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Health-related quality of life in pre-adolescent liver transplant recipients with biliary atresia: A cross-sectional study - 25/07/19

Doi : 10.1016/j.clinre.2018.10.018 
Mar Miserachs a, b, Arpita Parmar a, Agnieszka Bakula c, Loreto Hierro d, Lorenzo D’Antiga e, Imeke Goldschmidt f, Dominique Debray g, Valérie A McLin h, Valeria Casotti e, Joanna Pawłowska c, Carmen Camarena d, Anthony R Otley i, j, Ulrich Baumann f, Vicky L Ng a,
a Transplant and regenerative medicine centre, division of pediatric gastroenterology, hepatology and nutrition, the hospital for sick children, university of Toronto, Toronto, Ontario, Canada 
b Universitat Autònoma de Barcelona, Bellatera, Spain 
c Instytut Pomnik-Centrum Zdrowia Dziecka, Warsaw, Poland 
d Service of pediatric hepatology and transplantation, hospital universitario La Paz, Madrid, Spain 
e Hospital Papa Giovanni XXIII, Bergamo, Italy 
f Medizinischen Hochschule Hannover, Hannover, Germany 
g Pediatric hepatology unit, AP–HP, hôpital Necker-Enfants Malades, université Paris Descartes, Paris, France 
h Hôpitaux universitaires de Genève, Geneva, Switzerland 
i Division of gastroenterology and nutrition, department of pediatrics, IWK Health Centre, Halifax, Nova Scotia, Canada 
j Department of pediatrics, faculty of medicine, Dalhousie university, Halifax, Nova Scotia, Canada 

Corresponding author at: The hospital for sick children, 555, University avenue, M5G 1X8 Toronto, Ontario, CanadaThe hospital for sick children555, University avenueTorontoOntarioM5G 1X8Canada

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Highlights

Generic HRQOL in pre-adolescent LT recipients is poorer compared with healthy norms.
The PeLTQL questionnaire revealed lower scores in the Coping and Adjustment domain.
Simplified immunosuppression regimens were associated with better HRQOL.
Sports participation after LT in pre-adolescents may have a protective role on HRQOL.

Il testo completo di questo articolo è disponibile in PDF.

Summary

Objective

Pediatric recipients of liver transplantation (LT) often report lower Health-Related Quality of Life (HRQOL) than healthy controls when assessed on generic HRQOL measurement tools. The recent addition of the Pediatric Liver Transplant Quality of Life (PeLTQL), a novel disease-specific HRQOL instrument for pediatric LT recipients, into the clinical armamentarium of tools now routinely available to clinical care teams, provides the unique opportunity to identify disease-related challenges in children who have undergone this life-saving intervention. This study assesses HRQOL in pre-adolescent aged patients with a primary diagnosis of biliary atresia (BA) who underwent LT as an infant, using both generic and disease-specific HRQOL instruments validated for children. We also examined modifiable factors associated with HRQOL after pediatric LT.

Methods

HRQOL was the primary outcome of this study assessed using the disease-specific PeLTQL and the generic Pediatric Quality of Life Inventory 4.0 (PedsQL). Exposure variables of interest included medication status (e.g., monotherapy, dual therapy) and participation in sports.

Results

A total of 70 (56% female, mean age 9.89 ± 1.25 years) pediatric LT recipients (mean interval since LT was 9.0 ± 1.26 years) comprised the study cohort. LT recipients reported significantly lower PedsQL Scores relative to the general population. Immunosuppression monotherapy was associated with higher patient-reported PeLTQL Scores, and sports participation was associated with higher parent-reported PedsQL Scores.

Conclusions

Pre-adolescents who underwent LT as an infant with BA, self-report low HRQOL on both disease-specific and generic HRQOL tools. Further research targeting sports participation and simplifying immunosuppression may further optimize quality of life years restored by life-saving LT.

Il testo completo di questo articolo è disponibile in PDF.

Keywords : Children, Patient-reported outcome, Immunosuppression, Sports and adherence


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Vol 43 - N° 4

P. 427-435 - Agosto 2019 Ritorno al numero
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