ARFI and transient elastography for characterization of cystic fibrosis related liver disease: First longitudinal follow-up data in adult patients

Prospective studies concerning liver disease in pediatric cystic fibrosis patients are scarce. The present study aimed to describe the prevalence and clinical expression of cystic fibrosis - related liver disease, in a cohort of 62 pediatric patients. Descriptive study, resulting from the prospective evaluation, between 1994 and 2009, of 62 pediatric patients (age <18 years) with cystic fibrosis. The follow-up protocol included a clinical assessment every 2 months, liver function tests every 6 months and annual liver ultrasonography. The cumulative prevalence of liver disease was 11.2% (7/62 cases). All patients had ΔF508 mutation and pancreatic insufficiency, none had meconium ileus. The liver involvement became clinically evident at a mean age of 8 years (3-15 years), revealed by hepatomegaly or hepatosplenomegaly (3 cases) and/ or abnormalities of liver function tests (3 cases) changes of liver ultrasound (7 cases) with evidence of portal hypertension (2 cases). Four patients were submitted to liver biopsy; biliary fibrosis was documented in one case, focal biliary cirrhosis in 2 cases and multilobular cirrhosis in another case. Within a median 11.6 years follow-up period (all patients under UDCA therapy after liver disease diagnosis), progression of liver disease was observed in 2 patients; one patient developed refractory variceal bleeding and progressive hepatic failure, requiring liver transplant. The results of the present study agree with those of previous pediatric studies, further documenting clinical expression of liver disease in CF patients, which is usually detected in the first decade of life and emphasize the contribution of ultrasound to early diagnosis of liver involvement. Moreover, although advanced liver disease is a relatively rare event, early isolated liver transplantation may have to be considered at this age group.

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Correlation of Transient Elastography With Severity of Cystic Fibrosis–related Liver Disease

Journal of Pediatric Gastroenterology and Nutrition ◽

10.1097/mpg.0000000000001448 ◽

2017 ◽

Vol 64 (4) ◽

pp. 505-511 ◽

Cited By ~ 23

Author(s):

Amal Aqul ◽

Maureen M. Jonas ◽

Sarah Harney ◽

Roshan Raza ◽

Gregory S. Sawicki ◽

...

Keyword(s):

Cystic Fibrosis ◽

Liver Disease ◽

Transient Elastography ◽

Related Liver Disease

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Non-Invasive Evaluation of Cystic Fibrosis Related Liver Disease in Adults with ARFI, Transient Elastography and Different Fibrosis Scores

PLoS ONE ◽

10.1371/journal.pone.0042139 ◽

2012 ◽

Vol 7 (7) ◽

pp. e42139 ◽

Cited By ~ 31

Author(s):

Thomas Karlas ◽

Marie Neuschulz ◽

Annett Oltmanns ◽

Andrea Güttler ◽

David Petroff ◽

...

Keyword(s):

Cystic Fibrosis ◽

Liver Disease ◽

Transient Elastography ◽

Non Invasive ◽

Related Liver Disease ◽

Invasive Evaluation

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Acoustic Radiation Force Elastography and Transient Elastography in Cystic Fibrosis: Liver Stiffness Is not increased in CF-Related Liver Disease in Adults

Ultrasound in Medicine & Biology ◽

10.1016/j.ultrasmedbio.2011.05.080 ◽

2011 ◽

Vol 37 (8) ◽

pp. S13

Author(s):

T. Karlas ◽

M. Neuschulz ◽

A. Oltmanns ◽

A. Güttler ◽

H. Wirtz ◽

...

Keyword(s):

Cystic Fibrosis ◽

Liver Disease ◽

Acoustic Radiation ◽

Transient Elastography ◽

Liver Stiffness ◽

Radiation Force ◽

Acoustic Radiation Force ◽

Related Liver Disease

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Non-invasive Diagnostic Tests in Cystic Fibrosis-Related Liver Disease: A Diagnostic Test Accuracy Network Meta-Analysis

Frontiers in Medicine ◽

10.3389/fmed.2021.598382 ◽

2021 ◽

Vol 8 ◽

Author(s):

Ágnes Rita Martonosi ◽

Alexandra Soós ◽

Zoltán Rumbus ◽

Péter Hegyi ◽

Vera Izsák ◽

...

Keyword(s):

Cystic Fibrosis ◽

Liver Disease ◽

Diagnostic Test ◽

Diagnostic Performance ◽

Transient Elastography ◽

Meta Analysis ◽

Test Accuracy ◽

Non Invasive ◽

Related Liver Disease ◽

New Criteria

Background and Aims: Cystic fibrosis-related liver disease (CFLD) is one of the leading causes of morbidity and mortality in cystic fibrosis (CF). Several non-invasive diagnostic methods have been proposed as screening tools for CFLD. Our aim was to rank all available non-invasive modalities for diagnostic performance.Methods: A systematic search was performed in five medical databases to find studies which reported on any single or composite non-invasive diagnostic test (as an index test) compared to the Debray, the EuroCare or the Colombo criteria (as a reference standard). Ranking was carried out with a Bayesian diagnostic test accuracy network meta-analysis based on superiority indices, calculated for pooled sensitivity (Se) and specificity (Sp) with a 95% confidence interval (CI). The study was registered under CRD42020155846 in PROSPERO.Results: Fifteen studies with 15 index tests and a combination of them were included. The New criteria proposed by Koh et al. – which represent a composite diagnostic definition for CFLD including liver biochemistry, ultrasonography, transient elastography and fibrosis markers—had the best performance for detecting CFLD (Se:94%[CI:58–100], Sp:72%[CI:52–84]); while transient elastography (Se:65%[CI:56–74], Sp:88%[CI:84–91]) and a combination of it with a tissue inhibitor of metalloproteinase-4 measurement (Se:78%[CI:30–100], Sp:64%[CI:18–95%]) proved to be the second and third best options, respectively. In the imaging techniques subgroup, transient elastography (Se:66%[CI:57–72], Sp:88%[CI:85–91%]), acoustic radiation force impulse in the right lobe (Se:54%[CI:33–74], Sp:88%[CI:66–96]) and that in the left lobe (Se:55%[CI:23–81], Sp:82%[CI:50–95]) were ranked the highest. Comparing biochemical markers/fibrosis indices, the measurement of the Forns index (Se:72%[CI:25–99], Sp:63%[CI:16–94]), the aspartate aminotransferase-to-platelet ratio (Se:55%[CI:41–68], Sp:83%[CI:66–89]) and alkaline phosphatase (Se:63%[CI:18–93], Sp:64%[CI:19–95]) were ranked the highest.Conclusion: The New criteria show the best diagnostic performance. In clinical practice, transient elastography seems to be a simple, cheap and non-invasive tool, outperforming imaging, biochemical and fibrosis tests for detecting CFLD. Further studies are needed to validate our findings.

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