MCID: PRM158
MIFTS: 19

Primary Intestinal Lymphangiectasia malady

Categories: Genetic diseases, Rare diseases, Gastrointestinal diseases, Immune diseases

Aliases & Classifications for Primary Intestinal Lymphangiectasia

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Aliases & Descriptions for Primary Intestinal Lymphangiectasia:

Name: Primary Intestinal Lymphangiectasia 46 66
Familial Waldmann's Disease 46 66
Waldmann Disease 46 66
 
Primary Intestinal Lymphangiectasis 46
Waldmann's Disease 46

Classifications:



Summaries for Primary Intestinal Lymphangiectasia

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NIH Rare Diseases:46 Primary intestinal lymphangiectasia is a digestive disorder in which the lymph vessels supplying the lining of the small intestine are enlarged. the cause of the condition is still unknown. the signs and symptoms include swelling of the legs and abdominal discomfort, loss of lymphatic fluid into the gastrointestinal tract, protein-losing enteropathy, too little albumin in the blood, reduced levels of antibodies, and immunodeficiency. treatment involves a special long-term diet. last updated: 10/6/2015

MalaCards based summary: Primary Intestinal Lymphangiectasia, also known as familial waldmann's disease, is related to lymphangiectasia, intestinal and lymphoma. Affiliated tissues include small intestine, b cells and liver.

Related Diseases for Primary Intestinal Lymphangiectasia

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Diseases in the Lymphangiectasia, Intestinal family:

primary intestinal lymphangiectasia Secondary Intestinal Lymphangiectasia

Diseases related to Primary Intestinal Lymphangiectasia via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 13)
idRelated DiseaseScoreTop Affiliating Genes
1lymphangiectasia, intestinal11.5
2lymphoma10.1
3osteomalacia10.0
4lymphangiectasis10.0
5b-cell lymphomas10.0
6protein-losing enteropathy10.0
7osteoporosis9.9
8diffuse large b-cell lymphoma9.8
9nephrotic syndrome9.8
10tuberous sclerosis9.8
11lymphangioma9.8
12adenocarcinoma9.8
13refractory anemia9.8

Graphical network of diseases related to Primary Intestinal Lymphangiectasia:



Diseases related to primary intestinal lymphangiectasia

Symptoms for Primary Intestinal Lymphangiectasia

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Drugs & Therapeutics for Primary Intestinal Lymphangiectasia

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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Primary Intestinal Lymphangiectasia

Genetic Tests for Primary Intestinal Lymphangiectasia

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Anatomical Context for Primary Intestinal Lymphangiectasia

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MalaCards organs/tissues related to Primary Intestinal Lymphangiectasia:

34
Small intestine, B cells, Liver, Skin

Animal Models for Primary Intestinal Lymphangiectasia or affiliated genes

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Publications for Primary Intestinal Lymphangiectasia

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Articles related to Primary Intestinal Lymphangiectasia:

(show top 50)    (show all 62)
idTitleAuthorsYear
1
Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions: A Case Report. (26962779)
2016
2
Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy. (26908672)
2016
3
How to treat an extensive form of primary intestinal lymphangiectasia? (26109821)
2015
4
Severe Refractory Anemia in Primary Intestinal Lymphangiectasia. A Case Report. (26405709)
2015
5
Primary intestinal lymphangiectasia with generalized warts. (26217101)
2015
6
Gastroenterology: Primary intestinal lymphangiectasia of the terminal ileum. (26211692)
2015
7
Primary intestinal lymphangiectasia: Minireview. (25325063)
2014
8
Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic. (25276285)
2014
9
The lymphoscintigraphic manifestation of (99m)Tc-dextran lymphatic imaging in primary intestinal lymphangiectasia. (24488065)
2014
10
Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. (23626516)
2013
11
Evaluation of primary intestinal lymphangiectasia by capsule endoscopy. (23526519)
2013
12
Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: a case report. (23316917)
2013
13
Diffuse large B-cell lymphoma recurrence complicating primary intestinal lymphangiectasia. (23591283)
2013
14
Late-onset primary intestinal lymphangiectasia successfully managed with octreotide: a case report. (23555496)
2012
15
Primary intestinal lymphangiectasia or Waldmann's disease: a rare cause of lower gastrointestinal bleeding. (22980601)
2012
16
Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. (21191781)
2011
17
Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. (22110841)
2011
18
Forty years of shortness of breath and lower extremity edema. Diagnosis: Primary intestinal lymphangiectasia (Waldmann's disease). (21641905)
2011
19
Digital clubbing in primary intestinal lymphangiectasia: a case report. (20812055)
2010
20
Primary intestinal lymphangiectasia: four case reports and a review of the literature. (20198428)
2010
21
Primary intestinal lymphangiectasia in children: is octreotide an effective and safe option in the treatment? (20512058)
2010
22
A rare case of primary intestinal lymphangiectasia diagnosed by double balloon endoscopy. (21332012)
2010
23
Partial enterectomy: treatment for primary intestinal lymphangiectasia in four cases. (20368101)
2010
24
Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. (19853733)
2009
25
Increased surface receptor Fas (CD95) levels on CD4+ lymphocytes in patients with primary intestinal lymphangiectasia. (18855225)
2009
26
Medical image. Recurrent muscle cramps in chronic diarrhoea. Primary intestinal lymphangiectasia. (19966872)
2009
27
Primary intestinal lymphangiectasia (Waldmann's disease). (18294365)
2008
28
Photoclinic: primary intestinal lymphangiectasia. (18588385)
2008
29
A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: a case report. (17465517)
2007
30
Recurrent and opportunistic infections in children with primary intestinal lymphangiectasia. (17325562)
2007
31
Primary intestinal lymphangiectasia as a component of autoimmune polyglandular syndrome type I: a report of 2 cases. (18431016)
2007
32
Use of capsule endoscopy in children with primary intestinal lymphangiectasia. (16996365)
2006
33
Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia. (16292099)
2005
34
High-resolution magnifying video endoscopy in primary intestinal lymphangiectasia: a new role for endoscopy? (15933946)
2005
35
Primary Intestinal Lymphangiectasia. (14723832)
2004
36
Operative ascitic drainage in a patient with primary intestinal lymphangiectasia. (12648135)
2003
37
Primary intestinal lymphangiectasia successfully treated with octreotide. (11227670)
2001
38
Primary intestinal lymphangiectasia: a rare disease in the differential diagnosis of acute abdomen. (11698774)
2001
39
Ultrasonographic features of long-standing primary intestinal lymphangiectasia. (9700215)
1998
40
Intestinal leakage of technetium-99m-MDP in primary intestinal lymphangiectasia. (8691257)
1996
41
Endoscopic features of long-standing primary intestinal lymphangiectasia. (7615236)
1995
42
Immunological study in primary intestinal lymphangiectasia. (7509299)
1994
43
A case of primary intestinal lymphangiectasia. (8268148)
1993
44
Adenocarcinoma of unknown origin in a patient with primary intestinal lymphangiectasia. (1500667)
1992
45
Endoscopic diagnosis of primary intestinal lymphangiectasia using a high-fat meal. (3720681)
1986
46
Primary intestinal lymphangiectasia in three dogs: a morphological and immunopathological investigation. (3984156)
1985
47
Primary intestinal lymphangiectasia: clinical and CT findings. (4019833)
1985
48
Primary intestinal lymphangiectasia. Case report with radiological and ultrastructural study. (7227669)
1981
49
Primary intestinal lymphangiectasia. Comparison between endoscopic and radiological findings. (7451231)
1980
50
Primary intestinal lymphangiectasia: surgical implications. (5026674)
1972

Variations for Primary Intestinal Lymphangiectasia

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Expression for genes affiliated with Primary Intestinal Lymphangiectasia

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Search GEO for disease gene expression data for Primary Intestinal Lymphangiectasia.

Pathways for genes affiliated with Primary Intestinal Lymphangiectasia

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GO Terms for genes affiliated with Primary Intestinal Lymphangiectasia

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Sources for Primary Intestinal Lymphangiectasia

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet