Download PDF
Eur J Pediatr Surg 2016; 26(04): 349-356
DOI: 10.1055/s-0035-1554913
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Overall Quality of Life in Adult Biliary Atresia Survivors with or without Liver Transplantation: Results from a National Cohort

Willemien de Vries
1   Section of Gastroenterology/Hepatology, Department of Paediatrics, University Medical Center Groningen, Groningen, The Netherlands
*   The authors, Dr. de Vries and Dr. Lind, contributed equally to the manuscript.
,
Robert C. Lind
2   Section of Paediatric Surgery, Department of Surgery, University Medical Center Groningen, Groningen, The Netherlands
*   The authors, Dr. de Vries and Dr. Lind, contributed equally to the manuscript.
,
Yuk-Kueng Sze
2   Section of Paediatric Surgery, Department of Surgery, University Medical Center Groningen, Groningen, The Netherlands
,
Alida F. W. van der Steeg
3   Department of Pediatric Surgery, Pediatric Surgical Center of Amsterdam, Emma Children's Hospital AMC and VU University Medical Center, Amsterdam, The Netherlands
4   Department of Medical Psychology, Center of Research on Psychology in Somatic Diseases, Tilburg University, Tilburg, The Netherlands
,
Egbert Sieders
5   Department of Hepatobiliary Surgery and Liver Transplantation, University Medical Center Groningen, Groningen, The Netherlands
,
Robert Jack Porte
5   Department of Hepatobiliary Surgery and Liver Transplantation, University Medical Center Groningen, Groningen, The Netherlands
,
Henkjan J. Verkade
1   Section of Gastroenterology/Hepatology, Department of Paediatrics, University Medical Center Groningen, Groningen, The Netherlands
,
Jan B. F. Hulscher
6   Section of Paediatric Surgery, Department of Surgery, University Medical Center Groningen, Groningen, The Netherlands
,
Josette E. H. M. Hoekstra-Weebers
7   Wenckebach Institute, University Medical Center Groningen, Groningen, The Netherlands
› Author Affiliations
Further Information

Publication History

20 November 2015

08 April 2015

Publication Date:
27 May 2015 (online)

Also available at
    Permissions and Reprints

Abstract

Background Biliary atresia (BA) is a rare cholestatic disease of infancy. Kasai portoenterostomy and liver transplantation (LT) are the two sequential treatment options. An increasing number of patients survive into adulthood. Little is known about their health-related quality of life (HRQOL). This study aims to compare HRQOL of transplanted and nontransplanted patients in a cohort of young adult BA survivors.

Patients and Methods RAND-36 and Liver Disease Index Score (LDSI) questionnaires were sent to eligible adult patients with BA. Clinical characteristics were obtained from the NeSBAR (Netherlands Study group on Biliary Atresia Registry) and the national pediatric LT database. RAND-36 domain and summary scores were compared with those of an age-matched Dutch reference group. The correlations between several clinical variables and HRQOL were analyzed.

Results Mean RAND-36 domain and summary scores of transplanted (n = 15) and nontransplanted (n = 25) patients with BA (response 74%) were similar to the reference scores, with the exception of a decreased general health perception in nontransplanted patients (63 ± 21 vs. 75 ± 17; [p < 0.001], particularly in females. RAND-36 domain and summary scores were not significantly correlated to age at LT, time since LT, serum bilirubin, aspartate amino transferase or albumin levels, but were moderately to strongly correlated to LDSI total scores (r values 0.35–0.77).

Conclusions Overall, young adult patients with BA have a HRQOL similar to an age-matched reference group. However, general health perception of nontransplanted patients, particularly of females, was decreased. HRQOL is correlated to liver disease symptoms but not to liver biochemistry parameters. Nontransplanted females and patients suffering from liver disease–associated symptoms may be a target for tailored supportive interventions.

Keywords

quality of life - biliary atresia - liver transplantation
 

  • References

  • 1 Kasai M, Suzuki S. A new operation for “non-correctable” biliary atresia: portoenterostomy. Shujutsu 1959; 13: 733-739
    PubMedGoogle Scholar
  • 2 Serinet M-O, Broué P, Jacquemin E , et al. Management of patients with biliary atresia in France: results of a decentralized policy 1986-2002. Hepatology 2006; 44 (1) 75-84
    CrossrefPubMedGoogle Scholar
  • 3 Nio M, Ohi R, Miyano T, Saeki M, Shiraki K, Tanaka K ; Japanese Biliary Atresia Registry. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg 2003; 38 (7) 997-1000
    CrossrefPubMedGoogle Scholar
  • 4 Duffy JP, Kao K, Ko CY , et al. Long-term patient outcome and quality of life after liver transplantation: analysis of 20-year survivors. Ann Surg 2010; 252 (4) 652-661
    PubMedGoogle Scholar
  • 5 Fouquet V, Alves A, Branchereau S , et al. Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center. Liver Transpl 2005; 11 (2) 152-160
    CrossrefPubMedGoogle Scholar
  • 6 de Vries W, Homan-Van der Veen J, Hulscher JBF, Hoekstra-Weebers JE, Houwen RH, Verkade HJ ; Netherlands Study Group of Biliary Atresia Registry. Twenty-year transplant-free survival rate among patients with biliary atresia. Clin Gastroenterol Hepatol 2011; 9 (12) 1086-1091
    CrossrefPubMedGoogle Scholar
  • 7 Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology 2005; 41 (2) 366-371
    CrossrefPubMedGoogle Scholar
  • 8 Shinkai M, Ohhama Y, Take H , et al. Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children's hospital. J Pediatr Gastroenterol Nutr 2009; 48 (4) 443-450
    CrossrefPubMedGoogle Scholar
  • 9 Sebagh M, Rifai K, Féray C , et al. All liver recipients benefit from the protocol 10-year liver biopsies. Hepatology 2003; 37 (6) 1293-1301
    CrossrefPubMedGoogle Scholar
  • 10 Schreiber RA, Barker CC, Roberts EA , et al; Canadian Pediatric Hepatology Research Group. Biliary atresia: the Canadian experience. J Pediatr 2007; 151 (6) 659-665 , 665.e1
    CrossrefPubMedGoogle Scholar
  • 11 Davenport M, De Ville de Goyet J, Stringer MD , et al. Seamless management of biliary atresia in England and Wales (1999-2002). Lancet 2004; 363 (9418) 1354-1357
    CrossrefPubMedGoogle Scholar
  • 12 Marchesini G, Bianchi G, Amodio P , et al; Italian Study Group for quality of life in cirrhosis. Factors associated with poor health-related quality of life of patients with cirrhosis. Gastroenterology 2001; 120 (1) 170-178
    CrossrefPubMedGoogle Scholar
  • 13 Younossi ZM, Kiwi ML, Boparai N, Price LL, Guyatt G. Cholestatic liver diseases and health-related quality of life. Am J Gastroenterol 2000; 95 (2) 497-502
    CrossrefPubMedGoogle Scholar
  • 14 Younossi ZM, Boparai N, Price LL, Kiwi ML, McCormick M, Guyatt G. Health-related quality of life in chronic liver disease: the impact of type and severity of disease. Am J Gastroenterol 2001; 96 (7) 2199-2205
    CrossrefPubMedGoogle Scholar
  • 15 Jay CL, Butt Z, Ladner DP, Skaro AI, Abecassis MM. A review of quality of life instruments used in liver transplantation. J Hepatol 2009; 51 (5) 949-959
    CrossrefPubMedGoogle Scholar
  • 16 Gross CR, Malinchoc M, Kim WR , et al. Quality of life before and after liver transplantation for cholestatic liver disease. Hepatology 1999; 29 (2) 356-364
    CrossrefPubMedGoogle Scholar
  • 17 Alonso EM, Limbers CA, Neighbors K , et al; Studies of Pediatric Liver Transplantation (SPLIT) Functional Outcomes Group (FOG). Cross-sectional analysis of health-related quality of life in pediatric liver transplant recipients. J Pediatr 2010; 156 (2) 270-6.e1
    CrossrefPubMedGoogle Scholar
  • 18 Tome S, Wells JT, Said A, Lucey MR. Quality of life after liver transplantation. A systematic review. J Hepatol 2008; 48 (4) 567-577
    CrossrefPubMedGoogle Scholar
  • 19 Howard ER, MacLean G, Nio M, Donaldson N, Singer J, Ohi R. Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and England. J Pediatr Surg 2001; 36 (6) 892-897
    CrossrefPubMedGoogle Scholar
  • 20 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 (4) 1052-7.e2
    CrossrefPubMedGoogle Scholar
  • 21 Lind RC, de Vries W, Keyzer-Dekker CM , et al. Health status and quality of life in adult biliary atresia patients surviving with their native livers. Eur J Pediatr Surg 2015; 25 (1) 60-65
    Article in Thieme ConnectPubMedGoogle Scholar
  • 22 van der Plas SM, Hansen BE, de Boer JB , et al. The Liver Disease Symptom Index 2.0; validation of a disease-specific questionnaire. Qual Life Res 2004; 13 (8) 1469-1481
    CrossrefPubMedGoogle Scholar
  • 23 de Vries W, de Langen ZJ, Aronson DC , et al; NeSBAR. Mortality of biliary atresia in children not undergoing liver transplantation in the Netherlands. Pediatr Transplant 2011; 15 (2) 176-183
    PubMedGoogle Scholar
  • 24 Potgieser ARE, de Vries W, Sze YK , et al; NeSBAR. Course of life into adulthood of patients with biliary atresia: the achievement of developmental milestones in a nationwide cohort. J Adolesc Health 2012; 50 (6) 641-644
    CrossrefPubMedGoogle Scholar
  • 25 VanderZee KI, Sanderman R, Heyink JW, de Haes H. Psychometric qualities of the RAND 36-Item Health Survey 1.0: a multidimensional measure of general health status. Int J Behav Med 1996; 3 (2) 104-122
    CrossrefPubMedGoogle Scholar
  • 26 Stam H, Grootenhuis MA, Caron HN, Last BF. Quality of life and current coping in young adult survivors of childhood cancer: positive expectations about the further course of the disease were correlated with better quality of life. Psychooncology 2006; 15 (1) 31-43
    CrossrefPubMedGoogle Scholar
  • 27 Cohen J. Statistical Power Analysis for the Behavioural Sciences. New York, NY: Academy Press; 1988
    Google Scholar
  • 28 Deurloo JA, Ekkelkamp S, Hartman EE, Sprangers MA, Aronson DC. Quality of life in adult survivors of correction of esophageal atresia. Arch Surg 2005; 140 (10) 976-980
    CrossrefPubMedGoogle Scholar
  • 29 Schwartz CE, Sprangers MA. Methodological approaches for assessing response shift in longitudinal health-related quality-of-life research. Soc Sci Med 1999; 48 (11) 1531-1548
    CrossrefPubMedGoogle Scholar
  • 30 Wijnhoven HAH, Kriegsman DMW, Snoek FJ, Hesselink AE, de Haan M. Gender differences in health-related quality of life among asthma patients. J Asthma 2003; 40 (2) 189-199
    CrossrefPubMedGoogle Scholar
  • 31 Goodman E, Amick BC, Rezendes MO, Tarlov AR, Rogers WH, Kagan J. Influences of gender and social class on adolescents' perceptions of health. Arch Pediatr Adolesc Med 1997; 151 (9) 899-904
    CrossrefPubMedGoogle Scholar
  • 32 Mrus JM, Williams PL, Tsevat J, Cohn SE, Wu AW. Gender differences in health-related quality of life in patients with HIV/AIDS. Qual Life Res 2005; 14 (2) 479-491
    CrossrefPubMedGoogle Scholar
  • 33 Arrington-Sanders R, Yi MS, Tsevat J, Wilmott RW, Mrus JM, Britto MT. Gender differences in health-related quality of life of adolescents with cystic fibrosis. Health Qual Life Outcomes 2006; 4: 5
    CrossrefPubMedGoogle Scholar
  • 34 Tuinman MA, Gazendam-Donofrio SM, Hoekstra-Weebers JE. Screening and referral for psychosocial distress in oncologic practice: use of the Distress Thermometer. Cancer 2008; 113 (4) 870-878
    CrossrefPubMedGoogle Scholar
  • 35 van der Steeg AFW, De Vries J, Roukema JA. The value of quality of life and health status measurements in the evaluation of the well-being of breast cancer survivors. Eur J Surg Oncol 2008; 34 (11) 1225-1230
    CrossrefPubMedGoogle Scholar
  • 36 Gutteling JJ, Duivenvoorden HJ, Busschbach JJV, de Man RA, Darlington AS. Psychological determinants of health-related quality of life in patients with chronic liver disease. Psychosomatics 2010; 51 (2) 157-165
    CrossrefPubMedGoogle Scholar
  • 37 Zhang S-J, Huang L-H, Wen Y-L , et al. Impact of personality and coping mechanisms on health related quality of life in liver transplantation recipients. Hepatobiliary Pancreat Dis Int 2005; 4 (3) 356-359
    PubMedGoogle Scholar