MCID: BLD063
MIFTS: 42

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 57 53 72
Choledochal Cyst 12 53 59 44 15 72 33
Congenital Choledochal Cyst 12 53
Choledochocele 57 53
Congenital Cystic Dilatation of the Biliary Tract 59
Choledochal Cysts 57

Classifications:

Orphanet: 59  
Rare hepatic diseases


External Ids:

Disease Ontology 12 DOID:899
OMIM 57 603003
MeSH 44 D015529
NCIt 50 C2943
SNOMED-CT 68 30533003
ICD10 33 Q44.4
Orphanet 59 ORPHA480501
MedGen 42 C0008340
UMLS 72 C0008340 C0267931

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 DUOXA2 (Dual Oxidase Maturation Factor 2), and among its related pathways/superpathways are Aryl Hydrocarbon Receptor and Vemurafenib Pathway, Pharmacodynamics. The drugs Midazolam and Propofol have been mentioned in the context of this disorder. Affiliated tissues include liver, pancreas and kidney.

Wikipedia : 75 Choledochal cysts (a.k.a. bile duct cyst) are congenital conditions involving cystic dilatation of bile... more...

More information 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 231)
# Related Disease Score Top Affiliating Genes
1 biliary papillomatosis 30.6 KRAS HRAS
2 bile duct adenocarcinoma 29.9 KRAS HRAS
3 duodenal atresia 11.7
4 bile acid synthesis defect, congenital, 2 11.4
5 biliary atresia 10.8
6 pancreatitis 10.7
7 peritonitis 10.7
8 cholelithiasis 10.6
9 cholecystitis 10.5
10 cholangitis 10.5
11 liver cirrhosis 10.5
12 intestinal obstruction 10.5
13 adenocarcinoma 10.4
14 ileus 10.4
15 rhabdomyosarcoma 10.4
16 cholangiocarcinoma 10.4
17 intrahepatic cholangiocarcinoma 10.4
18 recurrent acute pancreatitis 10.4
19 pancreas, annular 10.3
20 gastritis 10.3
21 adenoma 10.3
22 intussusception 10.3
23 biliary atresia, extrahepatic 10.3
24 neonatal jaundice 10.3
25 embryonal rhabdomyosarcoma 10.3
26 duodenal obstruction 10.3
27 toxic shock syndrome 10.2
28 cystadenoma 10.2
29 tubular adenocarcinoma 10.2
30 appendicitis 10.2
31 sigmoid neoplasm 10.2 KRAS HRAS
32 varicose veins 10.1
33 ascites, chylous 10.1
34 pancreatic cancer 10.1
35 colonic atresia 10.1
36 pontine tegmental cap dysplasia 10.1
37 vitamin k deficiency bleeding 10.1
38 hepatitis a 10.1
39 neuroendocrine tumor 10.1
40 papillary adenocarcinoma 10.1
41 liver disease 10.1
42 biliary dyskinesia 10.1
43 mirizzi syndrome 10.1
44 splenomegaly 10.1
45 posttransplant acute limbic encephalitis 10.1
46 apocrine adenoma 10.1 KRAS HRAS
47 paronychia 10.1 KRAS HRAS
48 periampullary adenoma 10.1 KRAS HRAS
49 schimmelpenning-feuerstein-mims syndrome 10.1 KRAS HRAS
50 aggressive digital papillary adenocarcinoma 10.1 KRAS HRAS

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 37)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Midazolam Approved, Illicit Phase 4 59467-70-8 4192
2
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
3
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 5754
4
Etomidate Approved Phase 4 33125-97-2 36339 667484
5
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
6 Neurotransmitter Agents Phase 4
7 Tranquilizing Agents Phase 4
8 Anti-Anxiety Agents Phase 4
9 Adjuvants, Anesthesia Phase 4
10 GABA Agents Phase 4
11 Hydrocortisone-17-butyrate Phase 4
12 Anti-Inflammatory Agents Phase 4
13 Central Nervous System Depressants Phase 4
14 Hypnotics and Sedatives Phase 4
15 Anesthetics, General Phase 4
16 GABA Modulators Phase 4
17 Psychotropic Drugs Phase 4
18 Anesthetics, Intravenous Phase 4
19 Hydrocortisone hemisuccinate Phase 4
20 Hydrocortisone 17-butyrate 21-propionate Phase 4
21
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
22
Gemcitabine Approved Phase 2 95058-81-4 60750
23
Fluorouracil Approved Phase 2 51-21-8 3385
24
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
25
Pancrelipase Approved, Investigational Phase 2 53608-75-6
26
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
27 pancreatin Phase 2
28 Cola Phase 2
29 Albumin-Bound Paclitaxel Phase 2
30 Anti-Infective Agents Phase 2
31 Immunosuppressive Agents Phase 2
32 Liver Extracts Phase 2
33 Antimetabolites Phase 2
34 Antiviral Agents Phase 2
35 Antimetabolites, Antineoplastic Phase 2
36 Immunologic Factors Phase 2
37 Anesthetics

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase 1 Study of Circadian Rhythm of Salivary Cortisol in Health Children ;Phase 2 Study of Circadian Rhythm of Salivary Cortisol of Children Undergoing Surgery Using Etomidate or Not Using Etomidate. Unknown status NCT02013986 Phase 4 etomidate;midazolam;propofol
2 Definitive Chemoradiation With Gemcitabine and Continuous 5- FU (Fluorouracil)Followed by High Dose Rate Brachytherapy or Stereotactic Body Radiation Therapy Boost in Locally Advanced Intra or Extrahepatic Cholangiocarcinoma Terminated NCT00983541 Phase 2 Fluorouracil (5-FU);Gemcitabine
3 Role of Complete Blood Picture in Predicting the Etiology of Extrahepatic Cholestasis Completed NCT03237247
4 Cytological Evaluation of Biliary Epithelium After Previous Endoscopic Sphincterotomy for Benign Disease Completed NCT01135732
5 The RCT Study of Two Surgical Biliary-enteric Reconstruction Minimally Invasive Treatment of Bile Duct Dilatation Recruiting NCT03401424
6 Bilioenteric Anastomosis by Magnetic Compressive Technique: A Prospective, Randomized Controlled Trial Recruiting NCT03774589
7 Superior Bilioenteric Anastomosis by Magnetic Compressive Technique: A Multicenter, Prospective, Randomized Controlled Trial Recruiting NCT02801500
8 COMPLICATIONS OF ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY- AN INDIAN PERSPECTIVE Active, not recruiting NCT04062617
9 Endoscopic Access Loop With Bilio-enteric Anastomosis: A Prospective Randomized Comparison Between Gastric and Subcutaneous Accesses Not yet recruiting NCT03252379
10 A Prospective Randomised Study on the Patency Period of the Plastic Anti-reflux Biliary Stent Terminated NCT01142921

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:

41
Liver, Pancreas, Kidney, Colon, Testes, Appendix, Skin

Publications for Bile Duct Cysts

Articles related to Bile Duct Cysts:

(show top 50) (show all 1816)
# Title Authors PMID Year
1
Familial occurrence of congenital bile duct cysts. 38 8
9570247 1998
2
Choledochal cyst--clinical features and classification. 38 8
4025281 1985
3
Discordance for anomalous pancreaticobiliary ductal junction and congenital biliary dilatation in a set of monozygotic twins. 8
1469575 1992
4
The choledochocele: correlation of radiological, clinical and pathological findings. 8
1244667 1976
5
Letter to editor re: Baba T, Mukai M, Masuya R, et al. (2019) The long-term health-related quality of life in patients operated for choledochal cyst. Pediatr Surg Int 35:539-546. 38
31197445 2019
6
Diagnostic yield of endoscopic ultrasonography for a dilation of the common bile duct of an indeterminate cause. 38
31373506 2019
7
Early complications after laparoscopic resection of choledochal cyst. 38
31134322 2019
8
Laparoscopic Management of Choledochal Cysts Associated with Aberrant Hepatic Ducts. 38
31314676 2019
9
Trends in Routine and Complex Hepatobiliary Surgery Among General and Pediatric Surgical Residents: What is the Next Generation Learning and is it Enough? 38
30902561 2019
10
The Presence of Pericholedochal Hyaline Cartilage in Biliary Atresia: A Report and A Review. 38
31314631 2019
11
A degenerate Todani Ia choledochal cyst in a patient with pancreas divisum. 38
31317754 2019
12
Todani type IVa choledochal cyst. 38
30683445 2019
13
Per-operative modified rigid cholangioscopy for removal of intrahepatic stones associated with choledochal cyst in children. 38
29794365 2019
14
Robotic-assisted surgery for choledochal cyst in children: early experience at Vietnam National Children's Hospital. 38
31270674 2019
15
Short-term and long-term outcomes after Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy following laparoscopic excision of choledochal cyst in children. 38
31342261 2019
16
Acute pancreatitis with Cullen's sign presentation in a case of mixed type I and II choledochal cyst. 38
31262503 2019
17
Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy. 38
31204550 2019
18
Bile Plug Syndrome Mimicking Choledochal Cyst. 38
31209759 2019
19
Association between gallbladder agenesis and choledochal cyst: cause or coincidence? 38
31248898 2019
20
Female-restricted syndromic intellectual disability in a patient from Thailand. 38
30828969 2019
21
The long-term health-related quality of life in patients operated for choledochal cyst. 38
30762105 2019
22
Giant choledochal cyst. An unusual cause of obstructive jaundice in an adult. 38
31104867 2019
23
Development of adenocarcinoma at the remnant intrapancreatic cyst 16 years after resection of the choledochal cyst. 38
31225424 2019
24
A modified technique of single-incision laparoscopic hepaticojejunostomy for children with choledochal cysts. 38
30971256 2019
25
Cholangiocarcinoma in a Resected Biliary Cyst: Importance of Follow-up. 38
31263640 2019
26
Choledochal Cyst and Malignancy: A Plea for Lifelong Follow-Up. 38
29258149 2019
27
Coils migrate into the biliary-jejunum anastomosis: A case report. 38
30732121 2019
28
The Double Trouble: A Case of Duplicated Extrahepatic Bile Duct with Choledochal Cyst. 38
30209736 2019
29
ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation. 38
30019982 2019
30
Polypoid Undifferentiated Carcinoma With Osteoclast-like Giant Cells Arising in the Distal Common Bile Duct: A Rare Case Report. 38
30591492 2019
31
ERCP in infants, children, and adolescents-Different roles of the methods in different age groups. 38
30653580 2019
32
CD4+T Cell Subset Profiling in Biliary Atresia Reveals ICOS- Regulatory T Cells as a Favorable Prognostic Factor. 38
31355166 2019
33
Concomitant adenosquamous carcinoma and cystadenocarcinoma of the extrahepatic bile duct: A case report. 38
30705898 2019
34
The Pediatric Surgical Team: a Model for Increased Surgeon Index Case Exposure. 38
30765153 2019
35
Laparoscopic Surgery in the treatment of children with Choledochal Cyst. 38
31258599 2019
36
EBV Infection Unmasking a Choledochal Cyst in an Infant. 38
30838151 2019
37
Open surgical treatment of choledochocele: A case report and review of literature. 38
30510953 2018
38
Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts. 38
29288314 2018
39
Robot resection of a choledochal cyst with Roux-en-y hepaticojejunostomy in adults: Initial experiences with 22 cases and a comparison with laparoscopic approaches. 38
30588527 2018
40
Interpreting body MRI cases: classic findings in abdominal MRI. 38
29594465 2018
41
Introduction of resection of intrahepatic bile duct stenosis-causing membrane or septum into laparoscopic choledochal cyst excision. 38
30073480 2018
42
Long-term outcomes of surgery for choledochal cysts: a single-institution study focusing on follow-up and late complications. 38
29679145 2018
43
Laparoscopic evaluation and resection of type-II choledochal cyst arising from right hepatic duct mimicking gall bladder duplication. 38
30178764 2018
44
Imaging Diagnosis of Type 3 Choledochal Cyst: A Case Report. 38
30266111 2018
45
Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of bile duct cysts. 38
30139348 2018
46
Coexistence of Complete Pancreas Divisum and Anomalous Pancreaticobiliary Junction in a Patient With Type IA Choledochal Cyst. 38
30077786 2018
47
Icterus and abdominal pain: an unexpected, rare sonographic finding in a Peruvian Emergency Department. 38
29998373 2018
48
Single-incision laparoscopic hepaticojejunostomy for children with perforated choledochal cysts. 38
29340827 2018
49
Management of Pediatric Patients Presenting with Acute Abdomen Accompanying Dilatation of the Common Bile Duct. 38
29992121 2018
50
A Rare Case of a Common Hepatic Duct Stricture Secondary to an Anteriorly Crossing Right Hepatic Artery in an Infant. 38
30050268 2018

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 regulation of long-term neuronal synaptic plasticity GO:0048169 8.96 KRAS HRAS
2 response to isolation stress GO:0035900 8.62 KRAS HRAS

Sources for Bile Duct Cysts

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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