Utility of transient elastography in the non‐invasive evaluation of cystic fibrosis liver disease

Kitson, Matthew T.; Kemp, William; Iser, David; Paul, Eldho; Wilson, John W.; Roberts, Stuart K.

doi:10.1111/liv.12113

Cited by 51 publications

(61 citation statements)

References 32 publications

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“…Recent studies have tested the diagnostic accuracy of transient elastography, proteomic profiling, and a panel of circulating microRNAs 20-22 . The role of serum markers of fibrogenesis has also been studied 23 .…”

Section: Discussionmentioning

confidence: 99%

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

Stonebraker

Ooi

Pace

et al. 2016

Clinical Gastroenterology and Hepatology

107

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Background & Aims Liver disease is the third leading cause of death in patients with cystic fibrosis (CF), but features of patients with CF, severe liver disease, and portal hypertension have not been fully characterized. Methods We performed a retrospective analysis of data from 561 patients with CF (63% male, 99% with pancreatic insufficiency), liver disease (hepatic parenchymal abnormalities consistent with cirrhosis, confirmed by imaging), and portal hypertension (esophageal varices, portosystemic collaterals, or splenomegaly), with no alternate causes of liver disease. All patients were enrolled in the Genetic Modifier Study of Severe CF Liver Disease at 76 international centers, from January 1999 through July 2013. Results Male patients were diagnosed with liver disease at a younger age than female patients (10 vs 11 years; P=.01). Splenomegaly was observed in 99% of patients and varices in 71%. Levels of liver enzymes were near normal in most patients. Thrombocytopenia affected 70% of patients and was more severe in patients with varices (88×109/L vs 145×109/L; P<.0001). Ninety-one patients received liver transplants (16%), at a median age of 13.9 years. Compared to patients who did not receive liver transplants, patients who received liver transplants had lower platelet counts (78×109/L vs 113×100/L; P<.0001), higher international normalized ratios (P<.0001), and lower levels of albumin (P=.0002). The aminotransferase to platelet ratio index (APRi) and fibrosis index based on 4 factor (FIB-4) values were above diagnostic thresholds for CF liver disease in 96% and 90% of patients, respectively. Patients who received liver transplants or who had varices had higher APRi and FIB-4 values than patients who did not. Conclusions In patients with CF, severe liver disease develops early in childhood (around 10 years of age) and is more common in boys than girls. Patients with varices and those who receive liver transplants have more abnormal platelet counts and APRi and FIB-4 scores.

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Section: Discussionmentioning

confidence: 99%

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

Stonebraker

Ooi

Pace

et al. 2016

Clinical Gastroenterology and Hepatology

107

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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%

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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“…Its diagnostic performance is better for cirrhosis than for significant fibrosis, with mean area under the receiver operating characteristic curve (AUROC) values of 0.94 and 0.84, respectively (8). TE has also been found as a reliable method for assessment of chronic liver disease of non-viral etiology; such as alcoholic liver disease (9), nonalcoholic fatty liver disease (NAFLD) (10) and cystic fibrosis (11). A variety of direct serum markers of fibrosis, reflecting either the deposition or the removal of extracellular matrix in the liver, have been evaluated for their ability to assess liver fibrosis.…”

Section: Introductionmentioning

confidence: 99%

Egy-Score Predicts Severe Hepatic Fibrosis and Cirrhosis in Egyptians With Chronic Liver Diseases: A Pilot Study

et al. 2013

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BackgroundNon-invasive methods for assessment of hepatic fibrosis are increasingly needed. Recent studies showed that combined elevation of tumor markers CA 19-9 and CA 125 is predictive of severe hepatic fibrosis or cirrhosis with high specificity.ObjectivesWe aimed at developing a new panel of surrogate biomarkers for prediction of the stage of hepatic fibrosis by combining tumor markers with other known biomarkers of hepatic fibrosis.Patients and MethodsA total of 92 patients with different types of chronic liver diseases (chronic hepatitis B, chronic hepatitis C and autoimmune hepatitis), were prospectively enrolled in our cohort. They were subjected to: ALT, AST, GGT, ALP, total bilirubin, INR, total cholesterol, albumin, platelet count, cancer antigen 19-9 (CA 19-9), cancer antigen 125 (CA 125), cancer antigen 15-3 (CA 15-3), haptoglobin, alpha-2-macroglobulin, apolipoprotein A1, abdominal ultrasound, liver biopsy and histological staging of hepatic fibrosis using the METAVIR system.ResultsCombined elevation of CA 19-9 and CA 125 with a summated value > 37 U/mL is predictive of severe hepatic fibrosis or cirrhosis (stage F3-F4 METAVIR) with a probability of 77.6%. Multivariate analysis showed that the most relevant collection of biomarkers for prediction of stage of hepatic fibrosis is: CA 19-9, age, alpha-2- macroglobulin, total bilirubin, platelet count & albumin. We developed a new score, named the “Egy-Score”, using a regression equation composed of this panel of biomarkers. Egy-Score could differentiate no or early fibrosis (stage F0-F2 METAVIR) from severe fibrosis or cirrhosis (stage F3-F4 METAVIR) with 83.7% accuracy.ConclusionsNon-invasive assessment of hepatic fibrosis could be done using the Egy-Score. Egy-Score could differentiate no or early fibrosis (stage F0-F2 METAVIR) from severe fibrosis or cirrhosis (stage F3 - F4 METAVIR) with 83.7% accuracy.

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Utility of transient elastography in the non‐invasive evaluation of cystic fibrosis liver disease

Abstract: LSM is an accurate and reliable non-invasive tool in assessing CFLD and PHT. An LSM ≥ 6.8 kPa is highly suggestive of CFLD and an LSM <8.9 kPa reliably excludes PHT.

Cited by 51 publications

References 32 publications

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

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

Egy-Score Predicts Severe Hepatic Fibrosis and Cirrhosis in Egyptians With Chronic Liver Diseases: A Pilot Study

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