Original ArticleCharacterization and Outcomes of Young Infants with Acute Liver Failure
Section snippets
Methods
Data were obtained from the PALF Study Group, a multicenter, prospective study initiated in 1999 that collects data on children aged <18 years with ALF from 24 participating centers in the United States, Canada, and the United Kingdom. Entry criteria include evidence of acute liver injury combined with either severe coagulopathy (International Normalized Ratio [INR] >2.0 or prothrombin time [PT] >20 seconds) or encephalopathy with moderate coagulopathy (INR ≥1.5 or PT ≥15 seconds).
Results
Of the 841 subjects enrolled in the PALF registry, 148 were aged ≤90 days at enrollment (57.4% males; 73.0% Caucasian). The median age at enrollment was 18 days (Q1, 11 days; Q3, 37 days). The median weight at enrollment was 3.5 kg (Q1, 2.8 kg; Q3, 4.2 kg), and the median weight-for-age z score was –1.3 (Q1, –2; Q3, –0.2). The smallest infant weighed 1.7 kg (weight-for-age z score, –3.10), and the largest infant weighed 6.4 kg (weight-for-age z score, 3.32). The most common presenting symptoms
Discussion
This large prospective multicenter study characterized ALF in young infants. As might be expected, the etiology of ALF differed between young infants and older pediatric patients. The most common cause of ALF in young infants was NH, a disease limited to the neonatal period. Viral infections, mainly herpes simplex, were prominent as well (16%). A variety of metabolic diseases, dominated by galactosemia, also caused ALF. In this series, respiratory chain defects were more common than
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2022, Critical Care ClinicsCitation Excerpt :Treatment is through protein restriction and nitisinone supplementation, which prevents formation of 4-maleylacetoacetic acid and fumarylacetoacetic acid, precursors to succinylacetone.30 Galactosemia accounts for 1% of all cases of PALF and up to 8% of cases in infants.31 Galactosemia results from absent activity of galactose-1-phosphate uridyltransferase (GALT), galactokinase, or uridine diphosphate galactose epimerase enzymes.32
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2021, Digestive and Liver DiseaseCitation Excerpt :Conversely, search for viruses rarely associated with ALF (eg. rubella, paramyxovirus, Dengue, yellow fever) should be evaluated case by case, based on clinical presentation and epidemiological setting. HSV induced ALF is the most common viral etiology in neonates, and therefore should always be sought at this age [22–23]. While a positive nucleic acid testing for a given virus may be important to guide the diagnosis, it may not always represent the cause of ALF, that should be confirmed also by other clinical means.
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PALF Study Group member institutions are supported by the National Institutes of Health (Grants U01-DK 072146-04, ULI RR025014-01, UL1 RR024131, and UL1 RR024153) and General Clinical Research Centers (Grants M01 RR 08084, to the University of Cincinnati, and MO1 RR00069, to the University of Colorado). The authors declare no conflicts of interest.
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List of members of the Pediatric Acute Liver Failure Study Group is available at www.jpeds.com (Appendix).