Primary Intestinal Lymphangiectasia disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

Aliases & Classifications for Primary Intestinal Lymphangiectasia

Sources:
⁴⁸NIH Rare Diseases, ⁶⁸UMLS
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Aliases & Descriptions for Primary Intestinal Lymphangiectasia:

Name: Primary Intestinal Lymphangiectasia ⁴⁸ ⁶⁸

Familial Waldmann's Disease ⁴⁸ ⁶⁸

Waldmann Disease ⁴⁸ ⁶⁸

Primary Intestinal Lymphangiectasis ⁴⁸

Waldmann's Disease ⁴⁸

Classifications:

MalaCards categories:
Global: Genetic diseases, Rare diseases
Anatomical: Gastrointestinal diseases, Immune diseases

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Summaries for Primary Intestinal Lymphangiectasia

Sources:
³⁶MalaCards, ⁴⁸NIH Rare Diseases
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NIH Rare Diseases:⁴⁸ 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, and has symptoms including osteoporosis, lymphedema and thickened skin. Affiliated tissues include small intestine, b cells and skin.

Related Diseases for Primary Intestinal Lymphangiectasia

Sources:
²⁰GeneAnalytics, ²¹GeneCards
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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)

id	Related Disease	Score
1	lymphangiectasia, intestinal	11.7
2	lymphoma	10.0
3	osteomalacia	9.9
4	lymphangiectasis	9.9
5	b-cell lymphomas	9.9
6	protein-losing enteropathy	9.9
7	osteoporosis	9.8
8	diffuse large b-cell lymphoma	9.7
9	nephrotic syndrome	9.7
10	tuberous sclerosis	9.7
11	lymphangioma	9.7
12	adenocarcinoma	9.7
13	refractory anemia	9.7

Graphical network of diseases related to Primary Intestinal Lymphangiectasia:

Symptoms & Phenotypes for Primary Intestinal Lymphangiectasia

Sources:
⁶⁴The Human Phenotype Ontology
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Human phenotypes related to Primary Intestinal Lymphangiectasia:

⁶⁴ (show all 30)

id	Description	HPO Source Accession
1	osteoporosis⁶⁴	HP:0000939
2	lymphedema⁶⁴	HP:0001004
3	thickened skin⁶⁴	HP:0001072
4	seizures⁶⁴	HP:0001250
5	meningitis⁶⁴	HP:0001287
6	failure to thrive⁶⁴	HP:0001508
7	pericardial effusion⁶⁴	HP:0001698
8	hydrops fetalis⁶⁴	HP:0001789
9	weight loss⁶⁴	HP:0001824
10	lymphopenia⁶⁴	HP:0001888
11	iron deficiency anemia⁶⁴	HP:0001891
12	diarrhea⁶⁴	HP:0002014
13	nausea and vomiting⁶⁴	HP:0002017
14	malabsorption⁶⁴	HP:0002024
15	abdominal pain⁶⁴	HP:0002027
16	pleural effusion⁶⁴	HP:0002202
17	ileus⁶⁴	HP:0002595
18	immunodeficiency⁶⁴	HP:0002721
19	hypocalcemia⁶⁴	HP:0002901
20	hypoalbuminemia⁶⁴	HP:0003073
21	hypoproteinemia⁶⁴	HP:0003075
22	decreased antibody level in blood⁶⁴	HP:0004313
23	edema of the lower limbs⁶⁴	HP:0010741
24	b-cell lymphoma⁶⁴	HP:0012191
25	chylous ascites⁶⁴	HP:0012281
26	fatigue⁶⁴	HP:0012378
27	vaginal lymphocele⁶⁴	HP:0100676
28	gangrene⁶⁴	HP:0100758
29	abnormality of the lymphatic system⁶⁴	HP:0100763
30	verrucae⁶⁴	HP:0200043

Drugs & Therapeutics for Primary Intestinal Lymphangiectasia

Sources:
⁴ClinicalTrials
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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Primary Intestinal Lymphangiectasia

Genetic Tests for Primary Intestinal Lymphangiectasia

Anatomical Context for Primary Intestinal Lymphangiectasia

Sources:
³⁶MalaCards
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MalaCards organs/tissues related to Primary Intestinal Lymphangiectasia:

³⁶

Small intestine, B cells, Skin, Liver

Publications for Primary Intestinal Lymphangiectasia

Sources:
⁵⁷PubMed
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Articles related to Primary Intestinal Lymphangiectasia:

(show top 50) (show all 62)

id	Title	Authors	Year
1	Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy. (26908672)	Ozeki M.... Fukao T.	2016
2	Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions: A Case Report. (26962779)	Wang X.... Wu W.	2016
3	Surgical therapy of primary intestinal lymphangiectasia in adults. (26169531)	Huber T.... Kneist W.	2015
4	Primary intestinal lymphangiectasia with generalized warts. (26217101)	Lee S.J.... Hyun C.L.	2015
5	How to treat an extensive form of primary intestinal lymphangiectasia? (26109821)	Troskot R.... BrajkoviA8 I.	2015
6	Gastroenterology: Primary intestinal lymphangiectasia of the terminal ileum. (26211692)	Chiou F.K.... Lian D.W.	2015
7	Severe Refractory Anemia in Primary Intestinal Lymphangiectasia. A Case Report. (26405709)	Balaban V.D.... Jinga M.	2015
8	Primary intestinal lymphangiectasia treated with rapamycin in a child with tuberous sclerosis complex (TSC). (25943403)	Pollack S.F.... Shah U.	2015
9	Primary intestinal lymphangiectasia: Minireview. (25325063)	Ingle S.B.... Hinge Ingle C.R.	2014
10	Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic. (25276285)	Licinio R.... Leo A.D.	2014
11	The lymphoscintigraphic manifestation of (99m)Tc-dextran lymphatic imaging in primary intestinal lymphangiectasia. (24488065)	Wen Z.... Yang J.	2014
12	Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. (23626516)	Xinias I.... Imvrios G.	2013
13	Is there a risk of pubertal worsening in primary intestinal lymphangiectasia? (23698711)	Salpietro V.... Romano C.	2013
14	Diffuse large B-cell lymphoma recurrence complicating primary intestinal lymphangiectasia. (23591283)	Patel K.V.... Wong T.	2013
15	Evaluation of primary intestinal lymphangiectasia by capsule endoscopy. (23526519)	Ersoy O.... Buyukasik S.	2013
16	Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: a case report. (23316917)	Lai Y.... Chen Q.K.	2013
17	Primary intestinal lymphangiectasia or Waldmann's disease: a rare cause of lower gastrointestinal bleeding. (22980601)	Maamer A.B.... Cherif A.	2012
18	Late-onset primary intestinal lymphangiectasia successfully managed with octreotide: a case report. (23555496)	Suehiro K.... Hamano K.	2012
19	Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. (22110841)	Oh T.G.... Song S.Y.	2011
20	Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. (21191781)	Tang Q.Y.... Cai W.	2011
21	Forty years of shortness of breath and lower extremity edema. Diagnosis: Primary intestinal lymphangiectasia (Waldmann's disease). (21641905)	Choi E.H.... Coyle W.	2011
22	A rare case of primary intestinal lymphangiectasia diagnosed by double balloon endoscopy. (21332012)	A8eltA+k A.... Akpinar H.	2010
23	Primary intestinal lymphangiectasia in children: is octreotide an effective and safe option in the treatment? (20512058)	Sari S.... Dalgic B.	2010
24	Primary intestinal lymphangiectasia: four case reports and a review of the literature. (20198428)	Wen J.... Cai W.	2010
25	Digital clubbing in primary intestinal lymphangiectasia: a case report. (20812055)	Wiedermann C.J.... Mazzoleni G.	2010
26	Partial enterectomy: treatment for primary intestinal lymphangiectasia in four cases. (20368101)	Zhu L.H.... Wu J.G.	2010
27	Primary intestinal lymphangiectasia. (19887697)	Suresh N.... Sathiyasekaran M.	2009
28	Increased surface receptor Fas (CD95) levels on CD4+ lymphocytes in patients with primary intestinal lymphangiectasia. (18855225)	Vignes S.... Carcelain G.	2009
29	Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. (19853733)	Kim N.R.... Suh Y.L.	2009
30	Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. (19449286)	Desai A.P.... Carachi R.	2009
31	Medical image. Recurrent muscle cramps in chronic diarrhoea. Primary intestinal lymphangiectasia. (19966872)	Mohan P.... Venkataraman J.	2009
32	Primary intestinal lymphangiectasia (Waldmann's disease). (18294365)	Vignes S.... Bellanger J.	2008
33	Photoclinic: primary intestinal lymphangiectasia. (18588385)	Jamali R.... Sotoudeh M.	2008
34	Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal. (17453239)	Lee J.... Kong M.S.	2008
35	Primary intestinal lymphangiectasia as a component of autoimmune polyglandular syndrome type I: a report of 2 cases. (18431016)	Makharia G.K.... Tandon R.K.	2007
36	A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: a case report. (17465517)	Fang Y.H.... Chen C.X.	2007
37	Recurrent and opportunistic infections in children with primary intestinal lymphangiectasia. (17325562)	Dierselhuis M.P.... Wulffraat N.M.	2007
38	Use of capsule endoscopy in children with primary intestinal lymphangiectasia. (16996365)	Rivet C.... Lachaux A.	2006
39	High-resolution magnifying video endoscopy in primary intestinal lymphangiectasia: a new role for endoscopy? (15933946)	Cammarota G.... Gasbarrini G.	2005
40	Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia. (16292099)	Laharie D.... Amouretti M.	2005
41	Primary intestinal lymphangiectasia: a response to medium-chain triglyceride formula. (16188830)	Koo N.H.... Hwang J.S.	2005
42	Primary Intestinal Lymphangiectasia. (14723832)	Bliss C.M.... Schroy III P.C.	2004
43	Group G streptococcal empyema complicating primary intestinal lymphangiectasia. (12960729)	Hallevy C.... Almog Y.	2003
44	Operative ascitic drainage in a patient with primary intestinal lymphangiectasia. (12648135)	White S.M.	2003
45	Primary intestinal lymphangiectasia, nephrotic syndrome and clomid-induced triplet pregnancy. (19861780)	Al-Mushaikih A.A.	2001
46	Primary intestinal lymphangiectasia: a rare disease in the differential diagnosis of acute abdomen. (11698774)	UA9uralp S.... Mizrak B.	2001
47	Primary intestinal lymphangiectasia successfully treated with octreotide. (11227670)	Kuroiwa G.... Niitsu Y.	2001
48	Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts. (10896925)	Bouhnik Y.... Matuchansky C.	2000
49	Ultrasonographic features of long-standing primary intestinal lymphangiectasia. (9700215)	Maconi G.... Bianchi Porro G.	1998
50	Intestinal leakage of technetium-99m-MDP in primary intestinal lymphangiectasia. (8691257)	Lee K.H.... Koh C.S.	1996