Terlipressin in paediatric hepatorenal syndrome-acute kidney injury (HRS-AKI)

Children with chronic liver disease are at increased risk of acute kidney injury (AKI), which could be non-hepatorenal syndrome AKI (non-HRS-AKI) or hepatorenal syndrome AKI (HRS-AKI). Approximately 5-10% of these children develop HRS-AKI. In this cohort, portal hypertension leads to splanchnic vasodilatation, reduced mean arterial pressure, and compensatory renal arteriolar vasoconstriction. The reduction of mean arterial pressure and renal vasoconstriction reduces renal perfusion, causing functional renal failure and AKI (acute kidney injury). HRS-AKI may be precipitated by sepsis or inflammation, which may impact cardiac function in patients with cirrhotic cardiomyopathy. While liver transplantation remains curative, adult randomised controlled trials have demonstrated that treatment with terlipressin can potentially reverse HRS-AKI. Limited paediatric studies also suggest terlipressin may also be effective in children, though effects on mortality remain unexplored. In this review, we explore the prevalence and pathophysiology of HRS-AKI in paediatric liver disease, summarise evidence from adult trials supporting terlipressin use, highlight cautions and contraindications associated with its use in adults and children, and propose principles for its safe prescription. Additionally, we outline future research priorities, including the potential role of renal biomarkers in managing this high-risk population.