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Acute Liver Failure

Outcome and Value of Pediatric End-Stage Liver Disease Score in Pediatric Cases

Núñez-Ramos, Raquel MD*; Montoro, Soledad MD*; Bellusci, Marcello MD; del Fresno-Valencia, María Rosa MD*; Germán-Díaz, Marta MD*; Urruzuno, Pedro MD*; Medina, Enrique MD*; Manzanares, Javier MD*

doi: 10.1097/PEC.0000000000000884
Original Articles
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Purpose The aims of this study were to analyze the characteristics of patients with acute liver failure (ALF) in our center and evaluate the prognostic value of the Pediatric End-Stage Liver Disease (PELD) score calculated at admission.

Methods A retrospective analysis of patients with ALF younger than 15 years between 2005 and 2013 was performed. Information collected included age, sex, etiology of ALF, laboratory tests, PELD score, stage of encephalopathy, and need for liver support devices such as MARS and/or liver transplant (LT) and survival. A poor prognosis was defined as the need for LT or death.

Results Twenty patients (10 male patients, 50%) with a median age of 2.6 years (3 days-14.5 y old) were included. Acute liver failure was of indeterminate cause in 5 cases (25%). Within the recognized causes, the most frequent were viral hepatitis (herpes simplex virus, adenovirus, influenza B, Epstein-Barr virus), autoimmune hepatitis, and metabolopathies. Sixty percent presented with encephalopathy at diagnosis. Four patients were aided by a MARS liver support device. Six patients received a total of 7 transplants, all from deceased donors. The rate of spontaneous recovery was 45%. Currently 13 patients (65%) are living, 4 of them with an LT. Six patients died because of ALF. The mean PELD score of patients with spontaneous recovery was 15.31 (5.3–27.6) compared with a mean of 29.5 (17.2–39.4) in LT patients and 31.55 (15.8–52.4) for nonsurvivors (P = 0.013).

Conclusions High PELD scores at diagnosis were accurate predictors of a poor prognosis in our patients with ALF. This model may help in the clinical management of this entity, although prospective validation is needed.

From the Departments of *Pediatric Gastroenterology and Hepatology and

Pediatrics, University Hospital 12 de Octubre, Madrid, Spain.

Disclosure: The authors declare no conflict of interest.

Reprints: Raquel Núñez-Ramos, Department of Pediatric Gastroenterology and Hepatology, University Hospital 12 de Octubre, Av de Córdoba s/n, 28041 Madrid, Spain (e-mail: nunezramos.raquel@gmail.com).

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