Saturday, July 4, 2015

Peutz-Jeghers Syndrome & Juvenile Polyposis Syndrome News - July 2015

Peutz-Jeghers Syndrome & Juvenile Polyposis Syndrome News
Updated July 2015




The following resources are for two different rare genetic disorders Peutz-Jeghers Syndrome (PJS) & Juvenile Polyposis Syndrome (JPS). While both lead to gastrointestinal polyps and other medical challenges, they are different disorders, so please look before clicking on a link.

Warmest healing wishes,
Stephanie Sugars

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INFORMATION AND NEWS

All journal articles below are available free full text. For more titles and abstracts, please search PubMed

* The Peutz-Jeghers Syndrome & Juvenile Polyposis Syndrome Online Support Group

Please join us there by clicking on the link and following instructions to join. If you aren’t ready to join, please bookmark the website for future use.

* Peutz-Jeghers News blog by Stephanie Sugars 
http://peutz-jeghersnews.blogspot.com/

* Juvenile Polyposis Syndrome blog by Stephanie Sugars 
http://juvenilepolyposissyndromenews.blogspot.com

* 2015 Peutz-Jeghers Syndrome Meet & Greets are being planned for both Boston and Houston.

* 2015 Introductory article to PJS and JPS

* 2015 eMedicine article on PJS

* 2015 Genetic counseling practice guidelines that include PJS and JPS

* 2014 Storytelling event – I tell of PJS and its affect on my life with breast cancer and relationship with death

* 2014 Free online 40-minute radio show about PJS

* 2014 Introductory article PJS and JPS and similar polyposis syndromes

* 2014 Juvenile Polyposis syndrome entry at GeneReviews

* 2014 Juvenile Polyposis syndrome articles

* 2014 Cancer.Net PJS entry
and 2014 JPS entry

* 2014 Update on Imaging in PJS

* 2014 Intro to PJS for affected and their loved ones from St. Mark’s Hospital Polyposis Registry in London

* 2014 Intro to JPS for affected and their loved ones from St. Mark’s Hospital Polyposis Registry in London

* 2013 GeneReviews PJS entry
 Directories for genetic testing laboratories and clinics can be found at: http://www.genetests.org/

* 2013 Genetics Home Reference
Peutz-Jeghers syndrome entry

* 2012 Juvenile polyposis syndrome: a study of genotype, phenotype, and long-term outcome
free slide presentation

* 2012 PJS and family planning: the attitude towards prenatal genetic testing and pre-implantation genetic diagnosis

* The Summer 2011 issue of Familial Cancer journal focuses on
Peutz-Jeghers syndrome. The issue includes an article by me,
Peutz-Jeghers syndrome: a patient’s view. It can be purchased for
$34.95 at

Or read in an unformatted version at

* 2011 The third Peutz-Jeghers Syndrome Meet & Greet was held in Denver, Colorado. For a copy of the DVD, please contact Heidi at neverseenthat10@yahoo.com

* 2011 article about JPS - Juvenile polyposis syndrome

* 2010 radio show about the PJS Online Support Group
(click on Among Ourselves - PJS begins around minute 15)

* 2010 article from 2007 conference of European PJS specialists
Peutz–Jeghers syndrome: a systematic review and recommendations for Management
free full text
(note: this link may not work, if not please copy and paste the URL into your browser to reach the article.)

* 2010 free full text article includes PJS and Juvenile Polyposis
Syndrome - Hereditary hamartomatous polyposis syndromes: understanding the disease risks as children reach adulthood. by M. Manfredi

* 2010 report on Genetic mosaicism in PJS people. This is something patients and family members should discuss with genetic counselors…but they don't have the information yet.

*  2010 report – How to have a PJS-free baby

* 2009 Implications of Peutz-Jeghers Syndrome in Children and Adolescents

* 2009 Juvenile Polyposis entry from Cancer Syndromes textbook

* 2009 Juvenile Polyposis syndrome entry from Genetics Home Reference

* 2009 PJS in the news. This story is about mice, not people. And no, these drugs aren't currently available to PJS human patients, though there was a small trial of them in Utah. And the suggested FDG-PET scans for screening have several drawbacks including accuracy, expense & radiation exposure. Still, it's interesting that mouse PJS polyps show increased glucose metabolism. The PJS gene LKB1 affects both metabolism and cancer risk. Press release:  http://www.eurekalert.org/pub_releases/2009-06/si-hc061209.php

* 2008 50+ page PJS entry from Cancer Syndromes textbook
appropriate for patients, family, doctors and genetic counselors at

* 2006 Frequency and Spectrum of Cancers in the Peutz-Jeghers Syndrome.

* 2006 Peutz-Jeghers Syndrome and Management Recommendations. This article from Johns Hopkins includes suggested screening for PJS people & at-risk family members. Click on full-text tab for free full text.

TREATMENT OPTIONS
* Learn more about the PillCam capsule endoscopy for small bowel imaging and find a physician in your region at www.givenimaging.com

* Enteroscopy allows the entire small bowel to be scoped and replaces major and endoscopic surgery in some PJS patients. Interventional balloon enteroscopy at http://www.ncbi.nlm.nih.gov/pubmed/22586550

* Learn more about double balloon enteroscopy, a new surgical method and get referrals to doctors who use it at www.fujinonendoscopy.com 
Learn more about its use in adults at

* Olympus has technologies for reaching and visualizing the small bowel including endocapsule, spiral enteroscopy and single balloon enteroscopy. http://www.olympusamerica.com/msg_section/index.asp

GENETIC TESTING
* The genetic test method, MLPA, has increased the accuracy of genetic testing for PJS. Those with previously negative or inconclusive test results may wish to be retested. Clinic and laboratory directories for PJS http://www.ncbi.nlm.nih.gov/gtr/conditions/C0031269/

* Clinic and laboratory directories for JPS
And for JPHT
Juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome

* PJS genetic testing is available (no travel necessary) through Professor
Rodney Scott, Division of Genetics, Hunter Area Pathology Services,
John Hunter Hospital, Lookout Road, New Lambton, NSW 2305 Australia.
Check with Dr. Scott for cost Rodney.Scott@newcastle.edu.au

TRIALS AND STUDIES SEEKING PARTICIPANTS

* Pancreatic cancer screening studies open to PJS participants
Baltimore, Maryland (ask about study sites in Massachusetts, Ohio, and Pennsylvania)
White Plains, NY
Arkansas
Tampa Florida also has a pancreatic cancer screening study

* Dr. Stratakis, an endocrinologist with the NIH, continues to study PJS patients in Bethesda, MD, USA

* Study of people with PJS and JPS in France

ADDITIONAL RESOURCES
* European laboratories, research studies and information

* Many free, full-text, old articles on PJS are available at

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ONLINE SUPPORT GROUPS - INHERITED CANCER SYNDROMES

Peutz-Jeghers Syndrome & Juvenile Polyposis Syndrome Online Support Group   
The affected, family, friends, medical professionals and researchers are welcome to join the free online support group  https://www.smartpatients.com/communities/peutz-jeghers-syndrome

Peutz-Jeghers Syndrome Support – Facebook Group
Now a closed group

Sindrome de Peutz-Jeghers en Espanol

Peutz-Jeghers syndrome in China

Hereditary Colon Cancer Takes Guts                                                          
Familial Adenomatous Polyposis Foundation

FAP/Gardner syndrome

Lynch Syndrome or HNPCC

Li-Fraumeni

Cowden/PTEN

Stephanie Sugars









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