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

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

Sources:
²⁰GeneCards, ¹⁹GeneAnalytics
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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)

id	Related Disease	Score
1	intestinal lymphangiectasia	11.1
2	lymphoma	10.1
3	osteomalacia	10.0
4	lymphangiectasis	10.0
5	b-cell lymphomas	10.0
6	protein-losing enteropathy	10.0
7	osteoporosis	9.9
8	diffuse large b-cell lymphoma	9.8
9	nephrotic syndrome	9.8
10	tuberous sclerosis	9.8
11	lymphangioma	9.8
12	adenocarcinoma	9.8
13	refractory anemia	9.8

Graphical network of diseases related to Primary Intestinal Lymphangiectasia:

Symptoms for Primary Intestinal Lymphangiectasia

Sources:
⁵¹Orphanet
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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

Sources:
⁴⁴NIH Clinical Center, 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:
³³MalaCards
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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

Publications for Primary Intestinal Lymphangiectasia

Sources:
⁵⁴PubMed
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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	Gastroenterology: Primary intestinal lymphangiectasia of the terminal ileum. (26211692)	Chiou F.K.... Lian D.W.	2015
3	How to treat an extensive form of primary intestinal lymphangiectasia? (26109821)	Troskot R.... BrajkoviA8 I.	2015
4	Severe Refractory Anemia in Primary Intestinal Lymphangiectasia. A Case Report. (26405709)	Balaban V.D.... Jinga M.	2015
5	Surgical therapy of primary intestinal lymphangiectasia in adults. (26169531)	Huber T.... Kneist W.	2015
6	The lymphoscintigraphic manifestation of (99m)Tc-dextran lymphatic imaging in primary intestinal lymphangiectasia. (24488065)	Wen Z.... Yang J.	2014
7	Primary intestinal lymphangiectasia: Minireview. (25325063)	Ingle S.B.... Hinge Ingle C.R.	2014
8	Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic. (25276285)	Licinio R.... Leo A.D.	2014
9	Diffuse large B-cell lymphoma recurrence complicating primary intestinal lymphangiectasia. (23591283)	Patel K.V.... Wong T.	2013
10	Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. (23626516)	Xinias I.... Imvrios G.	2013
11	Is there a risk of pubertal worsening in primary intestinal lymphangiectasia? (23698711)	Salpietro V.... Romano C.	2013
12	Late-onset primary intestinal lymphangiectasia successfully managed with octreotide: a case report. (23555496)	Suehiro K.... Hamano K.	2012
13	Primary intestinal lymphangiectasia or Waldmann's disease: a rare cause of lower gastrointestinal bleeding. (22980601)	Maamer A.B.... Cherif A.	2012
14	Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. (22110841)	Oh T.G.... Song S.Y.	2011
15	Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. (21191781)	Tang Q.Y.... Cai W.	2011
16	Forty years of shortness of breath and lower extremity edema. Diagnosis: Primary intestinal lymphangiectasia (Waldmann's disease). (21641905)	Choi E.H.... Coyle W.	2011
17	Digital clubbing in primary intestinal lymphangiectasia: a case report. (20812055)	Wiedermann C.J.... Mazzoleni G.	2010
18	Primary intestinal lymphangiectasia: four case reports and a review of the literature. (20198428)	Wen J.... Cai W.	2010
19	Primary intestinal lymphangiectasia in children: is octreotide an effective and safe option in the treatment? (20512058)	Sari S.... Dalgic B.	2010
20	A rare case of primary intestinal lymphangiectasia diagnosed by double balloon endoscopy. (21332012)	A8eltA+k A.... Akpinar H.	2010
21	Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. (19853733)	Kim N.R.... Suh Y.L.	2009
22	Medical image. Recurrent muscle cramps in chronic diarrhoea. Primary intestinal lymphangiectasia. (19966872)	Mohan P.... Venkataraman J.	2009
23	Increased surface receptor Fas (CD95) levels on CD4+ lymphocytes in patients with primary intestinal lymphangiectasia. (18855225)	Vignes S.... Carcelain G.	2009
24	Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. (19449286)	Desai A.P.... Carachi R.	2009
25	Primary intestinal lymphangiectasia (Waldmann's disease). (18294365)	Vignes S.... Bellanger J.	2008
26	Photoclinic: primary intestinal lymphangiectasia. (18588385)	Jamali R.... Sotoudeh M.	2008
27	Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal. (17453239)	Lee J.... Kong M.S.	2008
28	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
29	A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: a case report. (17465517)	Fang Y.H.... Chen C.X.	2007
30	Recurrent and opportunistic infections in children with primary intestinal lymphangiectasia. (17325562)	Dierselhuis M.P.... Wulffraat N.M.	2007
31	Use of capsule endoscopy in children with primary intestinal lymphangiectasia. (16996365)	Rivet C.... Lachaux A.	2006
32	Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia. (16292099)	Laharie D.... Amouretti M.	2005
33	High-resolution magnifying video endoscopy in primary intestinal lymphangiectasia: a new role for endoscopy? (15933946)	Cammarota G.... Gasbarrini G.	2005
34	Primary intestinal lymphangiectasia: a response to medium-chain triglyceride formula. (16188830)	Koo N.H.... Hwang J.S.	2005
35	Primary Intestinal Lymphangiectasia. (14723832)	Bliss C.M.... Schroy III P.C.	2004
36	Group G streptococcal empyema complicating primary intestinal lymphangiectasia. (12960729)	Hallevy C.... Almog Y.	2003
37	Operative ascitic drainage in a patient with primary intestinal lymphangiectasia. (12648135)	White S.M.	2003
38	Primary intestinal lymphangiectasia successfully treated with octreotide. (11227670)	Kuroiwa G.... Niitsu Y.	2001
39	Ultrasonographic features of long-standing primary intestinal lymphangiectasia. (9700215)	Maconi G.... Bianchi Porro G.	1998
40	Intestinal leakage of technetium-99m-MDP in primary intestinal lymphangiectasia. (8691257)	Lee K.H.... Koh C.S.	1996
41	Endoscopic features of long-standing primary intestinal lymphangiectasia. (7615236)	Salomons H.A.... Schroy P.C.	1995
42	Immunological study in primary intestinal lymphangiectasia. (7509299)	Heresbach D.... Gosselin M.	1994
43	Adenocarcinoma of unknown origin in a patient with primary intestinal lymphangiectasia. (1500667)	Montalban C.... Garcia-Gonzalez R.	1992
44	Chylous reflux into abdominal skin simulating lymphangioma circumscriptum in a patient with primary intestinal lymphangiectasia. (2032374)	O'Driscoll J.B.... Warnes T.W.	1991
45	Endoscopic diagnosis of primary intestinal lymphangiectasia using a high-fat meal. (3720681)	Veldhuyzen van Zanten S.J.... Tytgat G.N.	1986
46	Primary intestinal lymphangiectasia in three dogs: a morphological and immunopathological investigation. (3984156)	Suter M.M.... Schenk H.	1985
47	Primary intestinal lymphangiectasia: clinical and CT findings. (4019833)	Fakhri A.... Siegelman S.S.	1985
48	Primary intestinal lymphangiectasia. Case report with radiological and ultrastructural study. (7227669)	Bujanover Y.... Thaler M.M.	1981
49	Primary intestinal lymphangiectasia. Comparison between endoscopic and radiological findings. (7451231)	Donzelli F.... Zacchello G.	1980
50	Primary intestinal lymphangiectasia: surgical implications. (5026674)	Muzaffar K.... Fox T.A.	1972