Total Bile Acid Concentration in Duodenal Fluid Is a Useful Preoperative Screening Marker to Rule Out Biliary Atresia

Fukuoka, Tetsuo; Bessho, Kazuhiko; Tachibana, Makiko; Satomura, Yutaka; Konishi, Akiko; Yasuda, Kie; Kimura, Takeshi; Hasegawa, Yasuhiro; Ueno, Takayoshi; Miyoshi, Yoko; Ozono, Keiichi

doi:10.1097/mpg.0000000000002037

Cited by 9 publications

(4 citation statements)

References 27 publications

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“…Median serum FGF19 levels were remarkably similar in both these BA studies, whereas unlike here, likely administered UDCA, with FXR antagonist activity 29 , 41 was the most predominant serum bile acid in the study by Johansson et al, potentially contributing to the missing correlation between bile acids and FGF19 in their study. 19 Although biliary secretion of bile acids into the intestine is negligible at the time of KPE because of widespread destruction of the extrahepatic bile ducts, 34 bile acids reach enterocytes also through systemic circulation. Theoretically, it remains possible that after escaping from the liver into the systemic circulation in high concentrations, 22 , 39 bile acids may also activate intestinal FXR-FGF19 from the basolateral side of enterocytes, 42 thereby contributing to the increased FGF19 serum levels.…”

Section: Discussionmentioning

confidence: 99%

“…In primary biliary cholangitis and alcoholic hepatitis high serum FGF19 levels associate with disease severity 28–30. Recent studies involving small number of BA patients indicate that liver expression and serum levels of FGF19 are increased both at the time of KPE and LT despite the diminished ileal exposure to luminal bile acids because of the severe cholestasis 19,20,34. Induction of FGF19 may thus represent another adaptative mechanism to cholestasis and therefore may be associated with postsurgical outcomes in BA.…”

Section: Introductionmentioning

confidence: 99%

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Serum FGF19 predicts outcomes of Kasai portoenterostomy in biliary atresia

et al. 2023

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Background and Aims: Outcomes after Kasai portoenterostomy (KPE) for biliary atresia remain highly variable for unclear reasons. As reliable early biomarkers predicting KPE outcomes are lacking, we studied the prognostic value of FGF19. Approach and Results: Serum and liver specimens, obtained from biliary atresia patients (N=87) at KPE or age-matched cholestatic controls (N=26) were included. Serum concentration of FGF19 and bile acids, liver mRNA expression of FGF19, and key regulators of bile acid synthesis were related to KPE outcomes and liver histopathology. Immunohistochemistry and in situ hybridization were used for the localization of liver FGF19 expression. Serum levels (223 vs. 61 pg/mL, p<0.001) and liver mRNA expression of FGF19 were significantly increased in biliary atresia. Patients with unsuccessful KPE (419 vs. 145 pg/mL, p=0.047), and those subsequently underwent liver transplantation (410 vs. 99 pg/mL, p=0.007) had significantly increased serum, but not liver, FGF19, which localized mainly in hepatocytes. In Cox hazard modeling serum FGF19 <109 pg/mL predicted native liver survival (HR: 4.31, p<0.001) also among patients operated <60 days of age (HR: 8.77, p=0.004) or after successful KPE (HR: 6.76, p=0.01). Serum FGF19 correlated positively with increased serum primary bile acids (R=0.41, p=0.004) and ductular reaction (R=0.39, p=0.004). Conclusions: Increased serum FGF19 at KPE predicted inferior long-term native liver survival in biliary atresia and was associated with unsuccessful KPE, elevated serum primary bile acids, and ductular reaction.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Serum FGF19 predicts outcomes of Kasai portoenterostomy in biliary atresia

et al. 2023

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“…Serum TBAs are typically low to normal in cholestasis due to bile acid synthesis disorders 55 . Duodenal biliary TBA (dTBA), dTBA/sTBA ratio, and dTBA/ serum GGT ratio were lower in infants with biliary atresia than healthy infants 56 . Bile acids have not been shown to correlate with the severity of pruritis in childhood cholestatic disorders 57 .…”

Section: Pediatric Cholestatic Disordersmentioning

confidence: 90%

A Current Understanding of Bile Acids in Chronic Liver Disease

Farooqui

Elhence

Shalimar

2022

Journal of Clinical and Experimental Hepatology

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This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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“…Less invasive tests assessing patency which can be performed before IOC include the hepatobiliary iminodiacetic acid (HIDA) scan (follows a tracer from liver to intestine), endoscopic retrograde cholangiopancreatography (ERCP) (injects dye into the ampulla of Vater via endoscopy), and the percutaneous cholangiogram (PTC) (injects dye into the gall bladder via a percutaneous approach). Patency can also be assessed indirectly by measuring bilirubin or bile acids in the intestine (duodenal aspirates), with presence of bilirubin or bile acids inconsistent with BA (40). A normal HIDA scan, ERCP, PTC, or duodenal aspirate can exclude BA and make an IOC unnecessary.…”

Section: Diagnosismentioning

confidence: 99%

Biliary Atresia as a Disease Starting In Utero

Mysore

Shneider

Harpavat

2019

J. pediatr. gastroenterol. nutr.

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Biliary atresia (BA) is the most common reason for pediatric liver transplant. BA's varied presentation, natural history, and treatment with the Kasai portoenterostomy have been well described; however, when BA starts relative to birth has not been clearly defined. In this review, we discuss laboratory, imaging, and clinical data which suggest that most if not all forms of BA may start before birth. This early onset has implications in terms of delivering treatments earlier and identifying possible factors underlying BA's etiology.

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Total Bile Acid Concentration in Duodenal Fluid Is a Useful Preoperative Screening Marker to Rule Out Biliary Atresia

Abstract: DTT evaluated by dTBA, dTBA/sTBA ratio, and dTBA/sGGT ratio had high accuracy to rule out BA and could avoid unnecessary surgery in some infants.

Cited by 9 publications

References 27 publications

Serum FGF19 predicts outcomes of Kasai portoenterostomy in biliary atresia

Serum FGF19 predicts outcomes of Kasai portoenterostomy in biliary atresia

A Current Understanding of Bile Acids in Chronic Liver Disease

Biliary Atresia as a Disease Starting In Utero

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