Cholelithiasis in Patients with Sickle Cell Disease and Sickle/Beta-Thalassemia in Akre City, Kurdistan Region of Iraq

Esraa Hasan Abdullah, Rawand P. Shamoon, N. Mohammad
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Abstract

Background and objectives: Sickle cell disease and sickle β-thalassemia are hemolytic diseases characterized by chronic destruction of the abnormal red cells with consequent hyperbilirubinemia. The present study aimed to determine the association of gallstone with Sickle cell disease and sickle β-thal patients compared to the control healthy group and to determine gallstone's association with several demographic and laboratory parameters among the patients themselves. Methods: Patients visiting the Akre thalassemia center for routine checks have been recruited for this study (patients=73; control=36). Blood samples were collected for biochemical and haematological analyses. All participants were subjected to abdominal ultrasonography for confirmation of gallstones. These parameters were compared with patients' subtype traits. Additional information regarding blood transfusion, hospital admission, and the use of chelating agents was also allocated. Results: The prevalence of gallstone was significantly higher in patients (12%) than in the control (3%) group (P<0.05), with similar prevalence in both trait; homozygous haemoglobin S (14%) and compound heterozygous of hemoglobin S and β-thalassemia (11%), together with significant differences in the biochemical and haematological parameters. The haemoglobin concentration was significantly (P<0.05) reduced in patients (8.5±0.86) versus control (12±1.6). Reticulocytes, lactate dehydrogenase enzyme, and bilirubin were significantly (P<0.05) elevated in patients compared to the control. No differences existed between control versus patients regarding hospital admission, blood transfusion, and the use of medication including chelating agent or hydroxyurea. Conclusion: Sickle-diseased and sickle beta-thalassemia patients have a higher risk of developing gallstones than the general population alongside interruption of metabolic and biochemical parameters with co-association with sub-traits of type of haemoglobin.
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伊拉克库尔德斯坦地区阿克雷市镰状细胞病和镰状/β-地中海贫血患者的胆石症
背景和目的:镰状细胞病和镰状β-地中海贫血是一种溶血性疾病,其特征是异常红细胞的慢性破坏和随之而来的高胆红素血症。本研究旨在确定与健康对照组相比,镰状细胞病和镰状β-地中海贫血患者胆结石的相关性,并确定胆结石与患者自身的一些人口统计学和实验室参数的相关性。研究方法本研究招募了到 Akre 地中海贫血中心进行常规检查的患者(患者=73;对照组=36)。采集血液样本进行生化和血液学分析。所有参与者都接受了腹部超声波检查,以确认是否患有胆结石。这些参数与患者的亚型特征进行了比较。此外,还分配了有关输血、入院和使用螯合剂的其他信息。结果显示胆结石患者的发病率(12%)明显高于对照组(3%)(P<0.05),两种性状的发病率相似;同型血红蛋白 S(14%)和血红蛋白 S 与 β 地中海贫血的复合杂合子(11%),生化和血液学参数也有显著差异。患者的血红蛋白浓度(8.5±0.86)明显低于对照组(12±1.6)(P<0.05)。与对照组相比,患者的网织红细胞、乳酸脱氢酶和胆红素明显升高(P<0.05)。对照组与患者在入院、输血和使用药物(包括螯合剂或羟基脲)方面不存在差异。结论镰刀型地中海贫血症和镰刀型β地中海贫血症患者患胆结石的风险高于普通人群,同时代谢和生化指标也会受到影响,这与血红蛋白类型的亚型有关。
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