Liver Disease in Cystic Fibrosis: Illuminating the Black Box

Colombo, Carla; Alicandro, Gianfranco

doi:10.1002/hep.30255

Cited by 14 publications

(20 citation statements)

References 11 publications

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“…There is insufficient evidence to justify its routine use in cystic fibrosis as a preventative measure. Present data suggesting that UDCA should be started before severe liver damage is present as it might be able to prevent the progression of CFLD and has the potential to induce a reversal of fibrosis [1,2,4,40,[49][50][51].…”

Section: Background Therapy Of Cystic Fibrosis Include Pancreas Enzymmentioning

confidence: 99%

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Liver Changes in the Course of Cystic Fibrosis

Więcek¹

2020

Cystic Fibrosis - Heterogeneity and Personalized Treatment

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Liver damage observed in cystic fibrosis is a complicated process comprised of fibrogenesis, inflammation, remodeling, apoptosis, and cholestasis. Complexity of processes which take place in the liver and bile ducts in the course of this disease are not clearly defined. Changes in the liver are observed in 5-20% of patients with diagnosed cystic fibrosis; however they can increase mortality, shorten time of life, and deteriorate quality of life. The aim of the chapter was to review the risk factors, clinical symptoms, diagnostic methods, and treatment of liver changes in the course of cystic fibrosis.

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Section: Background Therapy Of Cystic Fibrosis Include Pancreas Enzymmentioning

confidence: 99%

“…Cirrhosis is detected in about 10% of CF children under the age of 18 compared to 2% of adults with the same condition. The average age of the CF liver disease manifestation is about 10 years [1][2][3][4][5][6][7].…”

Section: Introductionmentioning

confidence: 99%

Liver Changes in the Course of Cystic Fibrosis

Więcek¹

2020

Cystic Fibrosis - Heterogeneity and Personalized Treatment

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“…Liver diseases are the most common extrapulmonary causes of death in patients with cystic fibrosis. They occur most often in the first decade of life [1][2][3][4]. Cirrhosis of the liver is detected in approximately 10% of children (<18 years) with cystic fibrosis, compared to only 2% of adults with this disease.…”

Section: Introductionmentioning

confidence: 99%

“…The role of pathophysiological lesions of bile acids and genetic and immunological factors is highlighted in the etiopathogenesis of hepatic lesions in cystic fibrosis [1,2,5]. Abnormal expression of cystic fibrosis transmembrane conductance regulator (CFTR) protein on the apical membranes of cholangiocytes within the epithelium of the bile ducts and the epithelium of the gallbladder leads to changes to the composition of bile, abnormalities to its transport, and the retention of toxic bile acids (taurine), which induces chemokines responsible for the processes of inflammation and fibrosis [2,5,6]. The most common lesions to the liver in the course of CF include focal liver fibrosis (72%), focal biliary cirrhosis (20-30%), multilobular biliary cirrhosis (5-15%), portal hypertension (2-5%), and/or cholelithiasis (14-24%).…”

Section: Introductionmentioning

confidence: 99%

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Assessment of Selected Parameters of Liver Fibrosis and Inflammation in Patients with Diagnosed Cystic Fibrosis

Więcek

Woś

Pogorzelski³

et al. 2020

Mediators of Inflammation

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Changes in the liver and bile ducts observed in patients diagnosed with cystic fibrosis result from inflammatory processes as well as fibrosis, remodeling, apoptosis, and cholestasis. As a consequence, portal hypertension, cirrhosis, and hepatic failure may develop. So far, the complexity of these processes has not been elucidated. Study Objectives. The aim of the study was to evaluate the selected parameters of hepatitis and fibrosis (Fibrotest, Actitest, and APRI) in patients diagnosed with cystic fibrosis. Material and Methods. The study included 79 patients with cystic fibrosis, aged 1 to 20 years (mean age 9.8 years), 49 girls (62%) and 30 boys (38%). The analysis involved the following: age, sex, clinical manifestations, laboratory tests evaluating pancreas function, parameters of liver damage, and cholestasis. Fibrotest, Actitest, and APRI were performed in all subjects. Results. Elevated parameters of hepatic cell damage (hypertransaminasemia) were found in 31/79 (39.2%) patients, while abnormal cholestasis parameters in 21/79 (26.6%). The abnormal results of Fibrotest were reported in 15% of patients (12/79), while of Actitest in 10% (8/79). In contrast, elevated APRI values were found in only 7.6% (6/79) of subjects. There was a statistically significant correlation between APRI and age (higher values were observed in younger children) and between Fibrotest and Actitest and pancreatic insufficiency (higher values were found in subjects without this abnormality). Moreover, Fibrotest values were significantly higher in girls. There was no correlation between Fibrotest, Actitest, and APRI values and the type of mutation. Conclusion. It appears that Fibrotest may be used as an early marker of liver fibrosis in patients with cystic fibrosis. Increased APRI values were only found in subjects with advanced hepatic lesions, most often in the form of portal hypertension.

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Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis

Leung

Molleston

et al. 2021

Hepatol Commun

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Methods to identify children with cystic fibrosis (CF) at risk for development of advanced liver disease are lacking. We aim to determine the association between liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) with research ultrasound (US) patterns and conventional hepatic markers as a potential means to follow liver disease progression in children with CF. ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study. We studied the association between LSM with research-grade US patterns (normal [NL], heterogeneous [HTG], homogeneous [HMG], or nodular [NOD]) and conventional hepatic markers. In a subgroup (n = 79), the association between controlled attenuation parameter (CAP) and US pattern was explored. Among 133 subjects undergoing VCTE, NOD participants (n = 26) had a significantly higher median (interquartile range) LSM of 9.1 kPa (6.3, 15.8) versus NL (n = 72, 5.1 kPa [4.2, 7.0]; P < 0.0001), HMG (n = 17, 5.9 kPa [5.2, 7.8]; P = 0.0013), and HTG (n = 18, 6.1 kPa [4.7, 7.0]; P = 0.0008) participants. HMG participants (n = 14) had a significantly higher mean CAP (SD) (270.5 dB/m [61.1]) compared with NL (n = 40, 218.8 dB/m [46.5]; P = 0.0027), HTG (n = 10, 218.1 dB/m [60.7]; P = 0.044), and NOD (n = 15, 222.7 dB/m [56.4]; P = 0.041) participants. LSM had a negative correlation with platelet count (r s = −0.28, P = 0.0071) and positive correlation with aspartate aminotransferase-toplatelet ratio index (r s = 0.38, P = 0.0002), Fibrosis-4 index (r s = 0.36, P = 0.0007), gamma-glutamyltransferase (GGT; r s = 0.35, P = 0.0017), GGT-to-platelet ratio (r s = 0.35, P = 0.003), and US spleen size z-score (r s = 0.27, P = 0.0073). Conclusion: VCTE is associated with US patterns and conventional markers in patients with liver disease with CF. (Hepatology Communications 2021;0:1-11).A dvanced cystic fibrosis-associated liver disease (CFLD) is evidenced by cirrhosis with or without portal hypertension, or nodular regenerative hyperplasia with portal hypertension, and occurs in approximately 7%-10% of patients with cystic fibrosis (CF). (1) Cirrhosis is seen primarily in childhood, (1,2) whereas noncirrhotic portal hypertension affects all ages. (3) More subtle liver involvement including hepatomegaly, persistently elevated aspartate aminotransferase (AST), alanine aminotransferase

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Liver Disease in Cystic Fibrosis: Illuminating the Black Box

Cited by 14 publications

References 11 publications

Liver Changes in the Course of Cystic Fibrosis

Liver Changes in the Course of Cystic Fibrosis

Assessment of Selected Parameters of Liver Fibrosis and Inflammation in Patients with Diagnosed Cystic Fibrosis

Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis

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