Biliary Atresia

Bassett, Mikelle; Murray, Karen F.

doi:10.1097/mcg.0b013e3181646730

Cited by 66 publications

(21 citation statements)

References 137 publications

(143 reference statements)

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“…Affected newborns exhibit evidence of biliary obstruction within the first few months of life. BA is manifested by impaired liver function and fibroinflammatory obliterative cholangiopathy of both intrahepatic and extrahepatic bile ducts[2,3]. Extrahepatic BA is the most common form of ductal cholestasis.…”

Section: Introductionmentioning

confidence: 99%

Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia

Udomsinprasert

Honsawek

Jirathanathornnukul

et al. 2016

WJH

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AIMTo investigate serum urokinase-type plasminogen activator receptor (uPAR) and liver stiffness in biliary atresia (BA) and examine the correlation of circulating uPAR, liver stiffness, and clinical outcomes in postoperative BA children.METHODSEighty-five postKasai BA children and 24 control subjects were registered. Circulating uPAR was measured using enzyme-linked immunosorbent essay. Liver stiffness was analyzed using transient elastography.RESULTSBA children had significantly greater circulating uPAR and liver stiffness scores than control subjects (P < 0.001). Circulating uPAR and liver stiffness were substantially higher in jaundiced BA children than non-jaundiced BA children (P < 0.001). In addition, circulating uPAR was positively associated with serum aspartate aminotransferase (r = 0.507, P < 0.001), alanine aminotransferase (r = 0.364, P < 0.001), total bilirubin (r = 0.559, P < 0.001), alkaline phosphatase (r = 0.325, P < 0.001), and liver stiffness scores (r = 0.508, P < 0.001).CONCLUSIONCirculating uPAR and liver stiffness values were greater in BA children than healthy controls. The increased circulating uPAR was associated with liver dysfunction in BA. As a consequence, serum uPAR and liver stiffness may be used as noninvasive biomarkers indicating the progression of liver fibrosis in postKasai BA.

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Section: Introductionmentioning

confidence: 99%

Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia

Udomsinprasert

Honsawek

Jirathanathornnukul

et al. 2016

WJH

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“…In addition, we found Interleukin 8, Interferon (IFN) receptor 1 and Interleukin receptor 10 were upregulated in gallbladder cells. The immunologic properties of bile duct cells have been long considered and various inflammatory diseases such as primary biliary cirrhosis (47) and biliary atresia (10) directly affect bile duct cells. They also play a causal role in liver allograft rejection (48).…”

Section: Discussionmentioning

confidence: 99%

“…The evaluation of human fetal gallbladder stem cells would have important ramifications for the study of congenital diseases such as biliary atresia (10) and gallbladder carcinoma, the most common malignancy of the bile duct system (11), because of the importance of stem cells in development, tissue regeneration and cancer (12). In addition, a comparison of human fetal gallbladder and IHBD cells would further elucidate the ontogeny of bile duct cells and represent the first time that the developmental differences between the human IHBD and EHBD cells have been explored.…”

Section: Introductionmentioning

confidence: 99%

Identification of a candidate stem cell in human gallbladder

Manohar

Fohrer

et al. 2015

Stem Cell Research

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There are currently no reports of the identification of stem cells in the human gallbladder. The differences between human gallbladder and intrahepatic bile duct (IHBD) cells have also not been explored. The goals of this study were to evaluate if human fetal gallbladder contains a candidate stem cell population and if fetal gallbladder cells are distinct from fetal IHBD cells. We found that EpCAM+CD44+CD13+ cells represent the cell population most enriched for clonal self-renewal from primary gallbladder. Primary EpCAM+CD44+CD13+ cells gave rise to EpCAM+CD44+CD13+ and EpCAM+CD44+CD13− cells in vitro, and gallbladder cells expanded in vitro exhibited short-term engraftment in vivo. Last, we found that CD13, CD227, CD66, CD26 and CD49b were differentially expressed between gallbladder and IHBD cells cultured in vitro indicating clear phenotypic differences between the two cell populations. Microarray analyses of expanded cultures confirmed that both cell types have unique transcriptional profiles with predicted functional differences in lipid, carbohydrate, nucleic acid and drug metabolism. In conclusion, we have isolated a distinct clonogenic population of epithelial cells from primary human fetal gallbladder with stem cell characteristics and found it to be unique compared to IHBD cells.

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“…La atresia de vías biliares (AVB) es una colangiopatía inflamatoria, progresiva y esclerosante, que afecta en grado variable segmentos de la vía biliar intra y extrahepática 1 . Es propia del período neonatal y se manifiesta con ictericia colestásica, acolia y hepatomegalia 2 .…”

Section: Introductionunclassified

“…La AVB es una enfermedad poco frecuente, su incidencia a nivel global varía entre 1/8.000 a 1/18.000 recién nacidos vivos 1 . Se ha descrito un leve predominio en pacientes de género femenino y en países de origen asiático 4 .…”

Section: Introductionunclassified

Seguimiento de pacientes con atresia biliar: cirugía tardía y desarrollo de lagunas biliares

Sáez

Almeida

Gana

et al. 2017

Rev. chil. pediatr.

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Palabras clave:Atresia vías biliares; portoenterostomía; lagunas biliares; colangitis ResumenLas dilataciones quísticas de las vías biliares, conocidas como lagunas biliares, se han descrito en el seguimiento de los pacientes sometidos a portoenterostomía o cirugía de Kasai por atresia de las vías biliares (AVB). Su aparición se ha asociado con el desarrollo de colangitis y consecuentemente a un peor pronóstico. Objetivo: Describir las características clínicas y pronóstico de pacientes con AVB, sometidos a cirugía de Kasai, con énfasis en aquellos que desarrollaron lagunas biliares. Pacientes y Método: Estudio retrospectivo de pacientes sometidos a portoenterostomía de Kasai por AVB durante los años 2008 a 2016. Se tabuló la información demográfica y variables asociadas a la portoenterostomía de Kasai, trasplante hepático, lagunas o quistes biliares y episodios de colangitis. Se crearon curvas de Kaplan Meier y comparación mediante el test de Log Rank para evaluar sobrevida global, libre de colangitis y con hígado nativo, considerando un valor p como significativo. Resultados: Se analizó el seguimiento de 13 pacientes. El tiempo promedio de realización del Kasai fue a los 85 meses (rango 42-193, DS 40,3), seis pacientes (46%) fueron sometidos a cirugía de Kasai después de los 90 días de vida por derivación tardía. Cuatro (31%) desarrollaron lagunas biliares múltiples, todos presentaron episodios de colangitis. La sobrevida libre de colangitis fue significativamente menor para los portadores de lagunas biliares. Nueve pacientes (69%) recibieron un trasplante hepático, en promedio a los 16 meses de edad (rango 6-40, DS 12,1), en 3 de ellos la causa fue colangitis recurrente. No se encontraron diferencias significativas en la sobrevida con hígado nativo ni en sobrevida global entre portadores y no portadores de lagunas biliares. Conclusiones: En esta cohorte, la incidencia de lagunas biliares luego de la portoenterostomía es similar a la descrita en la literatura. Los resultados concuerdan con la relación propuesta entre ellas y el desarrollo de colangitis. En los pacientes de nuestra serie, el diagnóstico y derivación de AVB fue realizado tardíamente, determinando un peor pronóstico.

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Biliary Atresia

Cited by 66 publications

References 137 publications

Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia

Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia

Identification of a candidate stem cell in human gallbladder

Seguimiento de pacientes con atresia biliar: cirugía tardía y desarrollo de lagunas biliares

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