Prognostic modeling in pediatric acute liver failure

Abstract

Liver transplantation (LT) is the only proven treatment for pediatric acute liver failure (PALF). However, over a period of time, spontaneous native liver survival is increasingly reported, making us wonder if we are overtransplanting children with acute liver failure (ALF). An effective prognostic model for PALF would help direct appropriate organ allocation. Only patients who would die would undergo LT, and those who would spontaneously recover would avoid unnecessary LT. Deriving and validating such a model for PALF, however, encompasses numerous challenges. In particular, the heterogeneity of age and etiology in PALF, as well as a lack of understanding of the natural history of the disease, contributed by the availability of LT has led to difficulties in prognostic model development. Several prognostic laboratory variables have been identified, and the incorporation of these variables into scoring systems has been attempted. A reliable targeted prognostic model for ALF in Wilson's disease has been established and externally validated. The roles of physiological, immunological, and metabolomic parameters in prognosis are being investigated. This review discusses the challenges with prognostic modeling in PALF and describes predictive methods that are currently available and in development for the future. Liver Transplantation 22 1418-1430 2016 AASLD.

Changing Etiologies and Prognostic Factors in Pediatric Acute Liver Failure

“…We can speculate that these findings might be explained by 2 reasons: HAV‐associated PALF infection has a hyperacute presentation with an associated mortality and need of LT of approximately 40% and 45%, respectively There have been some relevant improvements in the medical management of children with PALF …”

“…The ability to predict the need of LT in PALF and to determine the severity of illness is an unmet need in the clinical care of these patients. Several scoring systems have been developed to aid the decision whether to transplant a patient with acute liver failure (ALF) or not . The etiology of PALF differs worldwide, depending on geographical and socioeconomic characteristics.…”

Pediatric chronic liver failure-sequential organ failure assessment score and outcome of acute liver failure in children

“…So, there is an urgent need for dynamic prognostic scoring systems for PALF with potential inclusion of physiological and inflammatory parameters. However, determining the prognosis remains challenging [ 23 ].…”

Acute liver failure related to inherited metabolic diseases in young children

“…16 Decisions on transplantation are extremely difficult, a fact that is corroborated by the lack of validated pediatric ALF prognostic models, 17 especially for IMD etiology. LT may be contraindicated in cases in which ALF may resolve with specific medical and/or diet therapy, if the underlying disease involves other organs or systems or if there is a rapid progression of ALF with multiorgan failure or sepsis.…”