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Eur J Pediatr Surg 2020; 30(03): 261-272
DOI: 10.1055/s-0040-1712932
Review Article
Georg Thieme Verlag KG Stuttgart · New York

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

Lyan H. Rodijk
1   Department of Pediatric Surgery, University Medical Center Groningen, Groningen, The Netherlands
,
Eke M. W. Schins
1   Department of Pediatric Surgery, University Medical Center Groningen, Groningen, The Netherlands
,
Marieke J. Witvliet
2   Department of Pediatric Surgery, Wilhelmina Children’s Hospital, University Medical Center Utrecht, The Netherlands
,
Henkjan J. Verkade
3   Department of Pediatrics, Section of Gastroenterology/Hepatology, University Medical Center Groningen, Groningen, The Netherlands
,
Ruben H. de Kleine
4   Department of Hepato-Pancreatico-Biliary Surgery and Liver Transplantation, University Medical Center Groningen, Groningen, The Netherlands
,
Jan B. F. Hulscher
1   Department of Pediatric Surgery, University Medical Center Groningen, Groningen, The Netherlands
,
Janneke L. M. Bruggink
1   Department of Pediatric Surgery, University Medical Center Groningen, Groningen, The Netherlands
› Author Affiliations Funding This study was funded by the Dutch Digestive Foundation (Maag Lever Darm Stichting; JR/2016-098) and the University of Groningen.
Further Information

Publication History

10 April 2020

26 April 2020

Publication Date:
06 July 2020 (online)

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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.

Keywords

biliary atresia - health-related quality of life - liver transplantation - childhood
 

  • References

  • 1 Hays RD, Reeve BB. Measurement and modeling of health-related quality of life. Int Encycl Public Heal. 2008; 4: 241-252
    PubMedGoogle Scholar
  • 2 Bai G, Herten MH, Landgraf JM, Korfage IJ, Raat H. Childhood chronic conditions and health-related quality of life: findings from a large population-based study. PLoS One 2017; 12 (06) e0178539
    CrossrefPubMedGoogle Scholar
  • 3 Amin R, Knezevich M, Lingongo M. , et al. Long-term quality of life in neonatal surgical disease. Ann Surg 2018; 268 (03) 497-505
    CrossrefPubMedGoogle Scholar
  • 4 Parmar A, Vandriel SM, Ng VL. Health-related quality of life after pediatric liver transplantation: a systematic review. Liver Transpl 2017; 23 (03) 361-374
    CrossrefPubMedGoogle Scholar
  • 5 Haavisto A, Korkman M, Sintonen H. , et al. Risk factors for impaired quality of life and psychosocial adjustment after pediatric heart, kidney, and liver transplantation. Pediatr Transplant 2013; 17 (03) 256-265
    CrossrefPubMedGoogle Scholar
  • 6 Devine KA, Reed-Knight B, Loiselle KA, Simons LE, Mee LL, Blount RL. Predictors of long-term health-related quality of life in adolescent solid organ transplant recipients. J Pediatr Psychol 2011; 36 (08) 891-901
    CrossrefPubMedGoogle Scholar
  • 7 Robson VK, Stopp C, Wypij D. , et al. Longitudinal associations between neurodevelopment and psychosocial health status in patients with repaired D-transposition of the great arteries. J Pediatr 2019; 204: 38-45
    CrossrefPubMedGoogle Scholar
  • 8 Rodijk LH, den Heijer AE, Hulscher JBF. , et al. Long-term neurodevelopmental outcomes in children with biliary atresia. J Pediatr 2020; 217: 118-124
    CrossrefPubMedGoogle Scholar
  • 9 Cohen J. A coefficient of agreement for nominal scales. Educ Psychol Meas 1960; 20: 37-46
    CrossrefPubMedGoogle Scholar
  • 10 de Vries W, de Langen ZJ, Groen H. , et al; Netherlands Study Group of Biliary Atresia and Registry (NeSBAR). Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008. J Pediatr 2012; 160 (04) 638-644
    CrossrefPubMedGoogle Scholar
  • 11 Centraal Bureau voor de Statistiek (CBS) Den Haag. Standaard Onderwijsindeling; 2006 . Available at: https://www.cbs.nl/-/media/imported/onze%20diensten/methoden/classificaties/documents/2012/05/2006-pub-soi-2014-15.pdf . Accessed September, 2019
    PubMed
  • 12 Meinds RJ, van der Steeg AFW, Sloots CEJ. , et al. Long-term functional outcomes and quality of life in patients with Hirschsprung's disease. Br J Surg 2019; 106 (04) 499-507
    CrossrefPubMedGoogle Scholar
  • 13 Roorda D, Witvliet MJ, Wellens LM. , et al. Long-term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands. Colorectal Dis 2018; 20 (08) 719-726
    CrossrefPubMedGoogle Scholar
  • 14 The WHOQOL Group. Development of the World Health Organization WHOQOL-BREF quality of life assessment. Psychol Med 1998;28(03):551–558
  • 15 Landgraf JM, Abetz L, Ware JE. Child Health Questionnaire (CHQ): a user’s manual. Boston, Massachusetts: Health Institute, New England Medical Center; 1996
  • 16 Wulffraat N, van der Net JJ, Ruperto N. , et al; Paediatric Rheumatology International Trials Organisation. The Dutch version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ). Clin Exp Rheumatol 2001; 19 (04) (Suppl. 23) S111-S115
    PubMedGoogle Scholar
  • 17 Raat H, Landgraf JM, Bonsel GJ, Gemke RJBJ, Essink-Bot ML. Reliability and validity of the child health questionnaire-child form (CHQ-CF87) in a Dutch adolescent population. Qual Life Res 2002; 11 (06) 575-581
    CrossrefPubMedGoogle Scholar
  • 18 Landgraf JM, Abetz LN. Functional status and well-being of children representing three cultural groups: initial self-reports using the chq-cf87. Psychol Health 1997; 12: 839-854
    CrossrefPubMedGoogle Scholar
  • 19 Raat H, Bonsel GJ, Essink-Bot ML, Landgraf JM, Gemke RJBJ. Reliability and validity of comprehensive health status measures in children: the child health questionnaire in relation to the Health Utilities Index. J Clin Epidemiol 2002; 55 (01) 67-76
    CrossrefPubMedGoogle Scholar
  • 20 Raat H, Botterweck AM, Landgraf JM, Hoogeveen WC, Essink-Bot ML. Reliability and validity of the short form of the child health questionnaire for parents (CHQ-PF28) in large random school based and general population samples. J Epidemiol Community Health 2005; 59 (01) 75-82
    CrossrefPubMedGoogle Scholar
  • 21 Walker RE, Gauvreau K, Jenkins KJ. Health-related quality of life in children attending a cardiology clinic. Pediatr Cardiol 2004; 25 (01) 40-48
    CrossrefPubMedGoogle Scholar
  • 22 Cunningham MJ, Chiu EJ, Landgraf JM, Gliklich RE. The health impact of chronic recurrent rhinosinusitis in children. Arch Otolaryngol Head Neck Surg 2000; 126 (11) 1363-1368
    CrossrefPubMedGoogle Scholar
  • 23 Peetsold MG, Huisman J, Hofman VE, Heij HA, Raat H, Gemke RJ. Psychological outcome and quality of life in children born with congenital diaphragmatic hernia. Arch Dis Child 2009; 94 (11) 834-840
    CrossrefPubMedGoogle Scholar
  • 24 Dunbar-Masterson C, Wypij D, Bellinger DC. , et al. General health status of children with D-transposition of the great arteries after the arterial switch operation. Circulation 2001; 104 (12) (Suppl. 01) I138-I142
    CrossrefPubMedGoogle Scholar
  • 25 Peetsold MG, Heij HA, Deurloo JA, Gemke RJ. Health-related quality of life and its determinants in children and adolescents born with oesophageal atresia. Acta Paediatr 2010; 99 (03) 411-417
    CrossrefPubMedGoogle Scholar
  • 26 Speechley KN, Barrera M, Shaw AK, Morrison HI, Maunsell E. Health-related quality of life among child and adolescent survivors of childhood cancer. J Clin Oncol 2006; 24 (16) 2536-2543
    CrossrefPubMedGoogle Scholar
  • 27 Sabaz M, Cairns DR, Bleasel AF. , et al. The health-related quality of life of childhood epilepsy syndromes. J Paediatr Child Health 2003; 39 (09) 690-696
    CrossrefPubMedGoogle Scholar
  • 28 Goldmuntz E, Cassedy A, Mercer-Rosa L, Fogel MA, Paridon SM, Marino BS. Exercise performance and 22q11.2 deletion status affect quality of life in tetralogy of fallot. J Pediatr 2017; 189: 162-168
    CrossrefPubMedGoogle Scholar
  • 29 HealthActCHQ. The CHQ Scoring and Interpretation Manual. Cambridge, MA: HealthActCHQ; 2008
    Google Scholar
  • 30 Sundaram SS, Alonso EM, Haber B. , et al; Childhood Liver Disease Research and Education Network (ChiLDREN). Health related quality of life in patients with biliary atresia surviving with their native liver. J Pediatr 2013; 163 (04) 1052-1057
    CrossrefPubMedGoogle Scholar
  • 31 Ohnemus D, Neighbors K, Rychlik K. , et al; for Studies of Pediatric Liver Transplantation (SPLIT). Health-related quality of life and cognitive functioning in pediatric liver transplant recipients. Liver Transpl 2020; 26 (01) 45-56
    CrossrefPubMedGoogle Scholar
  • 32 Koo TK, Li MY. A guideline of selecting and reporting intraclass correlation coefficients for reliability research. J Chiropr Med 2016; 15 (02) 155-163
    PubMedGoogle Scholar
  • 33 Cohen J. Statistical Power Anaylsis for the Behavioural Science. 2nd ed. New York, NY: Lawrence Erlbaum; 1988
    Google Scholar
  • 34 Ellis PD. . The Essential Guide to Effect Sizes: Statistical Power, Meta-Analysis and the Interpretation of Search Results. New York, NY: Cambridge University Press; 2010
  • 35 Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data capture (REDCap)—a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 2009; 42 (02) 377-381
    CrossrefPubMedGoogle Scholar
  • 36 Parolini F, Boroni G, Milianti S. , et al. Biliary atresia: 20-40-year follow-up with native liver in an Italian centre. J Pediatr Surg 2019; 54 (07) 1440-1444
    CrossrefPubMedGoogle Scholar
  • 37 Lampela H, Pakarinen MP, Jahnukainen T, Jalanko H, Kosola S. Quality of life and parental worrying in a National Cohort of Biliary Atresia Children living with their native livers. J Pediatr Gastroenterol Nutr 2017; 64 (06) 883-887
    CrossrefPubMedGoogle Scholar
  • 38 Lind RC, de Vries W, Keyzer-Dekker CMG. , et al. Health status and quality of life in adult biliary atresia patients surviving with their native livers. Eur J Pediatr Surg 2015; 25 (01) 60-65
    Article in Thieme ConnectPubMedGoogle Scholar
  • 39 Ng VL, Haber BH, Magee JC. , et al; Childhood Liver Disease Research and Education Network (CHiLDREN). Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium. J Pediatr 2014; 165 (03) 539-546
    CrossrefPubMedGoogle Scholar
  • 40 Kikuchi R, Mizuta K, Urahashi T. , et al. Quality of life after living donor liver transplant for biliary atresia in Japan. Pediatr Int (Roma) 2018; 60 (02) 183-190
    CrossrefPubMedGoogle Scholar
  • 41 Miserachs M, Parmar A, Bakula A. , et al. Health-related quality of life in pre-adolescent liver transplant recipients with biliary atresia: a cross-sectional study. Clin Res Hepatol Gastroenterol 2019; 43 (04) 427-435
    CrossrefPubMedGoogle Scholar
  • 42 Wong CWY, Chung PHY, Tam PKH, Wong KKY. Long-term results and quality of life assessment in Biliary atresia patients: a 35-year experience in a tertiary hospital. J Pediatr Gastroenterol Nutr 2018; 66 (04) 570-574
    CrossrefPubMedGoogle Scholar
  • 43 de Vries W, Lind RC, Sze YK. , et al. Overall quality of life in adult biliary atresia survivors with or without liver transplantation: results from a National Cohort. Eur J Pediatr Surg 2016; 26 (04) 349-356
    Article in Thieme ConnectPubMedGoogle Scholar
  • 44 Lee WS, Ong SY. Health-related quality of life in children with biliary atresia living with native livers. Ann Acad Med Singapore 2016; 45 (02) 61-68
    PubMedGoogle Scholar
  • 45 Schwartz CE, Andresen EM, Nosek MA, Krahn GL. ; RRTC Expert Panel on Health Status Measurement. Response shift theory: important implications for measuring quality of life in people with disability. Arch Phys Med Rehabil 2007; 88 (04) 529-536
    CrossrefPubMedGoogle Scholar
  • 46 Cole CR, Bucuvalas JC, Hornung RW. , et al. Impact of liver transplantation on HRQOL in children less than 5 years old. Pediatr Transplant 2004; 8 (03) 222-227
    CrossrefPubMedGoogle Scholar
  • 47 Thijs A, van Leeuwen B, Zandbergen M. . Inclusive Education in the Netherlands. SLO Netherlands. Enschede: Institute for Curriculum Development; 2008
  • 48 Eiser C, Morse R. Can parents rate their child's health-related quality of life? Results of a systematic review. Qual Life Res 2001; 10 (04) 347-357
    CrossrefPubMedGoogle Scholar
  • 49 Albrecht GL, Devlieger PJ. The disability paradox: high quality of life against all odds. Soc Sci Med 1999; 48 (08) 977-988
    CrossrefPubMedGoogle Scholar