Intensive Care Management of Pediatric Acute Liver Failure

Lutfi, Riad; Abulebda, Kamal; Nitu, Mara; Molleston, Jean P.; Bozic, Molly; Subbarao, Girish

doi:10.1097/mpg.0000000000001441

Cited by 62 publications

(96 citation statements)

References 141 publications

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“…Although neurological signs were absent, a muscle biopsy, performed 6 months after transplantation, showed normal respiratory chain enzyme activities. Genetic testing for deoxyguanosine kinase (DGUOK), DNA polymerase γ (POLG) mutations and other genes commonly involved in mitochondrial DNA depletion [ 8 – 11 ], showed no pathogenic mutations [ 7 , 12 , 13 ].…”

Section: Case Presentationmentioning

confidence: 99%

NBAS mutations cause acute liver failure: when acetaminophen is not a culprit

et al. 2017

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BackgroundPediatric acute-liver-failure due to acetaminophen (APAP) administration at therapeutic dosage is rare, while viral infections and metabolic defects are the prevalent causes. Yet, as acetaminophen is routinely used in febrile illnesses, it may be mistakenly held responsible for the acute liver damage.Case presentationAn 11 month old boy had been on acetaminophen for 10 days (total dose 720 mg = 72 mg/kg) when he developed acute-liver-failure with encephalopathy. As he rapidly improved on N-acetylcysteine (NAC) infusion, it was concluded that chronic acetaminophen administration in an infant had lead to acute-liver-failure even at therapeutic doses, that N-acetylcysteine infusion had been life-saving and should be immediately started in similar circumstances. The child, however, had two further episodes of acute liver damage over a 34-month period, without having been given acetaminophen, as the parents carefully avoided using it. His clinical, laboratory and radiological findings between the acute episodes were unremarkable. His features and skeletal surveys were not suggestive of a syndromic condition. He then went on to suffer another episode of acute-liver-failure with multi-organ failure, necessitating an urgent liver transplant. All efforts to come to a diagnosis for the causes of his recurrent episodes of liver failure had been unsuccessful, until a biallelic mutation in the NBAS gene was reported to be associated with recurrent acute-liver-failure in children. The boy’s DNA analysis revealed compound heterozygous pathogenic mutations in the NBAS gene. Liver failure episodes in these patients are triggered and worsened by fever, most likely due to thermal susceptibility of hepatocytes, hence APAP, rather than being a culprit, is part of the supportive treatment.ConclusionsWe suggest that, in acute-liver-failure with a history of acetaminophen exposure at therapeutic dosage, clinicians should not be contented with administering NAC, but should consider an alternative etiology, above all if the episodes are recurrent, and actively start supportive and antipyretic treatment while seeking the advice of a specialist unit.

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Section: Case Presentationmentioning

confidence: 99%

NBAS mutations cause acute liver failure: when acetaminophen is not a culprit

et al. 2017

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“…Therapeutic modalities such as extracorporeal liver support (ELS) using albumin augmented clear- ance deserve consideration and assessment for safety and efficacy, as they may provide a bridge to transplant or recovery [4,5,7]. Clinically significant bleeding is seen in less than 5% of LF patients due to synthetic loss of both pro and anticoagulation factors [2]. Patients with acute chronic LF have higher rates of bleeding due to portal hypertension, hypersplenism and thrombocytopenia.…”

Section: Introductionmentioning

confidence: 99%

“…Patients frequently require extracorporeal modalities as part of their supportive care [1][2][3][4][5]. Due to limited treatment and transplant options, survival remains around 50% [1,2,6]. Therapeutic modalities such as extracorporeal liver support (ELS) using albumin augmented clear- ance deserve consideration and assessment for safety and efficacy, as they may provide a bridge to transplant or recovery [4,5,7].…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…Pediatric acute and acute-on-chronic liver failure (LF) is a poorly understood disease process that can lead to multiorgan failure with high morbidity and mortality [1,2]. Patients frequently require extracorporeal modalities as part of their supportive care [1][2][3][4][5].…”

Section: Introductionmentioning

confidence: 99%

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The Safety and Efficacy of Regional Citrate Anticoagulation in Albumin-Assisted Liver Dialysis for Extracorporeal Liver Support in Pediatric Patients

Lion¹,

Pekkucuksen²,

Srivaths³

et al. 2018

Blood Purif

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Aims: To establish the safety and efficacy of regional citrate anticoagulation (RCA) for pediatric liver failure (LF) patients receiving extracorporeal liver support (ELS) with albumin-assisted dialysis. Methods: Retrospective review of pediatric LF patients receiving ELS from April 2014 to December 2016 at a tertiary children’s hospital pediatric intensive care unit. Demographic and ELS data collected by chart review. Citrate accumulation (CA) was defined as total calcium (mmol/L): ionized calcium (mmol/L) > 2.5 (tCa:iCa). Efficacy was assessed by treatment duration. Safety was assessed by adverse events: bleeding, hemodynamic instability, arrhythmias, unplanned treatment discontinuation. Results: Fifteen patients (median age 3 [interquartile range (IQR) 0.7–8.0]) received 108 ELS treatments (median 5 [IQR 4–7.5]). Sixty-eight episodes of CA were identified. Of those, 6 coincided with intervention and 1 coincided with ELS discontinuation. There were no deaths attributed to ELS or RCA. Conclusion: RCA provides safe and effective anticoagulation for pediatric LF patients requiring ELS.

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Liver Transplant Listing in Pediatric Acute Liver Failure: Practices and Participant Characteristics

et al. 2018

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Liver transplant (LT) decisions in pediatric acute liver failure (PALF) are complex. Three phases of the PALF registry, containing data on 1,144 participants over 15 years, were interrogated to characterize clinical features associated with listing status. A decrease in the cumulative incidence of listing (P < 0.005) and receiving (P < 0.05) LT occurred without an increase in the cumulative incidence of death (P = 0.67). Time to listing was constant and early (1 day; quartiles 1-3 = 0-2; P = 0.88). The most frequent reasons for not listing were "not sick enough" and "medically unsuitable." Participants listed for LT were more likely male, with coma grade scores >0; had higher international normalized ratio, bilirubin, lactate, and venous ammonia; and had lower peripheral lymphocytes and transaminase levels compared to those deemed "not sick enough." Participants listed versus those deemed "medically unsuitable" were older; had higher serum aminotransferase levels, bilirubin, platelets, and albumin; and had lower lactate, venous ammonia, and lymphocyte count. An indeterminate diagnosis was more prevalent in listed participants. Ventilator (23.8%) and vasopressor (9.2%) support occurred in a significant portion of listed participants but less frequently than in those who were not "medically suitable." Removal from the LT list was a rare event. Conclusion: The cumulative incidence of listing for and receiving LT decreased throughout the PALF study without an increase in the cumulative incidence of death. While all participants fulfilled entry criteria for PALF, significant differences were noted between participants listed for LT and those deemed "not sick enough" as well as those who were "medically unsuitable." Having an indeterminate diagnosis and a requirement for cardiopulmonary support appeared to influence decisions toward listing; optimizing listing decisions in PALF may reduce the frequency of LT without increasing the frequency of death.

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Intensive Care Management of Pediatric Acute Liver Failure

Cited by 62 publications

References 141 publications

NBAS mutations cause acute liver failure: when acetaminophen is not a culprit

NBAS mutations cause acute liver failure: when acetaminophen is not a culprit

The Safety and Efficacy of Regional Citrate Anticoagulation in Albumin-Assisted Liver Dialysis for Extracorporeal Liver Support in Pediatric Patients

Liver Transplant Listing in Pediatric Acute Liver Failure: Practices and Participant Characteristics

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