Dataset Information

Factors Influencing Time-to-diagnosis of Biliary Atresia.

ABSTRACT:

Objectives

Diagnosing biliary atresia (BA) quickly is critical, because earlier treatment correlates with delayed or reduced need for liver transplantation. However, diagnosing BA quickly is also difficult, with infants usually treated after 60 days of life. In this study, we aim to accelerate BA diagnosis and treatment, by better understanding factors influencing the diagnostic timeline.

Methods

Infants born between 2007 and 2014 and diagnosed with BA at our institution were included (n = 65). Two periods were examined retrospectively: P1, the time from birth to specialist referral, and P2, the time from specialist referral to treatment. How sociodemographic factors associate with P1 and P2 were analyzed with Kaplan-Meier curves and Cox proportional hazard models. In addition, to better characterize P2, laboratory results and early tissue histology were studied.

Results

P1 associated with race/ethnicity, with shorter times in non-Hispanic white infants compared to non-Hispanic black and Hispanic infants (P = 0.007 and P = 0.004, respectively). P2 associated with referral age, with shorter times in infants referred after 30, 45, or 60 days of life (P < 0.001, P < 0.001, and P = 0.001, respectively). One potential reason for longer P2 in infants referred ≤30 days is that aminotransferase levels were normal or near-normal. However, despite reassuring laboratory values, tissue histology in early cases showed key features of BA.

Conclusions

Our findings suggest 2 opportunities to accelerate BA diagnosis and treatment. First, to achieve prompt referrals for all races/ethnicities, universal screening strategies should be considered. Second, to ensure efficient evaluations independent of age, algorithms designed to detect early features of BA can be developed.

SUBMITTER: Harpavat S

PROVIDER: S-EPMC5963977 | biostudies-literature | 2018 Jun

REPOSITORIES: biostudies-literature

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Publications

Factors Influencing Time-to-diagnosis of Biliary Atresia.

Harpavat Sanjiv S Lupo Philip J PJ Liwanag Loriel L Hollier John J Brandt Mary L ML Finegold Milton J MJ Shneider Benjamin L BL

Journal of pediatric gastroenterology and nutrition 20180601 6

<h4>Objectives</h4>Diagnosing biliary atresia (BA) quickly is critical, because earlier treatment correlates with delayed or reduced need for liver transplantation. However, diagnosing BA quickly is also difficult, with infants usually treated after 60 days of life. In this study, we aim to accelerate BA diagnosis and treatment, by better understanding factors influencing the diagnostic timeline.<h4>Methods</h4>Infants born between 2007 and 2014 and diagnosed with BA at our institution were incl ...[more]

PMID: 29315162

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Project description:BackgroundDespite surgical treatment, children with biliary atresia (BA) may face many problems which seriously affect their quality of life. However, there is a paucity of studies in China examining the quality of life of these children after BA treatment. This study investigated the current status of the quality of life of children after BA treatment and analyzed the influencing factors so as to provide a scientific basis for the development of targeted interventional measures.MethodsFrom May 1 to May 31, 2021, the STAR questionnaire method was used to conduct cross-sectional surveys in children following BA surgery in the West China Hospital of Sichuan University, China. The basic information questionnaire was used to investigate the general characteristics of the children. The children were also given quality of life questionnaires during follow-up sessions after surgical treatment for BA. In addition, a 12-point health survey was used to assess the physical and mental health of the children's caregivers. Age- and gender-matched healthy children were recruited as controls. Correlation analysis and multiple linear regression equations were established to explore the influencing factors of the quality of life of children after surgery for BA.ResultsAfter surgery, children with BA experienced significantly lower physical health, emotional function, social function, cognitive function, and quality of life compared to healthy children (P<0.050). Co-existing diseases had a statistically significant impact on the quality of life of children with BA (b=-12.566; t=-2.343; P=0.021), and the caregiver's understanding level of liver transplantation also had a statistically significant impact on the quality of life of these children (b=6.481; t=2.376; P=0.021).ConclusionsThe quality of life of children after BA surgery was significantly lower than that of age-matched healthy children. Co-existing diseases and the caregiver's understanding level of liver transplantation were the main factors affecting the quality of life of these children.

Project description:BACKGROUND: Young age at portoenterostomy has been linked to improved outcome in biliary atresia, but pre-existing biological factors may influence the rate of disease progression. In this study, we aimed to determine whether molecular profiling of the liver identifies stages of disease at diagnosis. METHODS: We examined liver biopsies from 47 infants with biliary atresia enrolled in a prospective observational study. Biopsies were scored for inflammation and fibrosis, used for gene expression profiles, and tested for association with indicators of disease severity, response to surgery, and survival at 2 years. RESULTS: Fourteen of 47 livers displayed prominent features of inflammation (N=9) or fibrosis (N=5), with the remainder showing similar levels of both simultaneously. Differential profiling of gene expression of the 14 livers displayed a unique molecular signature containing 150 gene probes. Applying prediction analysis models, the probes classified 29 of the remaining 33 livers into inflammation or fibrosis. Molecular classification into the two groups was validated by the findings of increased hepatic population of lymphocyte subsets or tissue accumulation of matrix substrates. The groups had no association with traditional markers of liver injury or function, response to surgery, or complications of cirrhosis. However, infants with an inflammation signature were younger, while those with a fibrosis signature had decreased transplant-free survival. CONCLUSION: Molecular profiling at diagnosis of biliary atresia uncovers a signature of inflammation or fibrosis in most livers. This signature may relate to staging of disease at diagnosis and has implications to clinical outcomes. Clinical and laboratory data and liver biopies were obtained from 47 infants at diagnosis of biliary atresia and every 3-6 month intervals until 2 years of age as part of a prospective, observational study of the Biliary Atresia Research Consortium. Liver biopsies underwent histological scoring and those specimens classified as inflammation or fibrosis were used to generate a group-specific gene expression profile. The profile was used to assign a molecular stages of inflammation or fibrosis to the entire cohort. Molecular groups were tested for biological plausibility using immunostaining to quantify hepatic inflammatory cells, and quantitative PCR to reproduce the expression of gene groups and the expression of collagen genes. They were also tested for clinical relevance by testing of association with indicators of liver function, complications of disease, and clinical outcome. This dataset is part of the TransQST collection.

Dataset Information

Factors Influencing Time-to-diagnosis of Biliary Atresia.

Objectives

Methods

Results

Conclusions

Publications

Factors Influencing Time-to-diagnosis of Biliary Atresia.

Similar Datasets

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