Neurodevelopmental Outcomes in Preschool and School Aged Children With Biliary Atresia and Their Native Liver

Squires, James E.; Ng, Vicky L.; Hawthorne, Kieran; Henn, Lisa; Sorensen, Lisa G.; Fredericks, Emily M.; Alonso, Estella M.; Murray, Karen F.; Loomes, Kathleen M.; Karpen, Saul J.; Cavallo, Laurel; Molleston, Jean P.; Bezerra, Jorge A.; Rosenthal, Philip; Squires, Robert H.; Wang, Kasper S.; Schwarz, Kathleen B.; Arnon, Ronen; Magee, John C.; Sokol, Ronald J.

doi:10.1097/mpg.0000000000002489

Cited by 18 publications

(19 citation statements)

References 40 publications

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“…15 In the current study, transplant recipients with biliary atresia had a mean FSIQ of 87.6 AE 10.9, which is lower than that in patients with biliary atresia with native liver (WPPSI-III, 104 AE 14; WISC-IV, 105 AE 12). 24 Children with congenital cholestasis transplanted before age 1 year had significantly lower FSIQ than those transplanted after age 1 year, indicating that age at transplantation, not diagnosis, is crucial. Age at transplantation was a significant factor associated with FSIQ also when adjusted for cholestasis.…”

Section: Original Articlesmentioning

confidence: 98%

Impaired Neurocognitive Performance in Children after Liver Transplantation

Østensen

Skarbø

Sanengen

et al. 2022

The Journal of Pediatrics

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Objectives To assess longitudinal neurocognitive development after liver transplantation and evaluate factors associated with neurocognitive performance.Study design Data from neurocognitive testing of 65 children (aged <18 years) who underwent liver transplantation at Oslo University Hospital between 1995 and 2018 were collected from the testing program after transplantation. The parent-reported version of the Behavior Rating Inventory of Executive Function was used to assess executive function.Results A total of 104 neurocognitive tests were conducted on 65 patients. At the first test, conducted at a median of 4.1 years (IQR, 1.5-5.3 years) after transplantation and at a median age of 6.7 years (IQR, 5.4-10.5 years), the mean full-scale IQ (FSIQ) was 91.7 AE 14, and the mean verbal comprehension index was 92.0 AE 14.5. In the 30 patients tested more than once, there was no significant difference in FSIQ between the first test at a median age of 5.8 years (IQR, 5.2-8.5 years) and the last test at a median age of 10.8 years (IQR, 9.8-12.9 years) (87.4 AE 12.9 vs 88.5 AE 13.2; P = .58). Compared with the patients who underwent transplantation a age >1 year (n = 35), those who did so at age <1 year (n = 30) had a lower FSIQ (87.1 AE 12.6 vs 96.6 AE 13.8; P = .005) and lower verbal comprehension index (87.3 AE 13.8 vs 95.4 AE 13.0; P = .020). Age at transplantation (P = .005; adjusted for cholestasis: P = .038) and transfusion of >80 mL/kg (P = .004; adjusted for age at transplantation: P = .046) were associated with FSIQ.Conclusions Young age at transplantation and large blood transfusions during transplantation are risk factors for poor neurocognitive performance later in life. Children who undergo transplantation before 1 year of age have significantly lower neurocognitive performance compared with those who do so later in childhood. Cognitive performance did not improve over time after transplantation.

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Section: Original Articlesmentioning

confidence: 98%

Impaired Neurocognitive Performance in Children after Liver Transplantation

Østensen

Skarbø

Sanengen

et al. 2022

The Journal of Pediatrics

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“…( 35 ) These neurodevelopmental delays improve over time, however, as preschool and school‐age children (aged 3‐12 years) with BA surviving with their native liver demonstrate a full‐scale intelligence quotient (FSIQ) equivalent to that of the normal population. ( 36 )…”

Section: Neurodevelopmental Outcomes and Health‐related Quality Of Li...mentioning

confidence: 99%

Malnutrition in Biliary Atresia: Assessment, Management, and Outcomes

et al. 2021

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Children with biliary atresia (BA), particularly infants, are at high risk for malnutrition attributed to a multitude of factors, including poor oral intake and intolerance of enteral feeding, fat malabsorption, abnormal nutrient metabolism, and increased caloric demand. Malnutrition and sarcopenia negatively impact outcomes in BA, leading to higher pretransplant and posttransplant morbidity and mortality. This review summarizes factors contributing to nutritional deficiencies in BA and offers an organized approach to the assessment and management of malnutrition in this vulnerable population.

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“…Last, with social distancing, closure of schools and recreational activities, and less in‐person access to developmental therapies, we may see disproportionate changes in developmental outcomes of children with inherited or acquired liver disease. ( 42 )…”

Section: Clinical Research In Special Populationsmentioning

confidence: 99%

“…Last, with social distancing, closure of schools and recreational activities, and less in-person access to developmental therapies, we may see disproportionate changes in developmental outcomes of children with inherited or acquired liver disease. (42) On a positive note, the COVID-19 pandemic has strengthened an already tight-knit pediatric hepatology community. The Society of Pediatric Liver Transplantation-The Transplantation Society and North American Society for Pediatric Gastroenterology, Hepatology & Nutrition have put forth a joint national registry for COVID-19 in children with chronic liver disease and post-liver transplantation.…”

Section: Clinical Research In Special Populations Pediatric Clinical Researchmentioning

confidence: 99%

Clinical Research in Hepatology in the COVID‐19 Pandemic and Post‐Pandemic Era: Challenges and the Need for Innovation

Verna

Serper

Chu³

et al. 2020

Hepatology

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The severe acute respiratory syndrome coronavirus 2 pandemic has drastically altered all facets of clinical care and research. Clinical research in hepatology has had a rich tradition in several domains, including the discovery and therapeutic development for diseases such as hepatitis B and C and studying the natural history of many forms of chronic liver disease. National Institutes of Health, foundation, and industry funding have provided important opportunities to advance the academic careers of young investigators while they strived to make contributions to the field. Instantaneously, however, all nonessential research activities were halted when the pandemic started, forcing those involved in clinical research to rethink their research strategy, including a shift to coronavirus disease 2019 research while endeavoring to maintain their preexisting agenda. Strategies to maintain the integrity of ongoing studies, including patient follow‐up, safety assessments, and continuation of investigational products, have included a shift to telemedicine, remote safety laboratory monitoring, and shipping of investigational products to study subjects. As a revamp of research is being planned, unique issues that face the research community include maintenance of infrastructure, funding, completion of studies in the predetermined time frame, and the need to reprogram career path timelines. Real‐world databases, biomarker and long‐term follow up studies, and research involving special groups (children, the homeless, and other marginalized populations) are likely to face unique challenges. The implementation of telemedicine has been dramatically accelerated and will serve as a backbone for the future of clinical research. As we move forward, innovation in clinical trial design will be essential for conducting optimized clinical research.

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Neurodevelopmental Outcomes in Preschool and School Aged Children With Biliary Atresia and Their Native Liver

Cited by 18 publications

References 40 publications

Impaired Neurocognitive Performance in Children after Liver Transplantation

Impaired Neurocognitive Performance in Children after Liver Transplantation

Malnutrition in Biliary Atresia: Assessment, Management, and Outcomes

Clinical Research in Hepatology in the COVID‐19 Pandemic and Post‐Pandemic Era: Challenges and the Need for Innovation

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