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Outcomes and Predictors for Re-stenosis of Esophageal Stricture in Epidermolysis Bullosa: A Multicenter Cohort Study
Indexado
WoS WOS:000570130600010
Scopus SCOPUS_ID:85090068999
DOI 10.1097/MPG.0000000000002820
Año 2020
Tipo artículo de investigación

Citas Totales

Autores Afiliación Chile

Instituciones Chile

% Participación
Internacional

Autores
Afiliación Extranjera

Instituciones
Extranjeras


Abstract



Background: Esophageal strictures are the common gastrointestinal complications in patients with epidermolysis bullosa (EB) requiring dilation. There is limited information on the best type of intervention, outcomes, and predictors for re-stenosis. Objectives: We aimed to investigate the frequency, clinical presentation of esophageal strictures in EB patients, and to ascertain the predictors of re-stenosis. Methods: We conducted a retrospective, multicenter cohort study involving 7 specialized, international EB centers on patients who were 0 to 50 years of age. Descriptive statistics and hazard risks for re-stenosis were calculated. Results: We identified 125 patients with 497 esophageal stricture episodes over a mean period of observation of 17 (standard deviation [SD] = 11.91) years. Dilations were attempted in 90.74% of episodes, using guided fluoroscopy 45.23%, retrograde endoscopy 33.04%, and antegrade endoscopy 19.07%. Successful dilation was accomplished in 99.33% of attempts. Patients experienced a median of 2 (interquartile range [IQR]: 1-7) stricture episodes with a median interval between dilations of 7 (IQR: 4-12) months. Predictors for re-stenosis included: number of strictures (2 vs 1 stricture: chi(2) = 4.293,P = 0.038, hazard ratio [HR] = 1.294 (95% confidence interval [CI]: 1.014--1.652 and 3 vs 1 stricture:chi(2) = 7.986,P = 0.005, HR = 1.785 [95% CI: 1.194, 2.667]) and a long (>= 1 cm) segment stricture (chi(2) = 4.599,P = 0.032, HR = 1.347 (95% CI: 1.026--1.769). Complications were more common with the endoscopic approach (8/86, antegrade endoscopy; 2 /149, retrograde endoscopy vs 2/204, fluoroscopy;chi(2) = 17.39,P-value <0.000). Conclusions: We found excellent dilation outcomes irrespective of the dilation procedure; however, with higher complications in the endoscopic approach. Long (>1 cm) segment involvement and multiple locations were predictive of stricture reoccurrence.

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Disciplinas de Investigación



WOS
Nutrition & Dietetics
Pediatrics
Gastroenterology & Hepatology
Scopus
Sin Disciplinas
SciELO
Sin Disciplinas

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Publicaciones WoS (Ediciones: ISSHP, ISTP, AHCI, SSCI, SCI), Scopus, SciELO Chile.

Colaboración Institucional



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Autores - Afiliación



Ord. Autor Género Institución - País
1 Pope, Elena Mujer Hosp Sick Children - Canadá
UNIV TORONTO - Canadá
Hospital for Sick Children University of Toronto - Canadá
University of Toronto - Canadá
The Hospital for Sick Children - Canadá
2 Mansour, Mark Hombre Western Univ - Canadá
Schulich School of Medicine &amp; Dentistry - Canadá
3 Berseneva, Maria Mujer Hosp Sick Children - Canadá
University of Toronto - Canadá
4 Wong, Carmen Liy Mujer Hosp Sick Children - Canadá
UNIV TORONTO - Canadá
Hospital for Sick Children University of Toronto - Canadá
University of Toronto - Canadá
The Hospital for Sick Children - Canadá
5 Salas, Julio Hombre Rio Plata & DEBRA - México
Rio de la Plata and DEBRA - México
6 FUENTES-SAN ROMAN, IGNACIO Mujer Universidad del Desarrollo - Chile
Fundación DEBRA Chile - Chile
Clínica Alemana - Chile
7 YUBERO-GONCALVES, MARIA JOAO Hombre Universidad del Desarrollo - Chile
Fundación DEBRA Chile - Chile
Facultad de Medicina Clínica Alemana Universidad del Desarrollo - Chile
Clínica Alemana - Chile
8 PALISSON-ETCHARREN, FRANCIS Hombre Universidad del Desarrollo - Chile
Fundación DEBRA Chile - Chile
9 Martinez, Anna Mujer Great Ormond St Hosp NHS Fdn Trust - Reino Unido
Great Ormond Street Hospital for Children NHS Foundation Trust - Reino Unido
10 Mellerio, J. E. Mujer Great Ormond St Hosp NHS Fdn Trust - Reino Unido
Great Ormond Street Hospital for Children NHS Foundation Trust - Reino Unido
11 Lara-Corrales, Irene Mujer Hosp Sick Children - Canadá
UNIV TORONTO - Canadá
Hospital for Sick Children University of Toronto - Canadá
University of Toronto - Canadá
The Hospital for Sick Children - Canadá
12 Yang, Anes Hombre Univ NSW - Australia
UNSW Sydney - Australia
13 Murrell, Dedee - Univ NSW - Australia
UNSW Sydney - Australia
14 Torres-Pradilla, Mauricio Hombre Fdn Univ Ciencias Salud & Hosp San Jose - Colombia
Hospital De San José, Bogotá, Colombia - Colombia
15 Lucky, Anne W. Mujer Cincinnati Childrens Hosp - Estados Unidos
Cincinnati Children's Hospital Medical Center - Estados Unidos

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Financiamiento



Fuente
Epidermolysis Bullosa Medical Research Foundation
Epidermolysis Bullosa Research Foundation

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Agradecimientos



Agradecimiento
This study was funded by Epidermolysis Bullosa Research Foundation.
This study was funded by Epidermolysis Bullosa Research Foundation.

Muestra la fuente de financiamiento declarada en la publicación.