Biliary atresia causes: Difference between revisions
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==Causes== | ==Causes== | ||
Biliary atresia does not have a clear identified cause. It was previously thought to be idiopathic, a destructive inflammatory process leaving fibrotic remnants at the porta hepatis. It is now thought it may be secondary to viral infections or an autoimmune-induced injury in some cases. | Biliary atresia does not have a clear identified cause. It was previously thought to be idiopathic, a destructive inflammatory process leaving fibrotic remnants at the porta hepatis. It is now thought it may be secondary to viral infections or an autoimmune-induced injury in some cases. The viruses thought to be involved are: | ||
*Reovirus, | *Reovirus, | ||
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**The highest association of a viral infection with biliary atresia is with CMV. There may be an inability to identify the virus because of a short timeframe to detect it, which leads to uncertainty in association. The viral infection of cholangiocytes may predispose to an aberrant autoimmune response, cascading to progressive biliary injury and cirrhosis. | **The highest association of a viral infection with biliary atresia is with CMV. There may be an inability to identify the virus because of a short timeframe to detect it, which leads to uncertainty in association. The viral infection of cholangiocytes may predispose to an aberrant autoimmune response, cascading to progressive biliary injury and cirrhosis. | ||
<br /> | === Genetic cause === | ||
Through a genome-wide study, an association between biliary atresia and the ''ADD3'' gene was detected in Chinese populations and confirmed in Thai populations. Anther possible association with the deletion of the ''GPC1'' gene has been reported, which encodes a glypican 1-a heparan sulfate proteoglycan.{{Cite book}} | |||
=== Environmental cause === | |||
Aflatoxin B1 exposure, and to a lesser extent aflatoxin B2 exposure during late pregnancy may cause biliary atresia. Maternal detoxification ability protects the fetus during intrauterine life, but it struggles with the aflatoxin in its blood and liver after birth, causing cholangitis in the baby.{{Cite journal|Kotb, Magd A. (March 2015). "Aflatoxins in Infants with Extrahepatic Biliary Atresia". The Medical Journal of Cairo University. 83 (1): 207–210.=https://scholar.cu.edu.eg/?q=magdkotb/files/aflatoxins_in_biliary_atresia.pdf}}<br /> | |||
==References== | ==References== | ||
Revision as of 18:59, 8 April 2022
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
There have been many theories about etiopathogenesis such as Reovirus 3 infection, congenital malformation, congenital CMV infection, autoimmune theory. This means that the etiology and pathogenesis of biliary atresia are largely unknown.
Causes
Biliary atresia does not have a clear identified cause. It was previously thought to be idiopathic, a destructive inflammatory process leaving fibrotic remnants at the porta hepatis. It is now thought it may be secondary to viral infections or an autoimmune-induced injury in some cases. The viruses thought to be involved are:
- Reovirus,
- Rotavirus,
- Cytomegalovirus (CMV)[1][2]
- The highest association of a viral infection with biliary atresia is with CMV. There may be an inability to identify the virus because of a short timeframe to detect it, which leads to uncertainty in association. The viral infection of cholangiocytes may predispose to an aberrant autoimmune response, cascading to progressive biliary injury and cirrhosis.
Genetic cause
Through a genome-wide study, an association between biliary atresia and the ADD3 gene was detected in Chinese populations and confirmed in Thai populations. Anther possible association with the deletion of the GPC1 gene has been reported, which encodes a glypican 1-a heparan sulfate proteoglycan.Empty citation (help)
Environmental cause
Aflatoxin B1 exposure, and to a lesser extent aflatoxin B2 exposure during late pregnancy may cause biliary atresia. Maternal detoxification ability protects the fetus during intrauterine life, but it struggles with the aflatoxin in its blood and liver after birth, causing cholangitis in the baby.Empty citation (help)
References
- ↑ Brindley SM, Lanham AM, Karrer FM, Tucker RM, Fontenot AP, Mack CL (2012). "Cytomegalovirus-specific T-cell reactivity in biliary atresia at the time of diagnosis is associated with deficits in regulatory T cells". Hepatology. 55 (4): 1130–8. doi:10.1002/hep.24807. PMC 3319336. PMID 22105891.
- ↑ Xu Y, Yu J, Zhang R, Yin Y, Ye J, Tan L; et al. (2012). "The perinatal infection of cytomegalovirus is an important etiology for biliary atresia in China". Clin Pediatr (Phila). 51 (2): 109–13. doi:10.1177/0009922811406264. PMID 22144720.