Liver disease in patients with cystic fibrosis

Kamal, Natasha; Surana, Pallavi; Koh, Christopher

doi:10.1097/mog.0000000000000432

Cited by 46 publications

(50 citation statements)

References 33 publications

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“…A previous study demonstrated that hepatic cytochrome P450 expression is reduced in rats subjected to DSS-induced colitis via LPS-induced downregulation [79]. Patients affected by cystic fibrosis lack the function of the cystic fibrosis transmembrane conductance regulator (CFTR), which is involved in bile flow and alkalinization, and may develop liver damage due to chronic cholestasis [80]. DSS-induced colitis has been demonstrated to cause biliary injury in Cftr knockout mice, which could be reversed by antibiotic treatment, and not by bile induction, via administration of 24-nor-ursodeoxycholic acid [81].…”

Section: Figurementioning

confidence: 99%

Gut–Liver Axis and Inflammasome Activation in Cholangiocyte Pathophysiology

Maroni

Ninfole

Pinto

et al. 2020

Cells

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The Nlrp3 inflammasome is a multiprotein complex activated by a number of bacterial products or danger signals and is involved in the regulation of inflammatory processes through caspase-1 activation. The Nlrp3 is expressed in immune cells but also in hepatocytes and cholangiocytes, where it appears to be involved in regulation of biliary damage, epithelial barrier integrity and development of fibrosis. Activation of the pathways of innate immunity is crucial in the pathophysiology of hepatobiliary diseases, given the strong link between the gut and the liver. The liver secretes bile acids, which influence the bacterial composition of the gut microbiota and, in turn, are heavily modified by microbial metabolism. Alterations of this balance, as for the development of dysbiosis, may deeply influence the composition of the bacterial products that reach the liver and are able to activate a number of intracellular pathways. This alteration may be particularly important in the pathogenesis of cholangiopathies and, in particular, of primary sclerosing cholangitis, given its strong association with inflammatory bowel disease. In the present review, we summarize current knowledge on the gut-liver axis in cholangiopathies and discuss the role of Nlrp3 inflammasome activation in cholestatic conditions.

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Section: Figurementioning

confidence: 99%

Gut–Liver Axis and Inflammasome Activation in Cholangiocyte Pathophysiology

Maroni

Ninfole

Pinto

et al. 2020

Cells

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“…A study aiming to identify patients with early fibrosis in CFLD demonstrated that vibration‐controlled transient elastography could differentiate patients with CFLD from those without CFLD (8.2 versus 4.5 kPA with an area under the curve of 0.91). It also found that an increase of >0.38 kPA/year in scores could identify those at greatest risk for progression . Incorporating transient elastography results into the NC improved the specificity and positive predictive value to diagnose CFLD, but sensitivity was limited because some degree of fibrosis was needed before the test results were positive for CFLD.…”

Section: Diagnosis Management and Treatment Of Liver Disease In Cfmentioning

confidence: 99%

“…It also found that an increase of >0.38 kPA/year in scores could identify those at greatest risk for progression. (29,30) Incorporating transient elastography results into the NC improved the specificity and positive predictive value to diagnose CFLD, (7) but sensitivity was limited because some degree of fibrosis was needed before the test results were positive for CFLD. On the basis of these data, we implemented a protocol in our lung transplant program to screen all patients with CF for liver disease (Tables 1 and 2).…”

Section: Fibrosis Assessmentmentioning

confidence: 99%

Cystic Fibrosis–Associated Liver Disease in Lung Transplant Recipients

Mallea¹,

Bolan²,

Cortese

et al. 2019

Liver Transpl

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Cystic fibrosis (CF) is an autosomal recessive disease characterized by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). CFTR gene mutations manifest as epithelial cell dysfunction in the airways, biliary tract, pancreas, gut, sweat glands, paranasal sinuses, and genitourinary tract. The clinical manifestations of this dysfunction include respiratory tract infections, bronchiectasis, pancreatic insufficiency, malabsorption, intestinal obstruction, liver disease, and male infertility. The liver disease manifestations of CF can include biliary disease, multilobular cirrhosis, and portal hypertension with and without cirrhosis. Pulmonary disease is the main cause for morbidity and mortality in individuals with CF, and according to the International Society for Heart and Lung Transplantation, CF is the third most common indication for lung transplantation in adults, accounting for 16% of procedures performed. The survival after lung transplantation in individuals with CF continues to improve and is now the highest among end‐stage lung diseases requiring transplant. The survival rate at 10 years is close to 50%. Given the potential presence of liver disease in CF patients undergoing an evaluation for lung transplantation and in lung transplant recipients, it is important to understand the manifestations of liver disease in CF patients and the recommended workup and follow‐up. This review aims to discuss the current literature and provide guidance in the management of these patients.

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“…84 This is a biliary tract epithelium-based cirrhosis that presents most frequently in teenagers, though it can occur de novo later in life. 85 Screening for liver disease is undertaken annually by the measurement of transaminases and the ultrasound/fibroscan imaging of the liver, though there is no strong evidence that this changes outcomes. 47 Management of CF-related liver disease should be in collaboration with an experienced hepatologist as it is a progressive condition that may ultimately require transplantation.…”

Section: Other Organ Diseasesmentioning

confidence: 99%

Adult Care in Cystic Fibrosis

Elborn

2019

Semin Respir Crit Care Med

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Cystic fibrosis (CF) is now more common in adults than children in countries with well-developed health care systems. The number of adults continues to increase and will further increase if the new cystic fibrosis transmembrane conductance regulator (CFTR) modulators are disease modifying. Most of the complex morbidity and almost all the mortality of CF occur in adults and will increasingly follow this pattern even with new effective modulator therapies. Maintaining good quality of life including social functioning and maximizing survival for adults are the key priorities. This requires a highly knowledgeable and adaptable multidisciplinary team, which, though focused on maintaining lung health, requires an increasing range of other disciplines and specialties to maximize well-being. Changes in health care systems will require current models of care to adapt to provide care for the large number of adult patients. With increasing survival and age, many are likely to have both CF morbidities and additional diseases of aging. New models are needed for health care delivery for this expanding population with complex medical conditions.

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Liver disease in patients with cystic fibrosis

Abstract: A better understanding of the pathophysiology of disease is critical to developing more disease-specific diagnostics and therapeutics.

Cited by 46 publications

References 33 publications

Gut–Liver Axis and Inflammasome Activation in Cholangiocyte Pathophysiology

Gut–Liver Axis and Inflammasome Activation in Cholangiocyte Pathophysiology

Cystic Fibrosis–Associated Liver Disease in Lung Transplant Recipients

Adult Care in Cystic Fibrosis

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