Reply: The utility of noninvasive imaging in cystic fibrosis liver disease
Abstract
We welcome the report by Manco et al. highlighting the importance of detecting and monitoring the severity of fibrosis and the need for reliable, noninvasive assessments of hepatic fibrosis in patients with cystic fibrosis liver disease (CFLD). In a study of an uncharacterized cohort of 40 children with cystic fibrosis (CF), they compared acoustic radiation force impulse (ARFI) imaging values from 12 children with evidence of portal hypertension to values from 28 children without portal hypertension. The sensitivity and specificity of an ARFI cutoff value of 1.3 m/s, which had been validated in adults with viral hepatitis, were 0.75 and 0.79, respectively, for the detection of portal hypertension in patients with CF; however, the relative importance of each value for a child with CF remains to be determined. Noninvasive assessments such as serum marker measurements, transient elastography, and ARFI imaging have been validated for the detection of liver fibrosis in adults with liver diseases (particularly chronic viral hepatitis). Studies in pediatric populations are complicated by the physicochemical influences of children's smaller size and growth. Children also have a different spectrum of etiologies for severe liver disease, with the majority of liver diseases that lead to transplantation being cholestatic in nature (including CF); extrahepatic cholestasis has been shown to increase liver stiffness (as measured by transient elastography), which may confound values…
How strong is the evidence?
Evidence Hub reads this as a cohort study, done in people. Its evidence level is 3 of 5 (Moderate · trial / cohort / secondary analysis). Trials without a large randomized design, cohorts and secondary analyses give moderate evidence. Reported size: 40 participants or records.
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