• Vol. 45 No. 2
  • 15 February 2016

Health-Related Quality of Life in Children with Biliary Atresia Living with Native Livers

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ABSTRACT

This study aimed to quantify and investigate factors affecting the healthrelated quality of life (HRQoL) in children with biliary atresia (BA) living with their native livers. Materials and Methods: A cross-sectional study on the HRQoL using the PedsQL4.0 generic core scales in children with BA aged between 2 to 18 years followed up at the University Malaya Medical Centre (UMMC) in Malaysia was conducted. Two groups, consisting of healthy children and children with chronic liver disease (CLD) caused by other aetiologies, were recruited as controls. Results: Children with BA living with their native livers (n = 36; median (range) age: 7.4 (2 to 18) years; overall HRQoL score: 85.6) have a comparable HRQoL score with healthy children (n = 81; median age: 7.0 years; overall HQRoL score: 87.4; P = 0.504) as well as children with CLD (n = 44; median age: 4.3 years; overall score: 87.1; P = 0.563). The HRQoL of children with BA was not adversely affected by having 1 or more hospitalisations in the preceding 12 months, the presence of portal hypertension, older age at corrective surgery (>60 days), a lower level of serum albumin (≤34 g/L) or a higher blood international normalised ratio (INR) (≥1.2). Children who had liver transplantation for BA did not have a signifi cantly better HRQoL as compared to those who had survived with their native livers (85.4 vs 85.7, P = 0.960). Conclusion: HRQoL in children with BA living with their native livers is comparable to healthy children.


Biliary atresia (BA), a progressive obliterative fi broinflammatory cholangiopathy, usually presents with jaundice in infancy. The defi nitive therapy for BA in the majority of infants is liver transplantation (LT).

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