Intraoperative view of complete extrahepatic biliary atresia[1]
Specialty
Pediatric surgery
Symptoms
Jaundice, pale stool, dark urine
Complications
Cirrhosis, portal hypertension, liver failure
Types
Congenital, acquired
Treatment
Surgery, liver transplantation
Frequency
1 in 5,000 (East Asia), 1 in 10,000-15,000 (US)
Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. It has an incidence of one in 10,000–15,000 live births in the United States,[2] and a prevalence of one in 16,700 in the British Isles.[3][4] Biliary atresia is most common in East Asia, with a frequency of one in 5,000.
The cause of biliary atresia in Egyptian infants has been proven to be as a result of aflatoxin induced cholangiopathy acquired prenatally in infants who have glutathione S transferase M1 deficiency. The biliary atresia phenotype caused by congenital aflatoxicosis in GST M1 deficient neonates is named Kotb disease.[5] Syndromic biliary atresia (e.g. Biliary Atresia Splenic Malformation (BASM)) has been associated with certain genes (e.g. Polycystic Kidney Disease 1 Like 1 - PKD1L1[6]), and some infants with isolated biliary atresia may arise as a result of an autoimmune inflammatory response, possibly due to a viral infection of the liver soon after birth.[7] In animals plant toxins have been shown to cause biliary atresia.[8] The only effective treatments[9] are operations such as the Kasai procedure and liver transplantation.[10]
^Suchy, Frederick J. (2015). "Anatomy, Histology, Embryology, Developmental Anomalies, and Pediatric Disorders of the Biliary Tract". In Feldman, Mark; Friedman, Lawrence S.; Brandt, Lawrence J. (eds.). Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management (10th ed.). Elsevier Health Sciences. pp. 1055–77. ISBN 978-1-4557-4989-8.
^McKiernan, Patrick J; Baker, Alastair J; Kelly, Deirdre A (2000). "The frequency and outcome of biliary atresia in the UK and Ireland". The Lancet. 355 (9197): 25–9. doi:10.1016/S0140-6736(99)03492-3. PMID 10615887. S2CID 25981400.
^Hartley, Jane L; Davenport, Mark; Kelly, Deirdre A (2009). "Biliary atresia". The Lancet. 374 (9702): 1704–13. doi:10.1016/S0140-6736(09)60946-6. PMID 19914515. S2CID 24191796.
^Kotb, Magd A.; Kotb, Ahmed (March 2015). "Extrahepatic Biliary Atresia is an Aflatoxin Induced Cholangiopathy in Infants with Null GSTM1 Genotype with Disrupted P53 and GSTPi to Mothers Heterozygous for GSTM1 Polymorphism: Damage Control is Mediated through Neutrophil Elastase and CD14+ Activated Monocytes: Kotb Disease" (PDF). The Medical Journal of Cairo University. 83 (2): 137–145.
^Berauer, John‐Paul; Mezina, Anya I.; Okou, David T.; Sabo, Aniko; Muzny, Donna M.; Gibbs, Richard A.; Hegde, Madhuri R.; Chopra, Pankaj; Cutler, David J.; Perlmutter, David H.; Bull, Laura N.; Thompson, Richard J.; Loomes, Kathleen M.; Spinner, Nancy B.; Rajagopalan, Ramakrishnan; Guthery, Stephen L.; Moore, Barry; Yandell, Mark; Harpavat, Sanjiv; Magee, John C.; Kamath, Binita M.; Molleston, Jean P.; Bezerra, Jorge A.; Murray, Karen F.; Alonso, Estella M.; Rosenthal, Philip; Squires, Robert H.; Wang, Kasper S.; Finegold, Milton J.; Russo, Pierre; Sherker, Averell H.; Sokol, Ronald J.; Karpen, Saul J. (September 2019). "Identification of Polycystic Kidney Disease 1 Like 1 Gene Variants in Children With Biliary Atresia Splenic Malformation Syndrome". Hepatology. 70 (3): 899–910. doi:10.1002/hep.30515. PMC 6642859. PMID 30664273.
^Mack, Cara (2007). "The Pathogenesis of Biliary Atresia: Evidence for a Virus-Induced Autoimmune Disease". Seminars in Liver Disease. 27 (3): 233–42. doi:10.1055/s-2007-985068. PMC 3796656. PMID 17682970.
^Koo, Kyung A.; Lorent, Kristin; Gong, Weilong; Windsor, Peter; Whittaker, Stephen J.; Pack, Michael; Wells, Rebecca G.; Porter, John R. (17 August 2015). "Biliatresone, a Reactive Natural Toxin from Dysphania glomulifera and D. littoralis: Discovery of the Toxic Moiety 1,2-Diaryl-2-Propenone". Chemical Research in Toxicology. 28 (8): 1519–1521. doi:10.1021/acs.chemrestox.5b00227. PMC 4755499. PMID 26175131.
^Superina, Riccardo (December 2017). "Biliary atresia and liver transplantation: results and thoughts for primary liver transplantation in select patients". Pediatric Surgery International. 33 (12): 1297–1304. doi:10.1007/s00383-017-4174-4. PMID 29030698. S2CID 23189323.
^Lien, Tien-Hau; Chang, Mei-Hwei; Wu, Jia-Feng; Chen, Huey-Ling; Lee, Hung-Chang; Chen, An-Chyi; Tiao, Mao-Meng; Wu, Tzee-Chung; Yang, Yao-Jong; Lin, Chieh-Chung; Lai, Ming-Wei; Hsu, Hong-Yuan; Ni, Yen-Hsuan (2011). "Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan". Hepatology. 53 (1): 202–8. doi:10.1002/hep.24023. PMID 21140377. S2CID 13397641.
Biliaryatresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or...
is the standard treatment for Anotia. Biliaryatresia (BA) is a rare disease marked by an unknown-origin biliary obstruction that manifests in the neonatal...
to relieve the symptoms of biliary obstruction as well as allows the bile duct to drain. In infants with biliaryatresia, hepatoportoenterostomy is an...
duct-paucity syndromes. It is contraindicated in obstruction of biliary tracts such as biliaryatresia. It is not effective in liver allograft rejection, and in...
been identified as toxins, including biliatresone which may cause biliaryatresia when infants are exposed to the plant product. The isoflavonoid group...
cholangitis) obstruction, perforation, fistula of biliary tract spasm of sphincter of Oddi biliary cyst biliaryatresia GBD 2013 Mortality and Causes of Death,...
condition. There are also many pediatric liver diseases, including biliaryatresia, alpha-1 antitrypsin deficiency, alagille syndrome, progressive familial...
pathologic causes, 35-40% of neonatal cholestasis cases are caused by biliaryatresia. This is one of the most common causes for neonatal cholestasis. Metabolic...
the biliary tree, which is responsible for bile production, even when the gall bladder is functional and morphologically normal. This biliaryatresia can...
jaundice. Pancreatic cancer of the pancreatic head Biliary tract strictures Biliaryatresia Primary biliary cholangitis Cholestasis of pregnancy Acute Pancreatitis...
PMC 2137053. PMID 18079549. Govindarajan, Krishna Kumar (2016-12-28). "Biliaryatresia: Where do we stand now?". World Journal of Hepatology. 8 (36): 1593–1601...
1963 by Dr. Thomas E. Starzl on a three-year-old male afflicted with biliaryatresia after perfecting the technique on canine livers. Baruch S. Blumberg...
jaundice is not resolved with simple phototherapy, other causes such as biliaryatresia, Progressive familial intrahepatic cholestasis, bile duct paucity,...
other developmental abnormalities such as intestinal malrotation or biliaryatresia, or cardiac abnormalities, such as dextrocardia. These accessory spleens...
Cardiac defects (such as TAPVR) Micrognathia (smaller jaw) Hernias Biliaryatresia Rarer malformations can affect almost any organ Intellectual disability...
liver transplantation, a hepato-pancreato-biliary surgeon, and the chairman of the Max Center of Liver and Biliary Sciences at Max Healthcare, Saket. Dr....
three children, Demelza, Timmy, and Tommy. Timmy was diagnosed with biliaryatresia upon birth. The condition required a liver transplant for survival...
choledochoceles (cystic dilation of the ampula of Vater (3–8 cm)), and biliaryatresia. Obstruction of the common bile duct and related jaundice has been...
more awareness of organ donation when their son, who suffered from biliaryatresia, urgently needed a transplant. While he also became the first child...
hepatoportoenterostomy, to address a life-threatening birth defect known as biliaryatresia. The modern form of the operation is still known as the Kasai procedure...
cases of intestinal malabsorption such as cystic fibrosis, congenital biliaryatresia, intestinal resection, and abetalipoproteinemia (Bassen-Kornzweig syndrome)...
and rarely with Parkinson's disease. Erythroblastosis fetalis and biliaryatresia are diseases which may cause teeth to appear green from the deposition...
that forms the pyloric sphincter, obstructing the passage of food. Biliaryatresia is a congenital defect where the common bile duct, which connects the...
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