MCID: BLD063
MIFTS: 44

Bile Duct Cysts

Categories: Rare diseases, Gastrointestinal diseases, Liver diseases

Aliases & Classifications for Bile Duct Cysts

MalaCards integrated aliases for Bile Duct Cysts:

Name: Bile Duct Cysts 53 49 69
Choledochal Cyst 12 49 55 41 14 69
Congenital Choledochal Cyst 12 49
Choledochocele 53 49
Congenital Cystic Dilatation of the Biliary Tract 55
Choledochal Cysts 53

Classifications:

Orphanet: 55  
Rare hepatic diseases


External Ids:

OMIM 53 603003
Disease Ontology 12 DOID:899
ICD10 32 Q44.4
MeSH 41 D015529
NCIt 46 C2943
Orphanet 55 ORPHA480501
MedGen 39 C0008340

Summaries for Bile Duct Cysts

MalaCards based summary : Bile Duct Cysts, also known as choledochal cyst, is related to choledocholithiasis and sigmoid neoplasm. An important gene associated with Bile Duct Cysts is WDR19 (WD Repeat Domain 19), and among its related pathways/superpathways are Agrin Interactions at Neuromuscular Junction and Aryl Hydrocarbon Receptor. The drugs Etomidate and Hydrocortisone have been mentioned in the context of this disorder. Affiliated tissues include liver and kidney.

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 51)
# Related Disease Score Top Affiliating Genes
1 choledocholithiasis 10.1
2 sigmoid neoplasm 10.1 HRAS KRAS
3 senior-loken syndrome 1 10.1 TMEM67 WDR19
4 apocrine adenoma 10.1 HRAS KRAS
5 periampullary adenoma 10.0 HRAS KRAS
6 schimmelpenning-feuerstein-mims syndrome 10.0 HRAS KRAS
7 paronychia 10.0 HRAS KRAS
8 aggressive digital papillary adenocarcinoma 10.0 HRAS KRAS
9 secondary hypertrophic osteoarthropathy 10.0 HRAS KRAS
10 nasal cavity adenocarcinoma 10.0 HRAS KRAS
11 nevus, epidermal 10.0 HRAS KRAS
12 lung adenoid cystic carcinoma 10.0 HRAS KRAS
13 ovary serous adenocarcinoma 10.0 HRAS KRAS
14 ampulla of vater neoplasm 10.0 HRAS KRAS
15 ovarian cystadenocarcinoma 10.0 HRAS KRAS
16 biliary papillomatosis 10.0 HRAS KRAS
17 pulmonic stenosis 10.0 HRAS KRAS
18 acneiform dermatitis 10.0 HRAS KRAS
19 colloid carcinoma of the pancreas 10.0 HRAS KRAS
20 ovary adenocarcinoma 10.0 HRAS KRAS
21 mixed cell type cancer 10.0 HRAS KRAS
22 adenosquamous cell lung carcinoma 10.0 HRAS KRAS
23 adenosquamous pancreas carcinoma 10.0 HRAS KRAS
24 duodenum cancer 10.0 HRAS KRAS
25 cecum adenocarcinoma 10.0 HRAS KRAS
26 core binding factor acute myeloid leukemia 10.0 HRAS KRAS
27 biliary tract neoplasm 10.0 HRAS KRAS
28 liver angiosarcoma 10.0 HRAS KRAS
29 lacrimal gland adenocarcinoma 9.9 HRAS KRAS
30 costello syndrome 9.9 HRAS KRAS
31 bile duct adenocarcinoma 9.9 HRAS KRAS
32 gastrointestinal system benign neoplasm 9.9 HRAS KRAS
33 exanthem 9.9 HRAS KRAS
34 intestinal benign neoplasm 9.9 HRAS KRAS
35 cardiofaciocutaneous syndrome 1 9.9 HRAS KRAS
36 duodenal atresia 9.8
37 bilirubin metabolic disorder 9.8
38 pancreatitis 9.8
39 situs inversus 9.8
40 duodenitis 9.8
41 malignant ovarian surface epithelial-stromal neoplasm 9.8 HRAS KRAS
42 ovary epithelial cancer 9.8 HRAS KRAS
43 cholangiocarcinoma 9.8
44 hepatitis 9.8
45 myoblastoma 9.8
46 kidney disease 9.8
47 autosomal dominant polycystic kidney disease 9.8
48 polycystic kidney disease 9.8
49 lung benign neoplasm 9.8 HRAS KRAS
50 cell type benign neoplasm 9.7 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 27)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Etomidate Approved Phase 4 33125-97-2 667484 36339
2
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 657311 5754
3
Midazolam Approved, Illicit Phase 4 59467-70-8 4192
4
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
5
Butyric Acid Experimental, Investigational Phase 4 107-92-6 264
6 Adjuvants, Anesthesia Phase 4
7 Anesthetics Phase 4
8 Anesthetics, General Phase 4
9 Anesthetics, Intravenous Phase 4
10 Anti-Anxiety Agents Phase 4
11 Anti-Inflammatory Agents Phase 4
12 Central Nervous System Depressants Phase 4
13 Cortisol succinate Phase 4
14 GABA Agents Phase 4
15 GABA Modulators Phase 4
16 Hydrocortisone 17-butyrate 21-propionate Phase 4
17 Hydrocortisone acetate Phase 4
18 Hydrocortisone-17-butyrate Phase 4
19 Hypnotics and Sedatives Phase 4
20 Neurotransmitter Agents Phase 4
21 Psychotropic Drugs Phase 4
22 Tranquilizing Agents Phase 4
23
Pancrelipase Approved, Investigational 53608-75-6
24 Alpha 1-Antitrypsin
25 Liver Extracts
26 pancreatin
27 Protein C Inhibitor

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 Cytological Evaluation of Biliary Epithelium After Endoscopic Sphincterotomy Completed NCT01135732
3 Superior Bilioenteric Anastomosis by Magnetic Compressive Technique Recruiting NCT02801500
4 Shear Wave Sonoelastography in Pediatric Liver Fibrosis Recruiting NCT02372682
5 UAB HRFD Core Center: Core A: The Hepato/Renal Fibrocystic Diseases Translational Resource Recruiting NCT01401998
6 Clinical and Molecular Investigations Into Ciliopathies Active, not recruiting NCT00068224
7 Endoscopic Access Loop With Bilio-enteric Anastomosis: A Prospective Randomized Comparison Between Gastric and Subcutaneous Accesses Not yet recruiting NCT03252379

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:

38
Liver, Kidney

Publications for Bile Duct Cysts

Articles related to Bile Duct Cysts:

(show top 50) (show all 651)
# Title Authors Year
1
Cystic metastasis from a mucinous adenocarcinoma of duodenum mimicking type II choledochal cyst: A case report. ( 29290919 )
2017
2
Different clinical presentations of choledochal cyst among infants and older children: A 10-year retrospective study. ( 28445267 )
2017
3
Choledochal Cyst with 17q12 Chromosomal Duplication. ( 28940454 )
2017
4
Spontaneous Rupture of a Choledochal Cyst During Post Partum: A Rare Presentation. ( 28496535 )
2017
5
Laparoscopic excision of the choledochal cyst in adult patients: An experience. ( 28872095 )
2017
6
Management of Complicated Choledochal Cyst in an Adult with Situs Inversus Totalis: Diagnostic Difficulties and Technical Notes. ( 29089712 )
2017
7
Ectopic Hepatocellular Carcinoma within a Choledochal Cyst Diagnosed Using Single-Operator Digital Cholangioscopy. ( 28766569 )
2017
8
Modified biliary-enteric anastomosis for congenital choledochal cyst: clinical and prognostic analysis of 91 cases. ( 28289881 )
2017
9
Laparoscopic Management of a Proximal Jejunal Gallstone Ileus with Patulous Ampulla and Choledochal Cyst-a Report of Unusual Presentation and a Review. ( 28331267 )
2017
10
Primary Epidermoid Cyst of Biliary Duct Presenting as Choledochal Cyst. ( 28508685 )
2017
11
Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts. ( 29288314 )
2017
12
External Drainage of Giant Infantile Choledochal Cyst before Definitive Repair: Is it Worth? ( 28892977 )
2017
13
Heterotopic pancreatic tissue associated with type 1 choledochal cyst, cystolithiasis and gall bladder stones: a rare entity with review of literature. ( 28396555 )
2017
14
Giant Choledochal Cyst in A Neonate. ( 28083508 )
2017
15
Cystic Biliary Atresia and Choledochal Cysts are Distinct Histopathologic Entities. ( 28079575 )
2017
16
Isolated dilatation of the cystic duct-Type VI choledochal cyst: a rare case presentation and review of the literature. ( 28458872 )
2017
17
Single-stage operation for perforated choledochal cyst. ( 28774506 )
2017
18
An impressive choledochal cyst and its surgical resection. ( 28273606 )
2017
19
The efficacy of resection of intrahepatic bile duct stenosis-causing membrane or septum for preventing hepatolithiasis after choledochal cyst excision. ( 28927985 )
2017
20
The distal classification and management of choledochal cyst in adults: Based on the relation between cyst and pancreatic duct. ( 28328818 )
2017
21
Embryonal rhabdomyosarcoma of the biliary tree: A rare cause of obstructive jaundice in children which can mimic choledochal cysts. ( 29089679 )
2017
22
A rare case of choledochal cyst with pancreas divisum: case presentation and literature review. ( 28317046 )
2017
23
Bile Duct Adenocarcinoma with Acute on Chronic Pancreatitis: A Rare Complication of Long Standing Choledochal Cyst. ( 28764249 )
2017
24
Duodenogastric regurgitation in hepaticoduodenostomy after excision of congenital biliary dilatation (choledochal cyst). ( 28410789 )
2017
25
A variant of type VI choledochal cyst: combined dilatation of cystic duct and common bile duct. ( 26941882 )
2016
26
Laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy. ( 27436941 )
2016
27
Type VI Choledochal Cyst-An Unusual Presentation of Jaundice. ( 27867293 )
2016
28
Percutaneous Transhepatic Angioplasty for Portal Vein Cavernous Transformation after Choledochal Cyst Surgery. ( 28064268 )
2016
29
Clinical effects in resection of congenital choledochal cyst of children and jejunum Roux-Y anastomosis by laparoscope. ( 27874945 )
2016
30
Metachronous cholangiocarcinoma 13A years post resection of choledochal cyst-is long-term follow-up useful?: a case study and review of the literature. ( 27307284 )
2016
31
Traumatic Rupture of Choledochal Cyst in Children: Use of Computed Tomography in the Emergency Department. ( 26928097 )
2016
32
Laparoscopic management of antenatally detected choledochal cyst: a 10-year review. ( 27129540 )
2016
33
Endoscopic Ultrasound Images of a Choledochal Cyst Mimicking a Pregnant Uterus. ( 27924091 )
2016
34
Choledochal cyst Todani IA case report. ( 27769027 )
2016
35
Increased survivin expression and its association with clinical parameters of congenital choledochal cysts. ( 27420945 )
2016
36
Cholangiocarcinoma in choledochal cyst after cystoenterostomy: how a mistreated choledochal cyst can progress to malignancy. ( 28261701 )
2016
37
Choledochal cyst with an aberrant right cholecysto-hepatic duct draining into cystic duct and a review of literature. ( 27695219 )
2016
38
Gallbladder Volvulus in a Patient with Type I Choledochal Cyst: A Case Report and Review of the Literature. ( 27747125 )
2016
39
Cosmetic selection of skin incision for resection of choledochal cyst in young female patients. ( 27621750 )
2016
40
Laparoscopic Treatment of Choledochal Cyst Using Barbed Sutures. ( 27200460 )
2016
41
Ciliated Foregut Cyst of the Common Hepatic Duct: An Unusual Mimic of a Type II Choledochal Cyst. ( 27166199 )
2016
42
Todani choledochal cyst type II: case report and review. ( 27813504 )
2016
43
Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts. ( 27358675 )
2016
44
Laparoscopic treatment of congenital choledochal cyst and hepaticojejunostomy with extracorporeal Roux-en-Y anastomosis: technical aspects and early experience with three cases. ( 27345604 )
2016
45
Treatment of choledochal cyst in a pediatric population. A single institution experience of 15-years. Case series. ( 26900456 )
2016
46
Intracholecystic papillary-tubular neoplasm in a patient with choledochal cyst: a link between choledochal cyst and gallbladder cancer? ( 27480698 )
2016
47
Dilatation of the Proximal Cystic Duct: Is It a Variant to "Type VI" Choledochal Cyst? ( 27504348 )
2016
48
Rupture of a Choledochal Cyst in an Adult Female: A Rare Consequence of Blunt Abdominal Trauma. ( 27647205 )
2016
49
Choledochal cyst: a future indication for peroral choledochoscopy? ( 27852091 )
2016
50
The presentation and management of choledochocele (type III choledochal cyst): A 40-year systematic review of the literature. ( 27829523 )
2016

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 cilium assembly GO:0060271 9.43 PCNT TMEM67 WDR19
2 ERBB2 signaling pathway GO:0038128 9.32 HRAS KRAS
3 regulation of long-term neuronal synaptic plasticity GO:0048169 9.16 HRAS KRAS
4 positive regulation of protein phosphorylation GO:0001934 9.13 AGER HRAS KRAS
5 response to isolation stress GO:0035900 8.62 HRAS KRAS

Sources for Bile Duct Cysts

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