Chronic cholestatic liver disorders are frequently associated with abnormal lipid metabolism, which may potentially raise cardiovascular (CV) risk, however data is limited. The study’s goal was to identify risk factors for cardiovascular disease (CVD) in children with Alagille syndrome (AGS) and biliary atresia (BA). Researchers studied 17 patients with AGS, ages 11.0 years, and 19 patients with BA, ages 13.5 years, and performed extensive biochemical testing, including lipid profiles and oxidative stress biomarkers, blood pressure (BP)—systolic, diastolic, and mean, carotid intima-media thickness (cIMT), and pulse wave velocity (PWV). Lipid profiles were abnormal in 82 percent of children with AGS and 52.6 percent of children with BA. Researchers found substantially greater levels of TC, LDL C, APO B, lower glutathione concentration and glutathione peroxidase activity, lower blood pressure, lower cIMT, cIMT-SDS, and PWV in the AGS group. However, they found high blood pressure in 2/19 BA patients and none with AGS, whereas cIMT-SDS was elevated in only 2/17 AGS patients and 6/19 BA patients, and aberrant PWV-SDS values were found in 3/17 AGS patients and 8/19 BA patients. Neither dyslipidemia nor Lp-X were found to be associated with vascular parameters.

Although children with BA and AGS may have elevated cardiovascular risk factors, vascular measures are not directly connected to lipid abnormalities. In these cholestatic diseases, cIMT and BP should be evaluated for clinical practice in order to identify patients at risk of CVD.