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INTRODUCTION: Neonatal cholestatic disorders are a group of hepatobiliary diseases occurring in the first 3 months of life. The most common causes of neonatal cholestasis are infantile hepatitis syndrome (IHS) and biliary atresia (BA). The clinical manifestations of the two diseases are ...
2019-06-25 | MTBLS689 | MetaboLights
Human intrahepatic biliary organoids were established from liver tissues of children with BA (n = 11), choledochal cysts (CC, n = 5), and histologically normal controls (NC, n = 5). We performed comprehensive multi-omics profiling, including transcriptomics, proteomics, phosphoproteomics, and untarg...
2026-09-02 | MTBLS15528 | MetaboLights

Maternal seeding of the microbiome in neonates promotes a long-lasting biological footprint, but how it impacts disease susceptibility in early life remains unknown. We hypothesized that feeding butyrate to pregnant mice influences the newborn’s susceptibility to biliary atresia, a severe cholang...

2022-01-11 | MTBLS2171 | MetaboLights
Liver biopsy samples were obtained from 64 infants with biliary atresia at the time of intraoperative cholangiogram. Liver biopsy samples were obtained from 14 age-matched infants with other causes of intrahepatic cholestasis, and from 7 deceased-donor children. GeneChip® Human Gene 1.0 ST Array (Af...
ORGANISM(S): Homo sapiens 
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...
ORGANISM(S): Homo sapiens 
Stool samples from four patients with Biliary atresia (BA) and three patients with neonatal cholestasis of other etiologies (non-BA) were analyzed by overlapping DIA-MS.
ORGANISM(S): Homo Sapiens (human) 
Biliary atresia (BA) is a rare cholestatic disease of unknown etiology that affects infants and shows an incidence of 1 out of 18,000 live births in Europe (1). The first therapeutic option is a timely performed portoenterostomy. However, the majority of patients suffer from a progressive inflammato...
ORGANISM(S): Mus musculus 
We report label-free quantification of xenobiotic metabolizing enzymes (XME), transporters, redox enzymes, proteases and nucleases in 25 human liver microsomal samples, taken from patients with biliary atresia. Nearly 3500 proteins were identified and quantified. These data can be used in physiolog...
ORGANISM(S): Homo sapiens (Human) 
2021-09-09 | PXD020974 | Pride
Early diagnosis of biliary atresia (BA) is crucial for improving the chances of survival and preserving the liver function of children with BA. To explore potential biomarkers for early diagnosis of BA with other non-BA jaundice cases, we performed proteomic analysis with data-independent acquisitio...
ORGANISM(S): Homo sapiens (Human) 
2024-06-22 | PXD048938 | Pride
Biliary organoids uncover delayed epithelial development and barrier function in biliary atresia.
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