MCID: BLR001
MIFTS: 50

Biliary Atresia

Categories: Gastrointestinal diseases, Liver diseases, Rare diseases

Aliases & Classifications for Biliary Atresia

MalaCards integrated aliases for Biliary Atresia:

Name: Biliary Atresia 12 74 52 36 54 43 15 71
Isolated Atresia of Bile Ducts 52
Non-Syndromic Biliary Atresia 52
Biliary Atresia, Congenital 12
Congenital Biliary Atresia 12
Isolated Biliary Atresia 52
Atresia of Bile Duct 12

Classifications:



External Ids:

Disease Ontology 12 DOID:13608
KEGG 36 H01855
ICD9CM 34 751.61
MeSH 43 D001656
NCIt 49 C34421
SNOMED-CT 67 77480004
ICD10 32 Q44.2
UMLS 71 C0005411

Summaries for Biliary Atresia

KEGG : 36 Biliary atresia (BA) is a congenital, fibro-obliterative obstructive cholangiopathy. It can be a devastating disease in infants invariably leading, if untreated, to cirrhosis, liver failure and death. Patients will be jaundiced and will have pale stools and dark urine. This is due to the inability to excrete conjugated bilirubin into the gastrointestinal tract, which is then excreted into the urine causing its color to darken. The cause of BA is not known with any degree of certainty, though there are a number of hypotheses. One prominent theory regarding the pathogenesis is that bile duct injury is initially caused by a viral infection, and then perpetuated by an autoimmune disorder. Surgical drainage, the only effective intervention, is successful only half of the time. Approximately 80% of patients require one or more liver transplantations with the associated morbidity and mortality issues caused by life-long transplant-related immunosuppression.

MalaCards based summary : Biliary Atresia, also known as isolated atresia of bile ducts, is related to biliary atresia, extrahepatic and bile acid synthesis defect, congenital, 2, and has symptoms including icterus An important gene associated with Biliary Atresia is GGT1 (Gamma-Glutamyltransferase 1), and among its related pathways/superpathways are Immune response IFN alpha/beta signaling pathway and Synthesis of bile acids and bile salts. The drugs Meloxicam and Cefoperazone have been mentioned in the context of this disorder. Affiliated tissues include liver, testes and bone, and related phenotypes are cardiovascular system and growth/size/body region

Disease Ontology : 12 A cholestasis characterized by blockage of the ducts that carry bile from the liver to the gallbladder.

NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 30391 Definition Biliary atresia is a rare, progressive obliterative cholangiopathy of the extrahepatic bile ducts, occuring in the embryonic/ perinatal period, leading to severe and persistent jaundice and acholic stool with an unfavorable course in the absence of treatment. Visit the Orphanet disease page for more resources.

Wikipedia : 74 Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a... more...

Related Diseases for Biliary Atresia

Diseases related to Biliary Atresia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 415)
# Related Disease Score Top Affiliating Genes
1 biliary atresia, extrahepatic 35.1 JAG1 GGT1
2 bile acid synthesis defect, congenital, 2 32.4 SLC10A1 NR1H4 ABCB11
3 esophageal varix 31.9 GPT GGT1 ALB
4 ascending cholangitis 31.8 GPT ALB
5 portal hypertension 31.8 TGFB1 NR1H4 GPT CCN2 ALB
6 cholangitis 31.5 NR1H4 GPT GGT1 ALB ABCB4 ABCB11
7 obstructive jaundice 31.5 TGFB1 HGF GPT GGT1 ALB
8 bile duct cysts 31.2 GPT GGT1 ATP8B1 ABCB4
9 bile duct disease 31.2 SLC10A2 SLC10A1 NR1H4 GPT GGT1 ATP8B1
10 liver cirrhosis 31.2 TIMP1 TGFB1 HGF GPT GGT1 CCN2
11 parenteral nutrition-associated cholestasis 31.0 GPT GGT1 ABCB4
12 hepatitis a 30.9 GPT GGT1 ALB
13 acute cholangitis 30.8 GPT GGT1 ALB
14 acute liver failure 30.7 HGF GPT ALB
15 bilirubin metabolic disorder 30.6 GPT GGT1 ATP8B1 ALB ABCB4 ABCB11
16 hepatic infarction 30.6 GPT ALB
17 patent ductus arteriosus 1 30.5 JAG1 CFC1 ALB
18 autoimmune hepatitis 30.5 GPT GGT1 ALB
19 protein-energy malnutrition 30.5 GPT GGT1 ALB
20 sclerosing cholangitis 30.5 NR1H4 GPT GGT1 ALB ABCB4 ABCB11
21 viral hepatitis 30.4 TGFB1 MX1 HGF GPT GGT1 ALB
22 hydronephrosis 30.4 TIMP1 TGFB1 ALB
23 hepatitis b 30.3 TGFB1 SLC10A1 MX1 GPT GGT1 ALB
24 gingival overgrowth 30.3 TIMP1 TGFB1 CCN2
25 kawasaki disease 30.3 TIMP1 GPT ALB
26 atrial heart septal defect 30.3 JAG1 CFC1 ALB
27 inherited metabolic disorder 30.2 NR1H4 GPT GGT1 ALB
28 alagille syndrome 1 30.2 JAG1 CFC1 ATP8B1 ABCB4 ABCB11
29 kidney disease 30.2 TGFB1 SPP1 HGF CCN2 ALB
30 extrahepatic cholestasis 30.2 SLC10A1 NR1H4 GPT GGT1 ABCB4 ABCB11
31 cholestasis 30.2 SLC10A2 SLC10A1 NR1H4 JAG1 GPT GGT1
32 splenic infarction 30.1 GPT ALB
33 alcoholic hepatitis 30.1 SPP1 HGF GPT GGT1 ALB
34 diarrhea 30.0 TGFB1 SLC10A2 GPT ALB
35 chronic kidney disease 30.0 TGFB1 SPP1 HGF CCN2 ALB
36 cholelithiasis 29.9 NR1H4 GPT GGT1 ALB ABCB4
37 atp8b1 deficiency 29.9 NR1H4 ATP8B1 ABCB11
38 pericholangitis 29.9 SLC10A2 SLC10A1 ABCB4 ABCB11
39 vascular disease 29.9 TIMP1 TGFB1 SPP1 NR1H4 ALB
40 liver disease 29.8 TGFB1 SLC10A1 NR1H4 HGF GPT GGT1
41 primary biliary cirrhosis 29.8 TIMP1 SLC10A1 NR1H4 GPT GGT1 ALB
42 non-alcoholic steatohepatitis 29.8 TGFB1 NR1H4 HGF GPT GGT1 CCN2
43 familial intrahepatic cholestasis 29.6 SLC10A2 NR1H4 GGT1 ATP8B1 ABCB4 ABCB11
44 progressive familial intrahepatic cholestasis 29.4 SLC10A2 SLC10A1 NR1H4 GGT1 ATP8B1 ABCB4
45 biliary tract disease 29.0 SLC10A2 SLC10A1 NR1H4 GPT GGT1 ATP8B1
46 hepatocellular carcinoma 28.9 TIMP1 TGFB1 SPP1 NR1H4 MMP7 JAG1
47 cholestasis, progressive familial intrahepatic, 3 28.8 SLC10A2 SLC10A1 NR1H4 JAG1 ATP8B1 ABCB4
48 cholangitis, primary sclerosing 28.6 SLC10A2 SLC10A1 NR1H4 JAG1 GPT GGT1
49 ichthyosis congenita with biliary atresia 12.5
50 biliary atresia with splenic malformation syndrome 12.5

Graphical network of the top 20 diseases related to Biliary Atresia:



Diseases related to Biliary Atresia

Symptoms & Phenotypes for Biliary Atresia

UMLS symptoms related to Biliary Atresia:


icterus

MGI Mouse Phenotypes related to Biliary Atresia:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.21 ABCB4 ADD3 ALB CCN2 HGF JAG1
2 growth/size/body region MP:0005378 10.13 ABCB11 ABCB4 ATP8B1 CCN2 GGT1 HGF
3 homeostasis/metabolism MP:0005376 10.07 ABCB11 ABCB4 ALB ATP8B1 CCN2 GGT1
4 endocrine/exocrine gland MP:0005379 10.02 ABCB4 ALB ATP8B1 CCN2 GGT1 JAG1
5 digestive/alimentary MP:0005381 10.01 ABCB4 ALB CCN2 JAG1 MMP7 NR1H4
6 liver/biliary system MP:0005370 9.9 ABCB11 ABCB4 ALB ATP8B1 CCN2 HGF
7 neoplasm MP:0002006 9.5 ABCB4 ALB MMP7 NR1H4 SPP1 TGFB1
8 skeleton MP:0005390 9.32 ABCB4 ATP8B1 CCN2 GGT1 GPT JAG1

Drugs & Therapeutics for Biliary Atresia

Drugs for Biliary Atresia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 83)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Meloxicam Approved, Vet_approved Phase 4 71125-38-7 54677470 5281106
2
Cefoperazone Approved, Investigational Phase 4 62893-19-0 44185
3
Meropenem Approved, Investigational Phase 4 96036-03-2, 119478-56-7 441130 64778
4
Teicoplanin Approved, Investigational Phase 4 61036-62-2
5
Tinidazole Approved, Investigational Phase 4 19387-91-8 5479
6 Anti-Inflammatory Agents Phase 4
7 Analgesics Phase 4
8 Analgesics, Non-Narcotic Phase 4
9 Cyclooxygenase 2 Inhibitors Phase 4
10 Cyclooxygenase Inhibitors Phase 4
11 Antirheumatic Agents Phase 4
12 Anti-Inflammatory Agents, Non-Steroidal Phase 4
13 Anti-Infective Agents Phase 4
14 Anti-Bacterial Agents Phase 4
15 Alkylating Agents Phase 4
16 Antiprotozoal Agents Phase 4
17 Antiparasitic Agents Phase 4
18 Antibiotics, Antitubercular Phase 4
19 Sulperazone Phase 4
20
Pentoxifylline Approved, Investigational Phase 2 6493-05-6 4740
21
Sargramostim Approved, Investigational Phase 2 83869-56-1, 123774-72-1
22
Lenograstim Approved, Investigational Phase 2 135968-09-1
23
Acetylcysteine Approved, Investigational Phase 2 616-91-1 12035
24
Cysteine Approved, Nutraceutical Phase 2 52-90-4 5862
25 Molgramostim Investigational Phase 2 99283-10-0
26 Phosphodiesterase Inhibitors Phase 2
27 Vasodilator Agents Phase 2
28 Radiation-Protective Agents Phase 2
29 Antibodies Phase 1, Phase 2
30 Immunoglobulins, Intravenous Phase 1, Phase 2
31 Rho(D) Immune Globulin Phase 1, Phase 2
32 gamma-Globulins Phase 1, Phase 2
33 Immunoglobulins Phase 1, Phase 2
34
Bilirubin Phase 2 69853-43-6, 635-65-4 21252250 5280352
35 Pharmaceutical Solutions Phase 2
36 Antioxidants Phase 2
37 Protective Agents Phase 2
38 Immunologic Factors Phase 2
39 Adjuvants, Immunologic Phase 2
40 polysaccharide-K Phase 2
41 Respiratory System Agents Phase 2
42 Antidotes Phase 2
43 Antiviral Agents Phase 2
44 Expectorants Phase 2
45 N-monoacetylcystine Phase 2
46 Bile Acids and Salts Phase 2
47
Vancomycin Approved Phase 1 1404-90-6 14969 441141
48
Propofol Approved, Investigational, Vet_approved 2078-54-8 4943
49
Zoledronic Acid Approved 118072-93-8 68740
50
Sevoflurane Approved, Vet_approved 28523-86-6 5206

Interventional clinical trials:

(show all 44)
# Name Status NCT ID Phase Drugs
1 Clinical Study About the Role of COX-2 Inhibitor in Liver Cirrhosis With Biliary Atresia Completed NCT02298218 Phase 4 Meloxicam
2 Antibiotics Treatment of Cholangitis Post-Kasai Portoenterostomy Not yet recruiting NCT04370145 Phase 4 Sulperazon;Teicoplanin;Meropenem Injection
3 Phase IIIb Study of Corticosteroids as Post-Operative Adjuvant Therapy in Biliary Atresia Unknown status NCT00539565 Phase 3 prednisolone;placebo
4 A Double-Blind, Randomized, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Odevixibat (A4250) in Children With Biliary Atresia Who Have Undergone a Kasai Hepatoportoenterostomy Recruiting NCT04336722 Phase 3 Odevixibat;Placebo
5 A Phase II Trial of Pentoxifylline in Newly-Diagnosed Biliary Atresia Unknown status NCT01774487 Phase 2 Pentoxifylline
6 A Phase 1/2A Trial of Intravenous Immunoglobulin (IVIG) Therapy Following Portoenterostomy in Infants With Biliary Atresia Completed NCT01854827 Phase 1, Phase 2 Intravenous immunoglobulin (IVIG)
7 Outcomes of Autologous Bone Marrow Mononuclear Stem Cell (BMMC) Transplantation for Children Suffering From Liver Cirrhosis Due to Biliary Atresia After Kasai's Operation: An Open Label Uncontrolled Clinical Trial Recruiting NCT03468699 Phase 2
8 Granulocyte-Colony Stimulating Factor Adjunct Therapy for Biliary Atresia: Part II of a Prospective, Randomized Controlled, Multi-Institutional Trial Recruiting NCT04373941 Phase 2 Filgrastim
9 A Phase 2 Trial of N-Acetylcysteine in Biliary Atresia After Kasai Portoenterostomy Recruiting NCT03499249 Phase 2 N-Acetyl cysteine
10 The Human Gastrointestinal Tract Microbiota in the Setting of Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin. Completed NCT01322386 Phase 1 Vancomycin
11 The Human Gastrointestinal Tract Microbiota in the Setting of Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Recruiting NCT02137668 Phase 1 Oral Vancomycin
12 The Effect of Sevoflurane or Propofol on Brain Injury and Neurocognitive in Pediatric Living Related Liver Transplantation Unknown status NCT03024840 Sevoflurane;Propofol
13 Impaired T-Lymphocyte Proliferative Function in Biliary Atresia Children With Prolonged Jaundice Unknown status NCT00155194
14 Randomised Control Trial to Investigate the Effectiveness of CoSeal® Surgical Sealant in Reducing Intra-abdominal Adhesions Following the Kasai Hepatoportoenterostomy for Biliary Atresia. Unknown status NCT01745991
15 Effects of Intraoperative Normal Saline vs Lactated Ringer on Outcomes in Pediatric Liver Transplantation: A Double-blind Randomized Trial Unknown status NCT03563378 Lactated Ringer;Normal saline
16 Clinical and Basic Study for Pediatric Liver Transplantation Unknown status NCT02503384
17 Molecular Genetic Study of Suspected Cases of Osteogenesis Imperfecta Attending Assiut University Children Hospital Unknown status NCT03169192 Zoledronic Acid
18 Comparison the Level of CTGF Protein and Related Cytokine in Pleural Effusion Among Tuberculous Pleurisy and Malignant Pleural Effusion Patients Unknown status NCT00313066
19 Prospective Trial on Survival With Own Liver After Conventional Versus Laparoscopic Kasai for Biliary Atresia Completed NCT01063699
20 the Correlation Between Hepatic Fibrotic Scores and ADC and Other Non-invasive Indicators in Patients With Biliary Atresia Completed NCT02848573
21 Biliary Atresia, Hepatic Buffer Response and Sevoflurane Completed NCT02471209 Sevoflurane
22 Study of Magnesium Sulfate in Children With Reduced Bone Density Secondary to Chronic Cholestatic Liver Disease Completed NCT00007033 magnesium gluconate;magnesium sulfate
23 Ultrasound Shear Wave Elastography Evaluation of Suspected and Known Biliary Atresia Completed NCT02652533
24 A Randomized, Double-Blinded, Placebo-Controlled Trial of Corticosteroid Therapy Following Portoenterostomy in Infants With Biliary Atresia Completed NCT00294684 Corticosteroids;Placebo
25 The Comparison of Desflurane and Sevoflurane on Postoperative Recovery and Hepatic Function of Biliary Atresia Patients During Kasai Operation Completed NCT01443572 desflurane anesthetics;sevoflurane anesthetics
26 Use of Probiotics to Prevent Cholangitis in Children With Biliary Atresia Completed NCT00166868 Lactobacillus casei rhamnosus (Lcr35);Neomycin
27 Granulocyte-Colony Stimulating Factor Adjunct Therapy for Biliary Atresia Completed NCT03395028 Early Phase 1 Granulocyte Colony-Stimulating Factor
28 Search for Maternal Microchimerism in Swollen Portal Lymph Nodes of Infants With Biliary Atresia. Completed NCT02292862
29 Remote Ischemic Preconditioning Protects Against Hepatic Ischemic and Reperfusion Injury in Pediatric Living Donor Liver Transplantation Completed NCT02830841
30 Increased Liver Stiffness: A Study of Acoustic Radiation Force Impulse (ARFI) Elastography Completed NCT03382119
31 POLYGEN DEFI-ALPHA : Genetic Polymorphisms Study in Children With Alpha-1 Antitrypsin Deficiency, Included in the DEFI-ALPHA Cohort Completed NCT01862211
32 Development and Validation of a Screening Score for the Biliary Atresia in Infantile Cholestasis: A Prospective Study Recruiting NCT03842150
33 Analysis of Gut Microbiome and Liver Imaging Including Elasticity in Biliary Atresia Patients With Cholangitis for the Prediction of Disease Activity and Improvement of Outcome Using Fecal Microbiota Transplantation Recruiting NCT04260503
34 Coordinating Center- Mapping Disease Pathways for Biliary Atresia Recruiting NCT03273049
35 Clinical Follow-up Study on Hepatitis B Virus Infection After Liver Transplantation in Children Recruiting NCT03865966
36 Psychosocial Situation of Children With Rare (Congenital) Pediatric Surgical Diseases and Their Families Recruiting NCT04382820
37 Dry Blood Spot Screening Test for Neonatal Cholestasis Patients Enrolling by invitation NCT03667534
38 Intestinal Microbiome Composition in Infants With Biliary Atresia Not yet recruiting NCT03890536
39 Molecular Characterization for Understanding Biliary Atresia Not yet recruiting NCT04272515
40 Dry Blood Spot Screening Test for Biliary Atresia and Other Cholestasis Diseases Not yet recruiting NCT03898765
41 Biliary Atresia Study in Infants and Children (BASIC) Suspended NCT00345553
42 Childhood Liver Disease Research Network (ChiLDReN): FibroScan™ in Pediatric Cholestatic Liver Disease (FORCE) Study Protocol Suspended NCT02922751
43 Childhood Liver Disease Research Network (ChiLDReN): A Prospective Database of Infants With Cholestasis Suspended NCT00061828
44 Longitudinal Study of Mitochondrial Hepatopathies Suspended NCT01148550

Search NIH Clinical Center for Biliary Atresia

Cochrane evidence based reviews: biliary atresia

Genetic Tests for Biliary Atresia

Anatomical Context for Biliary Atresia

MalaCards organs/tissues related to Biliary Atresia:

40
Liver, Testes, Bone, T Cells, Heart, Spleen, Lymph Node

Publications for Biliary Atresia

Articles related to Biliary Atresia:

(show top 50) (show all 4366)
# Title Authors PMID Year
1
Immunological investigation of the hepatic tissue from infants with biliary atresia. 61 54
19089432 2009
2
Abnormal activation of OPN inflammation pathway in livers of children with biliary atresia and relationship to hepatic fibrosis. 61 54
18704890 2008
3
Evaluation of hepatocyte growth factor in patients with biliary atresia. 61 54
18639691 2008
4
Improvement in accuracy of gamma-glutamyl transferase for differential diagnosis of biliary atresia by correlation with age. 54 61
18773671 2008
5
Adult to adult living-related liver transplant: report on an initial experience in Italy. 54 61
17337259 2007
6
Analysis of connective tissue growth factor promoter polymorphism in Thai children with biliary atresia. 54 61
17375628 2007
7
Association of serum levels of tissue inhibitors of metalloproteinase-1 with clinical outcome in children with biliary atresia. 61 54
17136882 2006
8
Expression of the interferon-induced Mx proteins in biliary atresia. 54 61
16769349 2006
9
Altered hepatobiliary gene expressions in PFIC1: ATP8B1 gene defect is associated with CFTR downregulation. 61 54
16628629 2006
10
Connective tissue growth factor expression is increased in biliary epithelial cells in biliary atresia. 61 54
16291159 2005
11
Kidney volume and plasma hepatocyte growth factor-transforming growth factor beta1 ratio among children with biliary atresia before and after liver transplantation: the reversibility of nephromegaly. 54 61
16253722 2005
12
Developmental expression of canalicular transporter genes in human liver. 61 54
15922475 2005
13
Matrilysin (MMP-7) is a major matrix metalloproteinase upregulated in biliary atresia-associated liver fibrosis. 54 61
15696117 2005
14
Expression of osteopontin correlates with portal biliary proliferation and fibrosis in biliary atresia. 54 61
15845635 2005
15
Connective tissue growth factor and progressive fibrosis in biliary atresia. 61 54
15459777 2005
16
Identification of transforming growth factors actively transcribed during the progress of liver fibrosis in biliary atresia. 54 61
15137003 2004
17
The value of cholinesterase activity after Kasai operation. 61 54
13680289 2003
18
Nephromegaly relates to hepatocyte growth factor dysregulation in biliary atresia. 61 54
12172775 2002
19
Immunosuppressive effect of chenodeoxycholic acid on natural killer cell activity in patients with biliary atresia and hepatitis C virus-related liver cirrhosis. 61 54
12018907 2002
20
Nephromegaly and elevated plasma hepatocyte growth factor-transforming growth factor-beta1 ratio in infants with fulminant hepatitis or biliary atresia. 54 61
11479153 2001
21
Cytokine-responsive gene-2/IFN-inducible protein-10 expression in multiple models of liver and bile duct injury suggests a role in tissue regeneration. 61 54
11418676 2001
22
Beneficial effect of a traditional herbal medicine (inchin-ko-to) in postoperative biliary atresia patients. 61 54
11527172 2001
23
Are stable postoperative biliary atresia patients really stable? 61 54
11315264 2001
24
Expression of collagens type I and IV, osteonectin and transforming growth factor beta-1 (TGFbeta1) in biliary atresia and paucity of intrahepatic bile ducts during infancy. 61 54
10453937 1999
25
Serum IFN-inducible protein-10: a new clinical prognostic predictor of hepatocyte death in biliary atresia. 61 54
10052811 1999
26
Value of gamma-glutamyl transpeptidase for early diagnosis of biliary atresia. 61 54
9884444 1998
27
Establishment of the enzyme-linked immunosorbent assay for connective tissue growth factor (CTGF) and its detection in the sera of biliary atresia. 61 54
9790981 1998
28
Contribution of hepatic parenchymal and nonparenchymal cells to hepatic fibrogenesis in biliary atresia. 61 54
9708812 1998
29
Hepatic growth hormone receptor, insulin-like growth factor I, and insulin-like growth factor-binding protein messenger RNA expression in pediatric liver disease. 61 54
9398004 1997
30
Hepatic basolateral sodium-dependent-bile acid transporter expression in two unusual cases of hypercholanemia and in extrahepatic biliary atresia. 61 54
9141436 1997
31
Nephromegaly and elevated hepatocyte growth factor in children with biliary atresia. 61 54
9016888 1997
32
Assay of gamma-glutamyl transpeptidase activity in amniotic fluid offers a possible prenatal diagnosis of biliary atresia in the rat model. 61 54
9021823 1997
33
Three-color risk stratification for improving the diagnostic accuracy for biliary atresia. 61
32162000 2020
34
Biliary Atresia Patients With Successful Kasai Portoenterostomy Can Present With Features of Obliterative Portal Venopathy. 61
32187144 2020
35
Investigation into multi-centre diagnosis and treatment strategies of biliary atresia in mainland China. 61
32444895 2020
36
Retraction Note: Interactions between Th1 cells and Tregs affect regulation of hepatic fibrosis in biliary atresia through the IFN-γ/STAT1 pathway. 61
31591471 2020
37
A Morphology-based Analysis of Biliary Ductules After Kasai Procedure and a Review of the Literature. 61
32155473 2020
38
Admission Characteristics Identify Risk of Pediatric Acute-on Liver Failure. 61
32443031 2020
39
Pregnancy Outcomes Following Pediatric Liver Transplantation: A Single-Center Experience in Japan. 61
32513910 2020
40
The Largest Single Center Report on Pediatric Liver Transplantation: Experiences and Lessons Learned. 61
32541224 2020
41
The Fecal Microbiome in Infants With Biliary Atresia Associates With Bile Flow After Kasai Portoenterostomy. 61
32443032 2020
42
Serum Trough Concentration and Effects of Mycophenolate Mofetil Based on Pathologic Findings in Infants After Liver Transplantation. 61
32571709 2020
43
Anatomical and histological characteristics of the hepatobiliary system in adult Sox17 heterozygote mice. 61
32478476 2020
44
Beta-amyloid deposition around hepatic bile ducts is a novel pathobiological and diagnostic feature of biliary atresia. 61
32553668 2020
45
Periportal thickening on magnetic resonance imaging for hepatic fibrosis in infantile cholestasis. 61
32550757 2020
46
Safety and Efficacy of Everolimus Rescue Treatment After Pediatric Living Donor Liver Transplantation. 61
32571711 2020
47
Evaluation of Thyroid Function Tests in Children with Chronic Liver Diseases 61
31486329 2020
48
Bilateral anophthalmia and intrahepatic biliary atresia, two unusual components of Fraser syndrome: a case report. 61
32522149 2020
49
Expression of CD56 is Not Limited to Biliary Atresia and Correlates with the Degree of Fibrosis in Pediatric Cholestatic Diseases. 61
32511036 2020
50
Ki67 expression at Kasai portoenterostomy as a prognostic factor in patients with biliary atresia. 61
32543770 2020

Variations for Biliary Atresia

Expression for Biliary Atresia

Search GEO for disease gene expression data for Biliary Atresia.

Pathways for Biliary Atresia

GO Terms for Biliary Atresia

Cellular components related to Biliary Atresia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.91 TIMP1 TGFB1 SPP1 MMP7 JAG1 HGF
2 extracellular matrix GO:0031012 9.62 TIMP1 TGFB1 MMP7 CCN2
3 extracellular space GO:0005615 9.61 TIMP1 TGFB1 SPP1 MMP7 HGF GPT
4 intercellular canaliculus GO:0046581 9.16 ABCB4 ABCB11
5 platelet alpha granule lumen GO:0031093 8.92 TIMP1 TGFB1 HGF ALB

Biological processes related to Biliary Atresia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 aging GO:0007568 9.67 TIMP1 TGFB1 MMP7 CCN2
2 response to organic substance GO:0010033 9.63 TIMP1 TGFB1 SPP1
3 negative regulation of cell-cell adhesion GO:0022408 9.48 TGFB1 JAG1
4 bile acid metabolic process GO:0008206 9.43 NR1H4 ATP8B1
5 cell activation GO:0001775 9.4 TIMP1 TGFB1
6 cellular response to bile acid GO:1903413 9.32 NR1H4 ABCB4
7 connective tissue development GO:0061448 9.26 TGFB1 CCN2
8 platelet degranulation GO:0002576 9.26 TIMP1 TGFB1 HGF ALB
9 connective tissue replacement involved in inflammatory response wound healing GO:0002248 9.16 TIMP1 TGFB1
10 bile acid and bile salt transport GO:0015721 9.02 SLC10A2 SLC10A1 NR1H4 ATP8B1 ABCB11

Molecular functions related to Biliary Atresia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 growth factor activity GO:0008083 9.02 TIMP1 TGFB1 JAG1 HGF CCN2
2 bile acid:sodium symporter activity GO:0008508 8.96 SLC10A2 SLC10A1

Sources for Biliary Atresia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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