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临床过程和管理的高甘油三血症,血症综合征:一个基于个案的系统性审查
Chane Choed-Amphai1, Pattranan Kusontammarat2, Supapitch Chanthong2
1Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand. chane.c@cmu.ac.th.
World journal of clinical pediatrics
|June 10, 2025
概括
过高甘油三血症 (hypertriglyceridemia thalassemia) 综合征,其特征是贫血和高甘油三,主要影响的是幼儿. 输血是一种有效的治疗方法,在长期监测下可以获得良好的结果.
科学领域:
- 血液学 血液学 血液学
- 儿科 儿科 儿科
- 代谢障碍 代谢障碍 代谢障碍
背景情况:
- 过高甘油三血症沙拉西米亚综合征是沙拉西米亚患者罕见的疾病,表现为严重的贫血和高甘油三.
- 关于这种综合征的临床结果和标准管理的数据有限.
研究的目的:
- 为了确定诊断出患有过高甘油三血症和血症综合征的患者的临床过程,治疗策略和结果.
主要方法:
- 通过使用Scopus,PubMed和Embase数据库进行了系统的文献审查.
- 包括所有出版日期的英语文章,重点关注患有甲血症,高甘油三血症的患者和临床数据.
- 该研究被注册 (CRD420250587918) 并分析了病例报告和病例系列的数据.
主要成果:
- 对14篇出版物的分析揭示了28例儿科病例,主要是β-thalassemia major,在输血之前呈现出贫血 (平均Hb 5.5 g/dL) 和严重的高甘油三血 (平均935 mg/dL).
- 常见的症状包括白色和肝炎;还注意到视网膜脂血和黄瘤.
- 对92.9%的患者进行了输血,包括或没有其他干预措施,在所有没有降脂剂的病例中都产生了良好的反应,在12个月后的存活率为85.7%.
结论:
- 过高甘油血症 (hypertriglyceridemia thalassemia) 综合征主要影响幼儿,在初次输血之前表现为贫血和严重的过高甘油血症.
- 通过输血治疗产生了积极的反应.
- 长期监测患者对于管理这种罕见疾病至关重要.
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