Liquid chromatography–mass spectroscopy as a tool in the rapid diagnosis of biliary atresia: a pilot study

Nguyen, Minh; Dossa, Avafia; Zagory, Jessica A.; Golden, Jamie; Roberts, Anne S.; Fu, Xiaowei; Wang, Kasper; Gayer, Christopher P.

doi:10.1016/j.jpedsurg.2016.02.053

Cited by 8 publications

(4 citation statements)

References 15 publications

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“…It was obvious that the function is complicated and difficult for clinical application; therefore, a composite score system was then established for easy prediction (Table 3). Of note, because the IBA value varied depending on the instruments or reagents used, [12,21] we standardized the serum bile acid value using the multiple of the median (MoM) value. Since we had tested the IBA concentrations of normal control, we could calculate the MoM, which is defined as the ratio of the actual measured value over the normal median value of IBA (Supplemental Table S1).…”

Section: Derivation Cohortmentioning

confidence: 99%

Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study

et al. 2020

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Background: Early distinguishing biliary atresia from other causes of infantile cholestasis remains a major challenge. We aimed to develop and validate a scoring system based on bile acid for identification of biliary atresia. Methods: In a prospective study, a total of 141 infants with cholestasis were enrolled in two sets (derivation cohort, n = 66; validation cohort, n = 75) from 2014 to 2018. Variables with significant difference between biliary atresia and non-biliary atresia infants were selected in the derivation cohort. Then, a scoring system including those variables was designed and validated. Results: Among 66 patients in the derivation cohort, 34 (51.5%) had biliary atresia. A scoring system was proposed with the following variables: glycochenodeoxycholic acid/chenodeoxycholic acid, clay stool, and gamma-glutamyl transferase. The total score ranged from 0 to 41, and a cutoff value of 15 identified biliary atresia with an area under receiver operating characteristic curve of 0.87 (95% confidence interval, 0.77-0.94), sensitivity of 85.3%, and specificity of 81.3% in the derivation cohort; these values were also confirmed in a validation cohort with a sensitivity of 90.0% and specificity of 80.0%. Conclusions: The proposed simple scoring system had good diagnostic accuracy for estimating the risk of biliary atresia in infants with cholestasis.

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Section: Derivation Cohortmentioning

confidence: 99%

Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study

et al. 2020

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“…Remarkably and unexpectedly, our data suggests that the microbiome before KP may be unique to BA and impact the success of the procedure, in particular, early features of bile flow. Although cholestatic liver diseases in infancy often have similar amounts of fecal bile acids present (33), we found a possibly unique microbiome associated with infants with BA. This suggests that other factors in addition to bile flow may be affecting the infant microbiome in BA.…”

Section: Discussionmentioning

confidence: 66%

The Fecal Microbiome in Infants With Biliary Atresia Associates With Bile Flow After Kasai Portoenterostomy

Tessier

Cavallo

Yeh

et al. 2020

J. pediatr. gastroenterol. nutr.

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Background: Biliary atresia's (BA) response to surgical Kasai portoenterostomy (KP) is uneven and dependent upon bile flow; 50% of infants require a liver transplant by 24 months. We hypothesized that the microbiome may identify and associate with outcomes in BA. Methods: Stool samples were collected from infants with cholestasis (n = 15), 8 of which with BA were followed longitudinally.16S sequencing was performed on all samples (n = 45). Whole Genome Sequencing (WGS) was performed on BA pre-KP samples (n = 8). Infants with BA, other forms of cholestasis, BA infants with very good bile flow (VGBF) and not (nVGBF) (VGBF dichotomized by TSBA <40 μmol/L by 6 months) were compared. Results: Of the 8 infants with BA, 4 infants had VGBF. Microbial richness was inversely proportional to degree of cholestasis (P = 0.046). Increased Bifidobacterium abundance associated with VGBF (P = 0.03) and decreased cholestasis (P < 0.01) at 1 month post-KP. Pre-KP, community structure differed in infants with BA versus other cholestasis. Interestingly, infants who subsequently achieved VGBF had increased diversity (P = 0.03) and different community structure at the pre-KP time point. WGS corroborated Bifidobacterium's pre-KP importance. Conclusions: The microbiome differs between infants with BA and other cholestasis. It additionally differs between infants with BA who have good and poor bile flow, and thus outcomes, post-KP. These differences are seen even before KP. These data suggest that bile influences the development of the infant microbiome and that there may be possible influences of the pre- and post-KP microbiome on bile flow after KP. Further larger studies are needed to confirm these findings.

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“…The metabolic and immune effects of secondary bile acids in liver disease require further investigation. In BA, serum bile acid profiles have been used as a diagnostic tool to discriminate between BA and non-BA infantile cholestasis, but increasingly studies have described a reduction in total and primary faecal bile acids (71,72). Molecular analysis has demonstrated a reduction in 7a-dehydroxylating bacteria, Lachnospiraceae, Blautia, and Clostridium XIVa in BA, although a concomitant reduction in secondary bile acids has yet to be described.…”

Section: Gut Microbiome-bile Acid Interactionmentioning

confidence: 99%

Gut Microbiome

Jain

Alexander

Burford

et al. 2021

J. pediatr. gastroenterol. nutr.

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Biliary atresia (BA) is a fibro-obliterative condition of the biliary tree, presenting in infancy. The bilioenteric conduit formed at Kasai portoenterostomy (KPE), achieves restoration of bile flow in approximately 60% of infants. Even if the operation is successful, cirrhosis and its associated complications are, however, common. BA remains the leading cause for liver transplantation (LT) in children. Antibiotic, choleretic, and steroid therapy post-KPE have not convincingly reduced LT rates. Advances in molecular technology have enabled characterisation of the encoded genes of the gut microbiota (gut microbiome). The gut microbiome plays an important role in host metabolism, nutrition, and immune function, with alterations in its diversity and/or composition, known as dysbiosis, being described in disease states, including liver disease. Liver-gut microbiome exploration in adulthood largely focuses on nonalcoholic liver disease, cirrhosis (mainly alcohol- or viral-based aetiology) and cholestatic liver diseases (eg, primary sclerosing cholangitis), with microbial signatures correlating to disease severity. Investigation of the gut microbiota in BA had been limited to culture-based methodology, but molecular studies are emerging, and although in their infancy, highlight a potential pathogenic role for Enterobacteriaceae and Streptococcus, and a potential beneficial role for Bifidobacteria. Bacterial translocation, and the production of gut microbiome-derived metabolites, are key host-microbiome-mechanistic pathways in liver disease pathogenesis. Microbiome-targeted therapeutics for liver disease are in development, with faecal microbiota transplantation showing promise in cirrhosis. Could the gut microbiome be a novel modifiable risk factor in BA, reducing the need for LT?

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Liquid chromatography–mass spectroscopy as a tool in the rapid diagnosis of biliary atresia: a pilot study

Cited by 8 publications

References 15 publications

Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study

Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study

The Fecal Microbiome in Infants With Biliary Atresia Associates With Bile Flow After Kasai Portoenterostomy

Gut Microbiome

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