Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study

Colombo, Carla; Alicandro, Gianfranco; Oliver, Mark; Lewindon, Peter; Ramm, Grant A.; Ooi, Chee Y.; Alghisi, Federico; Kashirskaya, N.; Kondratyeva, E.I.; Corti, Fabiola; Padoan, Rita; Asherova, I.; Evans, Helen; Monestrol, Isabelle de; Strandvik, Birgitta; Lindblad, Anders

doi:10.1016/j.jcf.2021.03.014

Cited by 27 publications

(16 citation statements)

References 21 publications

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“…A Cochrane review including 3 randomised controlled trials on the use of ursodeoxycholic acid for at least three months compared to placebo or no additional treatment (n = 118), reported no benefit and did not recommend UDCA for routine use in CF [131]. Colombo et al also reported no evidence of any benefit on the occurrence of portal hypertension [132]. On the other hand, a large longitudinal cohort study (n = 3417) suggested that UDCA had a positive effect on survival in patients with mild CFLD [7].…”

Section: Treatmentmentioning

confidence: 99%

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Dana

Debray

Beaufrère

et al. 2022

Journal of Hepatology

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Section: Treatmentmentioning

confidence: 99%

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Dana

Debray

Beaufrère

et al. 2022

Journal of Hepatology

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“…However, data to demonstrate the long‐term benefit of UDCA in preventing disease progression in CFLD populations is lacking 10 . Moreover, early use of UDCA has been shown to have no impact on the subsequent development of CFLD cirrhosis 10,73 . It is important to note, that these are cohort studies with potential selection bias and cause‐effect conclusions cannot be definitively made.…”

Section: Treatment Options In Cfldmentioning

confidence: 99%

“…10 Moreover, early use of UDCA has been shown to have no impact on the subsequent development of CFLD cirrhosis. 10,73 It is important to note, that these are cohort studies with potential selection bias and cause-effect conclusions cannot be definitively made. Improved characterisation of disease extent may define a sub-population that might derive benefit from UDCA.…”

Section: Ta B L E 1 Different Presentations Of Liver Disease In Cystic Fibrosismentioning

confidence: 99%

Review article: epidemiology, pathogenesis and management of liver disease in adults with cystic fibrosis

Scott

Jones

Hanley

et al. 2022

Aliment Pharmacol Ther

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Summary Background Cystic fibrosis‐related liver disease (CFLD) is the leading cause of death in cystic fibrosis (CF), after pulmonary disease. To improve identification and management of this condition requires an understanding of the underlying disease mechanism. Aims This review summarises the current understanding of CFLD epidemiology, pathology, diagnosis and management. Methods Relevant reports on cystic fibrosis liver disease were identified using a literature search and summarised. Results CFLD is a heterogeneous condition with several different co‐existent pathologies, including environmental and genetic factors. Incidence of clinically significant CFLD continues at a linear rate into early adulthood and has been described in up to 25% of CF patients. Diagnosis strategies lack precision and patient risk stratification needs to look beyond Childs‐Pugh scoring. Efficacious therapies are lacking and, at present, newer modulator therapies lack data in CFLD and carry an increased risk of hepatotoxicity. Outcomes of liver transplant are comparable to non‐CF transplant indications. Conclusions The incidence of CFLD increases with age and hence is increasingly important to adult patients with CF. Effective therapies are lacking. For progress to be made a better understanding of pathogenesis and disease detection are required.

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“…Some other longitudinal studies have shown an influence on growth, renal and liver function [55,58,67,68]. The progress of CF-related liver disease has shown an association to low LA [69,70], and Sweden, where the largest centres had provided LA supplementation for decades [4], had the lowest percentage of portal hypertension and cirrhosis compared to 10 other countries worldwide [71].…”

Section: Fatty Acid Abnormalities In Cfmentioning

confidence: 99%

Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations

Strandvik

2022

Nutrients

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Nutrition is important in cystic fibrosis (CF) because the disease is associated with a higher energy consumption, special nutritional deficiencies, and malabsorption mainly related to pancreatic insufficiency. The clinical course with deterioration of lung function has been shown to relate to nutrition. Despite general recommendation of high energy intake, the clinical deterioration is difficult to restrain suggesting that special needs have not been identified and specified. It is well-known that the CF phenotype is associated with lipid abnormalities, especially in the essential or conditionally essential fatty acids. This review will concentrate on the qualitative aspects of fat metabolism, which has mainly been neglected in dietary fat recommendations focusing on fat quantity. For more than 60 years it has been known and confirmed that the patients have a deficiency of linoleic acid, an n-6 essential fatty acid of importance for membrane structure and function. The ratio between arachidonic acid and docosahexaenoic acid, conditionally essential fatty acids of the n-6 and n-3 series, respectively, is often increased. The recently discovered relations between the CFTR modulators and lipid metabolism raise new interests in this field and together with new technology provide possibilities to specify further specify personalized therapy.

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Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study

Cited by 27 publications

References 21 publications

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Review article: epidemiology, pathogenesis and management of liver disease in adults with cystic fibrosis

Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations

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