WordNet

a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
United States jurist who served as chief justice of the Supreme Court (1890-1953) (同)Frederick Moore Vinson

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/04/07 09:23:08」(JST)

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Plummer–Vinson syndrome (PVS), also called Paterson–Brown–Kelly syndrome or sideropenic dysphagia, is a rare disease characterized by difficulty in swallowing, iron deficiency anemia, and esophageal webs.^[1] Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome.

Nowadays, this syndrome has become extremely rare.^[1] It generally occurs in postmenopausal women. Its identification and follow-up is considered relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx.^[1]

Presentation

PVS sufferers often complain of a burning sensation with the tongue and oral mucosa, and atrophy of lingual papillae produces a smooth, shiny, red dorsum of the tongue.

Symptoms include:

Dysphagia (difficulty in swallowing)
Pain
Weakness
Odynophagia (painful swallowing)
Atrophic glossitis
Angular stomatitis

Serial contrasted gastrointestinal radiography or upper gastrointestinal endoscopy may reveal the web in the esophagus. Blood tests show a hypochromic microcytic anemia that is consistent with an iron-deficiency anemia. Biopsy of involved mucosa typically reveals epithelial atrophy (shrinking) and varying amounts of submucosal chronic inflammation. Epithelial atypia or dysplasia may be present.

Causes and associated conditions

The cause of PVS is unknown; however, genetic factors and nutritional deficiencies may play a role. It is more common in women,^[2] particularly in middle age (peak age is over 50). In these patients, esophageal squamous cell carcinoma risk is increased;^[1] therefore, it is considered a premalignant process.

The condition is associated with koilonychia, glossitis, inflammation of the lips (cheilitis), and splenomegaly.

Eponym

The disease is named after two Americans: the physician Henry Stanley Plummer and the surgeon Porter Paisley Vinson.^[3]^[4]^[5]

It is preferentially known as Kelly-Paterson syndrome in the UK, after Derek Brown-Kelly and Donald Ross Paterson.^[3]^[6]^[7]

Treatment

Treatment is primarily aimed at correcting the iron-deficiency anemia. Patients with PVS should receive iron supplementation in their diet. This may improve dysphagia and pain.^{[citation needed]}

If not, the web can be dilated during upper endoscopy to allow normal swallowing and passage of food.^[8]

Prognosis

Patients generally respond well to treatment. Iron supplementation usually resolves the anemia, and corrects the glossodynia (tongue pain).^{[citation needed]}

Complications

There is risk of perforation of the esophagus with the use of dilators for treatment. Furthermore it is one of the risk factors for developing squamous cell carcinoma of the oral cavity, esophagus, and hypopharynx.^[9]

Prevention

Good nutrition with adequate intake of iron may prevent this disorder.^{[citation needed]}

References

^ ^a ^b ^c ^d Novacek G (2006). "Plummer-Vinson syndrome". Orphanet J Rare Dis 1: 36. doi:10.1186/1750-1172-1-36. PMC 1586011. PMID 16978405.
^ "Plummer-Vinson syndrome: MedlinePlus Medical Encyclopedia". 2011.
^ ^a ^b synd/1777 at Who Named It?
^ H. S. Plummer. Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm). A report of ninety-one cases. Journal of the American Medical Association, Chicago, 1912, 58: 2013-2015.
^ P. P. Vinson. A case of cardiospasm with dilatation and angulation of the esophagus. Medical Clinics of North America, Philadelphia, PA., 1919, 3: 623-627.
^ A. B. Kelly. Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 34: 285-289.
^ D. R. Paterson. A clinical type of dysphagia. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 24: 289-291.
^ Enomoto M, Kohmoto M, Arafa UA et al. (2007). "Plummer-Vinson syndrome successfully treated by endoscopic dilatation". J. Gastroenterol. Hepatol. 22 (12): 2348–51. doi:10.1111/j.1440-1746.2006.03430.x. PMID 18031398.
^ "Plummer-Vinson Syndrome url=http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002143/". PubMed Health. Missing or empty |url= (help); |accessdate= requires |url= (help)

External links

00325 at CHORUS
PVS Resource

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 食道輪および食道ウェブ esophageal rings and webs
2. 成人における鉄欠乏性貧血の原因および診断 causes and diagnosis of iron deficiency anemia in the adult
3. 成人における嚥下障害の概要 overview of dysphagia in adults
4. 食道の良性病変 benign lesions of the esophagus
5. 早期（ステージIおよびII）の頭頸部癌の治療：下咽頭 treatment of early stage i and ii head and neck cancer the hypopharynx

English Journal

Plummer-Vinson syndrome and reactive thrombocytosis mask a JAK2-V617F positive myeloproliferative neoplasm.

Scott EC, Schuster S.SourceUniversity of Pennsylvania, USA.
Blood.Blood.2011 Dec 1;118(23):5988.
PMID 22238785

Esophageal web resolved by endoscopic incision in a patient with Plummer-Vinson syndrome.

Seo MH, Chun HJ, Jeen YT, Park SC, Keum B, Kim YS, Seo YS, Lee HS, Um SH, Kim CD, Ryu HS.SourceDivision of Gastroenterology and Hepatology, Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea.
Gastrointestinal endoscopy.Gastrointest Endosc.2011 Nov;74(5):1142-3. Epub 2011 Aug 6.
PMID 21824614

Japanese Journal

内視鏡的バルーン拡張術にて治療しえた Plummer-Vinson 症候群の2例

冨永直之,岩切龍一,鶴岡ななえ,萬年孝太郎,坂田資尚,下田良,綱田誠司,坂田祐之,水口昌伸,藤本一眞
日本消化器内視鏡学会雑誌 = Gastroenterological endoscopy 52(7), 1671-1676, 2010-07-20
… 症例は78歳女性と43歳女性．主訴は嚥下困難．長期間の鉄欠乏性貧血の既往があった．上部消化管内視鏡検査および食道造影検査にて，頸部食道に食道膜様狭窄（web）が認められ，Plummer-Vinson症候群と診断した．内視鏡的バルーン拡張術を施行し，症状は速やかに改善した．本治療は安全，簡便であり，症状改善に有用な治療法であると考えた． …
NAID 10026910638

嚥下内視鏡検査のピットフォール

佐藤公則
耳鼻咽喉科臨床 103(5), 459-463, 2010-05-01
… These consisted of 2 subjects with esophageal cancer (2%) and 1 each of esophageal achalasia (1%), ossification of the anterior longitudinal ligament (1%), osteophyte of the cervical spine (1%), thyroid tumor (1%), lateral pharyngeal diverticulum (1%), postoperative complication due to anterior cervical fusion (1%), and Plummer-Vinson syndrome (1%). …
NAID 10026217467

Related Pictures

★リンクテーブル★

リンク元	「100Cases 55」「咽頭癌」「プランマー・ビンソン症候群」
関連記事	「syndrome」

「100Cases 55」

Plummer–Vinson syndrome
Classification and external resources
ICD-10	D50.1
ICD-9	280.8
DiseasesDB	10134
MedlinePlus	001158
eMedicine	med/3431
MeSH	D011004

　　[★]

ヒント：タイトルに騙されるな！

45歳　女性

主訴：肺炎

現病歴：過去6ヶ月の間、咳、発熱、および膿性痰をともなう3回のエピソードがあった。このうち1回のエピソードでは、右側の胸膜炎性胸痛があった。一連のエピソードはgeneral practitionerの外来で治療していた。これらのエピソードに加え、5年間の嚥下困難の既往がある。嚥下困難は最初は中等度であったが、だんだんと増悪している。食べ物が胸骨後部の下方に刺さる様だと言っている。どういう固形物であってもこのような症状がでる。体重は過去2ヶ月で5kg減少した。嚥下困難は食事中に改善することがあるようだ。最近、形のはっきりとした食物を嘔吐する問題を抱えている。

　3年前外来で施行した上部消化管内視鏡では問題は認められなかった。器質的な問題がないことが保証されたが、症状はひどくなってきた。排尿障害はない。便秘傾向があるが、最近少し悪くなってきた。

既往歴：無し

家族歴：無し

社会歴：10年前まで4年間、アメリカ合衆国の北西の沿岸部にすんでいた。店員として働いている。

嗜好歴：喫煙なし。飲酒は週に5units以下。

服用薬：

身体所見 examination

　やせて見える。右の肺底部にcrackleを認める。心血管系、消化器系、およびそのほかの臓器系に異常を認めない。

検査所見 investigations

　胸部単純X線写真(供覧)

問題

　診断名は？