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Health-Related Quality of Life in Children With Autoimmune Liver Disease

Gulati, Reema*; Radhakrishnan, Kadakkal R.*; Hupertz, Vera*; Wyllie, Robert*; Alkhouri, Naim*; Worley, Sarah; Feldstein, Ariel E.

Journal of Pediatric Gastroenterology and Nutrition: October 2013 - Volume 57 - Issue 4 - p 444–450
doi: 10.1097/MPG.0b013e31829ef82c
Original Articles: Hepatology and Nutrition

Background and Aim: Health-related quality of life (HRQOL), a pivotal outcome indicator of health care interventions, has not been evaluated in children with autoimmune liver disease (AILD). The aim of this study was to determine HRQOL in children with AILD and the factors affecting it.

Methods: The Pediatric Quality Of Life Inventory, generic core scale, was used to collect HRQOL data on children with AILD. Specific liver disease–related questions were added.

Results: Survey responses were received from 30 of 40 patients. Patients’ mean age at diagnosis was 11.6 ± 4.5 years, with M:F ratio of 1:1.3, and AILD for average of 4.6 ± 4.3 years. Seventy-three percent of patients had advanced liver disease. Mean overall health summary scores for the group per child and parent reports were 71.6 ± 19.0 and 71.3 ± 17.1, respectively, which were lower than healthy controls: 83.9 ± 12.5 and 82.3 ± 15.6 (P = 0.002). Frequent liver-related symptoms were associated with impaired physical and school functioning by child (P = 0.034 and 0.047) and parent reports (P = 0.051 and P = 0.018). Abdominal pain, fatigue, and psychological symptoms were found to adversely affect the HRQOL. Although it was difficult to estimate the effect of individual features of advanced liver disease such as cirrhosis, history of upper gastrointestinal bleed, and portal hypertension on the HRQOL, because of a relatively small sample size, the presence of ascites revealed lower social functioning score per parent report (P = 0.036). In an analysis of patients with any of the above complications versus those without, however, children reported lower social functioning scores (P = 0.018). There were no differences in HRQOL scores in children with autoimmune hepatitis versus primary sclerosing cholangitis versus autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome.

Conclusions: First study to date shows that AILD in children significantly affects HRQOL, especially with frequent liver disease–related symptoms, even in early stages of disease. Findings need to be validated in larger, multicenter studies and will help practitioners understand their patients better and optimize care.

*Division of Pediatric Gastroenterology and Nutrition

Department of Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH

Division of Pediatric Gastroenterology and Nutrition, UC San Diego, Rady Children's Hospital, San Diego, CA.

Address correspondence and reprint requests to Reema Gulati, MD, Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195 (e-mail:

Received 2 February, 2012

Accepted 4 June, 2013

The authors report no conflicts of interest.

© 2013 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,