MCID: BLR001
MIFTS: 58

Biliary Atresia

Categories: Fetal diseases, Gastrointestinal diseases, Liver diseases, Rare diseases
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Aliases & Classifications for Biliary Atresia

MalaCards integrated aliases for Biliary Atresia:

Name: Biliary Atresia 11 19 75 53 43 14 71 33
Isolated Atresia of Bile Ducts 19 58
Non-Syndromic Biliary Atresia 19 58
Congenital Biliary Atresia 11 33
Isolated Biliary Atresia 19 58
Congenital Bile Duct Atresia 33
Biliary Atresia, Congenital 11
Ba - [biliary Atresia] 33
Atresia of Common Duct 33
Atresia of Bile Ducts 33
Atresia of Bile Duct 11
Impervious Bile Duct 33
Biliary Duct Atresia 33
Bile Ductal Atresia 33
Cystic Duct Atresia 33
Bile Duct Atresia 33

Characteristics:


Inheritance:

Isolated Biliary Atresia: Multigenic/multifactorial 58

Prevelance:

Isolated Biliary Atresia: 1-5/10000 (Specific population, Germany, Netherlands, Switzerland, Ukraine, Worldwide, French Polynesia) 1-9/100000 (Europe, Europe, Belgium, France, Italy, Norway, Spain, United Kingdom, Japan, Australia) 58

Age Of Onset:

Isolated Biliary Atresia: Antenatal,Infancy,Neonatal 58

Classifications:

Orphanet: 58  
Rare hepatic diseases
Developmental anomalies during embryogenesis


Summaries for Biliary Atresia

GARD: 19 A rare, biliary tract disease characterized by progressive obliterative cholangiopathy of the intra- and extrahepatic bile ducts, occuring in the embryonic/ perinatal period, leading to severe and persistent neonatal jaundice and acholic stool.

MalaCards based summary: Biliary Atresia, also known as isolated atresia of bile ducts, is related to biliary atresia, extrahepatic and portal hypertension, and has symptoms including icterus An important gene associated with Biliary Atresia is GPT (Glutamic--Pyruvic Transaminase), and among its related pathways/superpathways are Nuclear receptors meta-pathway and Overview of interferons-mediated signaling pathway. The drugs Meloxicam and Analgesics have been mentioned in the context of this disorder. Affiliated tissues include liver, spleen and pancreas, and related phenotypes are hepatomegaly and elevated hepatic transaminase

Orphanet: 58 A rare, biliary tract disease characterized by progressive obliterative cholangiopathy of the intra- and extrahepatic bile ducts, occuring in the embryonic/ perinatal period, leading to severe and persistent neonatal jaundice and acholic stool.

Disease Ontology: 11 A cholestasis characterized by blockage of the ducts that carry bile from the liver to the gallbladder.

Wikipedia: 75 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 522)
# Related Disease Score Top Affiliating Genes
1 biliary atresia, extrahepatic 33.4 JAG1 GGT1
2 portal hypertension 31.8 NR1H4 GPT GGT1 ALB
3 cholangitis 31.6 NR1H4 GPT GGT1 ALB ABCB4 ABCB11
4 obstructive jaundice 31.6 GPT GGT1 ALB ABCB11
5 ascending cholangitis 31.4 GPT ALB ABCB4
6 liver cirrhosis 31.3 TIMP1 TGFB1 HGF GPT GGT1 CCN2
7 cholestasis 31.2 SLC10A2 SLC10A1 NR1H4 JAG1 GPT GGT1
8 bile duct cysts 31.0 GPT CFC1 ALB ADD3 ABCB4 ABCB11
9 hepatitis 31.0 TGFB1 SLC10A1 GPT GGT1
10 bile duct disease 30.9 SLC10A2 SLC10A1 NR1H4 MIR142 GPT ALB
11 cholangiocarcinoma 30.8 TGFB1 SPP1 MMP7 HGF GGT1
12 alagille syndrome 1 30.7 JAG1 CFC1 ABCB4 ABCB11
13 autoimmune hepatitis 30.6 GPT GGT1 ALB
14 bilirubin metabolic disorder 30.6 SLC10A2 SLC10A1 NR1H4 GPT GGT1 ALB
15 viral hepatitis 30.6 MX1 GPT GGT1 ALB
16 acute cholangitis 30.5 GPT ALB ABCB4
17 intrahepatic cholestasis 30.4 SLC10A2 NR1H4 JAG1 GPT GGT1 ALB
18 gingival overgrowth 30.4 TIMP1 TGFB1 CCN2
19 hepatic infarction 30.3 GPT ALB
20 cholecystitis 30.3 GPT ALB ABCB4
21 gallbladder disease 30.3 NR1H4 MIR142 GPT ALB ABCB4 ABCB11
22 hydronephrosis 30.3 TIMP1 TGFB1 ALB
23 liver disease 30.3 SLC10A2 SLC10A1 NR1H4 MIR142 HGF GPT
24 hepatitis b 30.2 TGFB1 SLC10A1 MX1 GPT GGT1 ALB
25 duodenal obstruction 30.2 GPT ALB
26 patent ductus arteriosus 1 30.2 JAG1 CFC1 ALB
27 arteriovenous malformation 30.2 TGFB1 JAG1 ALB
28 sclerosing cholangitis 30.2 SLC10A2 SLC10A1 NR1H4 GPT ALB ABCB4
29 hypertension, essential 30.1 TIMP1 TGFB1 SPP1 GPT CCN2 ALB
30 progressive familial intrahepatic cholestasis 30.1 SLC10A2 SLC10A1 NR1H4 JAG1 ABCB4 ABCB11
31 crigler-najjar syndrome, type i 30.1 HGF ALB ABCB11
32 alcoholic hepatitis 30.1 TGFB1 HGF GPT GGT1 ALB
33 pulmonary fibrosis 30.0 TIMP1 TGFB1 MMP7 HGF CCN2
34 syphilis 30.0 MIR142 GPT ALB
35 cholestasis, progressive familial intrahepatic, 1 30.0 SLC10A2 SLC10A1 NR1H4 JAG1 GGT1 ABCB4
36 cholelithiasis 30.0 NR1H4 GPT ALB ABCB4
37 chronic kidney disease 29.9 TGFB1 SPP1 HGF CCN2 ALB
38 extrahepatic cholestasis 29.9 SLC10A2 SLC10A1 NR1H4 GPT GGT1 ABCB4
39 diarrhea 29.9 TGFB1 SLC10A2 GPT ALB
40 cholangitis, primary sclerosing 29.8 TIMP1 SLC10A2 SLC10A1 NR1H4 JAG1 GPT
41 pulmonary disease, chronic obstructive 29.8 TIMP1 TGFB1 MIR142 CCN2 ALB
42 non-alcoholic steatohepatitis 29.7 TIMP1 NR1H4 MIR142 GPT ALB
43 kidney disease 29.7 TIMP1 TGFB1 SPP1 HGF GGT1 CCN2
44 cholestasis, progressive familial intrahepatic, 3 29.6 SLC10A2 SLC10A1 NR1H4 JAG1 ABCB4 ABCB11
45 non-alcoholic fatty liver disease 29.5 NR1H4 GPT GGT1 ALB
46 primary biliary cholangitis 29.4 TIMP1 SLC10A2 SLC10A1 NR1H4 MIR142 GPT
47 type 2 diabetes mellitus 29.4 TGFB1 NR1H4 MIR142 HGF GPT GGT1
48 interstitial lung disease 2 29.4 TIMP1 TGFB1 SPP1 MMP7 MIR142 HGF
49 biliary tract disease 29.3 SLC10A2 SLC10A1 NR1H4 MIR142 GPT ALB
50 pericholangitis 29.3 SPP1 SLC10A2 SLC10A1 NR1H4 GPT ABCB4

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



Diseases related to Biliary Atresia

Symptoms & Phenotypes for Biliary Atresia

Human phenotypes related to Biliary Atresia:

58 30 (show all 30)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hepatomegaly 58 30 Frequent (33%) Frequent (79-30%)
HP:0002240
2 elevated hepatic transaminase 58 30 Frequent (33%) Frequent (79-30%)
HP:0002910
3 fat malabsorption 58 30 Frequent (33%) Frequent (79-30%)
HP:0002630
4 prolonged prothrombin time 58 30 Frequent (33%) Frequent (79-30%)
HP:0008151
5 prolonged neonatal jaundice 58 30 Frequent (33%) Frequent (79-30%)
HP:0006579
6 acholic stools 58 30 Frequent (33%) Frequent (79-30%)
HP:0011985
7 conjugated hyperbilirubinemia 58 30 Frequent (33%) Frequent (79-30%)
HP:0002908
8 decreased liver function 58 30 Frequent (33%) Frequent (79-30%)
HP:0001410
9 severe failure to thrive 58 30 Frequent (33%) Frequent (79-30%)
HP:0001525
10 atretic gallbladder 58 30 Frequent (33%) Frequent (79-30%)
HP:0011984
11 dark yellow urine 58 30 Frequent (33%) Frequent (79-30%)
HP:0040321
12 elevated circulating alkaline phosphatase concentration 30 Frequent (33%) HP:0003155
13 elevated gamma-glutamyltransferase level 30 Frequent (33%) HP:0030948
14 seizure 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001250
15 hypothyroidism 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000821
16 splenomegaly 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001744
17 abnormal facial shape 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001999
18 cirrhosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001394
19 ophthalmoplegia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000602
20 pruritus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000989
21 small for gestational age 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001518
22 hypopituitarism 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0040075
23 periportal fibrosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001405
24 bile duct proliferation 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001408
25 xanthelasma 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001114
26 failure to thrive 58 Very frequent (99-80%)
27 jaundice 58 Very frequent (99-80%)
28 cholestasis 58 Very frequent (99-80%)
29 elevated alkaline phosphatase 58 Frequent (79-30%)
30 elevated gamma-glutamyltransferase activity 58 Frequent (79-30%)

UMLS symptoms related to Biliary Atresia:


icterus

MGI Mouse Phenotypes related to Biliary Atresia:

45 (show all 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.36 ABCB11 ABCB4 CCN2 CFC1 GGT1 HGF
2 homeostasis/metabolism MP:0005376 10.35 ABCB11 ABCB4 ALB CCN2 GGT1 JAG1
3 liver/biliary system MP:0005370 10.31 ABCB11 ABCB4 ALB CCN2 CFC1 HGF
4 cardiovascular system MP:0005385 10.24 ABCB4 ADD3 ALB CCN2 CFC1 HGF
5 digestive/alimentary MP:0005381 10.14 ABCB4 ALB CCN2 CFC1 JAG1 MMP7
6 endocrine/exocrine gland MP:0005379 10.13 ABCB4 ALB CCN2 GGT1 JAG1 NR1H4
7 neoplasm MP:0002006 10.11 ABCB4 ALB MMP7 NR1H4 SPP1 TGFB1
8 immune system MP:0005387 10.1 ABCB4 ALB CFC1 GGT1 MIR142 MMP7
9 skeleton MP:0005390 10.03 ABCB4 CCN2 CFC1 GGT1 GPT HGF
10 vision/eye MP:0005391 9.81 ABCB11 CCN2 GGT1 JAG1 MMP7 MX1
11 hematopoietic system MP:0005397 9.8 ABCB4 ADD3 CFC1 GGT1 HGF JAG1
12 mortality/aging MP:0010768 9.53 ABCB11 ALB CCN2 CFC1 GGT1 HGF

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 Analgesics Phase 4
3 Antirheumatic Agents Phase 4
4 Cyclooxygenase Inhibitors Phase 4
5 Cyclooxygenase 2 Inhibitors Phase 4
6 Anti-Inflammatory Agents, Non-Steroidal Phase 4
7 Analgesics, Non-Narcotic Phase 4
8 Anti-Inflammatory Agents Phase 4
9
Norepinephrine Approved Phase 3 51-41-2 439260
10 Neurotransmitter Agents Phase 3
11 Adrenergic alpha-Agonists Phase 3
12 Adrenergic Agonists Phase 3
13 Adrenergic Agents Phase 3
14 Vasoconstrictor Agents Phase 3
15 Anesthetics Phase 3
16 Sympathomimetics Phase 3
17 Bile Acids and Salts Phase 3
18
Lenograstim Approved, Investigational Phase 2 135968-09-1
19
Pentoxifylline Approved, Investigational Phase 2 6493-05-6 4740
20
Chenodeoxycholic acid Approved Phase 2 474-25-9 10133
21
Acetylcysteine Approved, Investigational Phase 2 616-91-1 581 12035
22
Cysteine Approved, Nutraceutical Phase 2 52-90-4 594 5862
23 Adjuvants, Immunologic Phase 2
24 polysaccharide-K Phase 2
25 Immunologic Factors Phase 2
26 Platelet Aggregation Inhibitors Phase 2
27 Vasodilator Agents Phase 2
28 Radiation-Protective Agents Phase 2
29 Phosphodiesterase Inhibitors Phase 2
30 Liver Extracts Phase 2
31
bilirubin Phase 2 635-65-4 5280352
32 Anti-Infective Agents Phase 2
33 Gastrointestinal Agents Phase 2
34 Protective Agents Phase 2
35 Cathartics Phase 2
36 Laxatives Phase 2
37 Pharmaceutical Solutions Phase 2
38 Antioxidants Phase 2
39 Antiviral Agents Phase 2
40 Expectorants Phase 2
41 Antidotes Phase 2
42 N-monoacetylcystine Phase 2
43 Respiratory System Agents Phase 2
44
Vancomycin Approved Phase 1 1404-90-6 14969
45 Anti-Bacterial Agents Phase 1
46
Propranolol Approved, Investigational 318-98-9, 525-66-6 62882 4946
47
Prednisolone phosphate Approved, Vet_approved 302-25-0
48
Prednisolone acetate Approved, Vet_approved 52-21-1
49
Prednisolone Approved, Vet_approved 50-24-8 4894 5755
50
Methylprednisolone hemisuccinate Approved 2921-57-5 1875

Interventional clinical trials:

(show all 49)
# 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 Phase IIIb Study of Corticosteroids as Post-Operative Adjuvant Therapy in Biliary Atresia Unknown status NCT00539565 Phase 3 prednisolone;placebo
3 Prophylactic Use of Norepinephrine for Prevention of Intraoperative Hypotension in Infants Undergoing Kasai Portoenterostomy Operation for Biliary Atresia: Double Blinded Randomized Controlled Trial Recruiting NCT05521152 Phase 3 Norepinephrine Bitartrate;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 Active, not recruiting NCT04336722 Phase 3 Odevixibat;Placebo
5 An Open-label Extension Study to Evaluate Long-term Efficacy and Safety of Odevixibat (A4250) in Children With Biliary Atresia (BOLD-EXT) Enrolling by invitation NCT05426733 Phase 3 Odevixibat
6 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 Unknown status NCT03468699 Phase 2
7 Evaluation Safety and Efficacy of Umbilical Cord Derived Mesenchymal Stem Cell (UC -MSC) Transplantation for Children Suffering From Liver Cirrhosis Due to Biliary Atresia: A Matched Control Prospective Study Unknown status NCT04522869 Phase 1, Phase 2
8 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)
9 Granulocyte-Colony Stimulating Factor Adjunct Therapy for Biliary Atresia: Part II of a Prospective, Randomized Controlled, Multi-Institutional Trial Recruiting NCT04373941 Phase 2 Filgrastim
10 Randomized, Double-Blind, Placebo-Controlled Phase 2 Study to Evaluate the Efficacy and Safety of Maralixibat in the Treatment of Subjects With Biliary Atresia After Hepatoportoenterostomy Recruiting NCT04524390 Phase 2 Maralixibat
11 A Phase II Trial of Pentoxifylline in Newly-Diagnosed Biliary Atresia Recruiting NCT01774487 Phase 2 Pentoxifylline
12 A Multicenter, Open-Label, Single- and Multiple-Dose, Dose-Finding Study, With an Optional Open-Label Extension to Assess the Safety, Tolerability, and Pharmacokinetics of Obeticholic Acid in Pediatric Subjects With Biliary Atresia Active, not recruiting NCT05321524 Phase 2 OCA 0.1mg;OCA 1.5mg;OCA 5mg
13 A Phase 2 Trial of N-Acetylcysteine in Biliary Atresia After Kasai Portoenterostomy Active, not recruiting NCT03499249 Phase 2 N-Acetyl cysteine
14 Autologous Bone Marrow Mononuclear Cell Infusion for Liver Cirrhosis in Children With Biliary Atresia Completed NCT05517317 Phase 1
15 The Human Gastrointestinal Tract Microbiota in the Setting of Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin. Completed NCT01322386 Phase 1 Vancomycin
16 The Human Gastrointestinal Tract Microbiota in the Setting of Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Recruiting NCT02137668 Phase 1 Oral Vancomycin
17 Dry Blood Spot Screening Test for Biliary Atresia and Other Cholestasis Diseases Unknown status NCT03898765
18 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 Unknown status NCT04260503
19 Development and Validation of a Screening Score for the Biliary Atresia in Infantile Cholestasis: A Prospective Study Unknown status NCT03842150
20 Impaired T-Lymphocyte Proliferative Function in Biliary Atresia Children With Prolonged Jaundice Unknown status NCT00155194
21 A Multicenter Real World Study on Clinical Efficacy of Sodium Bicarbonate Ringer's Solution in Children With Biliary Atresia Unknown status NCT04506021 Bicarbonate Ringer's solution;Other Crystalloid
22 Propanolol for Primary Prophylaxis for Variceal Bleed in Biliary Atresia - An Open Label Randomized Controlled Study Unknown status NCT04494763 Propanolol
23 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
24 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
25 Clinical and Basic Study for Pediatric Liver Transplantation Unknown status NCT02503384
26 Search for Maternal Microchimerism in Swollen Portal Lymph Nodes of Infants With Biliary Atresia. Completed NCT02292862
27 Ultrasound Shear Wave Elastography Evaluation of Suspected and Known Biliary Atresia Completed NCT02652533
28 Prospective Trial on Survival With Own Liver After Conventional Versus Laparoscopic Kasai for Biliary Atresia Completed NCT01063699
29 Use of Probiotics to Prevent Cholangitis in Children With Biliary Atresia Completed NCT00166868 Lactobacillus casei rhamnosus (Lcr35);Neomycin
30 Biliary Atresia, Hepatic Buffer Response and Sevoflurane Completed NCT02471209 Sevoflurane
31 A Randomized, Double-Blinded, Placebo-Controlled Trial of Corticosteroid Therapy Following Portoenterostomy in Infants With Biliary Atresia Completed NCT00294684 Corticosteroids;Placebo
32 Granulocyte-Colony Stimulating Factor Adjunct Therapy for Biliary Atresia Completed NCT03395028 Early Phase 1 Granulocyte Colony-Stimulating Factor
33 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
34 the Correlation Between Hepatic Fibrotic Scores and ADC and Other Non-invasive Indicators in Patients With Biliary Atresia Completed NCT02848573
35 Investigation of Mortality, Morbidity and Risk Factors After Pediatric Liver Transplantation: A Retrospective Study Completed NCT04778735
36 Study of Magnesium Sulfate in Children With Reduced Bone Density Secondary to Chronic Cholestatic Liver Disease Completed NCT00007033 magnesium gluconate;magnesium sulfate
37 Molecular Characterization for Understanding Biliary Atresia Recruiting NCT04272515
38 Coordinating Center- Mapping Disease Pathways for Biliary Atresia Recruiting NCT03273049
39 Biliary Atresia Study in Infants and Children (BASIC) Recruiting NCT00345553
40 Study on High Medium-chain Triglyceride Nutritional Support in Infants With Biliary Atresia After Kasai Portoenterostomy Recruiting NCT05072626
41 BILACO Trial: Biliary Atresia - a Severe Complex Congenital Liver Disease With High Mortality, Compromised Neurological Development, Severe Malnutrition and Unknown Etiology Recruiting NCT05399745
42 A Peer Support Network System Construction for the Primary Caregivers of Children With Biliary Atresia Based on Social Support Theory: A Quasi-experimental Study Recruiting NCT05503394
43 Establishment and Validation of a Standardized Definition for Biliary Atresia Associated Cholangitis After Hepatic Portoenterostomy Recruiting NCT05464303 Intravenous Antibodies
44 Childhood Liver Disease Research Network (ChiLDReN): A Prospective Database of Infants With Cholestasis Recruiting NCT00061828
45 Predictive Models of Hepatic Decompensation and Survival Outcomes in Pediatric Patients With Cirrhosis Recruiting NCT05181332
46 Peri Kasai Portoenterostomy Anchoring of the Jejunal Loop Recruiting NCT04961034
47 Childhood Liver Disease Research Network (ChiLDReN): FibroScan™ in Pediatric Cholestatic Liver Disease (FORCE) Study Protocol Active, not recruiting NCT02922751
48 Dry Blood Spot Screening Test for Neonatal Cholestasis Patients Enrolling by invitation NCT03667534
49 Intestinal Microbiome Composition in Infants With Biliary Atresia Not yet recruiting NCT03890536

Search NIH Clinical Center for Biliary Atresia

Cochrane evidence based reviews: biliary atresia

Genetic Tests for Biliary Atresia

Anatomical Context for Biliary Atresia

Organs/tissues related to Biliary Atresia:

MalaCards : Liver, Spleen, Pancreas, Bone Marrow, Bone, T Cells, Smooth Muscle

Publications for Biliary Atresia

Articles related to Biliary Atresia:

(show top 50) (show all 5116)
# Title Authors PMID Year
1
Immunological investigation of the hepatic tissue from infants with biliary atresia. 53 62
19089432 2009
2
Abnormal activation of OPN inflammation pathway in livers of children with biliary atresia and relationship to hepatic fibrosis. 53 62
18704890 2008
3
Evaluation of hepatocyte growth factor in patients with biliary atresia. 53 62
18639691 2008
4
Improvement in accuracy of gamma-glutamyl transferase for differential diagnosis of biliary atresia by correlation with age. 53 62
18773671 2008
5
Adult to adult living-related liver transplant: report on an initial experience in Italy. 53 62
17337259 2007
6
Analysis of connective tissue growth factor promoter polymorphism in Thai children with biliary atresia. 53 62
17375628 2007
7
Association of serum levels of tissue inhibitors of metalloproteinase-1 with clinical outcome in children with biliary atresia. 53 62
17136882 2006
8
Expression of the interferon-induced Mx proteins in biliary atresia. 53 62
16769349 2006
9
Altered hepatobiliary gene expressions in PFIC1: ATP8B1 gene defect is associated with CFTR downregulation. 53 62
16628629 2006
10
Connective tissue growth factor expression is increased in biliary epithelial cells in biliary atresia. 53 62
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. 53 62
16253722 2005
12
Developmental expression of canalicular transporter genes in human liver. 53 62
15922475 2005
13
Matrilysin (MMP-7) is a major matrix metalloproteinase upregulated in biliary atresia-associated liver fibrosis. 53 62
15696117 2005
14
Expression of osteopontin correlates with portal biliary proliferation and fibrosis in biliary atresia. 53 62
15845635 2005
15
Connective tissue growth factor and progressive fibrosis in biliary atresia. 53 62
15459777 2005
16
Identification of transforming growth factors actively transcribed during the progress of liver fibrosis in biliary atresia. 53 62
15137003 2004
17
The value of cholinesterase activity after Kasai operation. 53 62
13680289 2003
18
Nephromegaly relates to hepatocyte growth factor dysregulation in biliary atresia. 53 62
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. 53 62
12018907 2002
20
Nephromegaly and elevated plasma hepatocyte growth factor-transforming growth factor-beta1 ratio in infants with fulminant hepatitis or biliary atresia. 53 62
11479153 2001
21
Beneficial effect of a traditional herbal medicine (inchin-ko-to) in postoperative biliary atresia patients. 53 62
11527172 2001
22
Cytokine-responsive gene-2/IFN-inducible protein-10 expression in multiple models of liver and bile duct injury suggests a role in tissue regeneration. 53 62
11418676 2001
23
Are stable postoperative biliary atresia patients really stable? 53 62
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. 53 62
10453937 1999
25
Serum IFN-inducible protein-10: a new clinical prognostic predictor of hepatocyte death in biliary atresia. 53 62
10052811 1999
26
Value of gamma-glutamyl transpeptidase for early diagnosis of biliary atresia. 53 62
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. 53 62
9790981 1998
28
Contribution of hepatic parenchymal and nonparenchymal cells to hepatic fibrogenesis in biliary atresia. 53 62
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. 53 62
9398004 1997
30
Hepatic basolateral sodium-dependent-bile acid transporter expression in two unusual cases of hypercholanemia and in extrahepatic biliary atresia. 53 62
9141436 1997
31
Nephromegaly and elevated hepatocyte growth factor in children with biliary atresia. 53 62
9016888 1997
32
Assay of gamma-glutamyl transpeptidase activity in amniotic fluid offers a possible prenatal diagnosis of biliary atresia in the rat model. 53 62
9021823 1997
33
Neonatal Dubin-Johnson Syndrome and its Differentiation from Biliary Atresia. 62
36406324 2023
34
Surgical approach to supradiaphragmatic inferior vena cava in patients with metallic stent in the hepatic vein during repeat liver transplantation. 62
36237167 2022
35
Advances in the Treatment of Neonatal Biliary Disease. 62
36328612 2022
36
Biochemical markers to predict the development of gastrointestinal bleeding and esophageal varices after portoenterostomy in biliary atresia. 62
36114864 2022
37
Anlotinib improves bile duct ligature-induced liver fibrosis in rats via antiangiogenesis regulated by VEGFR2/mTOR pathway. 62
36464837 2022
38
Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining "successful" portoenterostomy. 62
36454515 2022
39
Treatments and outcomes of intra-operative portal vein thrombosis in living-donor liver transplantation due to biliary atresia. 62
35810021 2022
40
Peripheral blood CD177+ cells as an early diagnostic marker for biliary atresia: A prospective multicentre study in pediatric patients with cholestasis. 62
35995126 2022
41
Latest diagnostic performance of different ultrasonic features for biliary atresia. 62
34854739 2022
42
Postnatal outcomes of fetuses with isolated gallbladder anomalies: be aware of biliary atresia. 62
34470134 2022
43
The preoperative lymphocyte ratio and postoperative C-reactive protein are related to the surgical outcome in biliary atresia: an analysis of serial ubiquitous markers of inflammation. 62
36098795 2022
44
Selective enlargement of left lateral segment liver volume as a potential diagnostic predictor for biliary atresia. 62
36109363 2022
45
Human poliovirus receptor contributes to biliary atresia pathogenesis by exacerbating natural-killer-cell-mediated bile duct injury. 62
36251580 2022
46
Adding a spur valve to laparoscopic portoenterostomy for patients with biliary atresia can achieve a high jaundice clearance rate and lower the number of episodes of cholangitis. 62
36104601 2022
47
Laparoscopic revision of Kasai portoenterostomy after initial laparoscopic portoenterostomy in patients with biliary atresia: a limited but positive effect on native liver survival. 62
36104599 2022
48
IL13 and periostin in active fibrogenic areas of the extrahepatic bile ducts in biliary atresia patients. 62
36149445 2022
49
Primary exploring the value of metagenomic next-generation sequencing in detecting pathogenic bacteria of cholangitis with biliary atresia after Kasai operation. 62
36269375 2022
50
Relationship between the expression levels of CD4+ T cells, IL-6, IL-8 and IL-33 in the liver of biliary atresia and postoperative cholangitis, operative age and early jaundice clearance. 62
36242601 2022

Variations for Biliary Atresia

Expression for Biliary Atresia

Search GEO for disease gene expression data for Biliary Atresia.

Pathways for Biliary Atresia

Pathways related to Biliary Atresia according to GeneCards Suite gene sharing:

(show all 18)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.57 TGFB1 SLC10A1 NR1H4 HGF GGT1 ABCB4
2
Show member pathways
12.45 TIMP1 TGFB1 MX1 HGF CCN2
3
Show member pathways
12.45 SLC10A2 SLC10A1 NR1H4 ALB ABCB11
4
Show member pathways
11.96 SLC10A2 SLC10A1 NR1H4 ALB ABCB4 ABCB11
5 11.78 TIMP1 TGFB1 HGF
6 11.67 TIMP1 TGFB1 MMP7 JAG1 HGF
7 11.58 TIMP1 TGFB1 HGF
8 11.57 TIMP1 TGFB1 HGF CCN2
9 11.41 TGFB1 HGF CCN2
10 11.2 NR1H4 ABCB4 ABCB11
11 11.15 TIMP1 TGFB1 SPP1 HGF CCN2
12 11.11 TGFB1 MMP7 HGF
13 10.84 TIMP1 TGFB1 SPP1 HGF CCN2
14 10.84 ABCB11 NR1H4 SLC10A1
15 10.74 SLC10A1 NR1H4 ABCB4 ABCB11
16 10.7 TGFB1 NR1H4
17 10.66 SLC10A2 SLC10A1 ABCB11
18 10.18 SLC10A1 NR1H4 ABCB4 ABCB11

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 space GO:0005615 10.11 TIMP1 TGFB1 SPP1 MMP7 MIR142 HGF
2 intercellular canaliculus GO:0046581 9.26 ABCB4 ABCB11
3 platelet alpha granule lumen GO:0031093 9.17 TIMP1 TGFB1 HGF ALB

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

# Name GO ID Score Top Affiliating Genes
1 response to organic substance GO:0010033 9.76 TIMP1 TGFB1 SPP1
2 cellular response to bile acid GO:1903413 9.73 NR1H4 ABCB4
3 bile acid and bile salt transport GO:0015721 9.73 SLC10A2 SLC10A1 ABCB11
4 connective tissue replacement involved in inflammatory response wound healing GO:0002248 9.58 TIMP1 TGFB1
5 regulation of bile acid secretion GO:0120188 9.54 SLC10A1 ABCB11
6 response to 17alpha-ethynylestradiol GO:1904486 9.46 SLC10A1 ABCB11
7 connective tissue development GO:0061448 9.32 TGFB1 CCN2
8 response to organic cyclic compound GO:0014070 9.26 TGFB1 SLC10A1 CCN2 ABCB11
9 bile acid signaling pathway GO:0038183 9.1 SLC10A1 NR1H4 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 9.32 TIMP1 TGFB1 JAG1 HGF CCN2
2 bile acid:sodium symporter activity GO:0008508 9.26 SLC10A2 SLC10A1

Sources for Biliary Atresia

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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