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

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

doi:10.1097/mpg.0b013e31829ef82c

Cited by 37 publications

(29 citation statements)

References 47 publications

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“…A similar study of HRQoL in 114 treatment naive children with chronic hepatitis C virus demonstrated that, relative to normative data, caregivers were more likely to believe their child’s health was poor, reported higher concern about their child’s health status, and these perceptions limited family activities (26). In studies of children with non-alcoholic fatty liver disease (27) and autoimmune hepatitis (28), decreases in caregivers’ quality of life were found, although use of different HRQoL instruments makes direct comparison difficult. Information on HRQoL in children with chronic HBV is limited.…”

Section: Discussionmentioning

confidence: 99%

Health‐related Quality of Life in Pediatric Patients With Chronic Hepatitis B Living in the United States and Canada

Schwarzenberg

Ling

Cloonan

et al. 2017

J. pediatr. gastroenterol. nutr.

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Objectives To determine if selected socio-demographic and HBV-specific clinical factors are associated with health-related quality of life (HRQoL) among pediatric patients chronically infected with hepatitis B virus (HBV). Methods Children with chronic HBV enrolled in the Hepatitis B Research Network completed the Child Health Questionnaire (CHQ) at study entry. Caregivers of children 5–<10 years completed the parent-reported PF50; youth 10–< 18 years completed the child-reported CF87. We examined univariable associations of CHQ scores with selected independent variables: sex, adoption status, maternal education, alanine aminotransferase (ALT, U/L), aspartate aminotransferase-to-platelet ratio index (APRI), and HBV-specific symptom count. Results 244 participants (83 young children 5–<10y, 161 youth 10–<18y) were included, all HBV treatment naïve. Among young children, increased ALT level was negatively associated with PF50 psychosocial summary T-Score (r=−0.28, p=0.01). No other subscale comparisons for young children were statistically significant. Among youth, adoption was associated with better physical functioning and general health (p<0.01). Higher maternal education was associated with better role/functioning-physical and -emotional scores (p<0.05). Maternal education and adoption status were linked, with adoption associated with higher maternal education. Increased symptom count in youth was associated with worse HRQoL in subscales measuring bodily pain, behavior, mental health, and self-esteem (p<0.01). Conclusions Although overall HRQoL is preserved in children with chronic HBV, some socio-demographic and HBV-related clinical factors were associated with impaired HRQoL in our pediatric patients at baseline. Measurement of HRQoL can focus resources on education and psychosocial support in children and families most in need.

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Section: Discussionmentioning

confidence: 99%

Health‐related Quality of Life in Pediatric Patients With Chronic Hepatitis B Living in the United States and Canada

Schwarzenberg

Ling

Cloonan

et al. 2017

J. pediatr. gastroenterol. nutr.

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“…(312,314) Low scores on health-related quality of life assessments have been strongly associated with glucocorticoid use. (319)(320)(321)(322) Pretreatment psychological disturbances, especially depression, may be intensified during glucocorticoid treatment. (321) The combined effects of depression, anxiety, and glucocorticoidrelated emotional lability may impact on treatment outcome.…”

Section: Pretreatment Counselingmentioning

confidence: 99%

Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases

et al. 2020

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US children, 9%-12% (14-16) UK children, 38% (13) Age at presentation Peripubertal and adults Usually under 14 years (153) Mode of presentation Chronic symptoms common Acute onset (~40%) Ascites or GI bleeding rare Acute liver failure possible (555,556) Asymptomatic in 25%-34% Relapse frequent (108) Acute in 25%-75% Acute severe in 2%-6% Laboratory features Hypergammaglobulinemia IgA levels may be reduced (153) Autoantibodies ANA Anti-LKM1 SMA, anti-actin [Anti-LC1, Anti-LKM3] SLA Concurrent immune diseases Autoimmune thyroiditis Autoimmune thyroiditis Rheumatic diseases Diabetes mellitus IBD Vitiligo Autoimmune overlap with PSC (ASC in children) Common in children Rare Atypical pANCA-positive Atypical pANCA-negative Overlap with PBC Seen in adults (not children) Not reported Cirrhosis at presentation Adults, 28%-33% (especially elderly) Rare Children, ≤33% Remission after drug withdrawal Possible Rare, usually need long-term immunosuppression Abbreviations: GI, gastrointestinal; IgA, serum immunoglobulin A. *Removed from marketplace. Abbreviation: anti-PD-L1, antibody to programmed death protein ligand 1. taBle 6. Features of Drug-Induced aIH-like Injury and aIH Clinical Features Drug Induced AIH-Like Injury AIH Gender Mainly women (187) Female predominance, but men also affected (2,384,467) Acute onset Majority (>60%) (231) <20% (2,136) Hypersensitivity (fever, rash, eosinophilia) Up to 30% (231,232,613) Unusual (2,384,467) Temporal relationship with drug Positive (231-234) Negative (2,56,188) HLA DRB1*03:01 or DRB1*04:01 association None (236) Common (29) Concurrent autoimmune diseases Unusual (187) Present in 14%-44% (129,149-152) Cirrhosis at presentation Rare (187) 28%-33% (9,104-107) Management Stop offending drug ± glucocorticoids (187,231,232) Glucocorticoids with AZA (2,384,467) Relapse after drug withdrawal Rare (187) 60%-87% (243,244) Progression to cirrhosis Rare (187) 7%-40% (105) Survival without transplantation 90%-100% (187,232) 10-year survival, 89%-91% (105,451

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Section: Pathogenesismentioning

confidence: 95%

“…The single study of quality of life in children in autoimmune liver disease showed worse scores than healthy controls across most domains and worse scores than diabetes, liver transplantation, obesity, and rheumatological diseases, with only asthma having lower scores. Parents often feel that their children are doing worse than the children themselves think . It is particularly difficult to enable self‐assessment in young children and the currently available tools are culture specific and of varying complexities (Child Health Questionnaire [CHQ], EuroQoL, Beck Youth Inventory [BYI‐2], Paeds‐QL4 including specific transplant module) .…”

Section: Pathogenesismentioning

confidence: 95%

Review article: unanswered clinical and research questions in autoimmune hepatitis‐conclusions of the International Autoimmune Hepatitis Group Research Workshop

Dyson

Martin²,

Dalekos³

et al. 2019

Aliment Pharmacol Ther

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Background: Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease that results in substantial morbidity and mortality with many unanswered clinical and research questions. Improved understanding of disease pathogenesis, including the extra-hepatic manifestations of AIH, may allow targeted treatments with greater efficacy and fewer associated adverse events. Aim:To identify the spectrum of unanswered clinical and research questions facing care providers in the management of patients with autoimmune hepatitis (AIH). Methods:The International Autoimmune Hepatitis Group initiated a series of research workshops to start to address these questions. Key issues were discussed in small group sessions with collation of all discussions to be summarised in this manuscript.Results: Key issues were identified as: the need for better understanding of disease pathogenesis, standardisation of the methods and assays used to evaluate autoantibodies in AIH, refinement of the histopathological criteria for "typical" or "compatible" AIH, focus on the interaction with non-alcohol related fatty liver disease, how to treat acute severe AIH, better assessment of quality of life in adults and paediatrics, standardising use of standard, third-line and experimental therapies in AIH and search for biomarkers early in the disease course that predict outcome. Conclusion:This workshop has outlined the key unanswered clinical and research questions to help to define the research agenda in AIH.

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

Cited by 37 publications

References 47 publications

Health‐related Quality of Life in Pediatric Patients With Chronic Hepatitis B Living in the United States and Canada

Health‐related Quality of Life in Pediatric Patients With Chronic Hepatitis B Living in the United States and Canada

Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases

Review article: unanswered clinical and research questions in autoimmune hepatitis‐conclusions of the International Autoimmune Hepatitis Group Research Workshop

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