MCID: MDN008
MIFTS: 31

Median Arcuate Ligament Syndrome

Categories: Rare diseases, Cardiovascular diseases, Muscle diseases

Aliases & Classifications for Median Arcuate Ligament Syndrome

MalaCards integrated aliases for Median Arcuate Ligament Syndrome:

Name: Median Arcuate Ligament Syndrome 12 53 59 15
Celiac Artery Compression Syndrome 12 53 59 73
Dunbar Syndrome 53 59 73
Mals 53 59
Harjola-Marable Syndrome 12
Celiac Access Syndrome 53
Marable's Syndrome 12

Characteristics:

Orphanet epidemiological data:

59
celiac artery compression syndrome
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Disease Ontology 12 DOID:9892
ICD10 33 I77.4
ICD9CM 35 447.4
SNOMED-CT 68 9250002
Orphanet 59 ORPHA293208
ICD10 via Orphanet 34 I77.4

Summaries for Median Arcuate Ligament Syndrome

NIH Rare Diseases : 53 Celiac artery compression syndrome is a rare disorder characterized by chronic, recurrent abdominal pain related to compression of the celiac artery (which supplies blood to the upper abdominal organs) by the median arcuate ligament (a muscular fibrous band of the diaphragm). It usually presents with symptoms of abdominal pain, weight loss, and an abdominal bruit (abnormal sound of a blood vessel when blocked or narrowed). The consequence of chronic celiac trunk compression is unknown. While no known correlation currently exists, future studies may find other gastrointestinal disorders in patients diagnosed with this syndrome. The cause is not fully understood; however, it is suspected that there could be a combination of vascular (blood supply) and neurogenic (neurological) components involved. Diagnosis is usually confirmed with imaging such as CT angiography, MRI, ultrasound, and arteriography. Surgery is currently the only treatment option and involves releasing the ligament.

MalaCards based summary : Median Arcuate Ligament Syndrome, also known as celiac artery compression syndrome, is related to mal de meleda and mal de debarquement syndrome. An important gene associated with Median Arcuate Ligament Syndrome is UHMK1 (U2AF Homology Motif Kinase 1). Affiliated tissues include liver, and related phenotypes are Decreased substrate adherent cell growth and Decreased substrate adherent cell growth

Wikipedia : 76 In medicine, the median arcuate ligament syndrome (MALS, also known as celiac artery compression... more...

Related Diseases for Median Arcuate Ligament Syndrome

Diseases related to Median Arcuate Ligament Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 63)
# Related Disease Score Top Affiliating Genes
1 mal de meleda 12.5
2 mal de debarquement syndrome 12.4
3 epilepsy, idiopathic generalized 10 11.7
4 pinta disease 11.4
5 childhood absence epilepsy 11.2
6 epilepsy, idiopathic generalized 11.2
7 naxos disease 11.1
8 epilepsy, myoclonic juvenile 11.0
9 malaria, mild 11.0
10 status epilepticus 11.0
11 salt and pepper developmental regression syndrome 11.0
12 epilepsy with generalized tonic-clonic seizures 10.8
13 papillon-lefevre syndrome 10.8
14 seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance 10.8
15 palmoplantar keratoderma, nagashima type 10.8
16 argentine hemorrhagic fever 10.8
17 juvenile absence epilepsy 10.8
18 benign neonatal seizures 10.8
19 squamous cell carcinoma, head and neck 10.5
20 cervical squamous cell carcinoma 10.5
21 tongue squamous cell carcinoma 10.5
22 aneurysm 10.4
23 colorectal adenoma 10.4
24 epilepsy 10.2
25 thrombosis 10.2
26 systemic lupus erythematosus 10.0
27 pancreatic cancer 10.0
28 deficiency anemia 10.0
29 crohn's disease 10.0
30 hepatitis 10.0
31 hereditary hemorrhagic telangiectasia 10.0
32 arteriovenous malformation 10.0
33 iron deficiency anemia 10.0
34 histoplasmosis 10.0
35 vascular disease 10.0
36 iron metabolism disease 10.0
37 ischemia 10.0
38 cerebritis 10.0
39 gastritis 10.0
40 pancreatitis 10.0
41 chronic intestinal vascular insufficiency 10.0
42 vasculitis 10.0
43 lupus erythematosus 10.0
44 retroperitoneal fibrosis 10.0
45 cerebral aneurysms 10.0
46 lymphoma 10.0
47 cervicitis 10.0
48 palmoplantar keratosis 10.0
49 mediastinitis 10.0
50 b-cell lymphomas 10.0

Graphical network of the top 20 diseases related to Median Arcuate Ligament Syndrome:



Diseases related to Median Arcuate Ligament Syndrome

Symptoms & Phenotypes for Median Arcuate Ligament Syndrome

GenomeRNAi Phenotypes related to Median Arcuate Ligament Syndrome according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased substrate adherent cell growth GR00193-A-1 9.33 HCK UHMK1
2 Decreased substrate adherent cell growth GR00193-A-3 9.33 UHMK1
3 Downregulation of NF-kappaB pathway after LMP1 stimulation GR00199-A-1 8.96 NCR3 UHMK1
4 Downregulation of NF-kappaB pathway after TNFalpha stimulation GR00199-A-2 8.62 NCR3 UHMK1

Drugs & Therapeutics for Median Arcuate Ligament Syndrome

Search Clinical Trials , NIH Clinical Center for Median Arcuate Ligament Syndrome

Genetic Tests for Median Arcuate Ligament Syndrome

Anatomical Context for Median Arcuate Ligament Syndrome

MalaCards organs/tissues related to Median Arcuate Ligament Syndrome:

41
Liver

Publications for Median Arcuate Ligament Syndrome

Articles related to Median Arcuate Ligament Syndrome:

(show top 50) (show all 73)
# Title Authors Year
1
A predictive model for patients with median arcuate ligament syndrome. ( 29845396 )
2018
2
Treatment of the Median Arcuate Ligament Syndrome in a Patient with Behcet's Disease. ( 29772313 )
2018
3
The Laparoscopic Management of Median Arcuate Ligament Syndrome and Its Long-Term Outcomes. ( 29781769 )
2018
4
Median arcuate ligament syndrome - imaging presentation and interdisciplinary management. ( 29975975 )
2018
5
Robotic-assisted approach to Median Arcuate Ligament Syndrome with left gastric artery originating directly from the aorta. Report of a case and review of the current mini-invasive treatment modalities. ( 29745060 )
2018
6
Psychological Factors and Outcomes in the Surgical Treatment of Pediatric Patients With Median Arcuate Ligament Syndrome. ( 29373439 )
2018
7
Laparoscopic approach to median arcuate ligament syndrome (with video). ( 29678321 )
2018
8
Celiac Plexus Block as a Predictor of Surgical Outcome for Sympathetically Mediated Abdominal Pain in a Case of Suspected Median Arcuate Ligament Syndrome: A Case Report. ( 29634547 )
2018
9
Median arcuate ligament syndrome and arterial anastomotic bleeding inducing hepatic artery thrombosis after liver transplantation: A case report. ( 29923979 )
2018
10
Hemodynamic benefits of celiac artery release for ruptured right gastric artery aneurysm associated with median arcuate ligament syndrome: a case report. ( 29183305 )
2017
11
Median Arcuate Ligament Syndrome: A Single-Center Experience with 23 Patients. ( 28050657 )
2017
12
Outcome predictors in median arcuate ligament syndrome. ( 28189355 )
2017
13
Median Arcuate Ligament Syndrome: It Is Not Always Gastritis. ( 28904981 )
2017
14
Laparoscopic Treatment of Median Arcuate Ligament Syndrome: Analysis of Long-Term Outcomes and Predictive Factors. ( 29185149 )
2017
15
Pancreaticoduodenal artery aneurysms due to median arcuate ligament syndrome: what we need to know. ( 29209989 )
2017
16
Median Arcuate Ligament Syndrome - Literature Review and Case Report. ( 29701344 )
2017
17
Exercise induced median arcuate ligament syndrome in athletes: case series. ( 28462569 )
2017
18
Laparoscopic Treatment of Median Arcuate Ligament Syndrome: A Rare Cause of Chronic Severe Abdominal Pain. ( 28082779 )
2017
19
Intraoperative measurement of pressure gradient in median arcuate ligament syndrome as a rationale for radical surgical approach. ( 28859519 )
2017
20
A New Laparoscopic Manoeuvre in Median Arcuate Ligament Syndrome. ( 29215344 )
2017
21
Open vascular treatment of median arcuate ligament syndrome. ( 28851450 )
2017
22
Median arcuate ligament syndrome - predictor of ischemic complications? ( 27560288 )
2016
23
Median arcuate ligament syndrome. ( 27095474 )
2016
24
Acute median arcuate ligament syndrome after pancreaticoduodenectomy. ( 27744644 )
2016
25
Median Arcuate Ligament Syndrome-Review of This Rare Disease. ( 26934394 )
2016
26
Median arcuate ligament syndrome: case presentation and video-illustrated laparoscopic management. ( 27980769 )
2016
27
Median arcuate ligament syndrome in athletes. ( 27177949 )
2016
28
Novel application of four-dimensional wide-area detector computed tomographic angiography for investigation of median arcuate ligament syndrome. ( 27601318 )
2016
29
Hybrid (laparoscopy + stent) treatment of celiac trunk compression syndrome (Dunbar syndrome, median arcuate ligament syndrome (MALS)). ( 28194242 )
2016
30
Pediatric Chronic Abdominal Pain and Median Arcuate Ligament Syndrome: A Review and Psychosocial Comparison. ( 27403674 )
2016
31
Lessons learned from a case of multivessel median arcuate ligament syndrome in the setting of an Arc of Buhler. ( 27594946 )
2016
32
Laparoscopic decompression as treatment for median arcuate ligament syndrome. ( 26320770 )
2015
33
Exercise-related transient abdominal pain secondary to median arcuate ligament syndrome: a case report. ( 26542078 )
2015
34
Median arcuate ligament syndrome. ( 25523198 )
2015
35
Surgical treatment of median arcuate ligament syndrome: case report and review of literature. ( 25845256 )
2015
36
An unusual case of left renal artery compression: a rare type of median arcuate ligament syndrome. ( 25940812 )
2015
37
Case report: median arcuate ligament syndrome diagnosed with computed tomography and Doppler ultrasonography. ( 25739105 )
2015
38
Median arcuate ligament syndrome. ( 25823773 )
2015
39
Doppler ultrasound diagnosis of an unusual variant of median arcuate ligament syndrome: concomitant involvement of celiac and superior mesenteric arteries. A case report. ( 26649356 )
2015
40
A case of pancreatic cancer with concomitant median arcuate ligament syndrome treated successfully using an allograft arterial transposition. ( 26715412 )
2015
41
Imaging in median arcuate ligament syndrome. ( 26655665 )
2015
42
Contemporary management of median arcuate ligament syndrome provides early symptom improvement. ( 25758451 )
2015
43
Technique and outcomes of robot-assisted median arcuate ligament release for celiac artery compression syndrome. ( 25600337 )
2015
44
Median Arcuate Ligament Syndrome Is Not a Vascular Disease. ( 26365109 )
2015
45
Pancreaticoduodenal Artery Aneurysms Secondary to Median Arcuate Ligament Syndrome May Not Need Celiac Artery Revascularization or Ligament Release. ( 24930977 )
2014
46
Pancreaticoduodenal artery aneurysm associated with median arcuate ligament syndrome. ( 24495330 )
2014
47
Clinical and radiologic review of uncommon cause of profound iron deficiency anemia: median arcuate ligament syndrome. ( 25053902 )
2014
48
Histoplasmosis as a possible cause of retroperitoneal fibrosis and median arcuate ligament syndrome: A case report. ( 24981164 )
2014
49
Abdominal pain in systemic lupus erythematosus: lupus enteritis, mesenteric thrombosis, or median arcuate ligament syndrome? ( 25379650 )
2014
50
Pediatric median arcuate ligament syndrome: surgical outcomes and quality of life. ( 24328507 )
2014

Variations for Median Arcuate Ligament Syndrome

Expression for Median Arcuate Ligament Syndrome

Search GEO for disease gene expression data for Median Arcuate Ligament Syndrome.

Pathways for Median Arcuate Ligament Syndrome

GO Terms for Median Arcuate Ligament Syndrome

Biological processes related to Median Arcuate Ligament Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein autophosphorylation GO:0046777 8.62 HCK UHMK1

Molecular functions related to Median Arcuate Ligament Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 signaling receptor binding GO:0005102 8.8 GCG HCK NCR3

Sources for Median Arcuate Ligament Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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