Cirrhosis associated with decreased survival and a 10-year lower median age at death of cystic fibrosis patients in the Netherlands

Pals, F.H.; Verkade, Henkjan J.; Gulmans, Vincent; Koning, Barbara A E de; Koot, Bart; Meij, Tim G. J. de; Hendriks, D.; Gierenz, N.; Vreugdenhil, A.C.E.; Houwen, Roderick H. J.; Bodewes, Frank

doi:10.1016/j.jcf.2018.11.009

Cited by 22 publications

(21 citation statements)

References 25 publications

(22 reference statements)

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“…There is ongoing debate as to whether CFLD shortens life expectancy in CF, with some authors suggesting that liver disease does not affect outcome [16][17][18] , while we and others have shown that CFLD is associated with a worse prognosis 5,8,19 . If life expectancy for PWCF is to be further improved, clearly identifying risk factors for mortality that may be amenable to treatment is important.…”

Section: Rationalementioning

confidence: 86%

“…Today, children born with CF in Europe or the USA can expect to survive at least into their fifth decade 4 . However, while the management of pulmonary complications of CF has greatly improved, complications such as cystic fibrosis liver disease (CFLD) contribute to increased morbidity and mortality in CF [5][6][7][8] .…”

Section: Rationalementioning

confidence: 99%

“…CFLD [19][20][21] . Compounding all of these factors is the complicated nature of multidisciplinary care in CF, the complexity of data capture and interpretation across the transition from paediatric to adult care.…”

Section: Revisedmentioning

confidence: 99%

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The impact of liver disease on mortality in cystic fibrosis - a systematic review protocol

et al. 2020

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Background Cystic fibrosis (CF) is a multiorgan disease affecting the lungs pancreas and gastrointestinal tract. Pulmonary complications are the most common manifestation of the disease. Recent advances in the treatment of pulmonary complications have resulted in substantial improvement in life expectancy. Less than 10% of persons with CF (PWCF) develop liver disease (CFLD). There is conflicting evidence about impact of liver disease on mortality in CF, with evidence suggesting that CFLD contributes to increased mortality in CF, while other studies suggest that the impact on mortality is limited. Understanding the contribution of liver disease to mortality in CF is essential if further improvements in life expectancy are to be achieved. Objective: To document the impact of liver disease on life expectancy for PWCF. Methods: This systematic review will be conducted in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Protocols (PRISMA-P 2015). PubMed, Medline and Embase will be searched for English language publications (1949-2020). Studies reporting the outcome for CFLD will be included where the definition of CFLD is outlined clearly in a CF population. Studies with and without a comparator will be evaluated. Clinical trials of ursodeoxycholic acid will be excluded as well as organ transplantation outcome studies. We will examine all-cause and specific causes of mortality.We will include transplantation in our estimates of all-cause mortality. The Axis Risk of Bias tool for observational will be used to evaluate the quality of studies. We will provide a narrative synthesis of our findings using tabular formats to highlight any impact of liver disease on mortality in CF. Conclusion: It is anticipated that this review will bring clarity to the question of whether CFLD shortens life expectancy in PWCF and stimulate new approaches to the management of CFLD.

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Section: Rationalementioning

confidence: 86%

Section: Rationalementioning

confidence: 99%

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The impact of liver disease on mortality in cystic fibrosis - a systematic review protocol

et al. 2020

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“…Once a patient is diagnosed with cirrhosis, an understanding of the mortality related to liver disease is important for patient prognostic counseling. In a retrospective analysis of Dutch CF Centers, patients with cirrhosis had lower 6‐year survival compared with those without (77% vs 93%) and a lower median age at death (27 years vs 37 years) with the majority still having respiratory failure as the cause of death . Thus, being able to screen for and identify liver abnormalities earlier, could hopefully lead to early treatment and therefore improved outcomes.…”

Section: Multisystem Effects Of Cystic Fibrosismentioning

confidence: 99%

“…IQR] 1301 kcal/d vs 686; P < .0001) and proportion of energy intake (median [IQR] 44%[34][35][36][37][38][39][40][41][42][43][44][45][46][47][48][49][50][51] vs 31%[24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43]; P < .0001). Thus, while children with CF met their energy requirements and ate ample fat, this was largely due to ingestion of EDNP foods.…”

mentioning

confidence: 99%

Cystic fibrosis year in review 2018, part 2

Savant

McColley

2019

Pediatric Pulmonology

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Pathophysiology of Cystic Fibrosis Liver Disease

Kasper,

Assis

2024

Pediatric Pulmonology

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Hepatobiliary complications of Cystic Fibrosis (CF) constitute a significant burden for persons with CF of all ages, with advanced CF liver disease in particular representing a leading cause of mortality. The causes of the heterogeneity of clinical manifestations, ranging from steatosis to focal biliary cholestasis and biliary strictures, are poorly understood and likely reflect a variety of environmental and disease‐modifying factors in the setting of underlying CFTR mutations. This review summarizes the current understanding of the pathophysiology of hepatobiliary manifestations of CF, and discusses emerging disease models and therapeutic approaches that hold promise to impact this important yet incompletely addressed aspect of CF care.

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Cirrhosis associated with decreased survival and a 10-year lower median age at death of cystic fibrosis patients in the Netherlands

Cited by 22 publications

References 25 publications

The impact of liver disease on mortality in cystic fibrosis - a systematic review protocol

The impact of liver disease on mortality in cystic fibrosis - a systematic review protocol

Cystic fibrosis year in review 2018, part 2

Pathophysiology of Cystic Fibrosis Liver Disease

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