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

Karlas, Thomas; Neuschulz, Marie; Oltmanns, Annett; Wirtz, Hubert; Keim, Volker; Wiegand, Johannes

doi:10.1016/j.jcf.2013.04.004

Cited by 21 publications

(25 citation statements)

References 4 publications

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“…Although defined criteria for the diagnosis and management of cystic fibrosis-associated liver disease have been published recently, they might miss early stages of CFLD, especially in the absence of significant fibrosis deposition within the liver [8]. As shown in previous reports from our group and others, the measurement of liver stiffness with transient elastography has a high diagnostic accuracy for the detection of CFLD in adults and children [4–6,12–14]. Thus, the approach of assessing of liver stiffness using TE sensitively and noninvasively, which directly reflects increased matrix deposition and fibrogenesis in the liver, can complement or even facilitate conventional clinically oriented diagnosis by enhancing specificity and improving early detection of CFLD.…”

Section: Discussionmentioning

confidence: 99%

“…Nevertheless, so far only two studies presented follow-up data for TE-assessment in CFLD patients [4,12]. Karlas et al described a stable course of CFLD in adult CF patients once they have passed adolescence [4].…”

Section: Discussionmentioning

confidence: 99%

“…Karlas et al described a stable course of CFLD in adult CF patients once they have passed adolescence [4]. Van Biervliet et al retrospectively showed stable liver stiffness in young CF patients without liver disease on the one hand, and progressively increasing stiffness in young CF patients with developing liver disease on the other hand [12].…”

Section: Discussionmentioning

confidence: 99%

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Assessment of pathologic increase in liver stiffness enables earlier diagnosis of CFLD: Results from a prospective longitudinal cohort study

et al. 2017

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About 30% of patients with Cystic Fibrosis (CF) develop CF-associated liver disease (CFLD). Recent studies have shown that transient elastography (TE), as a method to quantify liver stiffness, allows non-invasive diagnosis of CFLD in adults and children with CF. Within this study we aimed to prospectively identify patients at risk for development of CFLD by longitudinal analysis of liver stiffness and fibrosis scores in a 5-year follow-up. 36 pediatric and 16 adult patients with initial liver stiffness below the cut-off value indicative of CFLD (6.3 kPa) were examined by transient elastography for 4-5 years. TE, APRI-, and FIB-4-scores were assessed and compared by Kruskal-Wallis test and receiver operating characteristic (ROC)-analysis. Frequencies were compared by Chi 2 -test. Among the 36 patients participating in this study, a subgroup of 9 patients developed liver stiffness >6.3 kPa after 4-5 years with an increase of ΔTE >0.38 kPa/a (the group with increasing liver stiffness was labelled TE inc ). APRI-and FIB-4 scores confirmed the rationale for grouping. The frequency of CFLD assessed by conventional diagnosis was significantly higher in TE inc -group compared to the control group (TE norm ). None of the adult CF patients matched criteria for TE incgroup. For the first time it was shown that the non-invasive longitudinal assessment of TE allows identification of patients with progression of CFLD in a subgroup of juvenile but not in adult CF patients. Comparing TE to conventional fibrosis-scores underlined the strength of the continuous assessment of liver stiffness for the exact diagnosis of progressive CFLD. The newly described cut-off for pathologic increase of liver stiffness, ΔTE cutoff = 0.38kPa/a, might enable to detect developing CFLD using consequent follow up TE measurements before reaching the level of stiffness indicating established CFLD. Nevertheless, the limited size of the analyzed cohort should encourage a prospective, multi-center, long term follow up study to confirm the suggested cut-off for the rise in liver stiffness.PLOS ONE | https://doi.org/10.1371/journal.pone

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Assessment of pathologic increase in liver stiffness enables earlier diagnosis of CFLD: Results from a prospective longitudinal cohort study

et al. 2017

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“…Regions of evaluation are chosen by ultrasound, allowing avoidance of anatomic obstacles [19,20]. Several studies have assessed the use of ARFI in the evaluation of CFLD [21,22,23,24,25,26]. Shear Wave Velocity increased progressively from patients with no evidence of CFLD to patients with CFLD with no evidence of portal hypertension to those with signs of portal hypertension [21].…”

Section: Diagnosis Of Cfldmentioning

confidence: 99%

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

Staufer

Halilbasic

Trauner

et al. 2014

IJMS

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Due to improved medical care, life expectancy in patients with cystic fibrosis (CF) has veritably improved over the last decades. Importantly, cystic fibrosis related liver disease (CFLD) has become one of the leading causes of morbidity and mortality in CF patients. However, CFLD might be largely underdiagnosed and diagnostic criteria need to be refined. The underlying pathomechanisms are largely unknown, and treatment strategies with proven efficacy are lacking. This review focuses on current invasive and non-invasive diagnostic standards, the current knowledge on the pathophysiology of CFLD, treatment strategies, and possible future developments.

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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%

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

Abstract: Fig. 1. Elastography data from 41 patients with cystic fibrosis (median follow-up period 22.5 months). Nine cases fulfilled the definition of cystic fibrosis related liver disease (CFLD) at baseline examination.

Cited by 21 publications

References 4 publications

Assessment of pathologic increase in liver stiffness enables earlier diagnosis of CFLD: Results from a prospective longitudinal cohort study

Assessment of pathologic increase in liver stiffness enables earlier diagnosis of CFLD: Results from a prospective longitudinal cohort study

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

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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