Etiology of biliary atresia as a developmental anomaly: recent advances

Nakamura, Kazuaki; Tanoue, Akito
June 2013
Journal of Hepato -- Biliary -- Pancreatic Sciences;Jun2013, Vol. 20 Issue 5, p459
Academic Journal
Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy affecting the extra- and intrahepatic biliary tree to various degrees and resulting in obstructive bile flow, cholestasis and icterus in neonates. It is the most common cause of pediatric liver transplantation. The etiology of BA is still unclear, although there is some evidence pointing to viral, toxic, and multiple genetic factors. For new therapeutic options other than liver transplantation to be developed, a greater understanding of the pathogenesis of BA is indispensable. The fact that the pathology of BA develops during a period of biliary growth and remodeling suggests an involvement of developmental anomalies. Recent studies indicate an association of the etiology of BA with some genetic factors such as laterality genes, epigenetic regulation and/or microRNA function. In this paper, we present an overview of recent advances in the understanding of the disease focusing on bile duct developmental anomaly.


Related Articles

  • Cystic Biliary Atresia: A Wolf in Sheep's Clothing. HILL, SARAH J.; CLIFTON, MATTHEW S.; DERDERIAN, SARKIS C.; WULKAN, MARK L.; RICKETTS, RICHARD R. // American Surgeon;Sep2013, Vol. 79 Issue 9, p870 

    Neonatal obstructive jaundice is frequently explained by biliary atresia (BA) or the presence of a choledochal cyst (CC). Cystic biliary atresia (CBA) has been a proposed as a subtype of BA with projected improved outcomes. We aimed to characterize these lesions further. We conducted an...

  • Liver transplantation in a[sub 1] -antitrypsin deficiency. N�meth, A. // European Journal of Pediatrics;1999, Vol. 158 Issue 14, pS085 

    Abstract Only a minority of infants born with alpha[sub 1]-antitrypsin deficiency will develop serious liver disease during childhood, mostly but not always after neonatal cholestasis. Early prognosis is difficult and all children have to be followed up carefully. The liver disease progresses...

  • Partial internal biliary diversion for patients with progressive familial intrahepatic cholestasis type 1. Mochizuki, Kyoko; Obatake, Masayuki; Takatsuki, Mitsuhisa; Nakatomi, Akiko; Hayashi, Tomayoshi; Okudaira, Sadayuki; Eguchi, Susumu // Pediatric Surgery International;Jan2012, Vol. 28 Issue 1, p51 

    We herein report a case of progressive familial intrahepatic cholestasis with partial internal biliary diversion (PIBD). Although by using PIBD an external stoma can be avoided, exposure of the ileocecal junction to bile reflux as well as the effects of the direct bile flow on the colonic mucosa...

  • Recent trends in the diagnosis and management of biliary atresia in developing countries. Ramachandran, Priya; Safwan, Mohamed; Reddy, Mettu; Rela, Mohamed // Indian Pediatrics;Oct2015, Vol. 52 Issue 10, p871 

    Need and purpose of review: Biliary atresia is a progressive obstructive cholangiopathy and is fatal if left untreated within 2 years of life. Delay in referral is because of difficulties in differentiating it from physiologic jaundice and identifying an abnormal stool color. This paper presents...

  • Intrapulmonary vascular dilation in children with chronic liver diseases: pre- and post-liver transplantation. Anant Khositseth; Suporn Treepongkaruna; Khemika Khemakanok; Sumate Teeraratkul; Vichai Pansrimangkorn; Chollasak Thirapattaraphan; Surasak Leelaudomlipi // Annals of Hepatology: Official Journal of the Mexican Associatio;Jan/Feb2016, Vol. 15 Issue 1, p47 

    Background and study aims. Chronic liver disease (CLD) can cause hepatopulmonary syndrome (HPS), defined as triad of liver disease, hypoxemia, and intrapulmonary vascular dilation (IPVD). The aim of this study was to determine the evidence of IPVD in a cohort of pediatric patients with CLD pre-...

  • Biliary Atresia: 50 Years after the First Kasai. Wildhaber, Barbara E. // ISRN Surgery;2012, p1 

    Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of...

  • Experience with choledochal cysts in infants. Vijayaraghavan, Paari; Lal, Richa; Sikora, Sadiq; Poddar, Ujjal; Yachha, Surender // Pediatric Surgery International;Oct2006, Vol. 22 Issue 10, p803 

    The purpose of this analysis is to study the clinical features, liver histology and the outcome after surgery in infants with choledochal cysts (CDCs), and to draw conclusions on the management strategy accordingly. The clinical features, liver histology and the outcome after surgery in 14 cases...

  • Biliary atresia type I cyst and choledochal cust: can we differentiate or not? Suzuki, Tatsuya; Hashimoto, Takashi; Hussein, Mohamed; Hara, Fujio; Hibi, Masahito; Kato, Takazumi // Journal of Hepato -- Biliary -- Pancreatic Sciences;Jun2013, Vol. 20 Issue 5, p465 

    Background/purpose: It is difficult to discriminate between choledochal cust with obstructive jaundice and biliary atresia with a cyst at the porta hepatis in neonates or young infants. This review evaluates whether it is possible to differentiate between these two diseases. We here also provide...

  • Prednisolone.  // Reactions Weekly;7/21/2012, Issue 1411, p36 

    The article presents a case study of a male neonate who developed cholestasis after treatment with prednisolone for Kasabach-Merritt phenomenon.


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics