Sindrome de Peutz-Jeghers en Espanol

 Síndrome de Peutz-Jeghers

Informacion - Sindrome de Peutz-Jeghers

http://es.wikipedia.org/wiki/S%C3%ADndrome_de_Peutz-Jeghers

http://www.hon.ch/HONselect/RareDiseases/PT/C04.700.705.html

http://www.nlm.nih.gov/medlineplus/spanish/ency/article/000244.htm

Por personas en Europa

http://www.orpha.net/consor/cgi-bin/index.php?lng=ES

Slideshare Plus

http://www.slideshare.net/hilacha/sndrome-de-peutz-jeghers

Una madre de PJS hijo en Espana crea un blog y un grupo por personas con

PJS y sus familias. Ella nombre es Laura y ella email es

sindromedepeutzjeghers@gmail.com

El blog Síndrome de Peutz-Jeghers:

http://sindromedepeutzjeghers.blogspot.com/

El grupo de Síndrome de Peutz-Jeghers:

http://groups.google.es/group/sindromedepeutzjeghers

Un doctor en Uruquay:

Dr. Asadur Jorge Tchekmedyian

asadur@chasque.net

http://www.smu.org.uy/dpmc/pracmed/temas/peutz.html

Findings from the peutz-jeghers syndrome registry of uruguay. Tchekmedyian A, Amos CI, Bale SJ, Zhu D, Arold S, Berrueta J, Nabon N, McGarrity T. Gastroenterology, Pasteur Hospital, Ministry of Public Health, Montevideo, Uruguay. PLoS One. 2013 Nov 19;8(11):e79639. doi: 10.1371/journal.pone.0079639. http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0079639

Articulos en Espanol:

http://www.ncbi.nlm.nih.gov/sites/entrez

* [Entero-tomography and Peutz-Jeghers syndrome].

Dardik D, Levy Yeyati E, Ulla M. Medicina (B Aires). 2012;72(4):323. Spanish. PubMed PMID: 22892084.

http://www.medicinabuenosaires.com/PMID/22892084.pdf

http://www.scielo.org.ar/scielo.php?script=sci_arttext&pid=S0025-76802012000400009

* Síndrome de Peutz-Jeghers: comunicación de un caso con cirugías múltiples por poliposis hamartomatosa

Peutz-Jeghers syndrome: a case report with multiple surgical procedures due to hamartomatous polyposis

Caren Jocelyn Aquino Farrera, Shein Asariel Rodríguez Inzunza

Edición Octubre-Diciembre 2012 / Volumen 10-Número 4

Mexico

http://www.dcmq.com.mx/edicion-octubre-diciembre-2012-volumen-10-número-4/99-sindrome-de-peutz-jeghers-comunicacion-de-un-caso-con-cirugias-multiples-por-poliposis-hamartomatosa

* Triple invaginación intestinal en paciente con síndrome de Peutz-Jeghers

Santos Gómez, Idoia; Mast Vilaseca, Richard; Madrazo González, Zoilo; Cañas Lozano, Concepción; Hernández Gañán, Ganan

Publicado en Cir Esp. 2011;89:252-3. - vol.89 núm 04

http://www.elsevier.es/es/revistas/cirugia-española-36/triple-invaginacion-intestinal-paciente-sindrome-peutz-jeghers-90002319-cartas-cientificas-2011

* Invaginación intestinal en el síndrome de Peutz Jeghers - Semes

por MJP MORER - ‎2011

http://www.semes.org/revista/vol23_6/14.pdf

* SÍNDROME DE PEUTZ - JEGHERS, A PROPÓSITO DE UN CASO

2010 Bolivia

http://www.scielo.org.bo/scielo.php?pid=S1012-29662010000200014&script=sci_arttext

*  Papel de la Laparoscopia en el Tratamiento  del síndrome de Peutz Jeghers

Revisión de la Literatura

RESTREPO H., MD., SCC.; VALENCIA M., MD; TORO R, MD; HOYOS S., MD., SCC;

áLVAREZ O., MD.

29-abr-2009

http://www.encolombia.com/medicina/cirugia/cirugia15400re-papel.htm

* Síndrome de Peutz-Jeghers - SciELO Colombia

JO Nieto - ‎2009 -

http://www.scielo.org.co/pdf/rcg/v24n2/v24n2a13.pdf

* [Peutz-Jeghers syndrome]

Pérez Rodríguez MJ, Anaya Barea FM, Galiano Fernández E, de Diego Sierra

D, Condado Sánchez-Rojas I.

An Pediatr (Barc). 2008 Apr;68(4):369-72. Spanish.

PMID: 18394383

*  [Oral double-balloon enteroscopy reaching the cecum]

Pérez-Cuadrado E, Esteban Delgado P, Shanabo J.

Rev Esp Enferm Dig. 2007 Jun;99(6):351. Spanish.

PMID: 17883299

* Síndrome de Peutz - Jeghers Seguimiento de una familia

http://encolombia.com/medicina/cirugia/cirugia22_3_2007/Sindrome_de_Peutz.htm

*  [The diagnosis of Peutz-Jeghers syndrome]

González Muñoz JL, Angoso Clavijo M, Esteban Velasco C, Rodríguez Pérez

A, Muñoz Bellvis L, Gómez Alonso L.

Rev Esp Enferm Dig. 2007 Mar;99(3):167. Spanish.

PMID: 17516831 [PubMed - indexed for MEDLINE]

http://scielo.isciii.es/scielo.php?pid=S1130-01082007000300009&script=sci_arttext

* Double-balloon enteroscopy: a descriptive study of 50 explorations.

Pérez-Cuadrado E, Más P, Hallal H, Shanabo J, Muñoz E, Ortega I, López

Martín    A, Torrella E, López Higueras A, Martín A, Carballo F.

Rev Esp Enferm Dig. 2006 Feb;98(2):73-81. English, Spanish.

PMID: 16566639

*   [Non-small cell bronchogenic carcinoma and Peutz-Jeghers syndrome]

Estrada Trigueros G, López-Encuentra A, García Quero C.

Arch Bronconeumol. 2005 May;41(5):296. Spanish.

PMID: 15919014

* Síndrome de Peutz-Jeghers - edigraphic.com

JMA Francis - ‎2005 Mexico

http://www.medigraphic.com/pdfs/h-gral/hg-2005/hg052h.pdf

* Síndrome de Peutz-Jeghers y adenocarcinoma de colon

Peru

2004

http://www.scielo.org.pe/scielo.php?pid=S1022-51292004000400011&script=sci_arttext

* [Peutz-Jegher's syndrome]

Gutiérrez Benjumea A, Rojo García J, Aguilera Llovet MA, García Arqueza

C, Casanovas Lax J, Aguayo Maldonado J.

 An Esp Pediatr. 2001 Aug;55(2):161-4. Spanish.

PMID: 11472670

http://zl.elsevier.es/es/revista/anales-pediatria-37/sindrome-peutz-jeghers-13017038-notas-clinicas-2001

* Síndrome de Peutz-Jeghers

A Lozano 1996

http://sisbib.unmsm.edu.pe/bvrevistas/gastro/vol_16n1/sindrome_peutz_j.htm

My Article on Palliative Care - not PJS-specific

I Wish I'd Known Earlier...Palliative Care Is Not a Mandate Not to Treat

By Stephanie Sugars

Center for Advancing Health

August 13, 2013

http://www.cfah.org/blog/2013/i-wish-id-known-earlier-palliative-care-is-not-a-mandate-not-to-treat

How Old Is Old in PJS?

A recent discussion at the Peutz-Jeghers Syndrome Online Support Group – http://listserv.acor.org/archives/pjs.html – on life expectancy in people with PJS led me to do a little research on the topic. Here’s what I found from the medical literature. Fortunately several “older” members shared their ages – causing me to eat my words. I’m 57 years old.

GeneReviews PJS Entry updated July 25, 2013

Peutz-Jeghers Syndrome

Synonym: PJS

Thomas J McGarrity, MD, Christopher I Amos, PhD, Marsha L Frazier, PhD, and Chongjuan Wei, PhD

Initial Posting: February 23, 2001; Last Update: July 25, 2013.

Go to:

Summary

Disease characteristics. Peutz-Jeghers syndrome (PJS) is an autosomal-dominant condition characterized by the association of gastrointestinal polyposis, mucocutaneous pigmentation, and cancer predisposition. Peutz-Jeghers-type hamartomatous polyps are most common in the small intestine (in order of prevalence: in the jejunum, ileum, and duodenum) but can also occur in the stomach, large bowel, and extraintestinal sites including the renal pelvis, bronchus, gall bladder, nasal passages, urinary bladder, and ureters. Gastrointestinal polyps can result in chronic bleeding and anemia and also cause recurrent obstruction and intussusception requiring repeated laparotomy and bowel resection. Mucocutaneous hyperpigmentation presents in childhood as dark blue to dark brown macules around the mouth, eyes, and nostrils, in the perianal area, and on the buccal mucosa. Hyperpigmented macules on the fingers are common. The macules may fade in puberty and adulthood. Individuals with Peutz-Jeghers syndrome are at increased risk for a wide variety of epithelial malignancies (colorectal, gastric, pancreatic, breast, and ovarian cancers). Females are at risk for sex cord tumors with annular tubules (SCTAT), a benign neoplasm of the ovaries, and adenoma malignum of the cervix, a rare aggressive cancer. Males occasionally develop large calcifying Sertoli cell tumors (LCST) of the testes, which secrete estrogen and can lead to gynecomastia, advanced skeletal age, and ultimately short stature, if untreated.

Continued at:

http://www.ncbi.nlm.nih.gov/books/NBK1266/

Newsletter - Peutz-Jeghers Syndrome & Juvenile Polyposis Syndrome

OUTDATED!
FOR ARCHIVE ONLY!

May 2013

These resources are especially for those interested in PJS & JPS - the diagnosed, their friends, families, and health professionals. Please share this with any and all. A list of Online Support Groups for people interested in inherited cancer syndromes is at bottom of this email.

Warmest healing wishes,

Stephanie Sugars

pj4steph@aol.com

Jump to Newsletter

Management Guidelines from Europe

Peutz–Jeghers syndrome: a systematic review and recommendations for Management

Available online free full text
save $30!

http://diposit.ub.edu/dspace/bitstream/2445/18638/1/578379.pdf

(note: this link doesn’t always work, so you’ll need to copy and paste the URL into your browser to reach the article.)

Cancer risk in PJS – report from Italy

Cancer risk in PJS – report from Italy

Things are worse in Italy than elsewhere, especially for women. The risk of cancer in people with PJS is greatly increased over the risk for folks in the general population. Relative risk estimates are the number of times that a diagnosis is more likely to occur in someone with PJS over the general population.

So, yes, the risk of pancreatic cancer is 139.7 times higher in Italian folks with PJS. And for women, the rate is 245.4 times higher. While in men, it’s “only” 88.6 times higher.

Cancer risk increases with age for everyone, but for this group of Italians with PJS the cumulative risk (the risk of cancer by a certain age) is 20% by age 40; 43% by age 50; 71% by age 60 and 89% by age 65.

The authors included 119 PJS patients in the study and noted that 31 were diagnosed with cancers of gastrointestinal tract, female reproductive tract, pancreas, breast, thyroid (in order of frequency). There was one case each of liver cancer, skin cancer, brain cancer and testis cancer. The risk of pancreatic cancer and cervical cancer were especially high relative to the general population.

The authors discuss the possibility of surveillance for PJS patients and note that only 6 of 36 cancers were diagnosed during a PJS-specific surveillance, thus indicating that the management of these rare patients is still particularly difficult in our county.

Jump to article abstract –

PJS Day in Netherlands - 8 June, 2013

In the Erasmus MC in Rotterdam is a PJS information day organized on 8 June, 2013. The registration fee for this day will be € 5 per person and includes a lunch. Information and registration can be done via email address pjsinformatiedag@gmail.com

Pancreatic cancer risk in PJS - report from Netherlands

Pancreatic cancer risk in Peutz-Jeghers syndrome patients: a large cohort study

and implications for surveillance.

This recent report from the Netherlands follows 144 PJS patients for a combined total of 5640 person years. Seven patients developed pancreatic cancer and four patients were diagnosed with biliary duct or ampullary cancer.

From this data, the authors were able to estimate the risk of pancreatico-biliary cancer as 32% by age 70. This is 96 times the rate of pancreatic cancer for a person in the general population.

The authors also note in Table 1, Baseline characteristics of the Dutch PJS Cohort, that 48 of their 144 patients (33%) had died at the median age of 46 years and the median age of the 90 surviving patients was 37 years old. (note, six patients were lost to follow-up).

Based on the results of our current study, we recommend that PJS patients should be offered surveillance regardless of family history for PC, since all subjects with PC in our series had a negative family history of PC. Although the median age of PC onset in our cohort was 54 years, we propose that surveillance starts at the age of 30 years. This suggestion is based on the fact that two patients in our series developed cancer in the pancreatico-biliary region at a very young age (ages 35 and 36).

jump to article abstract –