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|MainCategory=Pathology
|MainCategory=Pathology
|SubCategory=Gastrointestinal
|SubCategory=Gastrointestinal
|Prompt=A 48 year old Caucasian woman with no past medical history presents to the physician's office complaining of inability to swallow solid food for several years. She explains that her condition has recently got worse, but she does not feel any pain while swallowing and has no difficulty swallowing liquids. Review of systems also reveals that she has recently been feeling tired all the time and has lost several pounds within a few months. Her vital signs show a temperature of 36.8 degrees C (98.2 degrees F), a heart rate of 102 bpm, and a blood pressure measuring 132/88 mmHg.  Physical examination is remarkable for skin pallor, glossitis, angular cheilitis, and koilonychia.  The physician decides to perform endoscopy. What is the most likely finding on endoscopy associated with this patient's condition?
|Prompt=A 48 year old Caucasian woman with no past medical history presents to the physician's office complaining of inability to swallow solid food for several years. She explains that her condition has recently got worse, but she does not feel any pain while swallowing and has no difficulty swallowing liquids. Review of systems also reveals that she has recently been feeling tired all the time and has lost several pounds within a few months. Her vital signs show a temperature of 36.8 degrees C (98.2 degrees F), a heart rate of 102 bpm, and a blood pressure measuring 132/88 mmHg.  Physical examination is remarkable for skin pallor, glossitis, angular cheilitis, and koilonychia.  The physician decides to perform endoscopy for further evaluation. What is the most likely finding on endoscopy associated with this patient's condition?
|Explanation=The patient is most likely to be diagnosed with Plummer-Vinson syndrome (also known as Paterson-Kelly syndrome). It is a rare triad of: Dysphagia due to esophageal webs, glossitis, and iron deficiency anemia (IDA). Patients usually are middle-aged women, but age range is very wide: The disease has been described in childhood and among elderly patients. Patients typically present with dysphagia to solids with no odynophagia that progresses over several years. Other signs and symptoms are due to associated conditions, such as skin pallor, fatigue, and tachycardia due to iron deficiency anemia. Additional findings on physical examination may include angular cheilitis (inflammation of labial commissures), koilonychia (nail spooning), palpable spleen, and/or palpable thyroid. The real cause of Plummer-Vinson syndrome is not yet identified, but several environmental and genetic predispositions have been described, including malnutrition and autoimmune disease. The syndrome can be treated by iron replacement and mechanical dilation of the esophageal webs.
|Explanation=The patient is most likely to be diagnosed with Plummer-Vinson syndrome (also known as Paterson-Kelly syndrome). It is a rare triad of: Dysphagia due to esophageal webs, glossitis, and iron deficiency anemia (IDA). Patients usually are middle-aged women, but age range is very wide: The disease has been described in childhood and among elderly patients. Patients typically present with dysphagia to solids with no odynophagia that progresses over several years. Other signs and symptoms are due to associated conditions, such as skin pallor, fatigue, and tachycardia due to iron deficiency anemia. Additional findings on physical examination may include angular cheilitis (inflammation of labial commissures), koilonychia (nail spooning), palpable spleen, and/or palpable thyroid. The real cause of Plummer-Vinson syndrome is not yet identified, but several environmental and genetic predispositions have been described, including malnutrition and autoimmune disease. The syndrome can be treated by iron replacement and mechanical dilation of the esophageal webs.



Revision as of 10:53, 7 November 2013

 
Author [[PageAuthor::Rim Halaby, M.D. [1]]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Gastrointestinal
Prompt [[Prompt::A 48 year old Caucasian woman with no past medical history presents to the physician's office complaining of inability to swallow solid food for several years. She explains that her condition has recently got worse, but she does not feel any pain while swallowing and has no difficulty swallowing liquids. Review of systems also reveals that she has recently been feeling tired all the time and has lost several pounds within a few months. Her vital signs show a temperature of 36.8 degrees C (98.2 degrees F), a heart rate of 102 bpm, and a blood pressure measuring 132/88 mmHg. Physical examination is remarkable for skin pallor, glossitis, angular cheilitis, and koilonychia. The physician decides to perform endoscopy for further evaluation. What is the most likely finding on endoscopy associated with this patient's condition?]]
Answer A AnswerA::Zenker's diverticulum
Answer A Explanation AnswerAExp::Zenker's diverticulum is not associated with Plummer-Vinson syndrome.
Answer B AnswerB::Esophageal webs
Answer B Explanation AnswerBExp::Esophageal webs is associated with Plummer-Vinson syndrome.
Answer C AnswerC::Esophageal strictures
Answer C Explanation AnswerCExp::Esophageal strictures are not associated with Plummer-Vinson syndrome.
Answer D AnswerD::Achalasia of the esophagus
Answer D Explanation AnswerDExp::Achalasia is associated with Chagas disease. It is not associated with Plummer-Vinson syndrome.
Answer E AnswerE::Esophageal esophagitis
Answer E Explanation AnswerEExp::Esophageal esophagitis is not associated with Plummer-Vinson syndrome.
Right Answer RightAnswer::B
Explanation [[Explanation::The patient is most likely to be diagnosed with Plummer-Vinson syndrome (also known as Paterson-Kelly syndrome). It is a rare triad of: Dysphagia due to esophageal webs, glossitis, and iron deficiency anemia (IDA). Patients usually are middle-aged women, but age range is very wide: The disease has been described in childhood and among elderly patients. Patients typically present with dysphagia to solids with no odynophagia that progresses over several years. Other signs and symptoms are due to associated conditions, such as skin pallor, fatigue, and tachycardia due to iron deficiency anemia. Additional findings on physical examination may include angular cheilitis (inflammation of labial commissures), koilonychia (nail spooning), palpable spleen, and/or palpable thyroid. The real cause of Plummer-Vinson syndrome is not yet identified, but several environmental and genetic predispositions have been described, including malnutrition and autoimmune disease. The syndrome can be treated by iron replacement and mechanical dilation of the esophageal webs.

Most importantly, Plummer-Vinson syndrome is associated with squamous cell carcinoma of the pharynx and the esophagus. Accordingly, patients diagnosed with the syndrome require close follow-up.

Educational Objective: Plummer-Vinson syndrome is a triad of glossitis, dysphagia due to esophageal webs, and iron deficiency anemia.

Reference: Novacek, G. Plummer-Vinson syndrome. Orphanet J Rare Dis. 2006; 1:36.
Educational Objective:
References: ]]

Approved Approved::No
Keyword WBRKeyword::Plummer, WBRKeyword::Vinson, WBRKeyword::Plummer-Vinson, WBRKeyword::syndrome, WBRKeyword::glossitis, WBRKeyword::esophageal, WBRKeyword::webs, WBRKeyword::esophagus, WBRKeyword::koilonychia, WBRKeyword::nail, WBRKeyword::spooning, WBRKeyword::cheilitis, WBRKeyword::labial, WBRKeyword::commissures, WBRKeyword::dysphagia, WBRKeyword::swallow, WBRKeyword::swallowing, WBRKeyword::iron, WBRKeyword::deficiency, WBRKeyword::anemia, WBRKeyword::pallor, WBRKeyword::tachycardia, WBRKeyword::fatigue, WBRKeyword::middle, WBRKeyword::aged, WBRKeyword::women, WBRKeyword::females, WBRKeyword::esophageal, WBRKeyword::esophagus, WBRKeyword::cancer, WBRKeyword::squamous, WBRKeyword::cell, WBRKeyword::endoscopy
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