Alagille syndrome and non-syndromic paucity of the intrahepatic bile ducts
نویسندگان
چکیده
منابع مشابه
A 10-year-old child presenting with syndromic paucity of bile ducts (Alagille syndrome): a case report
BACKGROUND Alagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests with five major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects, and peripheral pulmonary stenosis. Globally, only 500 cases have so far been reported, with only five cases reported in the Indian subcontinent. Rarely, Alagille syndro...
متن کاملPaucity of Intrahepatic Bile Ducts in Neonates: the First Case Series from Iran
OBJECTIVE Paucity of interlobular bile ducts (PIBD), defined as absence or marked decrease in the number of interlobular bile ducts, is one of the causes of neonatal cholestasis. Treatment includes treating the intractable pruritus caused by persistent cholestasis. PIBD can be part of a familial syndrome of cholestasis named Alagille syndrome (AGS). We report clinical status of a case series of...
متن کاملIntrahepatic Transposition of Bile Ducts
Objective. To describe the intrahepatic bile duct transposition (anatomical variation occurring in intrahepatic ducts) and to determine the frequency of this variation. Material and Methods. The researches were performed randomly on 100 livers of adults, both sexes. Main research methods were anatomical macrodissection. As a criterion for determination of variations in some parts of bile tree, ...
متن کاملCongenital absence of the intrahepatic bile ducts.
Routine histological examination of the liver during the acute stages of neonatal obstructive jaundice, whether of biopsy or of autopsy specimens, reveals a variety of different pathological conditions. Congenital absence of the intrahepatic bile ducts appears to be a rare cause of obstructive jaundice in the newborn. The purpose of this paper is to draw attention to the condition by reporting ...
متن کاملJagged1 in the portal vein mesenchyme regulates intrahepatic bile duct development: insights into Alagille syndrome.
Mutations in the human Notch ligand jagged 1 (JAG1) result in a multi-system disorder called Alagille syndrome (AGS). AGS is chiefly characterized by a paucity of intrahepatic bile ducts (IHBD), but also includes cardiac, ocular, skeletal, craniofacial and renal defects. The disease penetration and severity of the affected organs can vary significantly and the molecular basis for this broad spe...
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ژورنال
عنوان ژورنال: Translational Gastroenterology and Hepatology
سال: 2021
ISSN: 2415-1289
DOI: 10.21037/tgh-2020-03