Plummer-Vinson综合征
Plummer-Vinson syndrome
定义 英文原文（暂无中文）
A rare hematological disorder characterized by the classic triad of iron-deficiency anemia, dysphagia, and esophageal webs. It predominantly affects Caucasian women aged 40-70 years, although pediatric cases have been reported. Dysphagia is usually painless, intermittent or progressive over several years, and limited to solids; it is sometimes associated with weight loss, while anemia-related symptoms such as fatigue and pallor may predominate. Additional signs include glossitis, angular cheilitis, and koilonychia. Splenomegaly and thyroid enlargement may occur. Iron deficiency is considered a major potential etiological factor. The syndrome is associated with an increased risk of developing squamous cell carcinoma of the upper gastrointestinal tract.
别名
Kelly-Paterson综合征
基本事实
- 遗传方式
- 未知
- 发病年龄
- 成年期
- 患病率
- <1 / 1 000 000
临床表型 16
必现 100%5
- 血清铁蛋白降低 HP:0012343
- 吞咽困难 HP:0002015
- 食管蹼 HP:0100594
- 低色素性小红细胞性贫血 HP:0004840
- 缺铁性贫血 HP:0001891
极常见 99–80%3
- 易疲劳性 HP:0003388
- 舌炎 HP:0000206
- 苍白圈 HP:0000980
偶见 29–5%8
- 腹痛 HP:0002027
- 唇炎 HP:0100825
- 反甲 HP:0001598
- 食土癖 HP:0025062
- 口内色素沉着 HP:0010284
- 小口畸形 HP:0000160
- 食欲不振 HP:0004396
- 舌萎缩 HP:0012473
外部标识与链接
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本页数据来源
- 疾病定义、同义词、基因、表型、流行病学：Orphanet（CC BY 4.0）
- 中文病名：Orphanet 中文包，冻结于 2020-06-01
- 表型中文标签：HPO 简体中文翻译（CHPO 上游成果）