Children with chronic liver disease (CLD) could maintain renal function for a long period of time, according to a new study published in Acta Paediatrica.

“Unlike in adults, pediatric CLD has less impact on renal function and in our long-term follow-up the kidney function remained stable, as long as the liver disease was compensated,” the authors said.

They used glomerular filtration rate (GFR) to investigate renal function. Patients with initial hyperfiltration, a condition that has been suggested as a prognostic factor for declining renal function, had the greatest decrease in GFR during the observation period. Despite the decrease observed, it rarely reached pathological levels.

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Moreover, patients with noncholestatic liver disease showed more alterations in GFR than those with cholestasis, suggesting that cholestasis could have a nephroprotective effect.

The results also showed that patients with Alagille syndrome (ALGS, n=23) had lower GFRs than control individuals or patients with primary sclerosing cholangitis (PSC, n=21) or progressive familial intrahepatic cholestasis (PFIC, n=18).

“Unlike the rest of the cholestatic patients in this study, the children with ALGS had a relatively low initial GFR followed by a further decreasing tendency, implying that the virtual protective role of chronic cholestasis could not counterbalance the disease-specific slow progress of deteriorating kidney function,” the authors wrote.

Additionally, patients with alpha-1 antitrypsin deficiency (AATD, n=10) had lower GFRs than patients with glycogen storage disease (GSD, n=20).

The study enrolled 289 children diagnosed with CLD, who were distributed across 7 diagnostic groups. The intrahepatic cholestasis group (n=67) included patients with ALGS, PFIC, PSC, and other forms of intrahepatic cholestasis of unknown etiology. The metabolic liver disease group (n=48) comprised patients with GSD, AATD with liver damage, and single cases of different metabolic disorders.


Berg UB, Häbel H, Németh A. Preserved renal function during long‐term follow‐up in children with chronic liver disease. Acta Paediatr. Published online February 21, 2022. doi:10.1111/apa.16306