Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease

Mueller-Abt, Peter R.; Frawley, Kieran; Greer, Ristan M.; Lewindon, Peter

doi:10.1016/j.jcf.2007.08.001

Cited by 71 publications

(47 citation statements)

References 29 publications

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“…However, our practice is to perform annual ultrasound of the abdomen in all patients with CF, as ultrasound is non-invasive, cost-effective and highly valuable for the diagnosis of hepatic steatosis, cirrhosis and complications of portal hypertension such as ascites and splenomegaly. Yet, ultrasound cannot reliably exclude early liver disease [9] and disparity between results of ultrasound and liver enzymes was observed in up to 24% of CF patients [10,11]. Several studies have demonstrated that clinical evaluation, serum liver enzymes and ultrasound are imprecise for detecting the presence and predicting the progression of liver disease [10,11,12,13].…”

Section: Diagnosis Of Cfldmentioning

confidence: 99%

“…Thus, for patients in whom diagnosis is still inconclusive after standard work-up (see above) including exclusion of other causes of liver disease (viral, autoimmune, metabolic, genetic) percutaneous liver biopsy representing the diagnostic gold standard, can be performed [8]. Although liver biopsy is invasive, complication rates after liver biopsy in CF patients seem to be low even when dual pass liver biopsies are performed (no bleeding, hospital admission, prolonged pain or surgery [9,13]. However, a substantial sampling error due to typically focally distributed lesions in CFLD can complicate diagnostic algorithms.…”

Section: Diagnosis Of Cfldmentioning

confidence: 99%

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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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Section: Diagnosis Of Cfldmentioning

confidence: 99%

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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show abstract

“…However, in many studies these diagnostic criteria were modified resulting in heterogeneous data of CFLD prevalence [1]. The focal pattern of liver involvement results in disaccordance between clinical findings and histological staging and a high proportion of sampling errors [8]. Therefore, although liver biopsy may help to identify patients at risk for development of clinically significant liver disease [9] it is not a standard procedure in CF [1].…”

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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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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“…Conventional ultrasound examination with B-mode seems to be insufficient in assessing liver condition in patients with CF [22]. Research conducted by Behrens et al and Menten et al compares respectively ARFI and TE value to B-mode.…”

Section: Cf-associated Liver Diseasementioning

confidence: 99%

Ultrasound Elastography - Review of Techniques and Its Clinical Applications in Pediatrics - Part 1

Pawluś¹,

Sokołowska-Dąbek²,

Szymańska

et al. 2015

Adv Clin Exp Med

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Sonoelastography is a novel technique that uses ultrasound waves to assess the elasticity of tissues noninvasively. It provides an ultrasound-based method to detect and display the relative stiffness of tissue. The main principle of sonoelastography is the measurement of tissue distortion in response to external compression. Changes in elasticity and tissue deformation elicited by compression are measured, processed and then shown in real time presentation with color-coded elastograms. One of the most important applications of sonoelastography is the evaluation of liver diseases, mainly liver fibrosis assessment and staging. Although in terms of definite diagnosis the liver biopsy still remains the golden standard, elastography seems to be a very inexpensive, repeatable and noninvasive method to evaluate most of liver conditions. The technique is also applicable in detection and differential diagnosis of focal lesions. It provides better imaging information and therefore more accurate evaluation of the lesions nature, e.g. in liver, lymphatic nodes or thyroid gland. Most of the applications mentioned above are well known and have been described in details in adults. Similarly, most of sonoelastographic studies are based on groups of adults. The purpose of this review article is to bring this technology closer to pediatric clinicians and to summarize some of its current clinical applications that are being pursued. In this part we focus on utility of elastography in liver assessment in pediatric patients (Adv Clin Exp Med 2015, 24, 3, 537-543).

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Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease

Cited by 71 publications

References 29 publications

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

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

Ultrasound Elastography - Review of Techniques and Its Clinical Applications in Pediatrics - Part 1

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