MCID: INT191
MIFTS: 50

Intestinal Lymphangiectasia malady

Cardiovascular diseases, Gastrointestinal diseases, Rare diseases, Immune diseases categories
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Summaries for Intestinal Lymphangiectasia

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33MalaCards
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MalaCards: Intestinal Lymphangiectasia, also known as lymphangiectasis, intestinal, is related to yellow nail syndrome and lymphedema, and has symptoms including neoplasms/tumors, osteomyelitis/osteitis/periostitis/spondylodisciitis and pericardium anomalies/pericarditis/absence/cysts/pericardial effusion. An important gene associated with Intestinal Lymphangiectasia is SOX18 (SRY (sex determining region Y)-box 18). The compounds hyaluronic acid and valproic acid have been mentioned in the context of this disorder. Affiliated tissues include skin, b cells and small intestine, and related mouse phenotypes are normal and liver/biliary system.

Aliases & Classifications for Intestinal Lymphangiectasia

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49Orphanet, 62UMLS, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

49
intestinal lymphangiectasia:
Age of onset: Variable


Aliases & Descriptions:

intestinal lymphangiectasia 49
lymphangiectasis, intestinal 62


External Ids:

MESH via Orphanet36 D008201
ICD10 via Orphanet26 I89.0
SNOMED-CT via Orphanet59 197260007
UMLS via Orphanet63 C0024215

Related Diseases for Intestinal Lymphangiectasia

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Intestinal Lymphangiectasia:



Diseases related to intestinal lymphangiectasia

Symptoms for Intestinal Lymphangiectasia

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49Orphanet
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Symptoms:

49 (show all 18)
  • neoplasms/tumors
  • osteomyelitis/osteitis/periostitis/spondylodisciitis
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • edema of the legs/lower limbs
  • anomalies of the lymphatic system
  • agammaglobulinemia/hypogammaglobulinemia/b-cell deficiency
  • lymphopenia
  • anomalies of the ribs
  • chronic skin infection/ulcerations/ulcers/cancrum
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • severe allergic reaction/atopy
  • hypocalcemia
  • failure to thrive/difficulties for feeding in infancy/growth delay
  • visual loss/blindness/amblyopia
  • thick skin/pachydermia/orange skin
  • ascitis
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • malabsorption/chronic diarrhea/steatorrhea

Drugs & Therapeutics for Intestinal Lymphangiectasia

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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Intestinal Lymphangiectasia

Search NIH Clinical Center for Intestinal Lymphangiectasia

Genetic Tests for Intestinal Lymphangiectasia

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Anatomical Context for Intestinal Lymphangiectasia

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33MalaCards
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MalaCards organs/tissues related to Intestinal Lymphangiectasia:

33
Skin, B cells, Small intestine, Liver, Bone marrow, Bone, Lymph node, Brain, Heart, Endothelial, Colon, Kidney, Breast, T cells

Animal Models for Intestinal Lymphangiectasia or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Intestinal Lymphangiectasia:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00028738.6ALB, FOXC2, FLT4, SOX18
2MP:00053708.6ALB, FOXC2, FLT4
3MP:00053858.6FOXC2, LYVE1, FLT4, SOX18
4MP:00053878.5SOX18, FLT4, LYVE1, FOXC2
5MP:00053788.3FOXC2, RELN, FLT4, SOX18
6MP:00053818.2ALB, FOXC2, RELN, FLT4
7MP:00053698.2FLT4, RELN, FOXC2, ALB
8MP:00107687.7SOX18, FLT4, RELN, FOXC2, ALB

Publications for Intestinal Lymphangiectasia

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52PubMed
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Articles related to Intestinal Lymphangiectasia:

(show top 50)    (show all 237)
idTitleAuthorsYear
1
Diffuse large B-cell lymphoma recurrence complicating primary intestinal lymphangiectasia. (23591283)
2013
2
Duodenal endoscopic findings and histopathologic confirmation of intestinal lymphangiectasia in dogs. (22827501)
2012
3
Intestinal lymphangiectasia associated with recurrence of histiocytosis X. (21947159)
2011
4
Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. (22110841)
2011
5
Secondary intestinal lymphangiectasia due to multiple myeloma. (21092952)
2011
6
Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. (21191781)
2011
7
Digital clubbing in primary intestinal lymphangiectasia: a case report. (20812055)
2010
8
Intestinal lymphangiectasia presenting with duodeno-jejunal polyposis: enteroscopic findings. (21086252)
2010
9
Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. (19853733)
2009
10
Primary intestinal lymphangiectasia (Waldmann's disease). (18294365)
2008
11
Intestinal lymphangiectasia presenting as abdominal mass. (17321261)
2007
12
Intestinal lymphangiectasia with protein-losing enteropathy in Waldenstrom macroglobulinemia. (17632262)
2007
13
Primary intestinal lymphangiectasia as a component of autoimmune polyglandular syndrome type I: a report of 2 cases. (18431016)
2007
14
Use of capsule endoscopy in children with primary intestinal lymphangiectasia. (16996365)
2006
15
Enteral nutrition as a primary therapy for intestinal lymphangiectasia: value of elemental diet and polymeric diet compared with total parenteral nutrition. (16110837)
2005
16
Bilateral breast edema in intestinal lymphangiectasia. (16174162)
2005
17
Focal intestinal lymphangiectasia. (14722552)
2004
18
Successful treatment of protein-losing enteropathy induced by intestinal lymphangiectasia in a liver cirrhosis patient with octreotide: a case report. (15201518)
2004
19
Successful resection of localized intestinal lymphangiectasia post-Fontan: role of (99m)technetium-dextran scintigraphy. (12949320)
2003
20
Congenital intestinal lymphangiectasia: CT demonstration in a young child. (12932684)
2003
21
Severe lymphedema, intestinal lymphangiectasia, seizures and mild mental retardation: further case of Hennekam syndrome with a severe phenotype. (12124738)
2002
22
Stable reversal of pathologic signs of primitive intestinal lymphangiectasia with a hypolipidic, MCT-enriched diet. (10758368)
2000
23
Ultrasonographic features of long-standing primary intestinal lymphangiectasia. (9700215)
1998
24
Intestinal lymphangiectasia. (9770894)
1998
25
Octreotide in the treatment of intestinal lymphangiectasia. (9744700)
1998
26
Intestinal lymphangiectasia due to recurrent giardiasis. (8607534)
1996
27
Intestinal lymphangiectasia. (9057388)
1996
28
Intestinal leakage of technetium-99m-MDP in primary intestinal lymphangiectasia. (8691257)
1996
29
Protein-losing enteropathy due to intestinal lymphangiectasia accompanied by mesenteric lymph node fibrosis. (8970198)
1996
30
Endoscopic features of long-standing primary intestinal lymphangiectasia. (7615236)
1995
31
Infantile intestinal lymphangiectasia and chylous ascites in Saudi Arabia. (17590685)
1993
32
Intestinal lymphangiectasia masquerading as coeliac disease. (1721792)
1991
33
The local mucosal immune system in intestinal lymphangiectasia. (3184157)
1988
34
Endoscopic diagnosis of primary intestinal lymphangiectasia using a high-fat meal. (3720681)
1986
35
Dietary management of intestinal lymphangiectasia complicated by short gut syndrome. (3700137)
1986
36
Intestinal lymphangiectasia in a dog. (16031026)
1984
37
Primary intestinal lymphangiectasia. Case report with radiological and ultrastructural study. (7227669)
1981
38
Severe enamel hypoplasia in a case of intestinal lymphangiectasia: a rare protein-losing enteropathy. (266150)
1977
39
Malignant lymphoma and extensive viral wart formation in a patient with intestinal lymphangiectasia and lymphocyte depletion. (604991)
1977
40
Primary intestinal lymphangiectasia: surgical implications. (5026674)
1972
41
Intestinal lymphangiectasia. (5280865)
1971
42
Intestinal lymphangiectasia. (5486217)
1970
43
Intestinal lymphangiectasia with normal immunological function. A case report. (4190697)
1970
44
Studies of the immunologic defects associated with intestinal lymphangiectasia, with some observations on dietary control of chylous ascites. (4181615)
1968
45
Intestinal lymphangiectasia with hypomagnesaemic tetany. (5684612)
1968
46
Chronic hypoalbuminemia and edema associated with intestinal lymphangiectasia. (5898076)
1965
47
INTESTINAL LYMPHANGIECTASIA MECHANISM OF ENTERIC LOSS OF PLASMA-PROTEIN AND FAT. (14234205)
1965
48
LOW FAT DIET IN INTESTINAL LYMPHANGIECTASIA. (14202400)
1964
49
INTESTINAL LYMPHANGIECTASIA. (14226225)
1964
50
SYSTEMIC LYMPHATIC ABNORMALITIES ASSOCIATED WITH GASTROINTESTINAL PROTEIN LOSS SECONDARY TO INTESTINAL LYMPHANGIECTASIA. (14134004)
1963

Variations for Intestinal Lymphangiectasia

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Expression for genes affiliated with Intestinal Lymphangiectasia

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Intestinal Lymphangiectasia

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Pathways for genes affiliated with Intestinal Lymphangiectasia

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Compounds for genes affiliated with Intestinal Lymphangiectasia

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45Novoseek, 24HMDB, 51PharmGKB, 11DrugBank
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Compounds related to Intestinal Lymphangiectasia according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1hyaluronic acid45 2410.2LYVE1, ALB
2valproic acid45 51 24 1112.0RELN, ALB
3lipid458.6RELN, FOXC2, ALB
4glucose458.6LYVE1, FOXC2, ALB

GO Terms for genes affiliated with Intestinal Lymphangiectasia

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16Gene Ontology
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Biological processes related to Intestinal Lymphangiectasia according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1embryonic heart tube developmentGO:0350509.7FOXC2, SOX18
2vascular endothelial growth factor receptor signaling pathwayGO:0480109.6FLT4, FOXC2
3vasculature developmentGO:0019449.5SOX18, FLT4
4positive regulation of endothelial cell migrationGO:0105959.4FOXC2, FLT4
5lymphangiogenesisGO:0019469.2FOXC2, FLT4, SOX18
6peptidyl-tyrosine phosphorylationGO:0181089.1RELN, FLT4
7negative regulation of apoptotic processGO:0430668.6ALB, FOXC2, FLT4

Molecular functions related to Intestinal Lymphangiectasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1transcription regulatory region DNA bindingGO:0442129.6FOXC2, SOX18
2RNA polymerase II core promoter proximal region sequence-specific DNA binding transcription factor activity involved in positive regulation of transcriptionGO:0010779.3FOXC2, SOX18

Products for genes affiliated with Intestinal Lymphangiectasia

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Intestinal Lymphangiectasia

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet