Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study

Ng, Vicky L.; Sorensen, Lisa G.; Alonso, Estella M.; Fredericks, Emily M.; Ye, Wen; Moore, Joseph; Karpen, Saul J.; Shneider, Benjamin L.; Molleston, Jean P.; Bezerra, Jorge A.; Murray, Karen F.; Loomes, Kathleen M.; Rosenthal, Philip; Squires, Robert H.; Wang, Kasper; Arnon, Ronen; Schwarz, Kathleen B.; Turmelle, Yumirle P.; Haber, Barbara; Sherker, Averell H.; Magee, John C.; Sokol, Ronald J.

doi:10.1016/j.jpeds.2017.12.048

Cited by 48 publications

(68 citation statements)

References 30 publications

(36 reference statements)

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“…In a study conducted before the widespread availability of LT intervention, Stewart et al 1 reported that children with chronic LD scored lower than typically developing children of the same age on both IQ scores and physical variables -particularly height. Similarly, Ng et al 3 have reported poorer cognitive outcomes in children with biliary atresia, including an association between height measured at 18 months and a set of broad-based neurocognitive variables assessed longitudinally at 24 months. These data are consistent with our finding of the relationship between pre-transplant percentiles for height, and IQ measured at long-term follow-up.…”

Section: Discussionmentioning

confidence: 87%

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Long‐term Effects of Cholestatic Liver Disease in Childhood on Neuropsychological Outcomes and Neurochemistry

Talcott

Beath²,

Patel

et al. 2019

J. pediatr. gastroenterol. nutr.

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OBJECTIVES. Children with liver disease have increased risk of long-term cognitive deficits. We differentiated between the effects of chronic liver disease from that associated with transplantation by recruiting children with cholestatic liver disease with and without transplantation. METHODS. Psychometric measures and magnetic resonance spectroscopy were obtained for 3 groups of children: stable liver disease (SLD) without transplantation; cholestatic liver disease (CLD) from birth with transplantation; and individuals healthy to 18 months of age, prior to transplantation for acute liver failure (ALF). RESULTS. Cognitive outcomes between children with different disease histories were significantly associated with the duration of liver disease but not the effects of transplantation, including that of immune suppression. Lower intellectual ability was most frequently observed in the CLD group, whereas all of the ALF group scored within the normal range. Myo-inositol and glutamate/glutamine concentrations in cortex were significantly associated with disease duration across the cohort. Neuro-metabolite profiles in SLD were consistent with subclinical encephalopathy. Impaired growth in early childhood was associated with later cognitive performance. CONCLUSION. Children with prolonged liver disease had the poorest cognitive outcomes despite successful transplantation, suggesting that prolonged cholestasis before transplantation adversely impacts neurodevelopment, and reinforces the need for timely interventions.

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Section: Discussionmentioning

confidence: 87%

“…Chronic liver disease is associated with an array of negative symptoms 1,2 . Improvements in nutritional support, treatment delivery and access to liver transplantation (LT) has resulted in children with liver disease (LD) surviving illnesses that were normally fatal in infancy 2,3 .…”

Section: Introductionmentioning

confidence: 99%

Long‐term Effects of Cholestatic Liver Disease in Childhood on Neuropsychological Outcomes and Neurochemistry

Talcott

Beath²,

Patel

et al. 2019

J. pediatr. gastroenterol. nutr.

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“…As a result, several national and international BA research consortiums and registries have been set up to foster scientific progress with this rare condition (Table ). The relatively massive resources devoted to the ChiLDReN network in the USA is a good example of this, though one could argue that little novel has come of it, beyond consolidation of what was already known or at least inferred . The amazing discovery of biliatresone and its role in ovine BA owes more to insight and scientific perseverance than to a centrally‐directed research committee .…”

Section: Discussionmentioning

confidence: 99%

Biliary atresia: a scientometric analysis of the global research architecture and scientific developments

Friedmacher

Ford

Davenport

2019

J Hepato Biliary Pancreat

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Biliary atresia (BA) is a rare cholangiopathy of largely unknown etiology and unpredictable outcome. There has been an increasing number of BA-related publications, which may challenge researchers to determine their actual scientific value. This study aimed to evaluate the global research activity and developments relating to BA using a combination of scientometric methodologies and visualization tools. A comprehensive search strategy for the Web of Science TM database was designed to obtain bibliographic data on scientific BA publications for the timespan 1900-2018. Research output of countries, institutions, individual authors and collaborative networks was analyzed. Semi-qualitative research measures including citation rate and h-index were assessed. Choropleth mapping and network diagrams were used to visualize results. In total, 4,459 publications on BA were identified (88.5% in English), originating from 63 countries. The largest number was published by the USA (n = 991; 22.2%), Japan (n = 667; 15.0%) and the UK (n = 294; 6.6%). The USA combined the highest number of cooperation articles (n = 140). The most productive collaborative network was established between the USA and Canada (n = 17). Scientific papers from the UK received the highest average citation rate (16.7), whereas the USA had the highest country-specific h-index (59). Eighty-eight (2.0%) items were published under the auspices of multicenter consortiums and registries. The most productive institutions and authors were based in the USA, the UK, Japan, France, Canada and Taiwan. BArelated research has constantly been progressing, becoming more multidisciplinary but with main research endeavors concentrated in a few high-income countries. Studies into pathogenesis of BA remain uncommon, but are sorely needed to foster true scientific progress with this rare disease. Hence, international collaborative and translational research should be strengthened to allow further evolution in this field.

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“…For cognition and executive functions, versions of the Wechsler Intelligence Scales (WISC) were the most used (15/17) (5,9,12,(14)(15)(16)(17)(18)(19)(20)(21)(22) . General, fine and gross motor development were assessed using the following instruments: Movement Assessment Battery for Children (M-ABC) (23) , Mullen Scales of Early Learning (MSEL) (10,24) , Bayley Development Scales (25,26) , Griffiths Mental Ability Scales (GMDS) (27) and General Development Scale of Minnesota Child Development Inventory (MCDI) (15) . Some of these instruments also evaluated general language (25) , receptive and expressive language (10,24) (TABLE 1).…”

Section: Study Characteristicsmentioning

confidence: 99%

Neuropsychomotor Development in Children and Adolescents With Liver Diseases: Systematic Review With Meta-Analysis

Santos

Saquetto

Neto

et al. 2021

Arq. Gastroenterol.

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BACKGROUND: The nature of liver disease, the evolutionary course and duration of liver diseases, as well as the degree of severity and disability can trigger multiple outcomes with repercussions on neuromotor acquisition and development. OBJECTIVE: To systematically review and conduct a meta-analysis to evaluate the effects of liver disease on the neuropsychomotor development of children and adolescents with their native livers and those who underwent liver transplantation. METHODS: Observational studies published since the early 1980s until June 2019 were sought in the PubMed and Scopus databases. An α value of 0.05 was considered significant. The statistical heterogeneity of the treatment effect between the studies was assessed by the Cochran’s Q test and the I2 inconsistency test, in which values above 25 and 50% were considered indicative of moderate and high heterogeneity, respectively. Analyses were performed with Review Manager 5.3. RESULTS: Twenty-five studies met the eligibility criteria, including 909 children and adolescents with liver disease. Meta-analyses showed deficits in total IQ -0.41 (95%CI: -0.51 to -0.32; N: 9,973), verbal IQ -0.38 (95%CI: -0.57 to -0.18; N: 10,284) and receptive language -0.85 (95%CI: -1.16 to -0.53; N: 921) in liver transplantation, and those with native livers who had symptoms early had total and verbal IQ scores (85±8.8; 86.3±10.6 respectively) lower than the scores of those with late manifestations (99.5±13.8; 96.2±9.2). Gross motor skill was reduced -46.29 (95%CI: -81.55 to -11.03; N: 3,746). CONCLUSION: Acute or chronic liver disease can cause declines in cognitive, motor and language functions. Although the scores improve after liver transplantation, children remain below average when compared to healthy children.

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Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study

Abstract: Clinicaltrials.gov: NCT00061828 and NCT00294684.

Cited by 48 publications

References 30 publications

Long‐term Effects of Cholestatic Liver Disease in Childhood on Neuropsychological Outcomes and Neurochemistry

Long‐term Effects of Cholestatic Liver Disease in Childhood on Neuropsychological Outcomes and Neurochemistry

Biliary atresia: a scientometric analysis of the global research architecture and scientific developments

Neuropsychomotor Development in Children and Adolescents With Liver Diseases: Systematic Review With Meta-Analysis

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