Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices

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http://hdl.handle.net/10138/328546

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Luoto , T T , Koivusalo , A & Pakarinen , M P 2020 , ' Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices ' , Journal of Pediatric Gastroenterology and Nutrition , vol. 70 , no. 5 , pp. 628-634 . https://doi.org/10.1097/MPG.0000000000002643

Title: Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices
Author: Luoto, Topi T.; Koivusalo, Antti; Pakarinen, Mikko P.
Contributor: University of Helsinki, Children's Hospital
University of Helsinki, HUS Children and Adolescents
University of Helsinki, HUS Children and Adolescents
Date: 2020-05
Language: eng
Number of pages: 7
Belongs to series: Journal of Pediatric Gastroenterology and Nutrition
ISSN: 0277-2116
URI: http://hdl.handle.net/10138/328546
Abstract: Objectives: Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed to assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices. Methods: All children with esophageal varices (n = 126) were identified from 14,144 single nationwide referral center endoscopy reports during 1987 to 2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded. Results: Nineteen underlying disorders included biliary atresia (35%), extrahepatic portal vein obstruction (35%), autosomal recessive polycystic kidney disease (7%), and other disorders (23%). During median follow-up of 15.2 (range 0.5-43.1) years patients underwent median 9 (1-74) upper gastrointestinal endoscopies. Esophageal varices were first observed at a median age of 4.0 (0.3-18.2) years, 112 (89%) patients underwent median 6 (1-56) sclerotherapy/banding sessions, and 61 (48%) experienced median 2 (range 1-20) variceal bleeding episodes. Forty-eight surgical shunt procedures were performed to 41 (36%) patients and 38% underwent liver transplantation. Portal hypertensive biliopathy was diagnosed in 4 patients. Hepatopulmonary syndrome necessitated liver transplantation in 2 patients, hepatic encephalopathy in 2, and hepatorenal syndrome in 1. No patient died of variceal bleeding. Patient-reported perception of health on a scale of 1 to 10 was 9 (range 4-10), and 86% reported no current symptoms attributable to esophageal varices. Conclusions: Pediatric-onset portal hypertension is a heterogeneous disease with significant long-term morbidity, requiring multimodal approach with considerable resources and continuation of follow-up in adulthood. Although mortality to variceal bleeding was avoided, bleeding episodes recurred also in adulthood, while patient-reported health of long-term survivors was encouraging.
Subject: children
hypersplenism
long-term
portal hypertension
shunt surgery
varices
POLYCYSTIC KIDNEY-DISEASE
QUALITY-OF-LIFE
PRIMARY PROPHYLAXIS
ESOPHAGEAL-VARICES
VEIN OBSTRUCTION
BILIARY ATRESIA
MESOPORTAL BYPASS
LIVER-DISEASE
SINGLE-CENTER
CHILDREN
3123 Gynaecology and paediatrics
3121 General medicine, internal medicine and other clinical medicine
3143 Nutrition
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