Characterization and Outcomes of Young Infants with Acute Liver Failure

Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, The Children's Hospital, University of Colorado School of Medicine, Aurora, CO, USA.
The Journal of pediatrics (Impact Factor: 3.79). 05/2011; 159(5):813-818.e1. DOI: 10.1016/j.jpeds.2011.04.016
Source: PubMed


To characterize infants aged ≤ 90 days enrolled in an international, multicenter, prospective registry of children aged < 18 years with acute liver failure (ALF).
The Pediatric Acute Liver Failure (PALF) Study Group collects prospective data on children from birth to 18 years. We analyzed data from infants aged ≤ 90 days enrolled in the PALF Study before May 18, 2009.
A total of 148 infants were identified in the PALF registry (median age, 18 days). Common etiologies of ALF were indeterminate (38%), neonatal hemochromatosis (13.6%), and herpes simplex virus (12.8%). Spontaneous survival occurred in 60% of the infants, 16% underwent liver transplantation, and 24% died without undergoing liver trsansplantation. Infants with indeterminate ALF were more likely to undergo liver transplantation than those with viral-induced ALF (P = .0002). The cumulative incidence of death without liver transplantation was higher in infants with viral ALF (64%) compared with those with neonatal hemochromatosis (16%) or indeterminate ALF (14%) (P = .0007).
ALF in young infants presents unique diagnostic considerations. Spontaneous survival is better than previously thought. Liver transplantation provides an additional option for care.

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Available from: Robert H Squires, Oct 09, 2015
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    • "The mortality rate of infants with ALF is up to 58% (Sundaram et al. 2011). The progression of HE in the setting of ALF due to HSV infection is erratic but may evolve to seizures and refractory ICP (Verma et al. 2006). "
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    ABSTRACT: Neurologic complications of pediatric acute liver failure (ALF) are a major determinant of outcome. Management of these complications, including increased intracranial pressure (ICP) is largely supportive. Although hypothermia is an effective treatment for perinatal asphyxia and is used to reduce ICP following traumatic brain injury, it has not been evaluated for neurologic complications of ALF in the newborn. Case report. We present a case of neonatal herpes simplex virus (HSV)-associated ALF with profound neurologic impairment and increased ICP. The patient was treated with selective head cooling, and monitored with transcranial doppler (TCD) studies of cerebral blood flow velocity, and electroencephalograms (EEG). The duration of head cooling was influenced by absent diastolic flow on TCDs, which subsequently improved during hypothermia. Continuous EEGs captured subclinical seizures, which improved with antiepileptic medications. Her death was attributed to a massive pulmonary hemorrhage and a hypoxemic cardiac arrest secondary to significant coagulopathy. This case demonstrates that selective head cooling may attenuate increased ICP in neonatal encephalopathy, and that TCDs may guide management in the absence of invasive monitoring.
    SpringerPlus 10/2013; 2(1):572. DOI:10.1186/2193-1801-2-572
  • Nature Reviews Gastroenterology &#38 Hepatology 11/2011; 8(11):606-7. DOI:10.1038/nrgastro.2011.190 · 12.61 Impact Factor
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    ABSTRACT: Although the etiologies of pediatric acute liver failure (ALF) are diverse, ultimate pathophysiologic pathways and management challenges for these disorders, usually lethal in the pre-transplant era, are similar. This review considers particularly the mechanisms of, and monitoring for, intracranial hypertension and coagulopathy; summarizes detailed advice for management of the ALF-associated failures of multiple body systems; and reviews the variety of prognostic scores available to guide management and assist in choosing the patients most apt to benefit from liver transplantation and the optimal timing for such transplantation.
    Current Gastroenterology Reports 04/2012; 14(3):262-9. DOI:10.1007/s11894-012-0260-x
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