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巴林镰状细胞病与肝胆影响之间关联的前瞻性研究。

A prospective study of the association between sickle cell disease and hepatobiliary effects in Bahrain.

作者信息

Abdulla Maheeba A M, Almoosa Fajer Juma, Almoosa Rana Juma, Al Qamish Jehad

机构信息

Department of Internal Medicine, Salmaniya Medical Complex.

Department of Internal Medicine, Ibn Al Nafees Hospital, Manama, Bahrain.

出版信息

Int J Gen Med. 2017 Aug 4;10:221-226. doi: 10.2147/IJGM.S139833. eCollection 2017.

Abstract

BACKGROUND

Sickle cell disease is a genetic disorder that leads to abnormally high levels of hemoglobin sickling in erythrocytes. Patients suffer debilitating and severe complications that affect multiple organs, though mainly the liver, gallbladder, spleen, bones, and kidneys. It has a significant impact on morbidity and mortality rates and is associated with substantial health care costs.

METHODS

For this study, the researchers prospectively reviewed the charts of 154 sickle cell disease patients who had been treated in Salmaniya Medical Complex hospital in Bahrain between September 2013 and December 2014.

RESULTS

The results obtained showed that 95% of patients had sickle cell hemoglobin type. Of the sample patients, 80% exhibited bone pain, with all patients exhibiting bone crisis. Biochemical tests revealed reduced hemoglobin concentration, elevated bilirubin, compromised liver function, and lower white blood cell counts. The incidence of hepatitis B and C was very low, at 0% and 9%, respectively. There were a large number of hospital admissions, with 11 days as an average length of stay. The most common ultrasound findings in this study were hepatomegaly, hepatosplenomegaly, cholelithiasis, gallbladder sludge, and splenomegaly.

CONCLUSION

Sickle cell disease had profound negative effects on multiple organs, with a particularly large number of complications related to the hepatobiliary system.

摘要

背景

镰状细胞病是一种遗传性疾病,会导致红细胞中血红蛋白异常高水平的镰变。患者会遭受影响多个器官的衰弱性严重并发症,主要累及肝脏、胆囊、脾脏、骨骼和肾脏。它对发病率和死亡率有重大影响,并与高昂的医疗费用相关。

方法

在本研究中,研究人员前瞻性地回顾了2013年9月至2014年12月期间在巴林萨勒曼尼亚医疗中心医院接受治疗的154例镰状细胞病患者的病历。

结果

所得结果显示,95%的患者为镰状细胞血红蛋白类型。在样本患者中,80%出现骨痛,所有患者均出现骨危象。生化检查显示血红蛋白浓度降低、胆红素升高、肝功能受损和白细胞计数降低。乙型和丙型肝炎的发病率非常低,分别为0%和9%。住院次数很多,平均住院时间为11天。本研究中最常见的超声检查结果为肝肿大、肝脾肿大、胆石症、胆囊泥沙样病变和脾肿大。

结论

镰状细胞病对多个器官有深远的负面影响,与肝胆系统相关的并发症尤其多。

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