کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8809574 | 1607029 | 2018 | 8 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Biliary atresia recent insight
ترجمه فارسی عنوان
بینش اخیر آترزی صفراوی
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کلمات کلیدی
ERCPNPVGGTKPEUDCAPPVMRCPEndoscopic retrograde cholangiopancreatography - cholangiopancreatography رتروگراد endoscopicKasai portoenterostomy - Kasai portoostostomyAlkaline phosphatase - آلکالین فسفاتاز یا فسفاتاز قلیاییHuman leukocyte antigen - آنتی ژن لوسکسی انسانHLA - آنتیژن گلبول سفید انسانیpositive predictive value - ارزش پیش بینی مثبتnegative predictive value - ارزش پیش بینی منفیUrsodeoxycholic acid - اورسودوکسی کولیک اسید، اورسودیولinterleukin - اینترلوکینmagnetic resonance cholangiopancreatography - تشدید مغناطیسی cholangiopancreatographyHUS - خانهBASM - داستانHAD - داشته استDDT - دیکرو دیفنیل تری کلرواتانHigh frequency ultrasound - سونوگرافی فرکانس بالاCMV - سیتومگالوویروسcytomegalovirus - سیتومگالوویروسBiliary Atresia Splenic Malformation - ضعف پلورالی آپریزی صفراویUltrasound - فراصوتLiver transplantation - پیوند کبدNeonatal cholestasis - کلستاز نوزادانgallbladder - کیسه صفراgamma-glutamyl transpeptidase - گاما گلوتامیل ترانسپپتیداز
موضوعات مرتبط
علوم پزشکی و سلامت
پزشکی و دندانپزشکی
پریناتولوژی (پزشکی مادر و جنین)، طب اطفال و بهداشت کودک
چکیده انگلیسی
Biliary atresia (BA) is a rare disease characterized by ascending obstruction of bile ducts that exclusively affects newborn infants. The etiology of the disease is not known. BA is considered to be a phenotype resulting from several pathogenic processes leading to obstruction of the biliary tree. It usually presents shortly after birth, characterized by persistent jaundice, hepatosplenomegaly, clay-colored stool, and dark urine. It affects both the extra-hepatic biliary ducts (EHBDs) and the intra-hepatic biliary system (IHBDs), but the former is more severely affected. Diagnosis of BA is a great challenge and must be achieved as early as possible to delay progression to cirrhosis. Laboratory tests reveal direct hyperbilirubinemia and, variable levels of transaminases, gamma-glutamyl transpeptidase (GGT), and alkaline phosphatase (ALP), which overlap significantly with other causes of neonatal cholestasis. The intraoperative cholangiogram is considered the gold standard for the diagnosis of BA and is performed routinely in many institutions. BA can be divided into correctable and non-correctable types; the former accounts for (10-15%) of cases, in which the proximal common hepatic duct is patent, allowing primary anastomosis of the EHBDs to the bowel. All patients are subjected to identical surgical and medical treatments; consisting of Kasai portoenterostomy (KPE), which entails removal of the atretic extra-hepatic tissue and a Roux-en-Y jejunal loop anastomosed to the hepatic hilum. Kasai portoenterstomy is considered a transition to liver transplantation, as the pathology may be still ongoing. BA is the most frequent indication for liver transplantation in infants, which is the only treatment that can definitively arrest the natural disease course. In conclusion: BA is a serious liver disease that needs to be further studied, and awareness of BA should be increased among the public and health care workers to prevent the complications of this disease.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Egyptian Pediatric Association Gazette - Volume 66, Issue 1, March 2018, Pages 1-8
Journal: Egyptian Pediatric Association Gazette - Volume 66, Issue 1, March 2018, Pages 1-8
نویسندگان
Ramy Mohamed Ghazy, Nermin M. Adawy, Mohamed Ahmed Khedr, Mohamed Mostafa Tahoun,