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某大学医院镰状细胞病中的胆结石及其并发症

Cholelithiasis and its complications in sickle cell disease in a university hospital.

作者信息

Martins Raquel Alves, Soares Renato Santos, Vito Fernanda Bernadelli De, Barbosa Valdirene de Fátima, Silva Sheila Soares, Moraes-Souza Helio, Martins Paulo Roberto Juliano

机构信息

Universidade Federal do Triângulo Mineiro (UFTM), Uberaba, MG, Brazil.

Universidade Federal do Triângulo Mineiro (UFTM), Uberaba, MG, Brazil.

出版信息

Rev Bras Hematol Hemoter. 2017 Jan-Mar;39(1):28-31. doi: 10.1016/j.bjhh.2016.09.009. Epub 2016 Oct 20.

Abstract

INTRODUCTION

The clinical manifestations of sickle cell disease are related to the polymerization of hemoglobin S. The chronic hemolysis caused by this condition often causes the formation of gallstones that can migrate and block the common bile duct leading to acute abdomen.

OBJECTIVE

This study aimed to evaluate the profile of patients with sickle cell disease and cholelithiasis.

METHODS

Patients with sickle cell disease were separated into groups according to the presence or absence of cholelithiasis. Socioepidemiological and clinical characteristics, such as gender, age, use of hydroxyurea and the presence of other hemoglobinopathies were researched in the medical records of patients.

RESULTS

A hundred and seven patients with sickle cell anemia were treated at the institution. Of these, 27 (25.2%) had cholelithiasis. The presence of cholelithiasis was higher in the 11-29 age group than in younger than 11 years and over 29 years. No association was found for the presence of cholelithiasis with gender, use of hydroxyurea or type of hemoglobinopathy (hemoglobin SS, hemoglobin SC or sickle beta-thalassemia). Sixteen of the patients had to be submitted to cholecystectomy with 14 of the surgeries being performed by laparoscopy. Complications were observed in three patients and one patient died for reasons unrelated to the surgery.

CONCLUSION

A quarter of patients with sickle cell disease had gallstones, more commonly in the 11- to 29-year age range. Patients should be monitored from childhood to prevent cholelithiasis with preoperative, intra-operative and postoperative care being crucial to reduce the risk of complications in these patients.

摘要

引言

镰状细胞病的临床表现与血红蛋白S的聚合有关。这种情况引起的慢性溶血常导致胆结石形成,结石可移动并阻塞胆总管,导致急腹症。

目的

本研究旨在评估镰状细胞病合并胆石症患者的情况。

方法

根据是否存在胆石症,将镰状细胞病患者分组。在患者的病历中研究社会流行病学和临床特征,如性别、年龄、羟基脲的使用情况以及其他血红蛋白病的存在情况。

结果

该机构共治疗了107例镰状细胞贫血患者。其中,27例(25.2%)患有胆石症。11至29岁年龄组的胆石症患病率高于11岁以下和29岁以上年龄组。未发现胆石症的存在与性别、羟基脲的使用或血红蛋白病类型(血红蛋白SS、血红蛋白SC或镰状β地中海贫血)之间存在关联。16例患者必须接受胆囊切除术,其中14例手术通过腹腔镜进行。3例患者出现并发症,1例患者因与手术无关的原因死亡。

结论

四分之一的镰状细胞病患者患有胆结石,更常见于11至29岁年龄范围。应从儿童期开始对患者进行监测,以预防胆石症,术前、术中和术后护理对于降低这些患者的并发症风险至关重要。

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