MCID: PRM158
MIFTS: 30

Primary Intestinal Lymphangiectasia malady

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

Aliases & Classifications for Primary Intestinal Lymphangiectasia

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Sources:
45NIH Rare Diseases, 51Orphanet, 65UMLS, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Primary Intestinal Lymphangiectasia:

Name: Primary Intestinal Lymphangiectasia 45 51 65
Waldmann Disease 45 51 65
Familial Waldmann's Disease 45 65
 
Primary Intestinal Lymphangiectasis 45
Waldmann's Disease 45

Characteristics:

Orphanet epidemiological data:

51
primary intestinal lymphangiectasia:
Age of onset: All ages; Age of death: any age

Classifications:



External Ids:

Orphanet51 90362
ICD10 via Orphanet28 I89.0
MESH via Orphanet37 C536567
UMLS via Orphanet66 C0267372, C2931241
UMLS65 C0267372, C2931242, C2931241

Summaries for Primary Intestinal Lymphangiectasia

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NIH Rare Diseases:45 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 waldmann disease, is related to intestinal lymphangiectasia and lymphoma, and has symptoms including edema of the legs/lower limbs, anomalies of the lymphatic system and agammaglobulinemia/hypogammaglobulinemia/b-cell deficiency. Affiliated tissues include small intestine, skin and b cells.

Wikipedia:68 Waldmann disease, also known as Waldmann\'s disease and Primary intestinal lymphangiectasia, is a rare... more...

Related Diseases for Primary Intestinal Lymphangiectasia

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Diseases in the Intestinal Lymphangiectasia 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
1intestinal lymphangiectasia11.1
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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Symptoms:

 51 (show all 28)
  • edema of the legs/lower limbs
  • anomalies of the lymphatic system
  • agammaglobulinemia/hypogammaglobulinemia/b-cell deficiency
  • lymphopenia
  • hypoproteinemia
  • nausea/vomiting/regurgitation/merycism/hyperemesis
  • acute abdominal pain/colic
  • malabsorption/chronic diarrhea/steatorrhea
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • failure to thrive/difficulties for feeding in infancy/growth delay
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • asthenia/fatigue/weakness
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • thick skin/pachydermia/orange skin
  • warts/papillomas
  • ascitis
  • intestinal obstruction/ileus
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • gangrena/necrosis
  • vaginal hydrocele/hematocele/pyocele/lymphocele/varicocele
  • meningitis/meningeal syndrome
  • seizures/epilepsy/absences/spasms/status epilepticus
  • osteoporosis/osteopenia/demineralisation/osteomalacia/rickets
  • ferropenic anemia
  • hypocalcemia
  • lymphoma
  • hydrops fetalis

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:

33
Small intestine, Skin, B cells, Lung, Prostate, Brain, Pituitary

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
Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy. (26908672)
2016
2
Gastroenterology: Primary intestinal lymphangiectasia of the terminal ileum. (26211692)
2015
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
Surgical therapy of primary intestinal lymphangiectasia in adults. (26169531)
2015
6
The lymphoscintigraphic manifestation of (99m)Tc-dextran lymphatic imaging in primary intestinal lymphangiectasia. (24488065)
2014
7
Primary intestinal lymphangiectasia: Minireview. (25325063)
2014
8
Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic. (25276285)
2014
9
Diffuse large B-cell lymphoma recurrence complicating primary intestinal lymphangiectasia. (23591283)
2013
10
Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. (23626516)
2013
11
Is there a risk of pubertal worsening in primary intestinal lymphangiectasia? (23698711)
2013
12
Late-onset primary intestinal lymphangiectasia successfully managed with octreotide: a case report. (23555496)
2012
13
Primary intestinal lymphangiectasia or Waldmann's disease: a rare cause of lower gastrointestinal bleeding. (22980601)
2012
14
Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. (22110841)
2011
15
Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. (21191781)
2011
16
Forty years of shortness of breath and lower extremity edema. Diagnosis: Primary intestinal lymphangiectasia (Waldmann's disease). (21641905)
2011
17
Digital clubbing in primary intestinal lymphangiectasia: a case report. (20812055)
2010
18
Primary intestinal lymphangiectasia: four case reports and a review of the literature. (20198428)
2010
19
Primary intestinal lymphangiectasia in children: is octreotide an effective and safe option in the treatment? (20512058)
2010
20
A rare case of primary intestinal lymphangiectasia diagnosed by double balloon endoscopy. (21332012)
2010
21
Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. (19853733)
2009
22
Medical image. Recurrent muscle cramps in chronic diarrhoea. Primary intestinal lymphangiectasia. (19966872)
2009
23
Increased surface receptor Fas (CD95) levels on CD4+ lymphocytes in patients with primary intestinal lymphangiectasia. (18855225)
2009
24
Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. (19449286)
2009
25
Primary intestinal lymphangiectasia (Waldmann's disease). (18294365)
2008
26
Photoclinic: primary intestinal lymphangiectasia. (18588385)
2008
27
Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal. (17453239)
2008
28
Primary intestinal lymphangiectasia as a component of autoimmune polyglandular syndrome type I: a report of 2 cases. (18431016)
2007
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
Use of capsule endoscopy in children with primary intestinal lymphangiectasia. (16996365)
2006
32
Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia. (16292099)
2005
33
High-resolution magnifying video endoscopy in primary intestinal lymphangiectasia: a new role for endoscopy? (15933946)
2005
34
Primary intestinal lymphangiectasia: a response to medium-chain triglyceride formula. (16188830)
2005
35
Primary Intestinal Lymphangiectasia. (14723832)
2004
36
Group G streptococcal empyema complicating primary intestinal lymphangiectasia. (12960729)
2003
37
Operative ascitic drainage in a patient with primary intestinal lymphangiectasia. (12648135)
2003
38
Primary intestinal lymphangiectasia successfully treated with octreotide. (11227670)
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
Adenocarcinoma of unknown origin in a patient with primary intestinal lymphangiectasia. (1500667)
1992
44
Chylous reflux into abdominal skin simulating lymphangioma circumscriptum in a patient with primary intestinal lymphangiectasia. (2032374)
1991
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
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet