Transient elastography and portal hypertension in pediatric patients with cystic fibrosis

Malbrunot-Wagner, A.C.; Bridoux, Laure; Nousbaum, Jean-Baptiste; Riou, C.; Dirou, Armel; Giniès, J.L.; Maurage, C.; Cagnard, B.; Pélatan, C.; Dabadie, A.

doi:10.1016/j.jcf.2011.04.004

Cited by 48 publications

(49 citation statements)

References 21 publications

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“…However, the prognostic role of CFLD in adults is less certain, and disease progression after adolescence is rare [7], [26]. Previous studies consisted of mixed patient cohorts with a large proportion of pediatric patients [12], [13] or of CF children with portal hypertension [14]. Hence, the reported increase of liver stiffness in these CFLD patients may be related to the more severe course of pediatric CFLD while our results reflect a milder degree of liver damage in adult CFLD cases.…”

Section: Discussionmentioning

confidence: 53%

“…First results of transient elastography (Fibroscan®, TE) in cystic fibrosis revealed an elevated liver stiffness in patients with CFLD classified according to pathologic ultrasound criteria [12] and biochemical or clinical definitions [13]. In a case series, TE could reliably identify children with CFLD and portal hypertension [14]. First data with the recently developed acoustic radiation force impulse imaging (ARFI) technique revealed a high accuracy for the detection of liver cirrhosis in pediatric CF patients with portal hypertension [15].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2012

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BackgroundCystic fibrosis-related liver disease (CFLD) is present in up to 30% of cystic fibrosis patients and can result in progressive liver failure. Diagnosis of CFLD is challenging. Non-invasive methods for staging of liver fibrosis display an interesting diagnostic approach for CFLD detection.AimWe evaluated transient elastography (TE), acoustic radiation force impulse imaging (ARFI), and fibrosis indices for CFLD detection.MethodsTE and ARFI were performed in 55 adult CF patients. In addition, AST/Platelets-Ratio-Index (APRI), and Forns' score were calculated. Healthy probands and patients with alcoholic liver cirrhosis served as controls.ResultsFourteen CF patients met CFLD criteria, six had liver cirrhosis. Elastography acquisition was successful in >89% of cases. Non-cirrhotic CFLD individuals showed elastography values similar to CF patients without liver involvement. Cases with liver cirrhosis differed significantly from other CFLD patients (ARFI: 1.49 vs. 1.13 m/s; p = 0.031; TE: 7.95 vs. 4.16 kPa; p = 0.020) and had significantly lower results than individuals with alcoholic liver cirrhosis (ARFI: 1.49 vs. 2.99 m/s; p = 0.002). APRI showed the best diagnostic performance for CFLD detection (AUROC 0.815; sensitivity 85.7%, specificity 70.7%).ConclusionsARFI, TE, and laboratory based fibrosis indices correlate with each other and reliably detect CFLD related liver cirrhosis in adult CF patients. CF specific cut-off values for cirrhosis in adults are lower than in alcoholic cirrhosis.

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Section: Discussionmentioning

confidence: 53%

Section: Introductionmentioning

confidence: 99%

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

et al. 2012

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“…Noninvasive methods such as transient elastography can also be used for the detection of fibrosis in patients with chronic liver disease [39,40] . Studies of pediatric patients have produced favorable evidence regarding the use of these techniques [40,[41][42][43] . A study evaluating the use of transient elastography in children with chronic liver disease found that this method had good capacity to discriminate between significant fibrosis, severe fibrosis and cirrhosis [40] .…”

Section: Investigationmentioning

confidence: 99%

Cirrhosis in children and adolescents: An overview

Pinto

Schneider

Silveira

2015

WJH

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Several conditions, especially chronic liver diseases, can lead to cirrhosis in children and adolescents. Most cases in clinical practice are caused by similar etiologies. In infants, cirrhosis is most often caused by biliary atresia and genetic-metabolic diseases, while in older children, it tends to result from autoimmune hepatitis, Wilson's disease, alpha-1-antitrypsin deficiency and primary sclerosing cholangitis. The symptoms of cirrhosis in children and adolescents are similar to those of adults. However, in pediatric patients, the first sign of cirrhosis is often poor weight gain. The complications of pediatric cirrhosis are similar to those observed in adult patients, and include gastrointestinal bleeding caused by gastroesophageal varices, ascites and spontaneous bacterial peritonitis. In pediatric patients, special attention should be paid to the nutritional alterations caused by cirrhosis, since children and adolescents have higher nutritional requirements for growth and development. Children and adolescents with chronic cholestasis are at risk for several nutritional deficiencies. Malnutrition can have severe consequences for both pre-and post-liver transplant patients. The treatment of cirrhosis-induced portal hypertension in children and adolescents is mostly based on methods developed for adults. The present article will review the diagnostic and differential diagnostic aspects of end-stage liver disease in children, as well as the major treatment options for this condition.

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“…However, detection of CF-associated liver disease (CFLD) is challenging. Many studies have been done in the recent years to assess the role of different non-invasive markers in assessing liver fibrosis and PHT in patients with CF [177][178][179][180][181][182] [181]. Furthermore, TIMP-1 had a high diagnostic accuracy for CFLD in adults.…”

Section: Cystic Fibrosismentioning

confidence: 99%

“…Furthermore, TIMP-1 had a high diagnostic accuracy for CFLD in adults. The diagnostic sensitivities were increased when TE and the respective biomarkers were combined for the detection of CFLD and EV [182].…”

Section: Cystic Fibrosismentioning

confidence: 99%

Asian-Pacific Association for the Study of the Liver (APASL) consensus guidelines on invasive and non-invasive assessment of hepatic fibrosis: a 2016 update

et al. 2016

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Transient elastography and portal hypertension in pediatric patients with cystic fibrosis

Abstract: Elastography should be recommended for all cystic fibrosis patients with liver disease to follow its progression. A prospective study is needed to define an elastography threshold value that predicts the presence of EV.

Cited by 48 publications

References 21 publications

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

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

Cirrhosis in children and adolescents: An overview

Asian-Pacific Association for the Study of the Liver (APASL) consensus guidelines on invasive and non-invasive assessment of hepatic fibrosis: a 2016 update

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