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

Aqul, Amal; Jonas, Maureen M.; Harney, Sarah; Raza, Roshan; Sawicki, Gregory S.; Mitchell, Paul D.; Fawaz, Rima

doi:10.1097/mpg.0000000000001448

Cited by 44 publications

(62 citation statements)

References 28 publications

(32 reference statements)

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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 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.…”

Section: Discussionmentioning

confidence: 99%

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: 99%

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

et al. 2017

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“…The role of magnetic resonance elastography of the liver during LT evaluation for patients with CF merits further investigation. Although advances in both shear‐wave elastography and TE are encouraging, they have not yet been validated to predict the need for transplantation or shown to detect small incremental changes . As such, their use in LT evaluation cannot be recommended at this time.…”

Section: The Evaluation For Lt 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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“…10 Five studies were prospective, [10][11][12]17,19 and 3 of the remaining 6 studies were retrospective. 14,16,20 The patient populations varied among the included studies, including 4 reports with patients who had various chronic or suspected liver diseases, 11,15,19,20 3 studies with cystic fibrosis cohorts, 10,16,17 and 4 studies with patients who had biliary atresia. [12][13][14]18 The age range of the patients in the 11 included studies was 3 months to 25 years.…”

Section: Quality Assessment Of the Included Studiesmentioning

confidence: 99%

“…Nine of the 11 included studies [10][11][12][13][14][15][16][17]19 compared the stiffness scores of the portal hypertension group with those of a control group, and we obtained the raw data for these measurements from the authors of the other 2 studies 18,20 and then evaluated the differences between the groups (Table 3). Among the 11 selected studies that evaluated liver stiffness, the representative value of the portal hypertension group ranged from 14.1 to 37.72 kPa in the 7 TE studies and 1.62 to 2.89 m/s in the 3 point SWE studies and was measured at 21.1 kPa in the real-time SWE study.…”

Section: Comparison Between the Portal Hypertension And Control Groupsmentioning

confidence: 99%

Diagnostic Performance of Ultrasound Elastography for Evaluating Portal Hypertension in Children: A Systematic Review and Meta‐analysis

Kim¹,

Yoon

Jung

et al. 2018

J of Ultrasound Medicine

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

Abstract: Liver stiffness, as determined by transient elastography, correlates with the presence and severity of CFLD. Although APRI provided some information regarding severity of liver disease, LSM performed better than APRI in this population.

Cited by 44 publications

References 28 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

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

Diagnostic Performance of Ultrasound Elastography for Evaluating Portal Hypertension in Children: A Systematic Review and Meta‐analysis

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