Observational study of cholelithiasis and its complications in sickle cell disease: experience from a tertiary care hospital in central India
DOI:
https://doi.org/10.18203/2349-2902.isj20253838Keywords:
Cholelithiasis, Gallstones, Hemolysis, Laparoscopic cholecystectomy, Sickle cell diseaseAbstract
Background: Sickle cell disease (SCD) is a genetic hemoglobinopathy associated with chronic hemolysis and vaso-occlusive crises. Pigment gallstone formation is among its most common hepatobiliary complications, causing cholelithiasis and resulting morbidity. Gallstones in patients with SCD have variable prevalence worldwide and are determined by genetic, hematologic, and environmental variables. Indian data are meager, and prospective hospital-based studies on clinical profiles, complications, and outcomes of management are minimal. This research sought to assess the prevalence, clinical spectrum, and prognosis of cholelithiasis among SCD patients presenting at a tertiary care center in central India.
Methods: This prospective observational study was conducted from August 2023 to January 2025 at a tertiary care hospital. A total of 91 adult patients with confirmed SCD presenting with symptoms of cholelithiasis were enrolled after ethical clearance and informed consent. Demographic, clinical, laboratory, and imaging data were collected. Management included laparoscopic cholecystectomy, ERCP, common bile duct (CBD) exploration, or conservative treatment. Data were analyzed using SPSS v27, with p<0.05 considered statistically significant.
Results: Gallstones were found in 26 patients (28.57%). Mixed stones (38.46%) were most frequent, followed by pigment (34.62%) and cholesterol stones (26.92%). Patients with gallstones had much lower hemoglobin (8.17±1.36 g/dl) and higher bilirubin (2.29±0.28 mg/dl) than those without (p<0.001). Complications were choledocholithiasis (53.85%), acute cholecystitis (46.15%), and acute pancreatitis (42.31%). Laparoscopic cholecystectomy was the most frequent intervention (42.31%), with a 9.09% conversion rate. All patients were recovered and discharged without in-hospital mortality.
Conclusions: Cholelithiasis is prevalent in SCD and strongly correlated with hemolysis-related indices. Early screening, aggressive surgical intervention, and mindful perioperative optimization are essential to minimize morbidity in this high-risk population.
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References
Serjeant GR. Sickle cell disease. Lancet. 1997;350(9079):725-30. DOI: https://doi.org/10.1016/S0140-6736(97)07330-3
Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-31. DOI: https://doi.org/10.1016/S0140-6736(10)61029-X
Al-Salem AH. Cholelithiasis in patients with sickle cell disease: A review. Ann Saudi Med. 1992;12(3):302-6.
Kato GJ, Piel FB, Reid CD, Gaston MH, Ohene-Frempong K, Krishnamurti L, et al. Sickle cell disease. Nature Revi Dis Prim. 2018;4(1):1-22. DOI: https://doi.org/10.1038/nrdp.2018.10
Diagne I, Moreira C, Signaté-Sy H, et al. Cholelithiasis in sickle cell disease in Senegal: prevalence and risk factors. Am J Hematol. 2000;63(1):1–6.
Mohanty D, Mukherjee MB. Sickle cell disease in India. Curr Opini Hematol. 2002;9(2):117-22. DOI: https://doi.org/10.1097/00062752-200203000-00006
Al-Salem AH. Hepatobiliary manifestations of sickle cell disease. Gastroenterol Hepatol Bed Bench. 2012;5(1):16-24.
Ebert EC, Nagar M. Gastrointestinal and hepatic complications of sickle cell disease. Clin Gastroenterol Hepatol. 2008;6(5):483-9. DOI: https://doi.org/10.1016/j.cgh.2010.02.016
Al-Salem AH. Cholecystectomy in children with sickle cell disease: experience with 300 cases. J Pediatr Surg. 2006;41(10):1903-8.
Martins RA, Soares RS, De Vito FB, de Fátima Barbosa V, Silva SS, Moraes-Souza H, et al. Cholelithiasis and complications in sickle cell disease in a university hospital. Rev Bras Hematol Hemoter. 2017;39(1):28-31. DOI: https://doi.org/10.1016/j.bjhh.2016.09.009
Oguntoye OO, Ndububa DA, Yusuf M, Bolarinwa RA, Ayoola OO. Hepatobiliary ultrasonographic abnormalities in adult patients with sickle cell anaemia. Pol J Radiol. 2017;82:1-8. DOI: https://doi.org/10.12659/PJR.899609
Al-Salem AH. Cholelithiasis in patients with sickle cell disease: A review. Ann Saudi Med. 1992;12(3):302-6. DOI: https://doi.org/10.5144/0256-4947.1992.387
Mohamed SO, Ibrahim OA, Mohammad DA, Ali AH. Correlates of gallbladder stones among patients with sickle cell disease: A meta-analysis. JGH Open. 2021;5(9):997-1003. DOI: https://doi.org/10.1002/jgh3.12622
Dutta S, Chakraborty AK. Cholelithiasis in sickle cell disease: experience from eastern India. Trop Gastroenterol. 2014;35(1):41-4.
Bond LR, Hatty SR, Horn ME, Dick M, Meire HB, Bellingham AJ. Gallstones in sickle cell disease in the United Kingdom. Br Med J (Clin Res Ed). 1987;295(6592):234-6. DOI: https://doi.org/10.1136/bmj.295.6592.234
Hamza HS, Medhat M, Kaddah N, Hegazy RA. Gallstones in children with sickle cell disease: incidence and relation to hemolysis. Pediatr Hematol Oncol. 2007;24(6):485-91.
Aranda-Narváez JM, González-Sánchez AJ, Montiel-Casado MC, et al. Complicated gallstone disease in sickle cell disease patients: role of laparoscopic surgery. Surg Endosc. 2007;21(10):1773-6.
Jain D, Arora NK, Aggarwal R, Malhotra V, Yachha SK, Naik SR. Hepatobiliary complications of sickle cell disease: an Indian experience. Indian Pediatr. 2001;38(6):661-6.
Haberkern CM, Neumayr LD, Orringer EP, Earles AN, Robertson SM, Black D, et al. Cholecystectomy in sickle cell disease: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Blood. 1997;89(5):1533-42.
Howard J, Malfroy M, Llewelyn C, Choo L, Hodge R, Johnson T, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) trial. Lancet. 2013;381(9870):930-8. DOI: https://doi.org/10.1016/S0140-6736(12)61726-7
Muroni M, Loi V, Lionnet F, Girot R, Galactéros F, Houry S. Elective laparoscopic cholecystectomy in adult sickle cell disease patients with cholelithiasis: a prospective cohort study. Int J Surg. 2015;22:62–6. DOI: https://doi.org/10.1016/j.ijsu.2015.07.708
Aliyu ZY, Gordeuk V, Sachdev V, et al. Cholelithiasis in sickle cell disease: a cohort study of prevalence and risk factors. Br J Haematol. 2007;136(2):249–52.
Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-23. DOI: https://doi.org/10.1016/S0140-6736(17)30193-9
Mohan N, Mishra P, Choudhry VP. Cholelithiasis in children with sickle cell disease. Ind Pediatr. 1993;30(8):1019-21.
Mukherjee MB, Surve RR, Gangakhedkar RR, Ghosh K, Colah RB, Mohanty D. Cholelithiasis in sickle cell disease. Ind J Pediatr. 1998;65(1):87-9.
Walker TM, Hambleton IR, Serjeant GR. Gallstones in sickle cell disease: Observations from The Jamaican Cohort Study. J Pediatr. 2000;136(1):80-5. DOI: https://doi.org/10.1016/S0022-3476(00)90054-4
Baheti AD, et al. Radiologic manifestations of hepatobiliary complications in sickle cell disease. Radiographics. 2017;37(1):76–93.
Al-Salem AH, Qaisaruddin S, Al-Abkari H, Al-Dabbous I. Cholelithiasis in sickle cell disease. Pediatr Surg Int.1996;11(5-6):471-3. DOI: https://doi.org/10.1007/BF00180085
Singhal A, Doherty JF, Rees DC, Serjeant GR. Clinical features of gallstones in sickle cell disease and the value of prophylactic cholecystectomy. Br J Haematol. 1993;82(4):744-9.
Ballas SK. Current issues in sickle cell pain and its management. Hematology Am Soc Hematol Educ Program. 2018;2018(1):142-9.