Well Preserved Renal Function in Children With Untreated Chronic Liver Disease

Area of Science:

• Pediatric Nephrology
• Hepatology
• Clinical Investigation

Background:

• Chronic liver disease (CLD) is known to affect renal function, particularly in adults with hepatorenal syndrome.
• Understanding renal function in pediatric CLD is crucial for early detection and management.

Purpose of the Study:

• To investigate renal function, including glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), in children diagnosed with CLD.
• To analyze renal function across different subgroups of pediatric liver diseases and disease severity.

Main Methods:

• Retrospective analysis of 277 children with CLD.
• Renal function assessed via clearance of inulin and para-amino hippuric acid or iohexol for GFR and ERPF.
• Data stratified by liver disease type and damage grade.

Main Results:

• Hyperfiltration observed in progressive familial intrahepatic cholestasis (44%), glycogenosis (75%), and acute fulminant liver failure (60%).
• Normal renal function in biliary atresia, metabolic diseases, intrahepatic cholestasis, vascular anomalies, and cryptogenic cirrhosis.
• Decreased renal function noted in Alagille's syndrome (64%).
• Elevated GFR and ERPF correlated with high transaminases, low prothrombin, high bile acids, and high AST/platelet ratio.

Conclusions:

• Most children with CLD maintain well-preserved renal function, with some showing hyperfiltration.
• Stable kidney function in decompensated liver disease suggests a lack of early hepatorenal syndrome indicators.
• Serum creatinine-based GFR estimation may miss hyperfiltration in pediatric CLD.