Background: Biliary atresia (BA) is a paediatric cholestatic disease characterized by a progressive fibro-inflammation of the biliary tree. Current treatment of choice is to establish good bile flow via the Kasai operation.
Aims: We aimed to identify outcome-predictive serum biomarkers in BA infant.
Methods: Thirty-three BA children recruited from 1986 to 2007 served as the baseline-study group. An additional 11 children recruited from 2008 to 2011 served as the validation group. Serum samples were collected immediately before and 6 months after the Kasai operation for the assessment of serum cytokines, including tumour necrosis factor-α (TNF-α), transforming growth factor-β (TGF-β), interferon-γ (IFN-γ), interleukin-2 (IL-2), IL-10, IL-12p40 and IL-12p70 as the candidate biomarkers.
Results: Increased serum TGF-β levels indicated a lower Knodell hepatitis activity index at Kasai operation. The serum TGF-β levels declined after the operation. Serum IL-12p40 levels before the Kasai operation were higher in the subjects with a 3-month jaundice-free status than in others (P = 0.001). A serum pre-operative IL-12p40 level of 33 pg/ml was predictive of a 3-month jaundice-free status after surgery (positive predictive value=81.0%; negative predictive value=83.3%). This biomarker was also predictive of a better outcome, in terms of 3-year survival with native liver (risk ratio [RR = 4.00]; P < 0.001), and 3-year jaundice-free survival with native liver (RR = 12.00; P < 0.001). We confirmed the predictive power of a high pre-operative IL-12p40 level on 3-month jaundice-free status in the validation group.
Conclusions: The pre-operative IL-12p40 level was a good predictive biomarker of clinical outcome in children with BA undergoing the Kasai operation.
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http://dx.doi.org/10.1111/liv.12001 | DOI Listing |
Sci Rep
January 2025
Laboratory of Pharmacology, Department of Pharmacy, Faculty of Pharmaceutical Sciences, Tokyo University of Science, Chiba, Japan.
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View Article and Find Full Text PDFSemin Pediatr Surg
January 2025
Swiss Pediatric Liver Center, Geneva University Hospitals, Geneva, Switzerland; Division of Child and Adolescent Surgery, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, Geneva, Switzerland.
Cholangitis, defined as the inflammation of the bile ducts, is the most frequent complication after Kasai hepatoportoenterostomy in patients with biliary atresia (BA). This review seeks to provide a comprehensive synthesis of current knowledge on diagnosing and managing BA-associated cholangitis while identifying gaps in the existing literature. A scoping literature review was conducted to gather global insights into the definition, evaluation, and management of post-Kasai cholangitis, illustrated through data from the Swiss Biliary Atresia Registry (SBAR).
View Article and Find Full Text PDFGenes (Basel)
January 2025
Department of Hearing Implant Sciences, Shinshu University School of Medicine, Matsumoto 390-8621, Japan.
belongs to the unconventional myosin superfamily, and the myosin IIIa protein localizes on the tip of the stereocilia of vestibular and cochlear hair cells. Deficiencies in have been reported to cause the deformation of hair cells into abnormally long stereocilia with an increase in spacing. is a rare causative gene of autosomal recessive sensorineural hearing loss (DFNB30), with only 13 cases reported to date.
View Article and Find Full Text PDFChildren (Basel)
January 2025
Department of Paediatric Surgery, Kings College Hospital, Denmark Hill, London SE5 9RS, UK.
Biliary atresia (BA) is an obliterative disease of the bile ducts affecting between 1 in 10,000-20,000 infants with a predominance in Asian countries. It is clinically heterogeneous with a number of distinct variants (e.g.
View Article and Find Full Text PDFBioengineering (Basel)
December 2024
Department of Pathology, University of Yamanashi, Yamanashi 409-3898, Japan.
The latest World Health Organization (WHO) classification of central nervous system tumors (WHO2021/5th) has incorporated molecular information into the diagnosis of each brain tumor type including diffuse glioma. Therefore, an artificial intelligence (AI) framework for learning histological patterns and predicting important genetic events would be useful for future studies and applications. Using the concept of multiple-instance learning, we developed an AI framework named GLioma Image-level and Slide-level gene Predictor (GLISP) to predict nine genetic abnormalities in hematoxylin and eosin sections: , , mutations, promoter mutations, homozygous deletion (CHD), amplification (amp), 7 gain/10 loss (7+/10-), 1p/19q co-deletion, and promoter methylation.
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