Comparison of Transient Elastography, ShearWave Elastography, Magnetic Resonance Elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with Cystic Fibrosis

Author(s):  
Jérémy Dana ◽  
Muriel Girard ◽  
Stéphanie Franchi-Abella ◽  
Laureline Berteloot ◽  
Martina Benoit-Cherifi ◽  
...  
Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Masaya Sugimoto ◽  
Hideharu Oka ◽  
Aya Kajihama ◽  
Kouichi Nakau ◽  
Hiroshi Azuma

Background: The incidence of late complications related to the liver such as fibrosis/cirrhosis is increasing in patients who have undergone the Fontan procedure and may contribute to morbidity and mortality. Recently, magnetic resonance elastography (MRE), a novel evaluation technique of liver fibrosis, has been attracting attention. However, few reports have described the use of MRE for evaluating liver fibrosis in children with congenital heart disease (CHD). Methods: Thirty-two children were examined and divided into 4 groups: 12 children with CHD who underwent intracardiac repair (ICR; median age, 13.0 years); 10 with CHD who underwent the Fontan procedure (Fontan; 15.3 years); 8 who were included in the control group (control; 15.8 years); and 2 children with cirrhosis (cirrhosis; 16.3 years). The liver stiffness (LS) was estimated by MRE. LS was measured 3 times consecutively, and the mean value was considered for further analysis. Central venous pressure (CVP) and cardiac index (C.I.) were determined by cardiac catheterization. The levels of cardiac biomarkers (NTproBNP and PIIIP) were determined at the same time. Results: Among the 4 groups, no significant differences were observed in age, C.I., and NTproBNP levels. The PIIIP levels in the cirrhosis group were significantly higher than those in the control, but no significant difference in PIIIP levels was found among the other groups (p < 0.01). LS in the Fontan and cirrhosis group was significantly higher than that in the control group (5.6, 15.3 vs. 2.4 kPa, respectively; Fig. 1). Furthermore, there was a strong correlation between LS and CVP (r = 0.802; Fig. 2). Conclusions: This study showed that LS is a direct function of CVP, which should be considered when assessing the degree of liver fibrosis in children with CHD. In particular, in the case of children who undergo the Fontan procedure, the highly sensitive MRE can be used to evaluate liver fibrosis and help detect LS earlier than cardiac biomarkers do.


Author(s):  
Eirini Kyrana ◽  
Anil Dhawan

The chapter on cystic fibrosis-associated liver disease discusses the pathophysiology and clinical features of this condition. In addition it covers diagnosis, including liver biopsy, transient elastography, the role of endoscopy and magnetic resonance retrograde cholangiopancreatography, and then discusses management including the role of transplantation.


2009 ◽  
Vol 54 (22) ◽  
pp. 6979-6990 ◽  
Author(s):  
Jennifer Oudry ◽  
Jonathan Vappou ◽  
Philippe Choquet ◽  
Rémy Willinger ◽  
Laurent Sandrin ◽  
...  

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