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Peutz-Jeghers syndrome
Peutz Jeghers syndrome

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We were unfortunately unable to download the information for this disease from OMIM.

Prevalence of clinical parameters (%)

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Pubmed id number as a reference Organ system affected
Number of patients in the reference Percent affected patients (Between 0 and 1, eg. 0.1 = 10%)
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List of symptoms

Symptom/sign Organ system Percent affected Pubmed id Added on(yyyy-mm-dd) Edit/add reference
Hamartomatous polyps digestive 100 % 20126809 2012-01-11
Perioral melanocytic macules integumentary 100 % 20126809 2012-01-11
Abdominal pain digestive 100 % 20126809 2012-01-11
Cancer multi 85 % 16707622 2012-01-11
Gastrointestinal bleeding digestive 42 % 20126809 2012-01-11

List of references:

Peutz-Jeghers syndrome: data from the Singapore Polyposis Registry and a shifting paradigm in management.
Veronique Km Tan, Poh Koon Koh, Carol Tt Loi, Kong Weng Eu, Choong Leong Tang,

Peutz-Jeghers Syndrome (PJS) is an uncommon autosomal dominant hamartomatous polyposis syndrome. Morbidity arises from polyp-related complications and increased risks of malignancy. We report on PJS patients registered in the Singapore Polyposis Registry, identified principal causes of morbidity and appraised current management strategies. A followup protocol based on recent literature has been proposed.

Annals of the Academy of Medicine, Singapore - Jan 2010

Frequency and spectrum of cancers in the Peutz-Jeghers syndrome.
Nicholas Hearle, Valérie Schumacher, Fred H Menko, Sylviane Olschwang, Lisa A Boardman, Johan J P Gille, Josbert J Keller, Anne Marie Westerman, Rodney J Scott, Wendy Lim, Jill D Trimbath, Francis M Giardiello, Stephen B Gruber, G Johan A Offerhaus, Felix W M de Rooij, J H Paul Wilson, Anika Hansmann, Gabriela Möslein, Brigitte Royer-Pokora, Tilman Vogel, Robin K S Phillips, Allan D Spigelman, Richard S Houlston,

Although an increased cancer risk in Peutz-Jeghers syndrome is established, data on the spectrum of tumors associated with the disease and the influence of germ-line STK11/LKB1 (serine/threonine kinase) mutation status are limited.

Clinical cancer research : an official journal of the American Association for Cancer Research - May 2006

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