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Protector effect of α-thalassaemia on cholecystitis and cholecystectomy in sickle cell disease
Robéria M Pontes1, Elaine S Costa1,2, Patrícia F R Siqueira1,3
1a Clinical Medicine Postgraduate Program, Faculty of Medicine , Rio de Janeiro Federal University (UFRJ) , Rio de Janeiro , Brazil.
Insights
Alpha-thalassemia significantly reduces cholecystitis and cholecystectomy needs in sickle cell disease (SCD) patients. This finding suggests improved splenic function may offer protection against gallstone complications in SCD.
Area of Science:
- Hematology
- Genetics
- Gastroenterology
Background:
- Cholecystitis is a common complication in sickle cell disease (SCD) patients.
- Symptomatic cholelithiasis contributes to significant morbidity in individuals with SCD.
Purpose of the Study:
- To investigate the protective role of single gene deletions of alpha-thalassemia against cholelithiasis and cholecystitis in SCD patients.
- To assess the impact of alpha-thalassemia on cholecystectomy requirements in SCD patients.
Main Methods:
- Determined alpha-globin genotype in 83 SCD patients using multiplex-polymerase chain reaction.
- Compared alpha-thalassemia status with clinical events, including cholelithiasis, cholecystitis, and cholecystectomy.
Main Results:
- Alpha-thalassemia (specifically -α3.7 deletion) was found in 23% of SCD patients.
- Concomitant alpha-thalassemia was an independent protective factor against cholecystitis (OR=0.07) and cholecystectomy (OR=0.14).
- Alpha-thalassemia did not significantly affect the risk of cholelithiasis.
Conclusions:
- This study is the first to demonstrate the protective effect of alpha-thalassemia on cholecystitis and cholecystectomy in SCD patients.
- The protective mechanism may be attributed to improved splenic function in individuals with both SCD and alpha-thalassemia.
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, -α3.7 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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