MCID: BLD063
MIFTS: 47

Bile Duct Cysts

Categories: Gastrointestinal diseases, Liver diseases, Rare diseases

Aliases & Classifications for Bile Duct Cysts

MalaCards integrated aliases for Bile Duct Cysts:

Name: Bile Duct Cysts 58 54 74
Choledochal Cyst 12 54 60 45 15 74
Congenital Choledochal Cyst 12 54
Choledochocele 58 54
Congenital Cystic Dilatation of the Biliary Tract 60
Choledochal Cysts 58

Classifications:

Orphanet: 60  
Rare hepatic diseases


External Ids:

Disease Ontology 12 DOID:899
OMIM 58 603003
MeSH 45 D015529
NCIt 51 C2943
SNOMED-CT 69 30533003
ICD10 34 Q44.4
Orphanet 60 ORPHA480501
MedGen 43 C0008340

Summaries for Bile Duct Cysts

MalaCards based summary : Bile Duct Cysts, also known as choledochal cyst, is related to biliary papillomatosis and bile duct adenocarcinoma. An important gene associated with Bile Duct Cysts is ADD3 (Adducin 3), and among its related pathways/superpathways are Bladder cancer and Central carbon metabolism in cancer. The drugs Hydrocortisone and Propofol have been mentioned in the context of this disorder. Affiliated tissues include pancreas, liver and kidney.

Wikipedia : 77 Choledochal cysts (a.k.a. bile duct cyst) are congenital conditions involving cystic dilatation of bile... more...

Description from OMIM: 603003

Related Diseases for Bile Duct Cysts

Diseases related to Bile Duct Cysts via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 147)
# Related Disease Score Top Affiliating Genes
1 biliary papillomatosis 30.5 HRAS KRAS
2 bile duct adenocarcinoma 29.9 HRAS KRAS
3 bile duct cancer 11.6
4 duodenal atresia 11.6
5 bile acid synthesis defect, congenital, 2 11.2
6 pancreatitis 10.7
7 biliary atresia 10.6
8 degos 'en cocarde' erythrokeratoderma 10.6
9 acute pancreatitis 10.5
10 adenocarcinoma 10.4
11 obstructive jaundice 10.4
12 choledocholithiasis 10.3
13 rhabdomyosarcoma 10.3
14 cholelithiasis 10.3
15 portal hypertension 10.3
16 peritonitis 10.3
17 papilloma 10.2
18 gallbladder cancer 10.2
19 embryonal rhabdomyosarcoma 10.2
20 cholangitis 10.2
21 sigmoid neoplasm 10.1 HRAS KRAS
22 apocrine adenoma 10.1 HRAS KRAS
23 periampullary adenoma 10.1 HRAS KRAS
24 paronychia 10.1 HRAS KRAS
25 schimmelpenning-feuerstein-mims syndrome 10.1 HRAS KRAS
26 aggressive digital papillary adenocarcinoma 10.1 HRAS KRAS
27 appendix disease 10.1 HRAS KRAS
28 lung adenoid cystic carcinoma 10.1 HRAS KRAS
29 nasal cavity adenocarcinoma 10.1 HRAS KRAS
30 secondary hypertrophic osteoarthropathy 10.1 HRAS KRAS
31 ascites, chylous 10.1
32 colonic atresia 10.1
33 cholecystitis 10.1
34 cystadenoma 10.1
35 duodenal obstruction 10.1
36 adenoma 10.1
37 anal canal adenocarcinoma 10.1 HRAS KRAS
38 ovarian cystadenocarcinoma 10.1 HRAS KRAS
39 acneiform dermatitis 10.1 HRAS KRAS
40 colloid carcinoma of the pancreas 10.1 HRAS KRAS
41 core binding factor acute myeloid leukemia 10.1 HRAS KRAS
42 mucinous lung adenocarcinoma 10.1 HRAS KRAS
43 duodenum cancer 10.1 HRAS KRAS
44 liver angiosarcoma 10.1 HRAS KRAS
45 cecum adenocarcinoma 10.1 HRAS KRAS
46 adenosquamous lung carcinoma 10.1 HRAS KRAS
47 gallbladder adenoma 10.1 HRAS PCNT
48 costello syndrome 10.1 HRAS KRAS
49 ampulla of vater cancer 10.1 HRAS KRAS
50 gastrointestinal system benign neoplasm 10.1 HRAS KRAS

Graphical network of the top 20 diseases related to Bile Duct Cysts:



Diseases related to Bile Duct Cysts

Symptoms & Phenotypes for Bile Duct Cysts

Clinical features from OMIM:

603003

Drugs & Therapeutics for Bile Duct Cysts

Drugs for Bile Duct Cysts (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 26)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 5754
2
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
3
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
4
Midazolam Approved, Illicit Phase 4 59467-70-8 4192
5
Etomidate Approved Phase 4 33125-97-2 667484 36339
6 Anesthetics, General Phase 4
7 Hydrocortisone hemisuccinate Phase 4
8 Central Nervous System Depressants Phase 4
9 GABA Modulators Phase 4
10 Adjuvants, Anesthesia Phase 4
11 Anesthetics Phase 4,Not Applicable
12 Anesthetics, Intravenous Phase 4
13 Anti-Anxiety Agents Phase 4
14 Hydrocortisone 17-butyrate 21-propionate Phase 4
15 Anti-Inflammatory Agents Phase 4
16 Psychotropic Drugs Phase 4
17 Hydrocortisone-17-butyrate Phase 4
18 Neurotransmitter Agents Phase 4
19 Tranquilizing Agents Phase 4
20 Hypnotics and Sedatives Phase 4
21 GABA Agents Phase 4
22
Pancrelipase Approved, Investigational 53608-75-6
23 pancreatin
24 Protein C Inhibitor
25 Alpha 1-Antitrypsin
26 Liver Extracts

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Effects of Etomidate on Postoperative Circadian Rhythm Changes of Salivary Cortisol in Children Unknown status NCT02013986 Phase 4 etomidate;midazolam;propofol
2 Superior Bilioenteric Anastomosis by Magnetic Compressive Technique Recruiting NCT02801500 Not Applicable
3 Endoscopic Access Loop With Bilio-enteric Anastomosis: A Prospective Randomized Comparison Between Gastric and Subcutaneous Accesses Not yet recruiting NCT03252379 Not Applicable
4 Cytological Evaluation of Biliary Epithelium After Endoscopic Sphincterotomy Completed NCT01135732
5 Bilioenteric Anastomosis by Magnetic Compressive Technique Recruiting NCT03774589 Not Applicable
6 Shear Wave Sonoelastography in Pediatric Liver Fibrosis Completed NCT02372682
7 UAB HRFD Core Center: Core A: The Hepato/Renal Fibrocystic Diseases Translational Resource Recruiting NCT01401998
8 Clinical and Molecular Investigations Into Ciliopathies Recruiting NCT00068224

Search NIH Clinical Center for Bile Duct Cysts

Cochrane evidence based reviews: choledochal cyst

Genetic Tests for Bile Duct Cysts

Anatomical Context for Bile Duct Cysts

MalaCards organs/tissues related to Bile Duct Cysts:

42
Pancreas, Liver, Kidney, Lung, Colon, Heart, Appendix

Publications for Bile Duct Cysts

Articles related to Bile Duct Cysts:

(show top 50) (show all 967)
# Title Authors Year
1
ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation. ( 30019982 )
2019
2
Giant choledochal cyst. An unusual cause of obstructive jaundice in an adult. ( 31104867 )
2019
3
The Double Trouble: A Case of Duplicated Extrahepatic Bile Duct with Choledochal Cyst. ( 30209736 )
2019
4
Todani type IVa choledochal cyst. ( 30683445 )
2019
5
The long-term health-related quality of life in patients operated for choledochal cyst. ( 30762105 )
2019
6
EBV Infection Unmasking a Choledochal Cyst in an Infant. ( 30838151 )
2019
7
Choledochal Cyst and Malignancy: A Plea for Lifelong Follow-Up. ( 29258149 )
2019
8
Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts. ( 29288314 )
2018
9
Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of bile duct cysts. ( 30139348 )
2018
10
Differentiation between cystic biliary atresia and choledochal cyst: A retrospective analysis. ( 29105184 )
2018
11
Cystic biliary atresia: A distinct clinical entity that may mimic choledochal cyst. ( 29904486 )
2018
12
Contribution of Imaging in the Diagnosis of Cholangiocarcinoma in Choledochal Cyst. ( 30643659 )
2018
13
Occurrence of signet-ring cell carcinoma with cholangiocarcinoma 25 years after choledochal cyst excision: A case report. ( 29465585 )
2018
14
Type VI Choledochal Cyst: An Unusual Case of a Bile Duct Anomaly. ( 29559068 )
2018
15
Mixed Adenoneuroendocrine Carcinoma (MANEC) of the Distal Common Bile Duct Arising from a Choledochal Cyst. ( 29580366 )
2018
16
Ruptured choledochal cyst during early pregnancy with successful fetomaternal outcome. ( 29654104 )
2018
17
Per-operative modified rigid cholangioscopy for removal of intrahepatic stones associated with choledochal cyst in children. ( 29794365 )
2018
18
Giant choledochal cyst presenting during third trimester of pregnancy. ( 29898912 )
2018
19
Choledochal cyst in the pediatric population: experience of 13 laparoscopic procedures in two years at a single institution. ( 29995153 )
2018
20
Introduction of resection of intrahepatic bile duct stenosis-causing membrane or septum into laparoscopic choledochal cyst excision. ( 30073480 )
2018
21
Coexistence of Complete Pancreas Divisum and Anomalous Pancreaticobiliary Junction in a Patient With Type IA Choledochal Cyst. ( 30077786 )
2018
22
Robotic-assisted surgery for pediatric choledochal cyst: Case report and literature review. ( 30079341 )
2018
23
Bilateral ultrasound-guided erector spinae plane block for postoperative analgesia in choledochal cyst resection surgery. ( 30100862 )
2018
24
Laparoscopic evaluation and resection of type-II choledochal cyst arising from right hepatic duct mimicking gall bladder duplication. ( 30178764 )
2018
25
Imaging Diagnosis of Type 3 Choledochal Cyst: A Case Report. ( 30266111 )
2018
26
An Unusual Case of Choledochal Cyst. ( 30370205 )
2018
27
Robot resection of a choledochal cyst with Roux-en-y hepaticojejunostomy in adults: Initial experiences with 22 cases and a comparison with laparoscopic approaches. ( 30588527 )
2018
28
Single-stage operation for perforated choledochal cyst. ( 28774506 )
2018
29
Choledochal Cyst with 17q12 Chromosomal Duplication. ( 28940454 )
2018
30
Choledochal cyst of the proximal cystic duct: a taxonomical and therapeutic conundrum. ( 29181996 )
2018
31
Clinical significance of intrapancreatic choledochal cyst excision in surgical management of type I choledochal cyst. ( 29322850 )
2018
32
Comparative analysis of different hepatico-jejunostomy techniques for treating adult type I choledochal cyst. ( 29479444 )
2018
33
Choledochal Cyst And Biliary Stone. ( 29504349 )
2018
34
Bile Duct Adenocarcinoma with Acute on Chronic Pancreatitis: A Rare Complication of Long Standing Choledochal Cyst. ( 28764249 )
2017
35
Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts: Results of the Multicenter Study of the French Surgical Association (AFC). ( 27620132 )
2017
36
Adult's congenital bile duct cysts. ( 29512796 )
2017
37
Cystic metastasis from a mucinous adenocarcinoma of duodenum mimicking type II choledochal cyst: A case report. ( 29290919 )
2017
38
Type I Choledochal Cyst Complicated With Acute Hemorrhagic Pancreatitis: A Case Report. ( 30065499 )
2017
39
Management of Complicated Choledochal Cyst in an Adult with Situs Inversus Totalis: Diagnostic Difficulties and Technical Notes. ( 29089712 )
2017
40
Laparoscopic Treatment of Choledochal Cyst Using Barbed Sutures. ( 27200460 )
2017
41
Evaluation of the learning curve of laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy in children: CUSUM analysis of a single surgeon's experience. ( 27338584 )
2017
42
The presentation and management of choledochocele (type III choledochal cyst): A 40-year systematic review of the literature. ( 27829523 )
2017
43
Laparoscopic resection of choledochal cyst with Roux-en-Y hepaticojejunostomy: a case report and review of the literature. ( 27873011 )
2017
44
Giant Choledochal Cyst in A Neonate. ( 28083508 )
2017
45
An impressive choledochal cyst and its surgical resection. ( 28273606 )
2017
46
Modified biliary-enteric anastomosis for congenital choledochal cyst: clinical and prognostic analysis of 91 cases. ( 28289881 )
2017
47
A rare case of choledochal cyst with pancreas divisum: case presentation and literature review. ( 28317046 )
2017
48
The distal classification and management of choledochal cyst in adults: Based on the relation between cyst and pancreatic duct. ( 28328818 )
2017
49
Laparoscopic Management of a Proximal Jejunal Gallstone Ileus with Patulous Ampulla and Choledochal Cyst-a Report of Unusual Presentation and a Review. ( 28331267 )
2017
50
Heterotopic pancreatic tissue associated with type 1 choledochal cyst, cystolithiasis and gall bladder stones: a rare entity with review of literature. ( 28396555 )
2017

Variations for Bile Duct Cysts

Expression for Bile Duct Cysts

Search GEO for disease gene expression data for Bile Duct Cysts.

Pathways for Bile Duct Cysts

GO Terms for Bile Duct Cysts

Biological processes related to Bile Duct Cysts according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 Ras protein signal transduction GO:0007265 9.32 HRAS KRAS
2 stimulatory C-type lectin receptor signaling pathway GO:0002223 9.26 HRAS KRAS
3 positive regulation of MAP kinase activity GO:0043406 9.16 HRAS KRAS
4 regulation of long-term neuronal synaptic plasticity GO:0048169 8.96 HRAS KRAS
5 response to isolation stress GO:0035900 8.62 HRAS KRAS

Molecular functions related to Bile Duct Cysts according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 GDP binding GO:0019003 8.62 HRAS KRAS

Sources for Bile Duct Cysts

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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