小结
定义
病史和体格检查
关键诊断因素
- 父/母确诊为镰状细胞贫血、其他镰状细胞病或镰状细胞遗传性状。
- 骨骼、胸部和/或腹部持续疼痛
- 指趾炎
其他诊断因素
- 高温
- 肺炎样综合征
- 骨痛
- 可见漂浮物
- 呼吸急促
- 生长迟缓
- 苍白
- 黄疸
- 心动过速
- 昏睡
- 上颌骨肥大伴覆咬合
- 隆凸腹，通常与脐疝同时出现
- 心脏收缩期血流杂音
- 休克
危险因素
- 遗传学
诊断性检查
首要检查
- 基于 DNA 的分析
- 血红蛋白等电聚焦 (Hb IEF)
- 醋酸纤维素电泳
- 高效液相色谱法 (HPLC)
- 血红蛋白溶解性试验
- 外周血涂片
- 全血细胞计数和网织红细胞计数
- 铁分析检测
需考虑的检查
- 脉搏血氧测定法
- 长骨 X 线平片检查
- 细菌培养
- 胸部 X 线检查
治疗流程
血管闭塞危象
急性胸部综合征
慢性疾病
撰稿人
作者
Sophie Lanzkron, MD, MHS
Director
Sickle Cell Center for Adults
Associate Professor of Medicine and Oncology
Johns Hopkins Medicine
Baltimore
MD
利益声明
SL has been the site principal investigator on several industry-funded studies with the following companies: Pfizer, Selexys, AstraZeneca, and Prolong. SL is a site principal investigator for Global Blood Therapeutics, Novartis, Shire, Ironwood, Imara and Bluebird Bio. She is chair of the Adjudication Committee for Novo Nordisk and Bluebird Bio and is a consultant for Pfizer. SL is an author of more than one reference in this topic. SL’s institution has received grants from Pfizer, Ironwood, Global Blood Therapeutics, NIH, PCORI and HRSA for research. She has an interest in a trust holding stocks in Pfizer and Teva.
鸣谢
Dr Sophie Lanzkron would like to gratefully acknowledge Dr Channing Paller, a previous contributor to this topic.
利益声明
CP declares that she has no competing interests.
同行评议者
James Bradner, MD
Instructor in Medicine
Division of Hematologic Neoplasia
Dana-Farber Cancer Institute
Boston
MA
利益声明
JB declares that he has no competing interests.
Adrian Stephens, MB BS, MD, FRCPath
Consultant Haematologist
University College London Hospitals
London
UK
利益声明
AS declares that he has no competing interests.
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