Outcome in patients with cystic fibrosis liver disease

Rowland, Marion; Gallagher, C.; Gallagher, Charles G.; Laoide, R. Ó.; Canny, Gerard J.; Broderick, A; Drummond, James L.; Greally, Peter; Slattery, Dubhfeasa; Daly, Leslie; McElvaney, Noel G.; Bourke, Billy

doi:10.1016/j.jcf.2014.05.013

Cited by 47 publications

(67 citation statements)

References 28 publications

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“…An international study based on 561 CF patients with severe CFLD (cirrhosis and portal hypertension) showed similar results . The complications were mostly cirrhosis and portal hypertension, as shown by others . As recently described, we also observed a subset of patients with portal hypertension but no cirrhosis .…”

Section: Discussionsupporting

confidence: 90%

“…Focal biliary cirrhosis is the most clinically relevant CFLD because extension of the initially focal fibrogenic process may lead to multilobular biliary cirrhosis with subsequent portal hypertension and related complications . Indeed, multilobular cirrhosis is recognized to have a significant impact on morbidity and accounts for ~2.5% of mortality in patients with CF (the third cause of death after respiratory failure and transplantation‐related complications) . The prevalence of CFLD remains controversial, with estimates ranging from 2% to 68% in young patients with CF, due to the lack of a consistent definition .…”

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confidence: 99%

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Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

et al. 2018

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CFLD occurs not only during childhood but also later in the lifetime of patients with CF; male sex, CFTR F508del homozygosity, and history of meconium ileus are independent risk factors for CFLD development; earlier use of UDCA over the last 20 years has not changed the incidence of severe CFLD, leading to questions about the use of this treatment in young children given its possible adverse effects. This article is protected by copyright. All rights reserved.

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Section: Discussionsupporting

confidence: 90%

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confidence: 99%

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

et al. 2018

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“…Liver disease is an independent risk factor for mortality 1 and the third leading cause of death in CF, accounting for an overall mortality of 2.5%. The risk of developing cirrhosis (3-5%) is likely related to non- CFTR genetic variation and environmental influences 2 .…”

Section: Introductionmentioning

confidence: 99%

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

Stonebraker

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Pace

et al. 2016

Clinical Gastroenterology and Hepatology

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Background & Aims Liver disease is the third leading cause of death in patients with cystic fibrosis (CF), but features of patients with CF, severe liver disease, and portal hypertension have not been fully characterized. Methods We performed a retrospective analysis of data from 561 patients with CF (63% male, 99% with pancreatic insufficiency), liver disease (hepatic parenchymal abnormalities consistent with cirrhosis, confirmed by imaging), and portal hypertension (esophageal varices, portosystemic collaterals, or splenomegaly), with no alternate causes of liver disease. All patients were enrolled in the Genetic Modifier Study of Severe CF Liver Disease at 76 international centers, from January 1999 through July 2013. Results Male patients were diagnosed with liver disease at a younger age than female patients (10 vs 11 years; P=.01). Splenomegaly was observed in 99% of patients and varices in 71%. Levels of liver enzymes were near normal in most patients. Thrombocytopenia affected 70% of patients and was more severe in patients with varices (88×109/L vs 145×109/L; P<.0001). Ninety-one patients received liver transplants (16%), at a median age of 13.9 years. Compared to patients who did not receive liver transplants, patients who received liver transplants had lower platelet counts (78×109/L vs 113×100/L; P<.0001), higher international normalized ratios (P<.0001), and lower levels of albumin (P=.0002). The aminotransferase to platelet ratio index (APRi) and fibrosis index based on 4 factor (FIB-4) values were above diagnostic thresholds for CF liver disease in 96% and 90% of patients, respectively. Patients who received liver transplants or who had varices had higher APRi and FIB-4 values than patients who did not. Conclusions In patients with CF, severe liver disease develops early in childhood (around 10 years of age) and is more common in boys than girls. Patients with varices and those who receive liver transplants have more abnormal platelet counts and APRi and FIB-4 scores.

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“…Approximately 5%‐10% of patients with cystic fibrosis (CF) will develop advanced liver disease with portal hypertension, representing the third leading cause of death among patients with CF . Cystic fibrosis with advanced liver disease and portal hypertension (CFLD) develops almost exclusively prior to 20 years of age and is an independent risk factor for mortality . Furthermore, adverse outcomes, such as variceal bleeding, liver transplantation (LT), liver‐related death, and all‐cause mortality, predominantly occur in the first 10 years after cirrhosis is identified .…”

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A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis–Associated Liver Disease

et al. 2019

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Approximately 5%‐10% of patients with cystic fibrosis (CF) will develop advanced liver disease with portal hypertension, representing the third leading cause of death among patients with CF. Cystic fibrosis with advanced liver disease and portal hypertension (CFLD) represents the most significant risk to patient mortality, second only to pulmonary or lung transplant complications in patients with CF. Currently, there is no medical therapy to treat or reverse CFLD. Liver transplantation (LT) in patients with CFLD with portal hypertension confers a significant survival advantage over those who do not receive LT, although the timing in which to optimize this benefit is unclear. Despite the value and efficacy of LT in selected patients with CFLD, established clinical criteria outlining indications and timing for LT as well as disease‐specific transplant considerations are notably absent. The goal of this comprehensive and multidisciplinary report is to present recommendations on the unique CF‐specific pre‐ and post‐LT management issues clinicians should consider and will face.

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Outcome in patients with cystic fibrosis liver disease

Abstract: Liver disease is an independent risk factor for mortality in CF.

Cited by 47 publications

References 28 publications

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis–Associated Liver Disease

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