Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations

Sellers, Zachary M.; Assis, David N.; Paranjape, Shruti M.; Sathe, Meghana; Bodewes, Frank; Bowen, Melissa; Cipolli, Marco; Debray, Dominique; Green, Nicole; Hughan, Kara S.; Hunt, William R.; Leey, Julio; Ling, Simon C.; Morelli, Giuseppe; Peckham, Daniel; Pettit, Rebeca S.; Philbrick, Alexander; Stoll, Janis; Vavrina, Kay; Allen, Stacy; Goodwin, Tara; Hempstead, Sarah E.; Narkewicz, Michael R.

doi:10.1097/hep.0000000000000646

Cited by 23 publications

(4 citation statements)

References 157 publications

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“…Consensus recommendations and guidance from the CF Foundation on screening, evaluation, and management of hepatobiliary disease in pwCF are expected to lead to improved CF outcomes through earlier detection and increased evidence-based approaches to monitoring and treatment. 180 CFLD occurs in ~30% of pwCF. 181 Liver disease in the setting of CF occurs because of impaired biliary flow as an effect of CFTR genetic variants.…”

Section: Hepatobiliary Disease and Cystic Fibrosis-related Liver Diseasementioning

confidence: 99%

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

McDonald,

Reid,

Pohl

et al. 2024

Nut in Clin Prac

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Cystic fibrosis (CF) is a progressive, genetic, multi‐organ disease affecting the respiratory, digestive, endocrine, and reproductive systems. CF can affect any aspect of the gastrointestinal (GI) tract, including the esophagus, stomach, small intestine, colon, pancreas, liver, and gall bladder. GI pathophysiology associated with CF results from CF membrane conductance regulator (CFTR) dysfunction. The majority of people with CF (pwCF) experience exocrine pancreatic insufficiency resulting in malabsorption of nutrients and malnutrition. Additionally, other factors can cause or worsen fat malabsorption, including the potential for short gut syndrome with a history of meconium ileus, hepatobiliary diseases, and disrupted intraluminal factors, such as inadequate bile salts, abnormal pH, intestinal microbiome changes, and small intestinal bacterial overgrowth. Signs and symptoms associated with fat malabsorption, such as abdominal pain, bloating, malodorous flatus, gastroesophageal reflux, nausea, anorexia, steatorrhea, constipation, and distal intestinal obstruction syndrome, are seen in pwCF despite the use of pancreatic enzyme replacement therapy. Given the association of poor nutrition status with lung function decline and increased mortality, aggressive nutrition support is essential in CF care to optimize growth in children and to achieve and maintain a healthy body mass index in adults. The introduction of highly effective CFTR modulator therapy and other advances in CF care have profoundly changed the course of CF management. However, GI symptoms in some pwCF may persist. The use of current knowledge of the pathophysiology of the CF GI tract as well as appropriate, individualized management of GI symptoms continue to be integral components of care for pwCF.

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Section: Hepatobiliary Disease and Cystic Fibrosis-related Liver Diseasementioning

confidence: 99%

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

McDonald,

Reid,

Pohl

et al. 2024

Nut in Clin Prac

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“…Several definitions have been used with Table 1 showing that proposed by the North American Cystic Fibrosis Foundation. 3,18 More recent categorizations by the Cystic Fibrosis Foundation Liver Disease Guideline Committee are further simplified to Advanced CF Liver Disease (aCFLD) or CF Hepatobiliary Involvement (CFHBI) 19 :…”

Section: Liver Involvement In Cystic Fibrosismentioning

confidence: 99%

“…Foundation provide clear recommendations on optimal strategies for Screening, Diagnosis, Monitoring and Management of persons with CF with possible liver involvment, 19 covered in more detail in an accompanying chapter in this series (Early Detection, Elastography, and Biomarkers).…”

Section: These Recently Published Guidelines By the Cystic Fibrosismentioning

confidence: 99%

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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“…53 The most recent guidelines published by the CFF recommend all PwCF with CFHBI undergo a baseline US that is repeated at least every 2 years. 1…”

Section: Ultrasound In Evaluation Of Cfhbimentioning

confidence: 99%

Early detection of hepatobiliary involvement in cystic fibrosis: Biomarkers, radiologic methods, and genetic influences

Sankararaman,

Freeman

2024

Pediatric Pulmonology

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Cystic fibrosis‐related hepatobiliary involvement (CFHBI) is a term used to describe a spectrum of hepatobiliary involvement ranging from a transient elevation of transaminase levels to advanced cystic fibrosis‐associated liver disease (aCFLD). While CFHBI is common among people with cystic fibrosis (PwCF), aCFLD is rare impacting only approximately 5%–10% of the CF population. After respiratory/cardiorespiratory issues and transplant‐related complications, aCFLD is now the 4th leading cause of mortality among PwCF. Additionally, aCFLD is an independent predictor of all‐cause mortality and is associated with significant morbidity. Despite this recognition, our ability to predict those patients at greatest risk for aCFLD, identify early aCFLD, and monitor the incremental progression of CFHBI is lacking. Here, we review the strengths and weaknesses of the common biomarkers and imaging modalities used in the evaluation and monitoring of CFHBI, as well as the current understanding of genetic modifiers related to aCFLD.

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Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations

Cited by 23 publications

References 157 publications

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

Pathophysiology of Cystic Fibrosis Liver Disease

Early detection of hepatobiliary involvement in cystic fibrosis: Biomarkers, radiologic methods, and genetic influences

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