Noninvasive Methods, including Transient Elastography, for the Detection of Liver Disease in Adults with Cystic Fibrosis

Sadler, Matthew D; Crotty, Pam; Fatovich, Linda; Wilson, Stephanie R.; Rabin, Harvey; Myers, Robert P.

doi:10.1155/2015/138530

Cited by 38 publications

(46 citation statements)

References 40 publications

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“…Another important finding in this study is the demonstration of the utility of incorporating VCTE (FibroScan) and noninvasive fibrosis markers into a diagnostic algorithm for CFLD. Recently, increasing evidence has suggested the potential utility of FibroScan in the detection of pediatric CFLD, with only a few studies in adults . This study adds to the growing body of evidence that VCTE may have utility in clinical practice in adult patients with CF.…”

Section: Discussionmentioning

confidence: 76%

“…Recently, increasing evidence has suggested the potential utility of Fibro-Scan in the detection of pediatric CFLD, (27)(28)(29)(30)(31) with only a few studies in adults. (11,32) This study adds to the growing body of evidence that VCTE may have utility in clinical practice in adult patients with CF. This is timely given that the FibroScan is approved by the US Food and Drug Administration for clinical use, which has resulted in increased availability in many advanced medical centers that routinely provide care to CF patients.…”

Section: Discussionmentioning

confidence: 90%

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Adult‐onset cystic fibrosis liver disease: Diagnosis and characterization of an underappreciated entity

et al. 2017

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Background & Aims Cystic fibrosis liver disease (CFLD), a leading cause of death in cystic fibrosis (CF), is mostly described in pediatric populations. Adult-onset CFLD lacks sufficient characterization and diagnostic tools. Methods A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with a newly proposed CFLD criteria. Results 36 CF patients were followed for a median of 24.5 years(IQR=15.6, 32.9). By the last follow-up, 11(31%) had died. With conventional criteria, 8(22%) patients had CFLD, and by new criterion, 17 (47%) had CFLD at a median age of 36.6 years(IQR=26.5, 43.2). By new criterion, those with CFLD had higher median ALT(42 vs 27, p=0.005), AST(26 vs 21, p=0.01), direct bilirubin(0.13 vs 0.1, p=0.01), PT(14.4 vs 12.4, p=0.002), and APRI(0.31 vs 0.23, p=0.003) over the last two years of follow-up. Subjects with a Fibroscan® >6.8kPa had higher ALT(42 vs 28U/L, p=0.02), AST(35 vs 25U/L, p=0.02), APRI(0.77 vs 0.25, p=0.0004), FIB-4(2.14 vs 0.74, p=0.0003) and lower platelet counts(205 vs 293, p=0.02). One CFLD patient had nodular regenerative hyperplasia. Longitudinally, mean platelet counts significantly declined in the CFLD group(310 to 230U/L, p=0.0005). Deceased CFLD patients had lower platelet counts than those alive with CFLD(143 vs 258 U/L, p=0.004) or those deceased with no CFLD(143 vs 327U/L, p=0.006). Conclusion Adult-onset CFLD may be more prevalent than previously described which suggests a later wave of CFLD that impacts morbidity. Routine liver tests, radiologic imaging, noninvasive fibrosis markers and fibroscan® can be utilized algorithmically to identify adult CFLD. Further evaluation in other CF cohorts should be performed for validation.

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Section: Discussionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 90%

Adult‐onset cystic fibrosis liver disease: Diagnosis and characterization of an underappreciated entity

et al. 2017

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“…Transient elastography (FibroScan, Echosens, France) using the M probe was used for all Fontan patients and was performed by a single experienced operator in a standardized manner reporting the median value of at least 10 measurements from the right lobe of the liver (16). All scans were interpreted by an expert hepatologist (RPM), and liver stiffness was reported in kilopascals.…”

Section: Methodsmentioning

confidence: 99%

Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients

Fidai

Dallaire

Alvarez

et al. 2017

Front. Cardiovasc. Med.

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Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8.4 ± 4.3 and 5.4 ± 4.1 years post-Fontan) and 8 adults (average age 31.5 ± 8.9 and 21.1 ± 4 years post-Fontan) using standard serum laboratory investigations assessing hepatic integrity and function, the FibroTest, liver ultrasound, and transient elastography (FibroScan). In adult Fontan patients, hemoglobin, C-reactive protein, and gamma-glutamyl transpeptidase were significantly increased, and white blood cell and platelet counts were significantly decreased in comparison to the pediatric cohort. International normalized ratio was mildly elevated in both children and adults. FibroTest results were suggestive of fibrosis regardless of time post-Fontan. FibroScan measurements were significantly correlated with time post-Fontan, but the incidence of ultrasound-detected liver abnormalities was variable. No cases of hepatocellular carcinoma were identified. Abnormalities suggestive of FALD occur in both children and adults post-Fontan. Select laboratory tests, and possibly ultrasound and FibroScan in some patients, appear to have the most promise for the non-invasive detection of FALD.

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“…In‐depth evaluation of biomarker indices, such as aspartate aminotransferase‐to‐platelet ratio index (APRI) and Fibrosis‐4, and advanced imaging modalities, such as transient elastography (TE) and acoustic radiation force impulse, have been shown to accurately reflect advanced liver disease/end‐stage fibrosis in CF. However, they have not yet been shown to predict outcomes, thus limiting their application in the selection of patients for LT …”

Section: Indications For Lt Evaluation In Patients With Cfldmentioning

confidence: 99%

A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis–Associated Liver Disease

et al. 2019

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Approximately 5%‐10% of patients with cystic fibrosis (CF) will develop advanced liver disease with portal hypertension, representing the third leading cause of death among patients with CF. Cystic fibrosis with advanced liver disease and portal hypertension (CFLD) represents the most significant risk to patient mortality, second only to pulmonary or lung transplant complications in patients with CF. Currently, there is no medical therapy to treat or reverse CFLD. Liver transplantation (LT) in patients with CFLD with portal hypertension confers a significant survival advantage over those who do not receive LT, although the timing in which to optimize this benefit is unclear. Despite the value and efficacy of LT in selected patients with CFLD, established clinical criteria outlining indications and timing for LT as well as disease‐specific transplant considerations are notably absent. The goal of this comprehensive and multidisciplinary report is to present recommendations on the unique CF‐specific pre‐ and post‐LT management issues clinicians should consider and will face.

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Noninvasive Methods, including Transient Elastography, for the Detection of Liver Disease in Adults with Cystic Fibrosis

Abstract: FS, APRI and FT were useful noninvasive methods for detecting CFLD in adults.

Cited by 38 publications

References 40 publications

Adult‐onset cystic fibrosis liver disease: Diagnosis and characterization of an underappreciated entity

Adult‐onset cystic fibrosis liver disease: Diagnosis and characterization of an underappreciated entity

Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients

A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis–Associated Liver Disease

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