成人遗传性球型红细胞增多症诱发重度高胆红素血症1例报告

阿卜杜萨拉木·图尔荪麦麦提; 张云飞; 姚刚; 沙地克·阿帕尔; 吐尔洪江·吐逊; 温浩

doi:10.3969/j.issn.1001-5256.2023.08.023

成人遗传性球型红细胞增多症诱发重度高胆红素血症1例报告

DOI: 10.3969/j.issn.1001-5256.2023.08.023

新疆医科大学第一附属医院消化血管外科中心/肝脏·腹腔镜外科，乌鲁木齐 830000

伦理学声明：本例报告已获得患者知情同意。

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：阿卜杜萨拉木·图尔荪麦麦提、张云飞负责课题设计，资料分析，撰写论文；阿卜杜萨拉木·图尔荪麦麦提参与收集数据，修改论文；张云飞、姚刚、沙地克·阿帕尔，吐尔洪江·吐逊，温浩负责提出修改意见及定稿。

详细信息

通信作者:
温浩，dr.wenhao@163.com (ORCID: 0000-0003-1016-1361)

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出版历程
- 收稿日期: 2022-09-19
- 录用日期: 2023-02-09
- 出版日期: 2023-08-20

Severe hyperbilirubinemia induced by hereditary spherocytosis: A case report

Digestive & Vascular Surgery Center/Department of Liver and Laparoscopic Surgery, The First Affiliated Hospital of Xinjiang Medical University, Urumqi 830000, China

More Information

Corresponding author: WEN Hao, dr.wenhao@163.com (ORDIC: 0000-0003-1016-1361)

摘要

摘要: 遗传性球型红细胞增多症是一种人体红细胞膜缺陷遗传性溶血疾病，主要以贫血、黄疸、脾大为主要症状，因临床症状不典型，容易误诊漏诊，目前临床上总胆红素及直接胆红素极高病例较少见，国内外很少有报道，文本患者入院接受治疗时总胆红素高达1 686.01 μmol/L，直接胆红素高达1 166.6 μmol/L。经外科治疗该患者顺利出院，术后随访一般情况佳，生活质量高，本文总结了外科治疗遗传性球型红细胞增多症的相关诊疗经验。
- 红细胞增多症 /
- 高胆红素血症 /
- 手术后并发症
Abstract: Hereditary spherocytosis is a type of hereditary hemolytic disease with human erythrocyte membrane defects and has the main symptoms of anemia, jaundice, and splenomegaly. Since its clinical symptoms are not typical, misdiagnosis or missed diagnosis is often observed. At present, there are rare cases with extremely high levels of total bilirubin and direct bilirubin in clinical practice, with few reports in China and globally. In this study, the patient had a total bilirubin level of 1 686.01 μmol/L and a direct bilirubin level of 1 166.6 μmol/L on admission. The patient was successfully discharged after surgical treatment and had good general conditions and high quality of life during follow-up. This article summarizes the experience in the diagnosis and surgical treatment of hereditary spherocytosis.
- Polycythemia /
- Hyperbilirubinemia /
- Postoperative Complications

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图 1 腹部超声及CT

注：a, 腹部超声; b, 腹部CT。

Figure 1. Abdominal ultrasound and CT

下载: 全尺寸图片幻灯片

图 2 患者胆红素及Cr水平术前术后变化

Figure 2. Changes in preoperative and postoperative bilirubin and creatinine levels in patients

下载: 全尺寸图片幻灯片

图 3 治疗期间影像检查

注：a，透析期间；b，术后1周复查；c，术后。

Figure 3. Imaging examination during treatment

下载: 全尺寸图片幻灯片

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