Abstract

Objectives: European consensus guidelines recommend annual screening for cystic fibrosis-related liver disease (CFLD) with annual clinical, biochemical and ultrasound (USS) evaluation. Abdominal USS is initiated from age 5 in many centres across the UK;however, it has been our practice to initiate from age 2. In this study we aim to determine the incidence of liver abnormalities in young children as recent reduced access to radiological services due to the COVID-19 pandemic has prompted us to re-evaluate our practice. Methods: We retrospectively evaluated abdominal USS reports of consecutive children aged 2–5 years with confirmed diagnosis of CF attending the NI regional CF centre between 2012 and 2020, along with relevant demographic and clinical data and liver function tests. All data was retrieved from the Northern Ireland Electronic Care record (NIECR). Results: Seventy 2–5-year-olds underwent a total of 199 scans between 2012 and 2020. In 2-year-olds, 43% of scans detected an abnormality, increasing to 70% in 5-year-olds, RR 1.61, 95% CI 1.03, 2.65, p = 0.03. 107 (53.4%) scans had one or more abnormality reported. Increased echogenicity of liver and/or pancreas was the most common abnormality identified in 72 of 107 scans (67.2%);hepatomegaly in 46 (42.9%);coarse heterogeneous echotexture in 24 (22.4%);increased periportal echogenicity in 6 (5.6%) and other incidental findings in 10 (9.3%) of scans, including cholelithiasis, splenic cyst, renal scarring, renal stones, and micro gallbladder. Conclusion: Abnormalities are present in over half of abdominal ultrasound scans in 2–5-year-olds. Increased echogenicity, liver enlargement and coarsened echotexture are indicative of hepatic steatosis, which has unclear significance in this age group. Longitudinal studies are needed to determine the role of early USS in detection of liver disease, and whether any particular abnormalities may identify those at risk of development of severe CFLD.

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