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United States circus clown (1898-1979) (同)Emmett Kelly, Weary Willie
United States dancer who performed in many musical films (1912-1996) (同)Gene Kelly, Eugene Curran Kelly
United States film actress who retired when she married into the royal family of Monaco (1928-1982) (同)Grace Kelly, Grace Patricia Kelly, Princess Grace of Monaco
a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
American Revolutionary leader (born in Ireland) who was a member of the Constitutional Convention (1745-1806) (同)William Patterson
a city of northeastern New Jersey

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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）』「2015/03/10 20:55:39」(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]}

§See also

List of hematologic conditions

§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

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1. 食道輪および食道ウェブ esophageal rings and webs
2. 成人の嚥下障害の評価法 approach to the evaluation of dysphagia in adults
3. 食道の良性病変 benign lesions of the esophagus
4. 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
5. 成人の鉄欠乏症および鉄欠乏性貧血の原因と診断 causes and diagnosis of iron deficiency and iron deficiency anemia in adults

English Journal

A case of plummer-vinson syndrome showing rapid improvement of Dysphagia and esophageal web after two weeks of iron therapy.

Tahara T1, Shibata T1, Okubo M1, Yoshioka D1, Ishizuka T1, Sumi K1, Kawamura T1, Nagasaka M1, Nakagawa Y1, Nakamura M2, Arisawa T2, Ohmiya N1, Hirata I1.
Case reports in gastroenterology.Case Rep Gastroenterol.2014 Jun 7;8(2):211-5. doi: 10.1159/000364820. eCollection 2014.
Plummer-Vinson syndrome (PVS) is a rare entity characterized by upper esophageal webs and iron deficiency anemia. We report a case of PVS whose esophageal web was rapidly improved by iron therapy. A 77-year-old woman was admitted to our hospital with complaints of dysphagia, vomiting, shortness of b
PMID 25028578

A case of plummer-vinson syndrome associated with Crohn's disease.

Park JM, Kim KO, Park CS, Jang BI.
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi.Korean J Gastroenterol.2014 Apr;63(4):244-7.
Plummer-Vinson syndrome manifests as cervical dysphagia, iron deficiency anemia, an upper esophageal web, and atrophic glossitis. The cause of the esophageal web is thought to be iron deficiency anemia; however, the cause of Plummer-Vinson syndrome has not been established. Crohn's disease is usuall
PMID 24755750

Plummer-Vinson syndrome with pectus carinatum mitral valve prolapsus and exotropia in an 18-year-old boy.

Sahin C1, Ozseker B, Rencuzogullari I, Zeybek A.
BMJ case reports.BMJ Case Rep.2014 Jan 22;2014. pii: bcr2013202047. doi: 10.1136/bcr-2013-202047.
The Plummer-Vinson syndrome is a clinical syndrome characterised by dysphagia, web or webs in upper oesophagus and iron-deficiency anaemia. The syndrome is often seen in women of age 40-70 years and rarely in adolescents. Plummer-Vinson syndrome might be associated with malignancy, myeloproliferativ
PMID 24451233