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Pulmmer-Vinson Syndrome

The triad of upper esophageal webs(small growth of tissue in esophagus) , dysphagia and iron deficiency anemia is called as Pulmmer-Vinson syndrome. It is also known as Paterson-Brown Kelly syndrome.

What causes the Pulmmer-Vinson syndrome?

The exact causes is still unknown but some believed that nutritional deficiencies (specially iron), genetic factor and autoimmune factor may play a role in Pulmmer-Vinson syndrome.

What are the signs and symptoms of Pulmmer-Vinson syndrome?

  1. Difficulty in swallowing(dysphagia)
  2. Burning sensation of tongue and mouth
  3. Weakness
  4. Light headache
  5. Fatigue
  6. Dyspnea(difficulty in breathing)
  7. Sore in tongue and angle of mouth( angular stomatitis and atrophic glossitis)
  8. Spoon nail( koilonychia)
  9. Pallor
  10. Weight loss
  11. Splenomegaly(enlarged spleen) , enlarged nodular thyroid.

How is Pulmmer-Vinson syndrome diagnosed ?

Diagnosis relies on clinical presentation, Laboratory test and radiological imaging .

  1. Laboratory test shows iron deficiency anemia.
  2. Radiological imaging which include barium esophagram, videofluorocopy and esophagogastroduodenoscopy may reveal the web in the esophagus.
  3. Biopsy of webs shows thin layer of normal squamous mucosa and submucosa cells .

How is Pulmmer-Vinson syndrome managed?

  1. Iron replacement with the dose of 235 mg PO once time a day help to correct anemia as well as improve dysphagia .
  2. If iron replacement cant help to improve dysphagia endoscopic web dilation is required .

What is the prognosis of the Pulmmer-Vinson syndrome?

Prognosis is good after treatment.

References:

  • http://www.emedicinehealth.com
  • http://en.wikipedia.org
  • http://www.nlm.nih.gov
  • http://www.medicinenet.com
  • Harrison’s Principles of Internal Medicine, 17th edition.
  • The Washington manual of surgery, 5th edition

AutoimmuneMom

From: Emedicinezone.com

This blog post was originally published by AutoimmuneMom.com, written by Emedicinezone.com and first published on Jan 20, 2011.

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