Liver Ultrasound Patterns in Children With Cystic Fibrosis Correlate With Noninvasive Tests of Liver Disease

Conclusions: Liver US patterns in children with CF correlate with platelet count, spleen size and indices of liver fibrosis. Multivariable models of these biomarkers have excellent discriminating ability for NL versus NOD, and good ability to distinguish other US patterns, suggesting that US patterns correlate with clinically relevant liver disease.
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research

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Learning point for cliniciansThis case illustrates the limitations of using non-invasive criteria for the diagnosis of hepatocellular carcinoma. The criteria may not be valid in rarer cause of cirrhosis such as cystic fibrosis. If the diagnosis precludes life-saving treatment such as liver transplantation a confirmatory liver biopsy may be wise.
Source: QJM - Category: Internal Medicine Source Type: research
Liver disease develops in 15% –72% of patients with cystic fibrosis, and 5%–10% develop cirrhosis or portal hypertension, usually during childhood. Transient elastography (TE) is a noninvasive method to measure liver stiffness. We aimed to validate its accuracy in detection of liver disease and assessment of fibrosis in chil dren with cystic fibrosis.
Source: Clinical Gastroenterology and Hepatology - Category: Gastroenterology Authors: Source Type: research
CONCLUSIONS: NC were able to identify 17.7% more CFLD patients compared to DC. The multiple biomarkers incorporated in NC may enhance the ability to detect CFLD. PMID: 30600723 [PubMed - as supplied by publisher]
Source: Scandinavian Journal of Gastroenterology - Category: Gastroenterology Tags: Scand J Gastroenterol Source Type: research
Up to 10% of patients with Cystic Fibrosis develop cirrhotic CF-related liver disease with portal hypertension: CF cirrhosis (CFC). In a nationwide study, we aimed to determine the role of CFC on survival in the Netherlands between 1 and 1-2009 and1-1-2015.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
(Children's Hospital Colorado) Children's Hospital Colorado (Children's Colorado) pediatric gastroenterologist, Michael Narkewicz, MD, recently shared results of the Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in Cystic Fibrosis (PUSH), which sought to determine if liver ultrasounds could identify children and adolescents with cystic fibrosis who are at greater risk of developing advanced liver disease.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news
Source: Clinical Gastroenterology and Hepatology - Category: Gastroenterology Authors: Source Type: research
Hepatobiliary complications commonly occur in cystic fibrosis (CF) with increasing prevalence due to longer life expectancies and widespread screening efforts. CF-related liver disease (CFLD) is a term used to describe a spectrum of hepatic diseases in CF including cirrhosis and portal hypertension [1]. Although regular screening by physical examination and liver enzyme level analysis (alanine transaminase (ALT) and aspartate transaminase (AST)) is widely performed, it is unclear whether these tools can predict patients who will develop CFLD or those who will progress to cirrhotic disease.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Letter to the Editor Source Type: research
Hepatobiliary complications commonly occur in cystic fibrosis (CF) with increasing prevalence due to longer life expectancies and widespread screening efforts. CF-related liver disease (CFLD) is a term used to describe a spectrum of hepatic diseases in CF including cirrhosis and portal hypertension [1]. Although regular screening by physical examination and liver enzyme level analysis (alanine transaminase (ALT) and aspartate transaminase (AST)) is widely performed, it is unclear whether these tools can predict patients who will develop CFLD or those who will progress to cirrhotic disease.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Letter to the Editor Source Type: research
Liver disease in cystic fibrosis has many causes, with biliary fibrosis due to abnormal CFTR protein predominating. Assessment requires aetiology to be defined. Biliary fibrosis may progress to cirrhosis and portal hypertension, which although initially asymptomatic, may cause varices and splenomegaly. Monitoring progression includes clinical and ultrasound assessment with endoscopic assessment of varices for those at risk. Extrapolated primarily from longitudinal assessment of viral hepatitis in adults, non-invasive elastography has a potential role.
Source: Paediatric Respiratory Reviews - Category: Respiratory Medicine Authors: Tags: The 2017 Royal Society Of Medicine Cystic Fibrosis Symposium Source Type: research
Purpose of review The aim of this study was to provide an overview of the current understanding of the pathophysiology, diagnosis and management of cystic fibrosis-liver disease (CFLD). Recent findings CFLD has a variety of manifestations. Previously, it was thought that patients progressed from mild cholestatic disease to cirrhosis to decompensated cirrhosis with portal hypertension. Newer evidence suggests that some patients may develop cirrhosis while others develop noncirrhotic portal hypertension. Advances in our understanding of the pathophysiology of disease necessitate modifications to the current diagnostic c...
Source: Current Opinion in Gastroenterology - Category: Gastroenterology Tags: LIVER: Edited by Don C. Rockey Source Type: research
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