Quality of life after living donor liver transplant for biliary atresia in Japan

Kikuchi, Ryota; Mizuta, Koichi; Urahashi, Taizen; Sanada, Yukihiro; Yamada, Naoya; Onuma, Erika; Sato, Iori; Kamibeppu, Kiyoko

doi:10.1111/ped.13442

Cited by 12 publications

(16 citation statements)

References 21 publications

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“…However, native liver survivors might be more frequently hospitalized due to ongoing liver cirrhosis and portal hypertension, compared with LTx recipients, which may influence their HrQoL. The most frequent reported factors in literature that are adversely associated with HrQoL include higher number of prescribed drugs, 40,41 adverse medical events, 30,40,42 and younger age of child. 30,40 Although adult patients with BA still report an impaired general health, 38 several studies showed that young adult BA patients, either with native liver or after LTx, did not experience an impaired HrQoL compared with their healthy peers.…”

Section: Discussionmentioning

confidence: 99%

“…We included nine studies on HrQoL in BA patients (n ¼ 984 children, n ¼ 102 adults). Four studies assessed patients who survived with their native liver, [36][37][38][39] two studies assessed LTx recipients with BA specifically, 40,41 and three studies assessed both 30,42,43 (►Table 1). There was a moderate agreement (Kappa 0.77) between the two screening authors L.H.R.…”

Section: Literature Search On Hrqol In Children With Bamentioning

confidence: 99%

“…The most frequently reported factors in literature, that are adversely associated with self-reported or parent-proxy HrQoL in children with BA, include adverse medical events, 30,40,42 higher number of types of prescribed drugs, 40,41 and younger age of child 30,40 (►Table 1).…”

Section: Factors Associated With Hrqol In Children With Bamentioning

confidence: 99%

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Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation

et al. 2020

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Abstract Introduction We aimed to assess health-related quality of life (HrQoL) in biliary atresia (BA) patients, based on original data and a literature review, and to determine factors associated with their HrQoL. Materials and Methods We reviewed available studies describing HrQoL in BA patients. We assessed HrQoL in Dutch BA patients (6–16 years) using the validated Child Health Questionnaire. We compared HrQoL scores in BA patients with healthy peers and with children who had undergone major surgery in infancy or children with chronic conditions. We determined the relationship between specific patient-related factors and HrQoL. Results Literature data indicated that HrQoL in children with BA is lower than in healthy peers. In Dutch BA patients (n = 38; age 10 ± 3 years), parent-proxy physical HrQoL (48 ± 11) was significantly lower compared with two reference groups of healthy peers (59 ± 4 and 56 ± 6, respectively, each p < 0.001), and lower than in children with attention deficit hyperactivity disorder (60 ± 5), asthma (54 ± 6), attending a cardiology clinic (52 ± n/r), congenital diaphragmatic hernia (53 ± 7) or D-transposition of the great arteries (54 ± 6; all p < 0.05). Psychosocial HrQoL (50 ± 9) was lower than in healthy peers (54 ± 6, p = 0.02, and 53 ± 6, p = 0.07) and children with asthma (54 ± 6, p = 0.02), and largely comparable to children with other chronic conditions. Parent-proxy physical HrQoL was adversely related to adverse medical event in the past year, special education, and motor impairments; psychosocial HrQoL was adversely related to behavioral problems. Conclusion Children with BA are at risk of impaired HrQoL. Special attention is warranted for children with adverse medical events and special education.

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Section: Discussionmentioning

confidence: 99%

Section: Literature Search On Hrqol In Children With Bamentioning

confidence: 99%

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Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation

et al. 2020

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“…Minimal experience with school, diverse types and dosages of preschool experiences, and minimal academic achievement expectations at young ages may have contributed to our findings for the School functioning domain. Third, our HRQL was based on parent‐proxy report, and some (though not all) studies suggest that self‐reported scores are lower in older children . Fourth, some variables were missing from our study.…”

Section: Discussionmentioning

confidence: 99%

“…Third, our HRQL was based on parent-proxy report, and some (though not all) studies suggest that self-reported scores are lower in older children. 16,41 Fourth, some variables were missing from our study. We did not record sleep disturbances and Note: FSIQ, full scale intelligence quotient; GAC, general adaptive composite score on the Adaptive Behavior Assessment System II; PedsQL, the health-related quality-of-life score; PIQ, performance intelligence quotient; VIQ, verbal intelligence quotient; VMI, visual-motor integration score.…”

Section: Times Expected Proportion (P-value)mentioning

confidence: 99%

Kindergarten‐age neurocognitive, functional, and quality‐of‐life outcomes after liver transplantation at under 6 years of age

Joffe

Wong

Bond

et al. 2019

Pediatric Transplantation

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Background We aimed to describe school‐entry age neurocognitive, functional, and HRQL outcomes and their predictors after liver transplant done at age <6 years. Methods A prospective cohort of all (n = 69) children surviving liver transplant from 1999 to 2014 were assessed at age 55.4 (SD 7.2) months and 38.6 (12.4) months after transplant. Assessment included: the Wechsler Preschool and Primary Scales of Intelligence, Beery‐Buktenica Developmental Test of VMI, Adaptive Behavior Assessment System caregiver‐completed questionnaire, and PedsQL 4.0 Generic Core Scales. Univariate and multiple linear regression determined predictors of outcomes at P < .05. Results Neurocognitive and functional outcomes were on average within 1 SD of population norms, although shifted to the left (P ≤ .03), with more patients than expected having scores >2 (3.7‐5.9 times more, P ≤ .007) SD below population norms. Total and Summary HRQL scores were statistically significantly lower than the healthy normative population (P ≤ .02) and a congenital heart disease group (P ≤ .02), but similar to children with other chronic health conditions; differences often exceeded the MCID and were lowest in the School functioning domain. There were few predictors on multiple linear regressions, and we could not confirm previous studies that suggested various inconsistent predictors of outcomes. Neurocognitive and functional outcomes scores were highly correlated with HRQL scores except for the School functioning domain, but did not fully explain them. Conclusions Long‐term follow‐up of this vulnerable population is important in order to facilitate support for the patient and family, and early intervention for any difficulties identified.

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Comprehensive analyses of neurodevelopmental outcomes and quality of life of children with biliary atresia

Satomura,

Tachibana,

Yasuda

et al. 2023

J. pediatr. gastroenterol. nutr.

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ObjectivesTo holistically evaluate neurodevelopmental outcomes and quality of life (QOL) of Japanese patients with biliary atresia (BA) and to investigate the factors associated with the outcomes.MethodsThis study enrolled patients with BA aged 5–18 years who visited Osaka University Hospital in 2021. Neurodevelopmental assessments were performed to evaluate intellectual ability, cognitive functions and adaptive skill levels. Furthermore, emotional and behavioral issues, characteristics of attention deficit hyperactivity disorder, and QOL were concomitantly assessed in the same cohort. Biochemical and social factors associated with the results were examined.ResultsFifty‐three patients, with a median age of 11.2 years were included in the analyses. Patients with BA had a significantly lower Full‐Scale Intelligence Quotient or developmental quotient (FSIQ/DQ) score and Vineland Adaptive Behavior Scale (VABS) composite score than the general Japanese population. Household education level and short stature were associated with low and borderline FSIQ/DQ and VABS composite scores, respectively. Among patients with low and borderline FSIQ/DQ scores, those with average or high VABS composite scores received significantly less neuroeducational care than those with low and borderline VABS composite scores. Despite the low FSIQ/DQ and VABS composite scores, the total QOL scores were higher than those of the general population.ConclusionPatients with BA had intellectual and behavioral impairments. Notably, patients with intellectual impairments are overlooked and not followed up, especially if adaptive skills are maintained.

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Quality of life after living donor liver transplant for biliary atresia in Japan

Abstract: The levels and factors of generic and transplant-specific HRQOL of Japanese pediatric and adolescent patients with BA after LDLT varied with reporter (i.e. patients or parents).

Cited by 12 publications

References 21 publications

Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation

Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation

Kindergarten‐age neurocognitive, functional, and quality‐of‐life outcomes after liver transplantation at under 6 years of age

Comprehensive analyses of neurodevelopmental outcomes and quality of life of children with biliary atresia

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