دورية أكاديمية
Cholestatic Liver Disease in Late Childhood: A Report of Two Rare Cases
| العنوان: | Cholestatic Liver Disease in Late Childhood: A Report of Two Rare Cases |
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| المؤلفون: | Sangeeta kini, Azmat Momin, Rima N Kamat, Sameet Patel, Pravin N Rathi |
| المصدر: | Journal of Clinical and Diagnostic Research, Vol 17, Iss 11, Pp 01-03 (2023) |
| بيانات النشر: | JCDR Research and Publications Private Limited, 2023. |
| سنة النشر: | 2023 |
| المجموعة: | LCC:Medicine |
| مصطلحات موضوعية: | benign recurrent intrahepatic cholestasis, progressive familial intrahepatic cholestasis type 3, Medicine |
| الوصف: | Cholestatic liver disease in late childhood has a comprehensive list of aetiologies, requiring a multidimensional approach. Among these, the genetic aetiology can range from having a self-limiting course to being associated with morbidity and mortality, requiring liver transplantation. Progressive Familial Intrahepatic Cholestasis (PFIC) and Benign Recurrent Intrahepatic Cholestasis (BRIC) are two rare inherited autosomal recessive cholestatic disorders. Both are related to mutations in the bile formation transport system and typically manifest in infancy or early childhood. Among the PFIC types, The PFIC type 3 rapidly progresses to end-stage liver disease, while BRIC follows a benign course with intermittent asymptomatic periods. Here, authors describe two (17-year-old male, 19-year-old male) such unusual inherited autosomal recessive cholestatic disorders in late childhood: PFIC (type 3) and BRIC, which have distinct clinical presentations and liver function derangements. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2249-782X 0973-709X |
| Relation: | https://www.jcdr.net/articles/PDF/18663/64846_CE[Ra1]_F(SS)_QC&Ref(AN_SS)_PF1(RI_OM)_PFA_NC(RI_KM)_PN(KM).pdf; https://doaj.org/toc/2249-782X; https://doaj.org/toc/0973-709X |
| DOI: | 10.7860/JCDR/2023/64846.18663 |
| URL الوصول: | https://doaj.org/article/cc4bcd4812244792b1e98ed092cc0cf7 |
| رقم الأكسشن: | edsdoj.4bcd4812244792b1e98ed092cc0cf7 |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 2249782X 0973709X |
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| DOI: | 10.7860/JCDR/2023/64846.18663 |