التفاصيل البيبلوغرافية
| العنوان: |
Progressive familial intrahepatic cholestasis—outcome and time to transplant after biliary diversion according to genetic subtypes |
| المؤلفون: |
Abdulla Sahloul, Elke Lainka, Simone Kathemann, Sandra Swoboda, Carola Dröge, Verena Keitel, Yahya Saleh Al-Matary, Michael Berger, Maren Schulze |
| المصدر: |
Frontiers in Surgery, Vol 10 (2023) |
| بيانات النشر: |
Frontiers Media S.A., 2023. |
| سنة النشر: |
2023 |
| المجموعة: |
LCC:Surgery |
| مصطلحات موضوعية: |
biliary diversion, morbus byler, pediatric liver transplantation (pediatric LT), progressive familial intrahepatic cholestasis (PFIC), pediatric surgery, liver disease, Surgery, RD1-811 |
| الوصف: |
BackgroundProgressive familial intrahepatic cholestasis (PFIC) is a heterogeneous disease characterized by progressive cholestasis in early childhood. Surgical therapy aims at preventing bile absorption either by external or internal biliary diversion (BD). Several different genetic subtypes encode for defects in bile transport proteins, and new subtypes are being discovered ongoingly. Overall, the literature is scarce, however, accumulating evidence points to PFIC 2 having a more aggressive course and to respond less favorable to BD. With this knowledge, we aimed to retrospectively analyze the long-term outcome of PFIC 2 compared to PFIC 1 following BD in children at our center.MethodsClinical data and laboratory findings of all children with PFIC, who were treated and managed in our hospital between 1993 and 2022, were analyzed retrospectively.ResultsOverall, we treated 40 children with PFIC 1 (n = 10), PFIC 2 (n = 20) and PFIC 3 (n = 10). Biliary diversion was performed in 13 children (PFIC 1, n = 6 and 2, n = 7). Following BD, bile acids (BA) (p = 0.0002), cholesterol (p |
| نوع الوثيقة: |
article |
| وصف الملف: |
electronic resource |
| اللغة: |
English |
| تدمد: |
2296-875X |
| Relation: |
https://www.frontiersin.org/articles/10.3389/fsurg.2023.1074229/full; https://doaj.org/toc/2296-875X |
| DOI: |
10.3389/fsurg.2023.1074229 |
| URL الوصول: |
https://doaj.org/article/b7161b171bf043bea082795a27b329ed |
| رقم الانضمام: |
edsdoj.b7161b171bf043bea082795a27b329ed |
| قاعدة البيانات: |
Directory of Open Access Journals |
Full Text Finder