Presenting Features and Prognosis of Ischemic and Nonischemic Neonatal Liver Failure

Zozaya, Carlos; Caamaño, B. Fernández; Bartolo, Gema Muñoz; Suso, Juan José Menéndez; Remacha, Esteban Frauca; Núñez, Eva Valverde

doi:10.1097/mpg.0000000000001501

Cited by 11 publications

(17 citation statements)

References 21 publications

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“…The differences in the frequency of causes of liver failure between the published series are probably related to the age range of infants included and the different referral patterns. The published series that are limited to neonates (1,6) did not include tyrosinemia, a disease that usually manifests after the neonatal period, whereas the most common cause of liver failure during neonatal period was NH, a disease limited to newborn and neonates. There were fewer neonates with NH in our case series (9%) than many of the other published series (13.5%-38%) possibly because our center is not a referral center for LT. On the other hand, the frequency of galactosemia and tyrosinemia cases (29%) seen in our center was greater than the frequency in published series from liver transplant centers, which are less likely to receive these cases that respond dramatically to specific treatment.…”

Section: Discussionmentioning

confidence: 99%

Liver Failure Among Young Saudi Infants

Lone¹,

Alsaleem²,

Asery³

et al. 2020

J. pediatr. gastroenterol. nutr.

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Objectives: The published data on early infantile liver failure (EILF) are scarce and limited to Caucasians. We conducted this study to describe the etiology and outcome of EILF among Arabs and identify prognostic factors. Methods: We retrospectively reviewed our database of 524 infants presenting with liver impairment from 2008 to 2018, and identified cases of EILF defined as presence of biochemical pattern of liver disease and INR ≥2 (unresponsive to vitamin K) with onset before 3 months of life. Primary outcomes included death or liver transplantation (LT) (poor outcome group) and survival with native liver (good outcome group). Results: Forty-two cases of EILF (22 girls) were identified (8%). The etiology was indeterminate in 14 (33.3%) and established in 27 (64.3%): galactosemia (7 cases, 16.6%), tyrosinemia (5, 12%), neonatal hemochromatosis (NH), and hemophagocytic lymphohistiocytosis (HLH) (4 each, 9.5%]) mitochondrial hepatopathy (3, 7%), and miscellaneous (5, 12%). LF resolved in 15 cases (35.7%), either spontaneously or in response to specific therapy, 23 (54.7%) died, and 4 underwent LT (9.5%). ROC analysis for the best cut-off value of serum total bilirubin for prediction of study outcomes was 120 μmol/L (sensitivity 81.5%, specificity 80%). Among the diagnostic groups, galactosemia and tyrosinemia predicted good outcome, whereas the idiopathic diagnosis predicted poor outcome (OR = 13). Conclusions: Similar to Western countries, galactosemia, tyrosinemia, NH, HLH, and mitochondrial hepatopathy are the main players in EILF in Saudi Arabia. Galactosemia and tyrosinemia predict good prognosis and idiopathic diagnosis predicts poor prognosis.

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Section: Discussionmentioning

confidence: 99%

Liver Failure Among Young Saudi Infants

Lone¹,

Alsaleem²,

Asery³

et al. 2020

J. pediatr. gastroenterol. nutr.

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“…In a pedigree study of GALD, survival was 9 of 20 (45%) in spite of IVIG AE ET (38). By contrast, among NH patients who received neither ET nor IVIG treatment, survival without LTx was reported to be only 10% to 20% (5,7,(37)(38)(39).…”

Section: Clinical Course and Outcomesmentioning

confidence: 99%

Early Exchange Transfusion to Treat Neonates With Gestational Alloimmune Liver Disease

Fischer

Staufner

Sallmon

et al. 2020

J. pediatr. gastroenterol. nutr.

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Objectives: Exchange transfusion (ET) and intravenous immunoglobulin are potentially life-saving treatment options in newborns with gestational alloimmune liver disease (GALD). Since 2008, early ET has been the standard of care for symptomatic neonates with suspected GALD in our unit. The present study's aim was to investigate the outcomes of this approach. Methods: From 2008 to 2018, all neonates who received ET for suspected GALD were identified, and their clinical course and outcomes were analyzed in a descriptive cohort study. In survivors, liver function parameters before ET and maximum values after ET and at discharge were compared. Results: During the 11-year period, 12 infants received ET for suspected GALD at a median (range) chronological age of 11 (1–23) days and gestational age of 38 (32–40) weeks. Signs of impaired liver function, most frequently postnatal hypoglycemia, hyperferritinemia, direct hyperbilirubinemia, and coagulopathy, were present in all infants. Survival without a liver transplant in the overall cohort was 10 of 12 (83.3%) and 7 of 9 (78%) in those fulfilling the criteria of acute liver failure. Two patients died, one of them after liver transplantation. Direct bilirubin typically increased after ET, even in survivors. All survivors recovered and were discharged from the pediatric hepatology outpatient clinic after 8 (3–11) months of follow-up. Conclusions: In newborns with suspected GALD, a limited diagnostic work-up followed by early ET may lead to favorable outcomes. More data are required to develop an evidence-based clinical approach to GALD.

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“…Diagnostic testing for these etiologies includes the newborn screen, galactose‐1‐phosphate uridyl transferase assay for galactosemia, and urine succinylacetone for tyrosinemia. Significant hyperammonemia (>80 umol/L and often >250 umol/L) may be indicative of urea cycle defects, with prognosis influenced by both duration and peak ammonia levels at presentation . Prompt discontinuation of protein, initiation of intravenous glucose to prevent catabolism, and involvement of metabolic/genetic specialists may optimize outcomes .…”

Section: Neonatal Alfmentioning

confidence: 99%

“…Significant hyperammonemia (>80 umol/L and often >250 umol/L) may be indicative of urea cycle defects, with prognosis influenced by both duration and peak ammonia levels at presentation . Prompt discontinuation of protein, initiation of intravenous glucose to prevent catabolism, and involvement of metabolic/genetic specialists may optimize outcomes . Mitochondrial liver disease, including respiratory chain defects and mitochondrial DNA (mtDNA) depletion syndromes, are increasingly recognized causes of neonatal ALF .…”

Section: Neonatal Alfmentioning

confidence: 99%

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Liver Diseases in the Perinatal Period: Interactions Between Mother and Infant

et al. 2020

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Liver diseases affecting the mother and infant dyad may present in the perinatal period from 20 weeks of gestation to 28 days of life. This review will focus on the current approach to neonatal acute liver failure and the progress made in the diagnosis and management of gestational alloimmune liver disease. It will highlight mother‐to‐child transmission of viral hepatitis, both management and public health implications. Emerging concepts implicating maternal obesity and nutrition in the development of a rapidly progressive nonalcoholic steatohepatitis phenotype in the offspring will be discussed. Finally, the presentation and management of acute fatty liver of pregnancy and intrahepatic cholestasis of pregnancy, and their impact on the fetus, will be reviewed.

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Presenting Features and Prognosis of Ischemic and Nonischemic Neonatal Liver Failure

Abstract: Neonatal liver failure should perhaps be considered in the differential diagnoses of any coagulopathy. ALT and international normalized ratio levels at diagnosis could predict prognosis in the short term. Ischemic liver failure appears to have a better prognosis.

Cited by 11 publications

References 21 publications

Liver Failure Among Young Saudi Infants

Liver Failure Among Young Saudi Infants

Early Exchange Transfusion to Treat Neonates With Gestational Alloimmune Liver Disease

Liver Diseases in the Perinatal Period: Interactions Between Mother and Infant

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