EHBA
MCID: BLR028
MIFTS: 35

Biliary Atresia, Extrahepatic (EHBA)

Categories: Fetal diseases, Liver diseases, Rare diseases

Aliases & Classifications for Biliary Atresia, Extrahepatic

MalaCards integrated aliases for Biliary Atresia, Extrahepatic:

Name: Biliary Atresia, Extrahepatic 57 75 55
Isolated Atresia of Bile Ducts 59
Non-Syndromic Biliary Atresia 59
Isolated Biliary Atresia 59
Biliary Atresia 72
Ehba 57

Characteristics:

Orphanet epidemiological data:

59
isolated biliary atresia
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/100000 (Europe),1-9/100000 (Belgium),1-9/100000 (France),1-5/10000 (Germany),1-9/100000 (Italy),1-5/10000 (Netherlands),1-9/100000 (Norway),1-9/100000 (Spain),1-5/10000 (Switzerland),1-9/100000 (United Kingdom),1-5/10000 (Ukraine),1-5/10000 (Worldwide),1-9/100000 (Japan); Age of onset: Infancy,Neonatal;

OMIM:

57
Inheritance:
multifactorial

Miscellaneous:
progressive disorder
onset in early infancy
most patients require liver transplantation within the first year of life
incidence of 1 in 10,000 live births
increased incidence in asian countries (e.g., 1.46 per 10,000 live births in taiwan)


HPO:

32
biliary atresia, extrahepatic:
Onset and clinical course infantile onset progressive


Classifications:



External Ids:

OMIM 57 210500
MESH via Orphanet 45 D001656
ICD10 via Orphanet 34 Q44.2
UMLS via Orphanet 73 C0005411
Orphanet 59 ORPHA30391
MedGen 42 C4520983
UMLS 72 C0005411

Summaries for Biliary Atresia, Extrahepatic

OMIM : 57 Biliary atresia is a disorder of infants in which there is progressive obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction of bile flow. Untreated, the resulting cholestasis leads to progressive conjugated hyperbilirubinemia, cirrhosis, and hepatic failure (Bates et al., 1998). Most patients require liver transplantation within the first year of life (Leyva-Vega et al., 2010). See also Alagille syndrome (118450), which includes biliary atresia as a feature. (210500)

MalaCards based summary : Biliary Atresia, Extrahepatic, also known as isolated atresia of bile ducts, is related to cholestasis and biliary atresia, and has symptoms including icterus An important gene associated with Biliary Atresia, Extrahepatic is GPC1 (Glypican 1). The drugs Meloxicam and Anti-Inflammatory Agents have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and testes, and related phenotypes are hepatomegaly and cirrhosis

Wikipedia : 75 Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a... more...

Related Diseases for Biliary Atresia, Extrahepatic

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

(showing 13, show less)
# Related Disease Score Top Affiliating Genes
1 cholestasis 29.4 JAG1 GGT1
2 biliary atresia 10.7
3 varicose veins 10.2
4 esophageal varix 10.2
5 hypersplenism 10.2
6 portal hypertension 10.1
7 obstructive jaundice 10.1
8 bilirubin metabolic disorder 10.1
9 cholangitis 10.1
10 cholangitis, primary sclerosing 10.0
11 sclerosing cholangitis 10.0
12 syndromic biliary atresia 10.0
13 intrahepatic cholestasis of pregnancy 9.2 JAG1 GGT1

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



Diseases related to Biliary Atresia, Extrahepatic

Symptoms & Phenotypes for Biliary Atresia, Extrahepatic

Human phenotypes related to Biliary Atresia, Extrahepatic:

32 (showing 11, show less)
# Description HPO Frequency HPO Source Accession
1 hepatomegaly 32 HP:0002240
2 cirrhosis 32 HP:0001394
3 elevated hepatic transaminase 32 HP:0002910
4 jaundice 32 HP:0000952
5 unconjugated hyperbilirubinemia 32 HP:0008282
6 acholic stools 32 HP:0011985
7 increased total bilirubin 32 HP:0003573
8 extrahepatic biliary duct atresia 32 HP:0005242
9 portal fibrosis 32 HP:0006580
10 bile duct proliferation 32 HP:0001408
11 atretic gallbladder 32 HP:0011984

Symptoms via clinical synopsis from OMIM:

57
Abdomen Liver:
hepatomegaly
cirrhosis
bile duct proliferation
liver biopsy shows portal and/or bridging fibrosis
bile duct plugging

Abdomen Gastrointestinal:
acholic stools

Abdomen Biliary Tract:
atretic gallbladder
biliary atresia, extrahepatic
fibrotic extrahepatic bile ducts

Skin Nails Hair Skin:
jaundice

Laboratory Abnormalities:
increased total bilirubin
abnormal liver enzymes
increased indirect bilirubin

Clinical features from OMIM:

210500

UMLS symptoms related to Biliary Atresia, Extrahepatic:


icterus

Drugs & Therapeutics for Biliary Atresia, Extrahepatic

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

(showing 86, show less)
# 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
3 Analgesics Phase 4
4 Peripheral Nervous System Agents Phase 4
5 Cyclooxygenase Inhibitors Phase 4
6 Analgesics, Non-Narcotic Phase 4
7 Cyclooxygenase 2 Inhibitors Phase 4
8 Anti-Inflammatory Agents, Non-Steroidal Phase 4
9 Antirheumatic Agents Phase 4
10
Pentoxifylline Approved, Investigational Phase 2 6493-05-6 4740
11
Acetylcysteine Approved, Investigational Phase 2 616-91-1 12035
12 Antibodies Phase 1, Phase 2
13 Rho(D) Immune Globulin Phase 1, Phase 2
14 Immunoglobulins Phase 1, Phase 2
15 Immunoglobulins, Intravenous Phase 1, Phase 2
16 gamma-Globulins Phase 1, Phase 2
17
Bilirubin Phase 2 635-65-4, 69853-43-6 5280352 21252250
18 Platelet Aggregation Inhibitors Phase 2
19 Pharmaceutical Solutions Phase 2
20 Antioxidants Phase 2
21 Free Radical Scavengers Phase 2
22 Phosphodiesterase Inhibitors Phase 2
23 Protective Agents Phase 2
24 Radiation-Protective Agents Phase 2
25 Vasodilator Agents Phase 2
26 Anti-Infective Agents Phase 2
27 N-monoacetylcystine Phase 2
28 Respiratory System Agents Phase 2
29 Antidotes Phase 2
30 cysteine Phase 2
31 Expectorants Phase 2
32 Antiviral Agents Phase 2
33 Bile Acids and Salts Phase 2
34
Vancomycin Approved Phase 1 1404-90-6 441141 14969
35 Anti-Bacterial Agents Phase 1
36
Propofol Approved, Investigational, Vet_approved 2078-54-8 4943
37
Zoledronic Acid Approved 118072-93-8 68740
38
Prednisolone phosphate Approved, Vet_approved 302-25-0
39
Methylprednisolone hemisuccinate Approved 2921-57-5
40
Methylprednisolone Approved, Vet_approved 83-43-2 6741
41
Prednisolone Approved, Vet_approved 50-24-8 5755
42
Sevoflurane Approved, Vet_approved 28523-86-6 5206
43
Magnesium Sulfate Approved, Investigational, Vet_approved 7487-88-9 24083
44
Desflurane Approved 57041-67-5 42113
45
Neomycin Approved, Vet_approved 1404-04-2 8378
46
Protein C Approved
47
Sargramostim Approved, Investigational Early Phase 1 83869-56-1, 123774-72-1
48
Lenograstim Approved, Investigational Early Phase 1 135968-09-1
49
Calcium Approved, Nutraceutical 7440-70-2 271
50
Serine Approved, Nutraceutical 56-45-1 5951
51
Prednisolone hemisuccinate Experimental 2920-86-7
52 Hypnotics and Sedatives
53 Anesthetics, Intravenous
54 Bone Density Conservation Agents
55 Hormone Antagonists
56 glucocorticoids
57 Hormones
58 Methylprednisolone Acetate
59 Antineoplastic Agents, Hormonal
60 Prednisolone acetate
61 Hormones, Hormone Substitutes, and Hormone Antagonists
62 Central Nervous System Depressants
63 Anesthetics
64 Anesthetics, General
65 Anesthetics, Inhalation
66 Tocolytic Agents
67 Anti-Arrhythmia Agents
68 Anticonvulsants
69 calcium channel blockers
70 Calcium, Dietary
71 Gastrointestinal Agents
72 Neuroprotective Agents
73 Antiemetics
74 Autonomic Agents
75 Acidophilus
76 Liver Extracts
77 Immunologic Factors Early Phase 1
78 Hematinics
79 Alpha 1-Antitrypsin
80
protease inhibitors
81 HIV Protease Inhibitors
82 Trypsin Inhibitors
83 Serine Proteinase Inhibitors
84 Protein C Inhibitor
85 Adjuvants, Immunologic Early Phase 1
86 polysaccharide-K Early Phase 1

Interventional clinical trials:

(showing 37, show less)
# 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 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)
4 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
5 A Phase II Trial of Pentoxifylline in Newly-Diagnosed Biliary Atresia Recruiting NCT01774487 Phase 2 Pentoxifylline
6 A Phase 2 Trial of N-Acetylcysteine in Biliary Atresia After Kasai Portoenterostomy Recruiting NCT03499249 Phase 2 N-Acetyl cysteine
7 The Human Gastrointestinal Tract Microbiota in the Setting of Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin. Completed NCT01322386 Phase 1 Vancomycin
8 The Human Gastrointestinal Tract Microbiota in the Setting of Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Recruiting NCT02137668 Phase 1 Oral Vancomycin
9 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
10 Clinical and Basic Study for Pediatric Liver Transplantation Unknown status NCT02503384
11 Remote Ischemic Preconditioning Protects Against Hepatic Ischemic and Reperfusion Injury in Pediatric Living Donor Liver Transplantation Unknown status NCT02830841
12 The Effect of Sevoflurane or Propofol on Brain Injury and Neurocognitive in Pediatric Living Related Liver Transplantation Unknown status NCT03024840 Sevoflurane;Propofol
13 Molecular Genetic Study of Suspected Cases of Osteogenesis Imperfecta Attending Assiut University Children Hospital Unknown status NCT03169192 Zoledronic Acid
14 Comparison the Level of CTGF Protein and Related Cytokine in Pleural Effusion Among Tuberculous Pleurisy and Malignant Pleural Effusion Patients Unknown status NCT00313066
15 Prospective Trial on Survival With Own Liver After Conventional Versus Laparoscopic Kasai for Biliary Atresia Completed NCT01063699
16 the Correlation Between Hepatic Fibrotic Scores and ADC and Other Non-invasive Indicators in Patients With Biliary Atresia Completed NCT02848573
17 Biliary Atresia, Hepatic Buffer Response and Sevoflurane Completed NCT02471209 Sevoflurane
18 Study of Magnesium Sulfate in Children With Reduced Bone Density Secondary to Chronic Cholestatic Liver Disease Completed NCT00007033 magnesium gluconate;magnesium sulfate
19 A Randomized, Double-Blinded, Placebo-Controlled Trial of Corticosteroid Therapy Following Portoenterostomy in Infants With Biliary Atresia Completed NCT00294684 Corticosteroids;Placebo
20 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
21 Use of Probiotics to Prevent Cholangitis in Children With Biliary Atresia Completed NCT00166868 Lactobacillus casei rhamnosus (Lcr35);Neomycin
22 Search for Maternal Microchimerism in Swollen Portal Lymph Nodes of Infants With Biliary Atresia. Completed NCT02292862
23 POLYGEN DEFI-ALPHA : Genetic Polymorphisms Study in Children With Alpha-1 Antitrypsin Deficiency, Included in the DEFI-ALPHA Cohort Completed NCT01862211
24 Development and Validation of a Screening Score for the Biliary Atresia in Infantile Cholestasis: A Prospective Study Recruiting NCT03842150
25 Ultrasound Shear Wave Elastography Evaluation of Suspected and Known Biliary Atresia Recruiting NCT02652533
26 Biliary Atresia Study in Infants and Children (BASIC) Recruiting NCT00345553
27 Effects of Intraoperative Normal Saline vs Lactated Ringer on Outcomes in Pediatric Liver Transplantation: A Double-blind Randomized Trial Recruiting NCT03563378 Lactated Ringer;Normal saline
28 Coordinating Center- Mapping Disease Pathways for Biliary Atresia Recruiting NCT03273049
29 Clinical Follow-up Study on Hepatitis B Virus Infection After Liver Transplantation in Children Recruiting NCT03865966
30 Childhood Liver Disease Research Network (ChiLDReN): A Prospective Database of Infants With Cholestasis Recruiting NCT00061828
31 Longitudinal Study of Mitochondrial Hepatopathies Recruiting NCT01148550
32 Increased Liver Stiffness: A Study of Acoustic Radiation Force Impulse (ARFI) Elastography Recruiting NCT03382119
33 Childhood Liver Disease Research Network (ChiLDReN): FibroScan™ in Pediatric Cholestatic Liver Disease Study Protocol Active, not recruiting NCT02922751
34 Granulocyte-Colony Stimulating Factor Adjunct Therapy for Biliary Atresia Active, not recruiting NCT03395028 Early Phase 1 Granulocyte Colony-Stimulating Factor
35 Dry Blood Spot Screening Test for Neonatal Cholestasis Patients Enrolling by invitation NCT03667534
36 Intestinal Microbiome Composition in Infants With Biliary Atresia Not yet recruiting NCT03890536
37 Dry Blood Spot Screening Test for Biliary Atresia and Other Cholestasis Diseases Not yet recruiting NCT03898765

Search NIH Clinical Center for Biliary Atresia, Extrahepatic

Genetic Tests for Biliary Atresia, Extrahepatic

Anatomical Context for Biliary Atresia, Extrahepatic

MalaCards organs/tissues related to Biliary Atresia, Extrahepatic:

41
Liver, Bone, Testes, Bone Marrow, Brain, Lymph Node, Thymus

Publications for Biliary Atresia, Extrahepatic

Articles related to Biliary Atresia, Extrahepatic:

(showing 168, show less)
# Title Authors PMID Year
1
Extrahepatic biliary atresia and associated anomalies: etiologic heterogeneity suggested by distinctive patterns of associations. 38 8
8456846 1993
2
Idiopathic extrahepatic biliary atresia: recurrence in sibs in two families. 38 8
3232705 1988
3
Genomic alterations in biliary atresia suggest region of potential disease susceptibility in 2q37.3. 8
20358598 2010
4
Epidemiological features of biliary atresia in Taiwan, a national study 1996-2003. 8
17725591 2008
5
Familial biliary atresia. 8
10406679 1999
6
Biliary atresia: pathogenesis and treatment. 8
9773428 1998
7
Discordance for biliary atresia in two sets of monozygotic twins. 8
2993572 1985
8
Biliary atresia in two sets of twins. 8
4040965 1985
9
Biliary atresia associated with prenatal infection by Listeria monocytogenes. 8
4625786 1972
10
FAMILIAL EXTRAHEPATIC BILIARY ATRESIA. 8
14244102 1964
11
Congenital biliary atresia; report of three cases; two occurring in one family. 8
14928160 1952
12
The significance of human jagged 1 mutations detected in severe cases of extrahepatic biliary atresia. 9 38
12297837 2002
13
DYSTROPHIC CALCIFICATION OF MAXILLARY SINUS IN PEDIATRIC PATIENTS WITH LIVER TRANSPLANTATION AND PIGMENTATION OF DENTAL ORGAN. 38
29166493 2018
14
Disturbance in Plasma Metabolic Profile in Different Types of Human Cytomegalovirus-Induced Liver Injury in Infants. 38
29146975 2017
15
Hepatic MDR3 expression impacts lipid homeostasis and susceptibility to inflammatory bile duct obstruction in neonates. 38
28355206 2017
16
The value of preoperative liver biopsy in the diagnosis of extrahepatic biliary atresia: A systematic review and meta-analysis. 38
26932252 2016
17
Diagnostic value of anti-smooth muscle antibodies and liver enzymes in differentiation of extrahepatic biliary atresia and idiopathic neonatal hepatitis. 38
27251654 2016
18
A study of associated congenital anomalies with biliary atresia. 38
26862288 2016
19
Etiologies of conjugated hyperbilirubinemia in infancy: a systematic review of 1692 subjects. 38
26589959 2015
20
Evaluation of blood levels of nitric oxide as a means of differentiation between neonatal hepatitis and extrahepatic biliary atresia: A pilot study. 38
26166985 2015
21
Diagnostic value of procalcitonin and apo-e in extrahepatic biliary atresia. 38
25793072 2014
22
Efficacy of the seven feature, fifteen point histological scoring system and CD56 in interpretation of liver biopsies in persistent neonatal cholestasis: a five-year study. 38
24943749 2014
23
Predictive value of hepatic ultrasound, liver biopsy, and duodenal tube test in the diagnosis of extrahepatic biliary atresia in Serbian infants. 38
25003677 2014
24
Rejection is less common in children undergoing liver transplantation for hepatoblastoma. 38
24341552 2014
25
Extrahepatic biliary atresia with choledochal cyst: Prenatal MRI predicted and post natally confirmed: A case report. 38
24347854 2013
26
Scottish outcomes for extra hepatic biliary atresia post-rationalisation of services. 38
23429893 2013
27
[Acute, severe liver insufficiency caused by extrahepatic biliary atresia in a newborn]. 38
23651786 2013
28
Extra hepatic biliary atresia associated with choledochal cyst: a diagnostic dilemma in neonatal obstructive jaundice. 38
26023431 2013
29
Extrahepatic biliary atresia: Correlation of histopathology and liver function tests with surgical outcomes. 38
23243365 2012
30
Subdural hemorrhage: A unique case involving secondary vitamin K deficiency bleeding due to biliary atresia. 38
22607980 2012
31
Medical management of chronic liver diseases (CLD) in children (part II): focus on the complications of CLD, and CLD that require special considerations. 38
21999650 2011
32
Spontaneously ruptured choledochal cyst: Rare diagnosis on hepatic scintigraphy. 38
23326070 2011
33
Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days. 38
21427309 2011
34
Is there any association of extra hepatic biliary atresia with cytomegalovirus or other infections? 38
21465946 2011
35
Bone marrow mononuclear stem cell infusion improves biochemical parameters and scintigraphy in infants with biliary atresia. 38
20857300 2011
36
Surgical outcome in relation to duct size at the porta hepatis and the use of cholagogues in patients with biliary atresia. 38
21560523 2010
37
Liver transplantation for syndromic biliary atresia with a pedunculated accessory hepatic lobe. 38
19496982 2010
38
Influence of substrate complexity on the diastereoselective formation of spiroiminodihydantoin and guanidinohydantoin from chromate oxidation. 38
20014751 2010
39
A histopathological study of liver and biliary remnants with clinical outcome in cases of extrahepatic biliary atresia. 38
20090233 2010
40
Histologic features of the portal plate in extrahepatic biliary atresia and their impact on prognosis--a Danish study. 38
19573659 2009
41
The development of intrapulmonary arteriovenous shunts as a poor prognostic factor following surgery for biliary atresia. 38
19760999 2009
42
Selected summary. Extrahepatic biliary atresia (EHBA) is still a significant medical and economic burden to patients and families. 38
19172135 2009
43
Ultrasound findings in biliary atresia: the role of triangular cord sign. 38
19579927 2009
44
Biliary atresia: recent progress. 38
18496390 2008
45
Improvement in accuracy of gamma-glutamyl transferase for differential diagnosis of biliary atresia by correlation with age. 38
18773671 2008
46
Detection of Epstein Barr virus by chromogenic in situ hybridization in cases of extra-hepatic biliary atresia. 38
18442403 2008
47
CD 56 staining in liver biopsies does not help in differentiating extrahepatic biliary atresia from other causes of neonatal cholestasis. 38
18321379 2008
48
Spiroiminodihydantoin as an oxo-atom transfer product of 8-oxo-2'-deoxyguanosine oxidation by chromium(V). 38
17915881 2007
49
Interlobular bile duct loss in pediatric cholestatic disease is associated with aberrant cytokeratin 7 expression by hepatocytes. 38
17929991 2007
50
Stem cells as a therapeutic modality in pediatric malformations. 38
17445576 2007
51
Biliary atresia associated with multiple unrelated anomalies: what about it? 38
17021739 2007
52
Hepatobiliary scintigraphy and gamma-GT levels in the differential diagnosis of extrahepatic biliary atresia. 38
17220819 2007
53
C4' sugar oxidation of deoxyribonucleotide triphosphates by chromium(V) complexes. 38
16890478 2006
54
Immunosuppression in infants with short bowel syndrome undergoing isolated liver transplantation. 38
16911490 2006
55
Predictors of clinical outcome in children undergoing orthotopic liver transplantation for acute and chronic liver disease. 38
16741901 2006
56
Management of a cadaveric orthotopic liver transplantation in a pediatric patient with complex congenital heart disease. 38
16719884 2006
57
Extrahepatic biliary atresia demonstrates abnormal persistence of HES1 protein in neonatal biliary epithelium: an immunohistochemical study. 38
16822088 2006
58
Bile acid metabolism in extrahepatic biliary atresia: lithocholic acid in stored dried blood collected at neonatal screening. 38
16553252 2006
59
MR cholangiography and dynamic examination of duodenal fluid in the differential diagnosis between extrahepatic biliary atresia and infantile hepatitis syndrome. 38
17357501 2006
60
Gastroschisis and biliary atresia in a neonate: uncommon presentation or common precipitant. 38
16418964 2005
61
Neonatal cholestasis in Thai infants. 38
16856419 2005
62
Pathogenesis of extrahepatic bile duct atresia (EHBA): comprehension from a surgical point of view. 38
16163590 2005
63
Immune-mediated liver injury: prognostic value of CD4+, CD8+, and CD68+ in infants with extrahepatic biliary atresia. 38
16080928 2005
64
Biliary atresia with situs inversus: an experience shared. 38
16164164 2005
65
Non-enzymatic phosphorylation of bovine serum albumin by Cr(V) complexes: role in Cr(VI)-induced phosphorylation and toxicity. 38
16335795 2005
66
Neonatal surgical jaundice revisited. 38
15973025 2005
67
Extrahepatic bile duct atresia and viral involvement. 38
15667615 2005
68
Answered and unanswered controversies in the surgical management of extra hepatic biliary atresia. 38
15598338 2004
69
Oxidative activation of the human carcinogen chromate by arsenite: a model for synergistic metal activation leading to oxidative DNA damage. 38
15465638 2004
70
The role of Notch receptor expression in bile duct development and disease. 38
15307138 2004
71
Sequential therapy in a case of extrahepatic biliary atresia associated with ventricular septal defect: liver transplantation followed by surgical closure of the septal defect. 38
15229777 2004
72
Biliary atresia in Turkish children. 38
15056241 2004
73
Biliary atresia revisited. 38
14994122 2004
74
X-ray absorption spectroscopic studies of chromium(V/IV/III)- 2-ethyl-2-hydroxybutanoato(2-/1-) complexes. 38
14753827 2004
75
Role of some viral infections in neonatal cholestasis. 38
16734127 2004
76
[Dynamic examination of duodenal fluid in the differential diagnosis of infantile cholestatic jaundice]. 38
14990109 2004
77
Apoptosis of lymphocytes induced by chromium(VI/V) is through ROS-mediated activation of Src-family kinases and caspase-3. 38
14572611 2003
78
Long-term survival following Kasai portoenterostomy: is chronic liver disease inevitable? 38
14508212 2003
79
A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy. 38
12844260 2003
80
Divergent patterns of extracellular matrix protein expression in neonatal versus adult liver fibrosis. 38
14692230 2003
81
[Extrahepatic biliary atresia: diagnostic methods]. 38
14502330 2003
82
Immunohistochemical and molecular biological investigations regarding the pathogenesis of extrahepatic biliary atresia. (Part 1: immunohistochemical studies). 38
12664408 2003
83
[Expanding the grafts pool for pediatric liver transplantation with segmental grafts from living donor and cadaveric reduced size or split grafts]. 38
12187551 2002
84
Gamma-glutamyltransferase: value of its measurement in paediatrics. 9
11853185 2002
85
Induction of bile ducts in embryonic liver by mesenchyme: a new perspective for the treatment of biliary atresia? 38
11807667 2001
86
Betamethasone in plus phenobarbitone prior to hepatobiliary scintigraphy increases diagnostic accuracy in infants with jaundice. 38
11770238 2001
87
Immunological gap in the infectious animal model for biliary atresia. 38
11676556 2001
88
Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia. 38
11666059 2001
89
Hepatic technetium-99m-mebrofenin iminodiacetate scans and serum gamma-glutamyl transpeptidase levels interpreted in series to differentiate between extrahepatic biliary atresia and neonatal hepatitis. 38
11683209 2001
90
Disproportionation of a model chromium(V) complex causes extensive chromium(III)-DNA binding in vitro. 38
11511167 2001
91
Apoptosis of lymphocytes in the presence of Cr(V) complexes: role in Cr(VI)-induced toxicity. 38
11478807 2001
92
Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia. 38
11431768 2001
93
AIIMS clinical score: a reliable aid to distinguish neonatal hepatitis from extra hepatic biliary atresia. 38
11519282 2001
94
Association of biliary atresia and urogenital sinus. 38
11283895 2001
95
Anatomy of the porta hepatis as a basis for extended hepatoporto-enterostomy for extrahepatic biliary atresia--a new surgical technique. 38
11370976 2001
96
Extrahepatic biliary atresia in Saudi Arabia: the importance of early diagnosis and referral. 38
11398239 2001
97
Fat absorption and metabolism in bile duct ligated rats. 38
11585978 2001
98
An investigation of the chromium oxidation state of a monoanionic chromium tris(catecholate) complex by X-ray absorption and EPR spectroscopies. 38
11170525 2001
99
[Kasai operation in the age of liver transplantation. Healing or merely palliative technique?]. 38
12601938 2000
100
Disproportionation and nuclease activity of bis[2-ethyl-2-hydroxybutanoato(2-)]oxochromate(V) in neutral aqueous solutions. 38
11229553 2000
101
Prognosis of extrahepatic bile-duct atresia after hepatoportoenterostomy. 38
10955561 2000
102
Anaesthesia, perioperative management and outcome of correction of extrahepatic biliary atresia in the infant: a review of 50 cases in the King's College Hospital series. 38
11119190 2000
103
Formation of modified cleavage termini from the reaction of chromium(V) with DNA. 38
10643657 1999
104
Infantile liver giant cells: immunohistological study of their proliferative state and possible mechanisms of formation. 38
10347279 1999
105
Expression of the stem cell factor receptor c-kit in normal and diseased pediatric liver: identification of a human hepatic progenitor cell? 38
10385646 1999
106
Electron Spin Relaxation in Chromium-Nitrosyl Complexes. 38
11671100 1999
107
In vitro plasmid DNA cleavage by chromium(V) and -(IV) 2-hydroxycarboxylato complexes. 38
10207127 1999
108
Need for liver transplantation in Indian children. 38
10717692 1999
109
Anatomy of the porta hepatis (PH) as rational basis for the hepatoporto-enterostomy (HPE). 38
10207697 1999
110
The influence of cholangitis on the prognosis of extrahepatic biliary atresia. 38
10207698 1999
111
Biliary atresia and cytomegalovirus infection: a DNA study. 38
9841700 1999
112
Hypervalent chromium mimics reactive oxygen species as measured by the oxidant-sensitive dyes 2',7'-dichlorofluorescin and dihydrorhodamine. 38
9860481 1998
113
Evaluation of mebrofenin hepatoscintigraphy in neonatal-onset jaundice. 38
9880637 1998
114
[Transjugular intrahepatic portosystemic shunt in children. Initial clinical experiences and literature review]. 38
9687952 1998
115
Progress in developing animal models for biliary atresia. 38
9676394 1998
116
Detection of reovirus RNA in hepatobiliary tissues from patients with extrahepatic biliary atresia and choledochal cysts. 38
9620316 1998
117
Extrahepatic bile duct atresia: how efficient is the hepatoporto-enterostomy? 38
9676397 1998
118
Permeability, cytotoxicity, and genotoxicity of chromium (V) and chromium (VI) complexes in V79 Chinese hamster lung cells. 38
9511903 1998
119
Ultrasonographic "triangular cord": the most definitive finding for noninvasive diagnosis of extrahepatic biliary atresia. 38
9550270 1998
120
Direct and hydrogen peroxide-induced chromium(V) oxidation of deoxyribose in single-stranded and double-stranded calf thymus DNA. 38
9437531 1997
121
Treatment of extrahepatic biliary atresia with interferon-alpha in a murine infectious model. 38
9357935 1997
122
New aspects in a murine model for extrahepatic biliary atresia. 38
9269968 1997
123
Hyaline cartilage at porta hepatis in extrahepatic biliary atresia. 38
9211551 1997
124
Recurrent intestinal bleeding from jejunojejunostomy caused by portal hypertension following hepatoportojejunostomy in extra hepatic biliary atresia (EHBA)--successful treatment by transjugular intrahepatic portosystemic shunt (TIPS). 38
9241499 1997
125
Different expression of HLA-DR and ICAM-1 in livers from patients with biliary atresia and Byler's disease. 38
9126800 1997
126
Biliary, anorectal and esophageal atresia: a new entity? 38
9149339 1997
127
Liver transplantation for extra hepatic biliary atresia. 38
9149346 1997
128
Extrahepatic biliary atresia in twins: zygosity determination by short tandem repeat loci. 38
9071076 1996
129
Pre-operative time course changes in liver function tests in biliary atresia: its usefulness in the discrimination of biliary atresia in early infancy. 9
8942012 1996
130
Neonatal cholestasis syndrome: an appraisal at a tertiary center. 38
9057399 1996
131
Sibling occurrence of biliary atresia and biliary dilatation. 38
8887110 1996
132
Biliary atresia, cytomegalovirus, and age at referral. 38
8657522 1996
133
Lack of evidence for seasonal variation in extrahepatic biliary atresia during infancy. 38
8771425 1996
134
Prognostic value of serum hyaluronic acid and type I and III procollagen propeptides in extrahepatic biliary atresia. 38
8559611 1995
135
TIPS: a new therapy for esophageal variceal bleeding caused by EHBA. 38
7577858 1995
136
The Kasai procedure in the treatment of biliary atresia. 38
7472936 1995
137
The slow progression of hepatic fibrosis in intrahepatic cholestasis as compared with extrahepatic biliary atresia. 38
7612587 1995
138
Hepatic concentrations of zinc, copper and manganese in infants with extrahepatic biliary atresia. 38
8846156 1995
139
Neonatal cholestasis. New approaches to diagnostic evaluation and therapy. 38
7936782 1994
140
Reovirus serotype 3 infection in infants with extrahepatic biliary atresia or neonatal hepatitis. 38
8054525 1994
141
In vitro DNA damage and mutations induced by a macrocyclic tetraamide chromium(V) complex: implications for the role of Cr(V) peptide complexes in chromium-induced cancers. 38
8403213 1993
142
Increased deposition and serum level of type IV collagen in patients with extrahepatic biliary atresia. 38
8392544 1993
143
The influence of portoenterostomy with stoma on morbidity in pediatric patients with biliary atresia undergoing orthotopic liver transplantation. 38
8468652 1993
144
Association between HLA and extrahepatic biliary atresia. 38
8450374 1993
145
Clinico-pathological studies on a transitional type between extrahepatic biliary atresia and paucity of the interlobular bile ducts. 38
8318784 1993
146
Biliary atresia--lessons from the Dublin experience. 38
1473950 1992
147
[Liver transplant in childhood: our experience]. 38
1298933 1992
148
Experimental reovirus type 3-induced murine biliary tract disease. 38
1640330 1992
149
[Extrahepatic bile duct atresia. Comparison of surgical and non-surgical therapy]. 38
1501618 1992
150
Recurrence of extrahepatic biliary atresia in two half sibs. 38
1605254 1992
151
The relative importance of familial, reproductive and environmental factors in biliary atresia: etiological implications and effect on patient survival. 38
1342598 1992
152
Liver transplantation in children: an update. 38
1727323 1992
153
Hyperinflation in children with liver disease due to alpha-1-antitrypsin deficiency. 38
1758742 1991
154
Biliary atresia: extrahepatic? Intrahepatic? Both? Neither? 38
2038544 1991
155
The therapy of biliary atresia combining the Kasai portoenterostomy with liver transplantation: a single center experience. 38
2299540 1990
156
Bone disease in chronic childhood cholestasis. II. Better absorption of 25-OH vitamin D than vitamin D in extrahepatic biliary atresia. 38
2296467 1990
157
Liver transplantation in children: the initial Toronto experience. 38
2553908 1989
158
Use of serum gamma glutamyl transpeptidase to differentiate between extrahepatic biliary atresia and neonatal hepatitis. 38
2571663 1989
159
Resting energy expenditure is increased in infants and children with extrahepatic biliary atresia. 38
2738818 1989
160
Late referral for biliary atresia--missed opportunities for effective surgery. 38
2563796 1989
161
Time-space distribution of extrahepatic biliary atresia in The Netherlands and West Germany. 38
3388982 1988
162
Human extrahepatic biliary atresia: portal connective tissue activation related to ductular proliferation. 38
3537604 1986
163
Effects of postoperative cholestyramine and phenobarbital administration on bile flow restoration in infants with extrahepatic biliary atresia. 38
3517284 1986
164
Gamma-glutamyl transpeptidase activity and its serial measurement in differentiation between extrahepatic biliary atresia and neonatal hepatitis. 38
2859361 1985
165
Liver enzyme ratios in neonatal liver disease. 38
6524089 1984
166
The prelaparotomy diagnosis of extrahepatic biliary atresia. 38
6137197 1983
167
The Rose Bengal test in neonatal cholestasis: diagnostic and prognostic value. 38
7274296 1981
168
Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants. 38
1087549 1976

Variations for Biliary Atresia, Extrahepatic

Expression for Biliary Atresia, Extrahepatic

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