de Janeiro , Brazil.
b Department of Pediatrics, Faculty of Medicine , Institute of Paediatrics and Puericulture Martagão Gesteira (IPPMG) UFRJ , Rio de Janeiro , Brazil.
出版信息
Hematology. 2017 Aug;22(7):444-449. doi: 10.1080/10245332.2017.1289325. Epub 2017 Feb 21.
OBJECTIVES
Cholecystitis is one of the complications of symptomatic cholelithiasis responsible for high levels of morbidity of sickle cell disease (SCD) patients. Here, we investigated the possible protective role of single gene deletions of α-thalassaemia in the occurrence of cholelithiasis and cholecystitis in SCD patients, as well as the cholecystectomy requirements.
METHODS
The α-globin genotype was determined in 83 SCD patients using the multiplex-polymerase chain reaction and compared with clinical events.
RESULTS
Overall, in 23% of patients, -α deletion was found. α-Thalassaemia concomitant to SCD was an independent protective factor to cholecystitis (OR = 0.07; 95% CI: 0.01-0.66; p = 0.020) and cholecystectomy requirement (OR = 0.14; 95% CI: 0.03-0.60; p = 0.008). The risk of cholelithiasis was not affected by the α-thalassaemia concomitance.
CONCLUSIONS
To the best our knowledge, our study is the first to show the protective effect of α-thalassaemia on cholecystitis and cholecystectomy requirements in SCD, which may be due to an improved splenic function.
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