Clinical use of shear-wave elastography for detecting liver fibrosis in children and adolescents with cystic fibrosis

Ultrasound elastography in children — nice to have for scientific studies or arrived in clinical routine?

“…The liver is generally the organ most frequently evaluated by elastography. In addition to some reference value studies in children [ 41 , 43 ], there are numerous clinical studies published on detecting and grading liver fibrosis as well as on diagnosis and treatment follow-up of biliary atresia in childhood ([ 16 , 32 ]; Kim JR et al [ 39 ];). Liver stiffness data can be expressed in kPa using TE or in m/s using pSWE or 2D-SWE.…”

Effects of CFTR Modulators on Serum Biomarkers of Liver Fibrosis in Children with Cystic Fibrosis

“…While it was being routinely performed prior to elexacaftor/tezacaftor/ivacaftor approval, the COVID-19 pandemic prevented many individuals from receiving a routine ultrasound prior to starting elexacaftor/tezacafttor/ivacaftor. With these caveats in mind, both cohorts likely consisted of those with mild-moderate liver involvement as assessed by biomarkers of fibrosis which correlate well with imaging findings (9). It is unclear if our findings are limited to those without advanced liver cirrhosis and/or portal hypertension, but the greatest opportunity to reverse disease likely lies prior to the development of multilobular cirrhosis.…”

“…In our liver involvement cohorts, both FEV1 and BMI percentile improved during treatment with elexacaftor/tezacaftor/ivacaftor, arguing that the lack of improvement in LFTs was not due to treatment failure or substantial medication noncompliance. Since all but one patient on lumacaftor/ivacaftor had normalization of GGT and GPR, sensitive markers of early liver fibrosis (9), this may have obscured our ability to detect any subsequent change with elexacaftor/tezacaftor/ivacaftor. The PROMISE study (Prospective Study to Evaluate Biological and Clinical Effects of Significantly Corrected CFTR Function) includes evaluation of liver function on elexacaftor/tezacaftor/ivacaftor and may provide further clarity on this question.…”

2D Shear Wave Elastography, a promising screening tool for Cystic Fibrosis liver disease, shows a correlation between vitamin D and liver stiffness

“…JID: JCF [m5G; July 3, 2022;14:35 ] Levitte et al compared 2D SWE to Magnetic Resonance (MR) elastography and found a significant correlation between these two methods. Additionally, they found that GT was a relevant biochemical marker in CFLD [20] . MR elastography is not routinely used at our CF center and the sole visual comparison was therefore with the abdominal ultrasound.…”