MCID: BLR001
MIFTS: 52

Biliary Atresia malady

Categories: Rare diseases, Gastrointestinal diseases, Liver diseases

Aliases & Classifications for Biliary Atresia

Aliases & Descriptions for Biliary Atresia:

Name: Biliary Atresia 12 50 29 52 42 14 69
Non-Syndromic Biliary Atresia 50
Biliary Atresia, Congenital 12
Congenital Biliary Atresia 12
Atresia of Bile Duct 12

Classifications:



External Ids:

Disease Ontology 12 DOID:13608
ICD10 33 Q44.2
ICD9CM 35 751.61
MeSH 42 D001656
UMLS 69 C0005411

Summaries for Biliary Atresia

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

MalaCards based summary : Biliary Atresia, also known as non-syndromic biliary atresia, is related to biliary atresia, extrahepatic and ichthyosis congenita biliary atresia, and has symptoms including icterus An important gene associated with Biliary Atresia is SLC10A1 (Solute Carrier Family 10 Member 1), and among its related pathways/superpathways are Apelin signaling pathway and Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Meloxicam and Anti-Inflammatory Agents have been mentioned in the context of this disorder. Affiliated tissues include liver, t cells and bone, and related phenotypes are cardiovascular system and growth/size/body region

Wikipedia : 71 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 134)
id Related Disease Score Top Affiliating Genes
1 biliary atresia, extrahepatic 12.3
2 ichthyosis congenita biliary atresia 11.9
3 biliary atresia with splenic malformation syndrome 11.9
4 biliary atresia intrahepatic non syndromic form 11.9
5 biliary atresia intrahepatic syndromic form 11.9
6 hepatoblastoma 11.3
7 lambert syndrome 11.2
8 bile duct disease 10.9
9 hyperbiliverdinemia 10.9
10 hepatitis 10.5
11 choledochal cyst 10.3
12 miliaria 10.2 CTGF TGFB1
13 aphthous stomatitis 10.2 ALB CTGF TGFB1
14 agammaglobulinemia 6 10.2 ALB CTGF TGFB1
15 acute monocytic leukemia 10.2 ALB GPT HGF
16 cholestasis 10.2
17 cholangitis 10.2
18 paranasal sinus sarcoma 10.2 ALB GPT HGF
19 zika fever 10.2 CTGF TGFB1 TIMP1
20 fiedler's myocarditis 10.2 ALB GPT TGFB1
21 acute necrotizing encephalitis 10.2 ALB CTGF TGFB1
22 cholera 10.2 CTGF TGFB1 TIMP1
23 noninfectious dermatoses of eyelid 10.1 ALB GPT TGFB1
24 germ cell and embryonal cancer 10.1 CTGF TGFB1 TIMP1
25 henoch-schoenlein purpura 10.1 ALB TGFB1 TIMP1
26 hemorrhagic cystitis 10.1 CTGF HGF TGFB1
27 borderline leprosy 10.1 ALB GPT NR1H4
28 calcinosis 10.1 ALB CTGF TGFB1
29 burns 10.1 MMP7 TGFB1 TIMP1
30 albinism, oculocutaneous, type ia 10.1 CTGF HGF TGFB1 TIMP1
31 hereditary spastic paraplegia 10.1 ALB GPT SLC10A1 TGFB1
32 renal tubular acidosis 10.1 CTGF HGF TGFB1 TIMP1
33 lymphoid leukemia 10.1 ALB GPT HGF TGFB1
34 benign focal seizures of adolescence 10.1 MMP7 TIMP1
35 geleophysic dysplasia 2 10.1 CTGF TGFB1
36 ascending cholangitis 10.1
37 esophagitis 10.1
38 heart aneurysm 10.0 ALB NR1H4 SLC10A1
39 cholestasis, intrahepatic, of pregnancy, 1 10.0 JAG1 NR1H4 SLC10A2
40 portal hypertension 10.0
41 situs inversus 10.0
42 alagille syndrome 10.0
43 ringed hair 10.0 GPC1 GPT JAG1 SLC10A1
44 trichodysplasia-xeroderma 10.0 ALB SPP1
45 proctitis 10.0 ALB MMP7 TGFB1
46 hepatocellular carcinoma 10.0
47 neuroectodermal endocrine syndrome 9.9 CTGF SPP1 TGFB1 TIMP1
48 chronic rheumatic pericarditis 9.9 ALB JAG1 NR1H4 SLC10A2
49 thrombosis 9.9
50 duodenitis 9.9

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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.06 ADD3 CTGF HGF JAG1 MMP7 NR1H4
2 growth/size/body region MP:0005378 10.02 CTGF HGF JAG1 MMP7 NR1H4 SLC10A2
3 hematopoietic system MP:0005397 10 NR1H4 SPP1 TGFB1 TIMP1 XPNPEP1 ADD3
4 digestive/alimentary MP:0005381 9.91 CTGF JAG1 MMP7 NR1H4 SLC10A2 TGFB1
5 homeostasis/metabolism MP:0005376 9.9 ALB CTGF GPC1 JAG1 MMP7 NR1H4
6 liver/biliary system MP:0005370 9.5 ALB HGF JAG1 NR1H4 SLC10A2 SPP1
7 neoplasm MP:0002006 9.17 ALB GPC1 MMP7 NR1H4 SPP1 TGFB1

Drugs & Therapeutics for Biliary Atresia

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

(show top 50) (show all 76)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Meloxicam Approved, Vet_approved Phase 4 71125-38-7 5281106 54677470
2 Anti-Inflammatory Agents Phase 4,Phase 3
3 Analgesics Phase 4
4 Analgesics, Non-Narcotic Phase 4
5 Anti-Inflammatory Agents, Non-Steroidal Phase 4
6 Antirheumatic Agents Phase 4
7 Cyclooxygenase 2 Inhibitors Phase 4
8 Cyclooxygenase Inhibitors Phase 4
9 Liver Extracts Phase 4,Phase 2
10 Peripheral Nervous System Agents Phase 4
11
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
12
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
13 Antineoplastic Agents, Hormonal Phase 3
14 glucocorticoids Phase 3
15 Hormone Antagonists Phase 3
16 Hormones Phase 3
17 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
18 Methylprednisolone acetate Phase 3
19 Methylprednisolone Hemisuccinate Phase 3
20 Prednisolone acetate Phase 3
21 Prednisolone hemisuccinate Phase 3
22 Prednisolone phosphate Phase 3
23
Pentoxifylline Approved, Investigational Phase 2 6493-05-6 4740
24 Antibodies Phase 1, Phase 2
25 gamma-Globulins Phase 1, Phase 2
26 Immunoglobulins Phase 1, Phase 2
27 Immunoglobulins, Intravenous Phase 1, Phase 2
28 Rho(D) Immune Globulin Phase 1, Phase 2
29
Bilirubin Phase 2 635-65-4 5280352
30 Platelet Aggregation Inhibitors Phase 2
31 Protective Agents Phase 2
32 Antioxidants Phase 2
33 Pharmaceutical Solutions Phase 2
34 Phosphodiesterase Inhibitors Phase 2
35 Radiation-Protective Agents Phase 2
36 Vasodilator Agents Phase 2
37 Fat Emulsions, Intravenous Phase 2
38 Parenteral Nutrition Solutions Phase 2
39 Soybean oil, phospholipid emulsion Phase 2
40 Omega 3 Fatty Acid Nutraceutical Phase 2
41 Soy Bean Nutraceutical Phase 2
42
Vancomycin Approved Phase 1 1404-90-6 441141 14969
43 Anti-Bacterial Agents Phase 1
44 Anti-Infective Agents Phase 1
45
Neomycin Approved, Vet_approved 1404-04-2 8378
46
Sevoflurane Approved, Vet_approved 28523-86-6 5206
47
Desflurane Approved 57041-67-5 42113
48
Isoflurane Approved, Vet_approved 26675-46-7 3763
49
Magnesium Sulfate Approved, Vet_approved 7487-88-9 24083
50
Pancrelipase Approved 53608-75-6

Interventional clinical trials:

(show all 28)
id Name Status NCT ID Phase
1 Clinical Study About the Role of COX-2 Inhibitor in Liver Cirrhosis With Biliary Atresia Completed NCT02298218 Phase 4
2 RCT of Steroids Following Kasai Portoenterostomy for Biliary Atresia. Unknown status NCT00539565 Phase 3
3 Safety Study of Intravenous Immunoglobulin (IVIG) Post-Portoenterostomy in Infants With Biliary Atresia Completed NCT01854827 Phase 1, Phase 2
4 Pentoxifylline Therapy in Biliary Atresia Recruiting NCT01774487 Phase 2
5 Compassionate Use of Omegaven in Children Recruiting NCT02370251 Phase 2
6 Gastrointestinal Microbiota in Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Completed NCT01322386 Phase 1
7 Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Recruiting NCT02137668 Phase 1
8 Evaluation of Immune Function in Biliary Atresia Children With Prolonged Jaundice Unknown status NCT00155194
9 Survival With Own Liver of Conventional Versus Laparoscopic Kasai for Biliary Atresia Completed NCT01063699
10 Use of Probiotics to Prevent Cholangitis in Children With Biliary Atresia After the Kasai Portoenterostomy Completed NCT00166868
11 Biliary Atresia, Hepatic Buffer Response and Sevoflurane Completed NCT02471209
12 Maternal Microchimerism in Lymph Nodes of Infants With Biliary Atresia at Time of Kasai's Operation Completed NCT02292862
13 A Randomized, Double-Blinded, Placebo-Controlled Trial of Corticosteroid Therapy Following Portoenterostomy Completed NCT00294684
14 The Comparison of Desflurane and Sevoflurane on Postoperative Recovery and Hepatic Function of Biliary Atresia Patients During Kasai Operation Completed NCT01443572
15 the Correlation Between Non-invasive Indicators in Patients With Biliary Atresia Completed NCT02848573
16 Study of Magnesium Sulfate in Children With Reduced Bone Density Secondary to Chronic Cholestatic Liver Disease Completed NCT00007033
17 Biliary Atresia Study in Infants and Children Recruiting NCT00345553
18 Ultrasound Shear Wave Elastography Evaluation of Suspected and Known Biliary Atresia Recruiting NCT02652533
19 Trail to Investigate the Effectiveness of CoSeal in Reducing Adhesions Following the Kasai Hepatoportoenterostomy for Biliary Atresia Recruiting NCT01745991
20 Biliary Atresia Research Consortium Recruiting NCT00061828
21 Clinical and Basic Study for Pediatric Liver Transplantation Recruiting NCT02503384
22 Polygen Defi-Alpha: Genetic Polymorphisms Study in Children With Alpha-1 Antitrypsin Deficiency, Included in the DEFI-ALPHA Cohort Recruiting NCT01862211
23 Shear Wave Sonoelastography in Pediatric Liver Fibrosis Recruiting NCT02372682
24 Evaluating the Genetic Causes and Progression of Cholestatic Liver Diseases (LOGIC) Recruiting NCT00571272
25 Omegaven for Compassionate Use in the Treatment of Parenteral Nutrition-Associated Liver Disease Available NCT03072667
26 FibroScan™ in Pediatric Cholestatic Liver Disease Study Protocol Enrolling by invitation NCT02922751
27 Transfusion Strategies for Pediatric Liver Transplantation Not yet recruiting NCT02951013
28 Prevention of Parenteral Nutrition-Associated Cholestasis With Cyclic Parenteral Nutrition in Infants Terminated NCT01062815

Search NIH Clinical Center for Biliary Atresia

Cochrane evidence based reviews: biliary atresia

Genetic Tests for Biliary Atresia

Genetic tests related to Biliary Atresia:

id Genetic test Affiliating Genes
1 Biliary Atresia 29

Anatomical Context for Biliary Atresia

MalaCards organs/tissues related to Biliary Atresia:

39
Liver, T Cells, Bone, Testes, Neutrophil, Nk Cells, Pancreas

Publications for Biliary Atresia

Articles related to Biliary Atresia:

(show top 50) (show all 764)
id Title Authors Year
1
Quality of Life and Parental Worrying in A National Cohort of Biliary Atresia Children Living with Their Native Livers. ( 28060195 )
2017
2
Embryonic cholecystitis and defective gallbladder contraction in the Sox17-haploinsufficient mouse model of biliary atresia. ( 28432216 )
2017
3
Outcomes of laparoscopic Kasai portoenterostomy for biliary atresia: A systematic review. ( 28007417 )
2017
4
Achondroplasia and Biliary Atresia: A Rare Association and Review of Literature. ( 28497003 )
2017
5
Effects of recipient size and allograft type on pediatric liver transplantation for biliary atresia. ( 27862929 )
2017
6
MicroRNA-19b Expression in Human Biliary Atresia Specimens and Its Role in BA-Related Fibrosis. ( 28083843 )
2017
7
Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis. ( 27830578 )
2017
8
Cystic Biliary Atresia and Choledochal Cysts are Distinct Histopathologic Entities. ( 28079575 )
2017
9
Association between Promoter Hypomethylation and Overexpression of Autotaxin with Outcome Parameters in Biliary Atresia. ( 28052132 )
2017
10
Liver stiffness measurements with supersonic shear wave elastography in the diagnosis of biliary atresia: a comparative study with grey-scale US. ( 28083694 )
2017
11
Newborn Screening for Biliary Atresia - Bilirubin or Bust? ( 27101537 )
2016
12
Prognostic values of serum bilirubin at 7th day post-Kasai for survival with native livers in patients with biliary atresia. ( 27476151 )
2016
13
Hepatic pathology of biliary atresia: A new comprehensive evaluation method using liver biopsy. ( 27210782 )
2016
14
Long-term outcomes after revision of Kasai portoenterostomy for biliary atresia. ( 27591751 )
2016
15
Loss of a Candidate Biliary Atresia Susceptibility Gene, add3a, Causes Biliary Developmental Defects in Zebrafish. ( 27526058 )
2016
16
Newborn Bilirubin Screening for Biliary Atresia. ( 27509119 )
2016
17
Interleukin-17 in a Mouse Model of Biliary Atresia and in Livers of Patients: The Study Control Matters. ( 27140493 )
2016
18
MicroRNA-499 Rs3746444 polymorphism and biliary atresia. ( 26795543 )
2016
19
Pediatric Liver Transplantation in Sickle Cell Anemia: A Case of Extrahepatic Biliary Atresia. ( 27169716 )
2016
20
Silver nanoparticle treatment ameliorates biliary atresia syndrome in rhesus rotavirus inoculated mice. ( 27890655 )
2016
21
Health-Related Quality of Life in Children with Biliary Atresia Living with Native Livers. ( 27125347 )
2016
22
Diagnostic and Prognostic Significance of Various Histopathological Features in Extrahepatic Biliary Atresia. ( 27504296 )
2016
23
Two Case Reports of FGF23-Induced Hypophosphatemia in Childhood Biliary Atresia. ( 27462066 )
2016
24
Expression of transforming growth factor-I^1 and connective tissue growth factor in congenital biliary atresia and neonatal hepatitis liver tissue. ( 26909983 )
2016
25
The dendritic cell-Th17-macrophage axis controls cholangiocyte injury and disease progression in murine and human biliary atresia. ( 27641439 )
2016
26
Incidence of biliary atresia associated congenital malformations: A retrospective multicenter study in China. ( 27210725 )
2016
27
Diffusion Tensor Imaging for Evaluating Biliary Atresia in Infants and Neonates. ( 27992499 )
2016
28
Common genetic variants of GPC1 gene reduce risk of biliary atresia in a Chinese population. ( 27373597 )
2016
29
Idiopathic neonatal hepatitis or extrahepatic biliary atresia? The role of liver biopsy. ( 27651555 )
2016
30
Infant Stool Color Card Screening Helps Reduce the Hospitalization Rate and Mortality of Biliary Atresia: A 14-Year Nationwide Cohort Study in Taiwan. ( 27015203 )
2016
31
Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia. ( 27957246 )
2016
32
Multiple orifices and cholangiography with a "fire-like" appearance after Kasai hepatoportoenterostomy for biliary atresia. ( 27502010 )
2016
33
Association between rs17095355 polymorphism on 10q24 and susceptibility to biliary atresia: a meta-analysis. ( 27557278 )
2016
34
The SRL peptide of rhesus rotavirus VP4 protein governs cholangiocyte infection and the murine model of biliary atresia. ( 27859498 )
2016
35
Ichthyosis congenita with biliary atresia: a rare association. ( 27824632 )
2016
36
Classification of biliary atresia in the laparoscopic era. ( 27655364 )
2016
37
The extended Kasai portoenterostomy for biliary atresia: A preliminary report. ( 27046977 )
2016
38
Liver transplantation in infants with biliary atresia: comparison of primary versus temporary abdominal closure. ( 27812908 )
2016
39
Role of CD56-expressing immature biliary epithelial cells in biliary atresia. ( 26937142 )
2016
40
FOXP3 PROMOTOR METHYLATION IMPAIRS SUPPRESSIVE FUNCTION of REGULATORY T CELLS in BILIARY ATRESIA. ( 27659419 )
2016
41
Biliary Atresia: Indications and Timing of Liver Transplantation and Optimization of Pre-Transplant Care. ( 27650268 )
2016
42
Impaired intention-to-treat survival after listing for liver transplantation in children with biliary atresia compared to other chronic liver diseases: 20A years' experience from the Nordic countries. ( 27957786 )
2016
43
Analysis of the prognostic factors of long-term native liver survival in survivors of biliary atresia. ( 27464487 )
2016
44
Biliary atresia: Where do we stand now? ( 28083081 )
2016
45
Hepatoblastoma in Explanted Livers of Patients With Biliary Atresia. ( 27457778 )
2016
46
Ultrasound for the Diagnosis of Biliary Atresia: A Meta-Analysis. ( 27010179 )
2016
47
Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre. ( 27859955 )
2016
48
Alagille Syndrome Candidates for Liver Transplantation: Differentiation from End-Stage Biliary Atresia Using Preoperative CT. ( 26901874 )
2016
49
Effect of microRNA-155 on the interferon-gamma signaling pathway in biliary atresia. ( 27817193 )
2016
50
Liquid chromatography-mass spectroscopy as a tool in the rapid diagnosis of biliary atresia: a pilot study. ( 26995515 )
2016

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:

id Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.85 ALB CFC1 CTGF GPC1 HGF JAG1
2 proteinaceous extracellular matrix GO:0005578 9.65 CTGF GPC1 MMP7 TGFB1 TIMP1
3 extracellular space GO:0005615 9.61 ALB CTGF GPC1 GPT HGF MMP7
4 platelet alpha granule lumen GO:0031093 8.92 ALB HGF TGFB1 TIMP1

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

id Name GO ID Score Top Affiliating Genes
1 Notch signaling pathway GO:0007219 9.58 JAG1 NR1H4 TGFB1
2 response to organic substance GO:0010033 9.5 SPP1 TGFB1 TIMP1
3 aging GO:0007568 9.46 CTGF MMP7 TGFB1 TIMP1
4 response to vitamin D GO:0033280 9.43 SPP1 TGFB1
5 extracellular matrix disassembly GO:0022617 9.43 MMP7 SPP1 TIMP1
6 cell activation GO:0001775 9.37 TGFB1 TIMP1
7 platelet degranulation GO:0002576 9.26 ALB HGF TGFB1 TIMP1
8 bile acid and bile salt transport GO:0015721 8.92 ALB NR1H4 SLC10A1 SLC10A2

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

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

Sources for Biliary Atresia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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