IPO
MCID: INT072
MIFTS: 53

Intestinal Pseudo-Obstruction (IPO) malady

Genetic diseases, Rare diseases, Neuronal diseases, Gastrointestinal diseases, Fetal diseases categories

Summaries for Intestinal Pseudo-Obstruction

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22Genetics Home Reference, 44NIH Rare Diseases, 66Wikipedia, 48OMIM, 34MalaCards
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NIH Rare Diseases:44 Intestinal pseudo-obstruction is a digestive disorder in which the intestinal walls are unable to contract normally (called hypomotility); the condition resembles a true obstruction, but no actual blockage exists. signs and symptoms may include abdominal pain; vomiting; diarrhea; constipation; malabsorption of nutrients leading to weight loss and/or failure to thrive; and other symptoms. it may be classified as neuropathic (from lack of nerve function) or myopathic (from lack of muscle function), depending on the source of the abnormality. the condition is sometimes inherited (in an x-linked recessive or autosomal dominant manner) and may be caused by mutations in the flna gene; it may also be acquired after certain illnesses. the goal of treatment is to provide relief from symptoms and ensure that nutritional support is adequate. last updated: 5/10/2011

MalaCards: Intestinal Pseudo-Obstruction, also known as congenital short bowel syndrome, is related to short bowel syndrome and pancreatitis. An important gene associated with Intestinal Pseudo-Obstruction is FLNA (filamin A, alpha), and among its related pathways is TCR signaling in naive CD4+ T cells. The compounds pyridostigmine and pirenzepine have been mentioned in the context of this disorder. Affiliated tissues include small intestine, liver and smooth muscle, and related mouse phenotypes are nervous system and digestive/alimentary.

Genetics Home Reference:22 Intestinal pseudo-obstruction is a condition characterized by impairment of the muscle contractions that move food through the digestive tract. The condition may arise from abnormalities of the gastrointestinal muscles themselves (myogenic) or from problems with the nerves that control the muscle contractions (neurogenic).

Wikipedia:66 Intestinal Pseudo-obstruction is a clinical syndrome caused by severe impairment in the ability of the... more...

Description from OMIM:48 615237,300048

Aliases & Classifications for Intestinal Pseudo-Obstruction

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22Genetics Home Reference, 63UMLS, 9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 46Novoseek, 48OMIM, 59SNOMED-CT, 36MeSH, 41NCIt
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Classifications:



Aliases & Descriptions:

intestinal pseudo-obstruction 9 44 22 11 46 63
congenital short bowel syndrome 22 48
hollow visceral myopathy 44 63
paralytic ileus 22 63
congenital idiopathic intestinal pseudoobstruction 63
chronic idiopathic intestinal pseudo-obstruction 22
pseudo-obstruction of gastrointestinal tract 63
pseudointestinal obstruction syndrome 22
pseudo-obstruction of intestine 9
intestinal pseudoobstruction 44
intestine pseudoobstruction 9
pseudoobstructive syndrome 22
enteric neuropathy 22
ciip 22
ipo 22


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Disease Ontology9 DOID:3878
MeSH36 D007418
SNOMED-CT59 235825006
NCIt41 C34733

Related Diseases for Intestinal Pseudo-Obstruction

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Sources:
18GeneCards, 19GeneDecks
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Diseases in the Intestinal Pseudo-Obstruction family:

Intestinal Obstruction

Diseases related to Intestinal Pseudo-Obstruction via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 161)
idRelated DiseaseScoreTop Affiliating Genes
1short bowel syndrome31.2SST
2pancreatitis30.5SST, SPINK1
3acute pancreatitis30.4SST, SPINK1
4paralytic ileus11.3
5systemic lupus erythematosus10.7
6lupus erythematosus10.7
7myopathy10.7
8neuropathy10.6
9amyloidosis10.6
10neuronitis10.6
11thymoma10.6
12intestinal pseudoobstruction neuronal chronic idiopathic x-linked10.5
13natal teeth, intestinal pseudoobstruction and patent ductus10.5
14peritonitis10.5
15visceral myopathy10.5
16appendicitis10.5
17myasthenia gravis10.5
18strongyloidiasis10.4
19intestinal obstruction10.4
20kawasaki disease10.4
21myotonic dystrophy10.4
22polymyositis10.4
23ileus10.4
24hypothyroidism10.4
25crohn's disease10.3
26gastroparesis10.3
27small cell carcinoma10.3
28patent ductus arteriosus10.3
29pneumatosis cystoides intestinalis10.3
30constipation10.3
31diarrhea10.3
32multiple sclerosis10.3
33ophthalmoplegia10.3
34scleroderma10.3
35pheochromocytoma10.3
36mitochondrial neurogastrointestinal encephalopathy disease10.3
37autonomic dysfunction10.3
38schizophrenia10.3
39ganglioneuroblastoma10.3
40hydrocephalus with congenital idiopathic intestinal pseudoobstruction10.3
41myopathic intestinal pseudoobstruction10.3
42unclassified intestinal pseudoobstruction10.3
43neurofibromatosis10.2
44myelitis10.2
45alcohol abuse10.2
46rectal prolapse10.2
47myxedema10.2
48steatorrhea10.2
49muscular atrophy10.2
50retroperitoneal leiomyosarcoma10.2

Graphical network of the top 20 diseases related to Intestinal Pseudo-Obstruction:



Diseases related to intestinal pseudo-obstruction

Symptoms for Intestinal Pseudo-Obstruction

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48OMIM
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Clinical features from OMIM:

615237,300048

Drugs & Therapeutics for Intestinal Pseudo-Obstruction

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

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Drug clinical trials:

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Search NIH Clinical Center for Intestinal Pseudo-Obstruction

Search CenterWatch for Intestinal Pseudo-Obstruction

Genetic Tests for Intestinal Pseudo-Obstruction

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Anatomical Context for Intestinal Pseudo-Obstruction

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34MalaCards
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MalaCards organs/tissues related to Intestinal Pseudo-Obstruction:

34
Small intestine, Liver, Smooth muscle, Colon, Lung, Lymph node

Animal Models for Intestinal Pseudo-Obstruction or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Intestinal Pseudo-Obstruction:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00036318.1ACHE, SST, FLNA, SOX10, B2M
2MP:00053818.1SST, FLNA, SOX10, B2M, SPINK1
3MP:00053787.5SPINK1, B2M, SOX10, FLNA, SST, ACHE
4MP:00053767.3SPINK1, B2M, SOX10, FLNA, SST, ACHE

Publications for Intestinal Pseudo-Obstruction

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53PubMed
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Articles related to Intestinal Pseudo-Obstruction:

(show top 50)    (show all 406)
idTitleAuthorsYear
1
Retinoblastoma protein prevents enteric nervous system defects and intestinal pseudo-obstruction. (24177421)
2013
2
Transgastric long tube placement following percutaneous endoscopic gastrostomy for severe chronic intestinal pseudo-obstruction related to systemic sclerosis. (24252025)
2013
3
Intestinal amyloidosis with intractable diarrhea and intestinal pseudo-obstruction. (23018539)
2012
4
Retraction of article. Eugen Florin Georgescu, Ion Vasile, Ana Claudia Georgescu. Intestinal pseudo-obstruction--a rare condition with heterogeneous etiology and unpredictable outcome. A case report. J Gastrointestin Liver Dis. March 2008; 17(1): 77-80. (22720315)
2012
5
Raising awareness about chronic intestinal pseudo-obstruction (CIPO): a case report showing CIPO as initial manifestation of atypical seronegative systemic sclerosis. (23059804)
2012
6
Prognostic yield of esophageal manometry in chronic intestinal pseudo-obstruction: a retrospective cohort of 116 adult patients. (22762287)
2012
7
An interesting case of intestinal pseudo-obstruction: MNGIE. (21922881)
2011
8
Intestinal pseudo-obstruction: initial manifestation of systemic lupus erythematosus. (22148114)
2011
9
Major gastrointestinal manifestations in lupus patients in Asia: lupus enteritis, intestinal pseudo-obstruction, and protein-losing gastroenteropathy. (20947549)
2010
10
Chronic intestinal pseudo-obstruction related to viral infections. (20172270)
2010
11
Frequency of mitochondrial defects in patients with chronic intestinal pseudo-obstruction. (19344718)
2009
12
Intestinal pseudo-obstruction associated with polymyositis successfully treated with somatostatin analog]. (18323156)
2008
13
Mitochondrial DNA A3243G mutation involved in familial diabetes, chronic intestinal pseudo-obstruction and recurrent pancreatitis. (18955007)
2008
14
Chronic intestinal pseudo-obstruction caused by an intestinal inflammatory myopathy: case report and review of the literature. (18004985)
2008
15
Fibrosing gastrointestinal leiomyositis as a cause of chronic intestinal pseudo-obstruction in an 8-month-old dog. (17197634)
2007
16
Chronic intestinal pseudo-obstruction due to buserelin-induced formation of anti-GnRH antibodies. (17241858)
2007
17
Intestinal pseudo-obstruction and neuroleptic malignant syndrome in a chronically constipated parkinsonian patient. (16618352)
2006
18
Inhibition of protein kinase A in murine enteric neurons causes lethal intestinal pseudo-obstruction. (16329126)
2006
19
Semiquantitative assessment of the intestinal motility in chronic intestinal pseudo-obstruction in systemic sclerosis and mixed connective tissue disease by Sitzmarks capsule. (17039319)
2006
20
Acute on chronic intestinal pseudo-obstruction as a cause of death in a previously healthy twenty-year-old male. (16614983)
2006
21
Does bcl2 immunostaining really help with pathological evaluation of intestinal pseudo-obstruction? (16861987)
2006
22
Caecal impaction and chronic intestinal pseudo-obstruction in a dog. (15703010)
2005
23
Multivisceral transplantation for pediatric intestinal pseudo-obstruction: single center's experience of 16 cases. (15050142)
2004
24
Prokinetics in the treatment of acute intestinal pseudo-obstruction. (15057661)
2004
25
Genetic background modifies intestinal pseudo-obstruction and the expression of a reporter gene in Hox11L1-/- mice. (14598259)
2003
26
SOX10 mutations in chronic intestinal pseudo-obstruction suggest a complex physiopathological mechanism. (12189494)
2002
27
Rare presentation of intestinal amyloidosis with acute intestinal pseudo-obstruction and perforation. (12401832)
2002
28
Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy. (10981652)
2000
29
Intestinal pseudo-obstruction due to neurofibromatosis responding to cisapride. (10812823)
2000
30
Chronic intestinal pseudo-obstruction: treatment and long term follow up of 44 patients. (10373127)
1999
31
Eosinophilic enterocolitis and visceral neuropathy with chronic intestinal pseudo-obstruction. (10372941)
1999
32
Myasthenia gravis, thymoma, intestinal pseudo-obstruction, and neuronal nicotinic acetylcholine receptor antibody. (10514242)
1999
33
Autonomic dysfunction in chronic intestinal pseudo-obstruction. (9869551)
1998
34
Desmin-rich smooth muscle bundles in chronic intestinal pseudo-obstruction. (9327377)
1997
35
Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults. (9414977)
1997
36
Manometric evaluation of gastrointestinal motility in children with chronic intestinal pseudo-obstruction syndrome. (8938348)
1996
37
Pancreatic somatostatinoma presenting with chronic intestinal pseudo-obstruction syndrome. (17912215)
1996
38
Cisapride as a successful treatment for acute intestinal pseudo-obstruction. (8701389)
1996
39
Small bowel resection for relief of chronic intestinal pseudo-obstruction. (7541935)
1995
40
Acute intestinal pseudo-obstruction due to malignant thymoma. (8266165)
1993
41
Erythromycin in chronic intestinal pseudo-obstruction. (2362109)
1990
42
Chronic intestinal pseudo-obstruction: diagnosis and treatment. (2642997)
1989
43
Intestinal pseudo-obstruction due to dialysis amyloidosis. (2612073)
1989
44
Idiopathic intestinal pseudo-obstruction and contaminated small bowel syndrome--treatment with metronidazole, ileostomy, and indomethacin. (6688501)
1983
45
Intestinal pseudo-obstruction as the presenting manifestation of small-cell carcinoma of the lung. A paraneoplastic neuropathy of the gastrointestinal tract. (6297344)
1983
46
Radiation-induced recurrent intestinal pseudo-obstruction. (6895001)
1981
47
Chronic intestinal pseudo-obstruction. A report of 27 cases and review of the literature. (6894476)
1981
48
Idiopathic intestinal pseudo-obstruction: a familial visceral neuropathy. (7438508)
1980
49
Intestinal pseudo-obstruction in alcohol abuse: report of two cases. (519478)
1979
50
Myxedema ileus. A form of intestinal pseudo-obstruction. (1242154)
1975

Variations for Intestinal Pseudo-Obstruction

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65UniProtKB/Swiss-Prot, 1 National Center for Biotechnology Information (Clinvar)
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UniProtKB/Swiss-Prot genetic disease variations for Intestinal Pseudo-Obstruction:

65
id Symbol AA change Variation ID SNP ID
1CLMPp.Val124AspVAR_069713

Clinvar genetic disease variations for Intestinal Pseudo-Obstruction:

1
id Gene Name Type Significance SNP ID Assembly Location
1FLNANM_001110556.1(FLNA): c.65_66delAC (p.Thr23Alafs)deletionPathogenicrs80338840GRCh37Chr X, 153599548: 153599549
2CLMPNM_024769.2(CLMP): c.230delA (p.Glu77Glyfs)deletionPathogenicGRCh37Chr 11, 122955378: 122955378
3CLMPNM_024769.2(CLMP): c.821G> A (p.Arg274Gln)single nucleotide variantPathogenicGRCh37Chr 11, 122945410: 122945410
4CLMPNM_024769.2(CLMP): c.664C> T (p.Arg222Ter)single nucleotide variantPathogenicGRCh37Chr 11, 122953808: 122953808
5CLMPNM_024769.2(CLMP): c.371T> A (p.Val124Asp)single nucleotide variantPathogenicGRCh37Chr 11, 122955237: 122955237

Expression for genes affiliated with Intestinal Pseudo-Obstruction

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Intestinal Pseudo-Obstruction

Search GEO for disease gene expression data for Intestinal Pseudo-Obstruction.

Pathways for genes affiliated with Intestinal Pseudo-Obstruction

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51PathCards, 39NCBI BioSystems Database
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Pathways related to Intestinal Pseudo-Obstruction according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
TCR signaling in naive CD8+ T cells39
9.5FLNA, B2M

Compounds for genes affiliated with Intestinal Pseudo-Obstruction

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46Novoseek, 12DrugBank, 30IUPHAR, 62Tocris Bioscience, 52PharmGKB, 25HMDB
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Compounds related to Intestinal Pseudo-Obstruction according to GeneCards/GeneDecks:

(show all 11)
idCompoundScoreTop Affiliating Genes
1pyridostigmine46 1210.7ACHE, SST
2pirenzepine46 30 1211.7ACHE, SST
3nitroglycerin46 1210.6B2M, SST
4atropine46 30 1211.6ACHE, SST
5captopril46 62 52 30 1213.5SST, B2M
6carboplatin46 52 1211.5SST, SOX10
7fluoride469.5B2M, SPINK1
8nifedipine46 30 52 1212.4SST, B2M
9dopamine46 30 25 1212.3FLNA, SST, ACHE
10sodium nitroprusside469.1SST, B2M
11serine468.5SPINK1, B2M, FLNA, ACHE

GO Terms for genes affiliated with Intestinal Pseudo-Obstruction

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17Gene Ontology
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Cellular components related to Intestinal Pseudo-Obstruction according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.0ACHE, SST, FLNA, B2M, SPINK1

Products for genes affiliated with Intestinal Pseudo-Obstruction

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Sources for Intestinal Pseudo-Obstruction

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet