Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis.
J Pediatr Gastroenterol Nutr
; 66(1): 122-127, 2018 01.
Article
em En
| MEDLINE
| ID: mdl-28906321
ABSTRACT
OBJECTIVES:
Cirrhosis occurs in 5% to 10% of cystic fibrosis (CF) patients, often accompanied by portal hypertension. We analyzed 3 adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver-related death (LD), and risk factors for these in CF Foundation Patient Registry subjects with reported cirrhosis.METHODS:
We determined 10-year incidence rates for VB, LT, LD, and all-cause mortality (ACM), and examined risk factors using competing risk models and Cox-proportional hazard regression.RESULTS:
From 2003 to 2012, 943 participants (41% females, mean age 18.1 years) had newly reported cirrhosis; 24.7% required insulin, 85% had previous pseudomonas. Seventy-three subjects had reported VB 38 with first VB and new cirrhosis reported simultaneously and 35 with VB after cirrhosis report. Ten-year cumulative VB, LT, and LD rates were 6.6% (95% confidence interval [CI] 4.0, 9.1%), 9.9% (95% CI 6.6%, 13.2%), and 6.9% (95% CI 4.0%, 9.8%), respectively, with an ACM of 39.2% (95% CI 30.8, 36.6%). ACM was not increased in subjects with VB compared to those without (hazard ratio [HR] 1.10, 95% CI 0.59, 2.08). CF-related diabetes (HR 3.141, 95% CI1.56, 6.34) and VB (HR 4.837, 95% CI 2.33, 10.0) were associated with higher LT risk, whereas only worse lung function was associated with increased LD in multivariate analysis. Death rate among subjects with VB was 24% with LT and 20.4% with native liver.CONCLUSIONS:
VB is an uncommon complication of CF cirrhosis and can herald the diagnosis, but does not affect ACM. Adverse liver outcomes and ACM are frequent by 10 years after cirrhosis report.
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Varizes Esofágicas e Gástricas
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Fibrose Cística
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Hemorragia Gastrointestinal
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Cirrose Hepática
Tipo de estudo:
Etiology_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adolescent
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Child
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Female
/
Humans
/
Male
Idioma:
En
Revista:
J Pediatr Gastroenterol Nutr
Ano de publicação:
2018
Tipo de documento:
Article