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The pattern of hepatobiliary complications among Egyptian sickle cell disease children

机译:埃及镰状细胞病患儿肝胆并发症的模式

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Background Hepatobiliary complications are frequent among sickle cell disease patients. Sickle cell disease has been extensively studied. However, data about hepatobiliary abnormalities among the pediatric age group are limited. Objective This study aimed at describing the pattern of hepatobiliary complications among sickle cell disease patients as detected clinically, by laboratory testing and by abdominal ultrasonography. Methods Seventy sickle cell disease patients, aged 2–8 years, of both SS and Sβ types were enrolled in this cross-sectional study. Thorough history taking, full clinical examination, hematological and biochemical parameters assessment and abdominal ultrasonographic studies were performed to all patients. Result The median age of the patients was 12.5 years (mean: 11.81 ± 5.1). Hepatomegaly was detected ultrasonographically in 24 patients (34.3%) and was more encountered among patients with increasing age (P = 0.003), patients with HbSβ type (P = 0.002) and high serum ferritin levels (P = 0.047). Gallbladder diseases were encountered in 19 patients (27.1%), and manifested as isolated stones in (7.1%), isolated mud in (5.7%), stones with mud in 2 patients (2.9%), and surgically removed stones in (11.4%). Cholecystectomy was done in 8 patients (11.4%). Gallbladder disease was more frequent among patients with older age (P = 0.041), more severe sickle cell disease attacks (P = 0.035), and longer duration of blood transfusion therapy (P = 0.041). Other hepatobiliary complications were chronic hepatitis C (12.9%), benign hyperbilirubinemia (1.4%), intrahepatic cholestasis (1.4%), hepatic sequestration(1.4%), hepatic crisis (1.4%), and liver cirrhosis (1.4%). Conclusion Hepatobiliary complications are frequent among sickle cell disease children especially gallbladder diseases. Repeated clinical and ultrasound screening is needed for early detection and treatment of these complications.
机译:背景在镰状细胞病患者中肝胆并发症多发。镰状细胞病已被广泛研究。但是,有关小儿年龄组肝胆异常的数据有限。目的本研究旨在描述临床,实验室检查和腹部超声检查所发现的镰状细胞病患者的肝胆并发症的类型。方法这项横断面研究纳入了70名2-8岁的SS型和Sβ型镰状细胞病患者。对所有患者进行了全面的病史记录,全面的临床检查,血液学和生化指标评估以及腹部超声检查。结果患者的中位年龄为12.5岁(平均:11.81±5.1)。超声检查发现24例肝肿大(34.3%),在年龄增长(P = 0.003),HbSβ型(P = 0.002)和血清铁蛋白水平高(P = 0.047)的患者中更为常见。胆囊疾病发生于19例患者(27.1%),表现为孤立的结石(7.1%),孤立的泥巴(5.7%),2例有泥巴的结石(2.9%)和手术切除的结石(11.4%) )。 8例(11.4%)进行了胆囊切除术。年龄较大的患者(P = 0.041),镰状细胞疾病发作的严重程度(P = 0.035)和输血治疗时间较长(P = 0.041)的患者中胆囊疾病更为常见。其他肝胆并发症是慢性丙型肝炎(12.9%),良性高胆红素血症(1.4%),肝内胆汁淤积症(1.4%),肝隔离症(1.4%),肝病危象(1.4%)和肝硬化(1.4%)。结论镰状细胞病患儿尤其是胆囊疾病患儿肝胆并发症多见。对于这些并发症的早期发现和治疗,需要进行反复的临床和超声检查。

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