MCID: PRM158
MIFTS: 25

Primary Intestinal Lymphangiectasia malady

Cardiovascular, Gastrointestinal, Immune categories

Summaries for Primary Intestinal Lymphangiectasia

Sources:
64Wikipedia, 47OMIM, 33MalaCards
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Wikipedia:64 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 waldmann disease, and has symptoms including nausea/vomiting/regurgitation/merycism/hyperemesis, seizures/epilepsy/absences/spasms/status epilepticus and failure to thrive/difficulties for feeding in infancy/growth delay. Affiliated tissues include skin, small intestine and b cells.

Description from OMIM:47 152800

Aliases & Classifications for Primary Intestinal Lymphangiectasia

Sources:
49Orphanet, 47OMIM, 61UMLS, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Cardiovascular, Gastrointestinal, Immune


Aliases & Descriptions:

primary intestinal lymphangiectasia 49 47 61
waldmann disease 49 61


External Ids:

MESH via Orphanet36 C536567
ICD10 via Orphanet26 I89.0
SNOMED-CT via Orphanet58 6124009
UMLS via Orphanet62 C0267372, C2931241
OMIM47 152800

Related Diseases for Primary Intestinal Lymphangiectasia

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Primary Intestinal Lymphangiectasia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

idRelated DiseaseScoreTop Affiliating Genes
1intestinal lymphangiectasia11.0
2waldmann disease10.5
3protein-losing enteropathy10.2
4adenocarcinoma10.2
5lymphoma, diffuse large cell10.2

Graphical network of diseases related to Primary Intestinal Lymphangiectasia:



Diseases related to primary intestinal lymphangiectasia

Clinical Features for Primary Intestinal Lymphangiectasia

Sources:
47OMIM, 49Orphanet
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Clinical features from OMIM:

152800

Symptoms:

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

Drugs & Therapeutics for Primary Intestinal Lymphangiectasia

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Primary Intestinal Lymphangiectasia

Drug clinical trials:

Search ClinicalTrials for Primary Intestinal Lymphangiectasia

Search NIH Clinical Center for Primary Intestinal Lymphangiectasia

Search CenterWatch for Primary Intestinal Lymphangiectasia

Genetic Tests for Primary Intestinal Lymphangiectasia

Anatomical Context for Primary Intestinal Lymphangiectasia

Sources:
33MalaCards
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MalaCards organs/tissues related to Primary Intestinal Lymphangiectasia:

33
Skin, Small intestine, B cells

Animal Models for Primary Intestinal Lymphangiectasia or affiliated genes

Sources:
28inGenious Targeting Laboratory
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Publications for Primary Intestinal Lymphangiectasia

Sources:
51PubMed
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Articles related to Primary Intestinal Lymphangiectasia:

(show top 50)    (show all 59)
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
Late-onset primary intestinal lymphangiectasia successfully managed with octreotide: a case report. (23555496)
2012
6
Primary intestinal lymphangiectasia or Waldmann's disease: a rare cause of lower gastrointestinal bleeding. (22980601)
2012
7
Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. (22110841)
2011
8
Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. (21191781)
2011
9
A rare cause of severe hypoalbuminemia in a patient with primary hypoparathyroidism: intestinal lymphangiectasia. (21837942)
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 in children: is octreotide an effective and safe option in the treatment? (20512058)
2010
13
A rare case of primary intestinal lymphangiectasia diagnosed by double balloon endoscopy. (21332012)
2010
14
Intestinal lymphangiectasia mimicking primary peritoneal carcinoma. (20801422)
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
Treatment with parenteral nutrition support and Chinese herbs in one case of primary small intestinal lymphangiectasia. (20120774)
2009
20
Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. (19449286)
2009
21
Primary intestinal lymphangiectasia. (19887697)
2009
22
Primary intestinal lymphangiectasia (Waldmann's disease). (18294365)
2008
23
Photoclinic: primary intestinal lymphangiectasia. (18588385)
2008
24
Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal. (17453239)
2008
25
Primary intestinal lymphangiectasia as a component of autoimmune polyglandular syndrome type I: a report of 2 cases. (18431016)
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
Enteral nutrition as a primary therapy for intestinal lymphangiectasia: value of elemental diet and polymeric diet compared with total parenteral nutrition. (16110837)
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 lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia. (11841381)
2002
35
Primary intestinal lymphangiectasia successfully treated with octreotide. (11227670)
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
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
Endoscopic and histopathological study on primary and secondary intestinal lymphangiectasia. (7238258)
1981
50
Primary intestinal lymphangiectasia. Comparison between endoscopic and radiological findings. (7451231)
1980

Genetic Variations for Primary Intestinal Lymphangiectasia

Expression for genes affiliated with Primary Intestinal Lymphangiectasia

Sources:
15Gene 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

Compounds for genes affiliated with Primary Intestinal Lymphangiectasia

GO Terms for genes affiliated with Primary Intestinal Lymphangiectasia

Products for genes affiliated with Primary Intestinal Lymphangiectasia

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  • Lysates
  • Antibodies

Sources for Primary Intestinal Lymphangiectasia

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet