MCID: PRM158
MIFTS: 30

Primary Intestinal Lymphangiectasia malady

Cardiovascular diseases, Gastrointestinal diseases, Rare diseases, Immune diseases categories

Summaries for Primary Intestinal Lymphangiectasia

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Sources:
66Wikipedia, 48OMIM, 34MalaCards
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Wikipedia:66 Waldmann disease, also known as Waldmann\'s disease and Primary intestinal lymphangiectasia, is a rare... more...

MalaCards: Primary Intestinal Lymphangiectasia, also known as waldmann disease, is related to intestinal lymphangiectasia and b-cell lymphomas, and has symptoms including abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax, pericardium anomalies/pericarditis/absence/cysts/pericardial effusion and gangrena/necrosis. Affiliated tissues include small intestine, skin and b cells.

Description from OMIM:48 152800

Aliases & Classifications for Primary Intestinal Lymphangiectasia

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Sources:
50Orphanet, 48OMIM, 63UMLS, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

50
primary intestinal lymphangiectasia:
Age of onset: Variable; Age of death: Any age


Aliases & Descriptions:

primary intestinal lymphangiectasia 50 48 63
waldmann disease 50 63


External Ids:

MESH via Orphanet37 C536567
ICD10 via Orphanet27 I89.0
SNOMED-CT via Orphanet60 6124009
UMLS via Orphanet64 C0267372, C2931241
OMIM48 152800

Related Diseases for Primary Intestinal Lymphangiectasia

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Sources:
18GeneCards, 19GeneDecks
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Diseases in the Primary Intestinal Lymphangiectasia family:

Intestinal Lymphangiectasia

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

idRelated DiseaseScoreTop Affiliating Genes
1intestinal lymphangiectasia11.0
2b-cell lymphomas10.3
3protein-losing enteropathy10.2
4diffuse large b-cell lymphoma10.2
5adenocarcinoma10.2
6lymphangioma10.2
7nephrotic syndrome10.2
8waldmann disease10.2

Graphical network of diseases related to Primary Intestinal Lymphangiectasia:



Diseases related to primary intestinal lymphangiectasia

Symptoms for Primary Intestinal Lymphangiectasia

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48OMIM, 50Orphanet
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Symptoms by clinical synopsis from OMIM:

152800

Clinical features from OMIM:

152800

Symptoms:

50 (show all 28)
  • 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
  • intestinal obstruction/ileus
  • ascitis
  • warts/papillomas
  • 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
  • edema of the legs/lower limbs

Drugs & Therapeutics for Primary Intestinal Lymphangiectasia

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Primary Intestinal Lymphangiectasia

Drug clinical trials:

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Search NIH Clinical Center for Primary Intestinal Lymphangiectasia

Search CenterWatch for Primary Intestinal Lymphangiectasia

Genetic Tests for Primary Intestinal Lymphangiectasia

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Anatomical Context for Primary Intestinal Lymphangiectasia

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Sources:
34MalaCards
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MalaCards organs/tissues related to Primary Intestinal Lymphangiectasia:

34
Small intestine, Skin, B cells

Animal Models for Primary Intestinal Lymphangiectasia or affiliated genes

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Publications for Primary Intestinal Lymphangiectasia

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Sources:
53PubMed
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Articles related to Primary Intestinal Lymphangiectasia:

(show all 50)
idTitleAuthorsYear
1
Diffuse large B-cell lymphoma recurrence complicating primary intestinal lymphangiectasia. (23591283)
2013
2
Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. (23626516)
2013
3
Is there a risk of pubertal worsening in primary intestinal lymphangiectasia? (23698711)
2013
4
Evaluation of primary intestinal lymphangiectasia by capsule endoscopy. (23526519)
2013
5
Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: a case report. (23316917)
2013
6
Late-onset primary intestinal lymphangiectasia successfully managed with octreotide: a case report. (23555496)
2012
7
Primary intestinal lymphangiectasia or Waldmann's disease: a rare cause of lower gastrointestinal bleeding. (22980601)
2012
8
Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. (22110841)
2011
9
Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. (21191781)
2011
10
Forty years of shortness of breath and lower extremity edema. Diagnosis: Primary intestinal lymphangiectasia (Waldmann's disease). (21641905)
2011
11
Digital clubbing in primary intestinal lymphangiectasia: a case report. (20812055)
2010
12
Primary intestinal lymphangiectasia: four case reports and a review of the literature. (20198428)
2010
13
Primary intestinal lymphangiectasia in children: is octreotide an effective and safe option in the treatment? (20512058)
2010
14
A rare case of primary intestinal lymphangiectasia diagnosed by double balloon endoscopy. (21332012)
2010
15
Partial enterectomy: treatment for primary intestinal lymphangiectasia in four cases. (20368101)
2010
16
Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. (19853733)
2009
17
Medical image. Recurrent muscle cramps in chronic diarrhoea. Primary intestinal lymphangiectasia. (19966872)
2009
18
Increased surface receptor Fas (CD95) levels on CD4+ lymphocytes in patients with primary intestinal lymphangiectasia. (18855225)
2009
19
Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. (19449286)
2009
20
Primary intestinal lymphangiectasia. (19887697)
2009
21
Primary intestinal lymphangiectasia (Waldmann's disease). (18294365)
2008
22
Photoclinic: primary intestinal lymphangiectasia. (18588385)
2008
23
Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal. (17453239)
2008
24
Primary intestinal lymphangiectasia as a component of autoimmune polyglandular syndrome type I: a report of 2 cases. (18431016)
2007
25
A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: a case report. (17465517)
2007
26
Recurrent and opportunistic infections in children with primary intestinal lymphangiectasia. (17325562)
2007
27
Use of capsule endoscopy in children with primary intestinal lymphangiectasia. (16996365)
2006
28
Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia. (16292099)
2005
29
High-resolution magnifying video endoscopy in primary intestinal lymphangiectasia: a new role for endoscopy? (15933946)
2005
30
Primary intestinal lymphangiectasia: a response to medium-chain triglyceride formula. (16188830)
2005
31
Primary Intestinal Lymphangiectasia. (14723832)
2004
32
Group G streptococcal empyema complicating primary intestinal lymphangiectasia. (12960729)
2003
33
Operative ascitic drainage in a patient with primary intestinal lymphangiectasia. (12648135)
2003
34
Primary intestinal lymphangiectasia successfully treated with octreotide. (11227670)
2001
35
Primary intestinal lymphangiectasia: a rare disease in the differential diagnosis of acute abdomen. (11698774)
2001
36
Primary intestinal lymphangiectasia, nephrotic syndrome and clomid-induced triplet pregnancy. (19861780)
2001
37
Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts. (10896925)
2000
38
Ultrasonographic features of long-standing primary intestinal lymphangiectasia. (9700215)
1998
39
Intestinal leakage of technetium-99m-MDP in primary intestinal lymphangiectasia. (8691257)
1996
40
Endoscopic features of long-standing primary intestinal lymphangiectasia. (7615236)
1995
41
Immunological study in primary intestinal lymphangiectasia. (7509299)
1994
42
A case of primary intestinal lymphangiectasia. (8268148)
1993
43
Adenocarcinoma of unknown origin in a patient with primary intestinal lymphangiectasia. (1500667)
1992
44
The distribution of lymphocyte subpopulations in an infant with primary intestinal lymphangiectasia. (1304188)
1992
45
Chylous reflux into abdominal skin simulating lymphangioma circumscriptum in a patient with primary intestinal lymphangiectasia. (2032374)
1991
46
Endoscopic diagnosis of primary intestinal lymphangiectasia using a high-fat meal. (3720681)
1986
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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Sources:
16Gene Expression Omnibus DataSets
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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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Compounds for genes affiliated with Primary Intestinal Lymphangiectasia

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GO Terms for genes affiliated with Primary Intestinal Lymphangiectasia

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Products for genes affiliated with Primary Intestinal Lymphangiectasia

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Primary Intestinal Lymphangiectasia

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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