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

Summaries for Primary Intestinal Lymphangiectasia

Sources:
⁶⁴Wikipedia, ⁴⁶OMIM, ³²MalaCards
See all sources

Wikipedia:⁶⁴ Waldmann disease, also known as Waldmann\'s disease and Primary intestinal lymphangiectasia, is a rare... more...

MalaCards based summary: Primary Intestinal Lymphangiectasia, also known as waldmann disease, is related to b-cell lymphomas and diffuse large b-cell lymphoma, and has symptoms including lymphopenia, hypoproteinemia and hypogammaglobulinemia. Affiliated tissues include small intestine, skin and b cells.

Description from OMIM:⁴⁶ 152800

Aliases & Classifications for Primary Intestinal Lymphangiectasia

Sources:
⁴⁸Orphanet, ⁶¹UMLS, ⁴⁶OMIM, ³⁵MESH via Orphanet, ²⁷ICD10 via Orphanet, ⁶²UMLS via Orphanet
See all sources

Primary Intestinal Lymphangiectasia, Aliases & Descriptions:

Name: Primary Intestinal Lymphangiectasia ⁴⁸ ⁴⁶ ⁶¹

Waldmann Disease ⁴⁸ ⁶¹

Classifications:

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

ICD10: ²⁷

Diseases of the circulatory system

Diseases of veins, lymphatic vessels and lymph nodes, not elsewhere classified

Other noninfective disorders of lymphatic vessels and lymph nodes

Lymphoedema, not elsewhere classified

Orphanet: ⁴⁸

Rare circulatory system diseases

Rare gastroenterological diseases

Characteristics (Orphanet epidemiological data):

⁴⁸

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

External Ids:

OMIM⁴⁶ 152800

MESH via Orphanet³⁵ C536567

ICD10 via Orphanet²⁷ I89.0

UMLS via Orphanet⁶² C0267372, C2931241

Related Diseases for Primary Intestinal Lymphangiectasia

Sources:
¹⁸GeneCards, ¹⁹GeneDecks
See all sources

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

id	Related Disease	Score
1	b-cell lymphomas	10.4
2	diffuse large b-cell lymphoma	10.2
3	protein-losing enteropathy	10.2
4	adenocarcinoma	10.2
5	lymphangioma	10.2
6	nephrotic syndrome	10.2
7	waldmann disease	10.2

Graphical network of diseases related to Primary Intestinal Lymphangiectasia:

Symptoms for Primary Intestinal Lymphangiectasia

Sources:
⁴⁶OMIM, ⁵⁸The Human Phenotype Ontology, ⁴⁸Orphanet
See all sources

Symptoms by clinical synopsis from OMIM:

152800

Clinical features from OMIM:

152800

Symptoms:

⁴⁸ (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

HPO human phenotypes related to Primary Intestinal Lymphangiectasia:

(show all 33)

id	Description	Frequency	HPO Source Accession
1	lymphopenia	hallmark (90%)	HP:0001888
2	hypoproteinemia	hallmark (90%)	HP:0003075
3	hypogammaglobulinemia	hallmark (90%)	HP:0004313
4	edema of the lower limbs	hallmark (90%)	HP:0010741
5	abnormality of the lymphatic system	hallmark (90%)	HP:0100763
6	weight loss	typical (50%)	HP:0001824
7	nausea and vomiting	typical (50%)	HP:0002017
8	malabsorption	typical (50%)	HP:0002024
9	abdominal pain	typical (50%)	HP:0002027
10	thickened skin	occasional (7.5%)	HP:0001072
11	seizures	occasional (7.5%)	HP:0001250
12	meningitis	occasional (7.5%)	HP:0001287
13	ascites	occasional (7.5%)	HP:0001541
14	abnormality of the pericardium	occasional (7.5%)	HP:0001697
15	hydrops fetalis	occasional (7.5%)	HP:0001789
16	iron deficiency anemia	occasional (7.5%)	HP:0001891
17	abnormality of the pleura	occasional (7.5%)	HP:0002103
18	lymphoma	occasional (7.5%)	HP:0002665
19	hypocalcemia	occasional (7.5%)	HP:0002901
20	reduced bone mineral density	occasional (7.5%)	HP:0004349
21	intestinal obstruction	occasional (7.5%)	HP:0005214
22	vaginal hernia	occasional (7.5%)	HP:0100672
23	gangrene	occasional (7.5%)	HP:0100758
24	verrucae	occasional (7.5%)	HP:0200043
25	autosomal dominant inheritance		HP:0000006
26	abnormality of the hair		HP:0001595
27	lymphopenia		HP:0001888
28	malabsorption		HP:0002024
29	intestinal lymphangiectasia		HP:0002593
30	igg deficiency		HP:0004315
31	prominent floating ribs		HP:0006641
32	neonatal hypoproteinemia		HP:0008360
33	edema of the lower limbs		HP:0010741

Drugs & Therapeutics for Primary Intestinal Lymphangiectasia

Sources:
⁴¹NIH Clinical Center, ⁶ClinicalTrials
See all sources

Drug clinical trials:

Search ClinicalTrials for Primary Intestinal Lymphangiectasia

Search NIH Clinical Center for Primary Intestinal Lymphangiectasia

Genetic Tests for Primary Intestinal Lymphangiectasia

Anatomical Context for Primary Intestinal Lymphangiectasia

Sources:
³²MalaCards
See all sources

MalaCards organs/tissues related to Primary Intestinal Lymphangiectasia:

³²

Small intestine, Skin, B cells, Liver

Animal Models for Primary Intestinal Lymphangiectasia or affiliated genes

Publications for Primary Intestinal Lymphangiectasia

Sources:
⁵¹PubMed
See all sources

Articles related to Primary Intestinal Lymphangiectasia:

(show top 50) (show all 54)

id	Title	Authors	Year
1	The lymphoscintigraphic manifestation of (99m)Tc-dextran lymphatic imaging in primary intestinal lymphangiectasia. (24488065)	Wen Z.... Yang J.	2014
2	Primary intestinal lymphangiectasia: Minireview. (25325063)	Ingle S.B.... Hinge Ingle C.R.	2014
3	Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic. (25276285)	Licinio R.... Leo A.D.	2014
4	Diffuse large B-cell lymphoma recurrence complicating primary intestinal lymphangiectasia. (23591283)	Patel K.V.... Wong T.	2013
5	Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. (23626516)	Xinias I.... Imvrios G.	2013
6	Is there a risk of pubertal worsening in primary intestinal lymphangiectasia? (23698711)	Salpietro V.... Romano C.	2013
7	Evaluation of primary intestinal lymphangiectasia by capsule endoscopy. (23526519)	Ersoy O.... Buyukasik S.	2013
8	Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: a case report. (23316917)	Lai Y.... Chen Q.K.	2013
9	Late-onset primary intestinal lymphangiectasia successfully managed with octreotide: a case report. (23555496)	Suehiro K.... Hamano K.	2012
10	Primary intestinal lymphangiectasia or Waldmann's disease: a rare cause of lower gastrointestinal bleeding. (22980601)	Maamer A.B.... Cherif A.	2012
11	Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. (22110841)	Oh T.G.... Song S.Y.	2011
12	Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia. (21191781)	Tang Q.Y.... Cai W.	2011
13	Forty years of shortness of breath and lower extremity edema. Diagnosis: Primary intestinal lymphangiectasia (Waldmann's disease). (21641905)	Choi E.H.... Coyle W.	2011
14	Digital clubbing in primary intestinal lymphangiectasia: a case report. (20812055)	Wiedermann C.J.... Mazzoleni G.	2010
15	Primary intestinal lymphangiectasia: four case reports and a review of the literature. (20198428)	Wen J.... Cai W.	2010
16	Primary intestinal lymphangiectasia in children: is octreotide an effective and safe option in the treatment? (20512058)	Sari S.... Dalgic B.	2010
17	A rare case of primary intestinal lymphangiectasia diagnosed by double balloon endoscopy. (21332012)	A8eltA+k A.... Akpinar H.	2010
18	Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. (19853733)	Kim N.R.... Suh Y.L.	2009
19	Medical image. Recurrent muscle cramps in chronic diarrhoea. Primary intestinal lymphangiectasia. (19966872)	Mohan P.... Venkataraman J.	2009
20	Increased surface receptor Fas (CD95) levels on CD4+ lymphocytes in patients with primary intestinal lymphangiectasia. (18855225)	Vignes S.... Carcelain G.	2009
21	Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. (19449286)	Desai A.P.... Carachi R.	2009
22	Primary intestinal lymphangiectasia (Waldmann's disease). (18294365)	Vignes S.... Bellanger J.	2008
23	Photoclinic: primary intestinal lymphangiectasia. (18588385)	Jamali R.... Sotoudeh M.	2008
24	Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal. (17453239)	Lee J.... Kong M.S.	2008
25	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
26	A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: a case report. (17465517)	Fang Y.H.... Chen C.X.	2007
27	Recurrent and opportunistic infections in children with primary intestinal lymphangiectasia. (17325562)	Dierselhuis M.P.... Wulffraat N.M.	2007
28	Use of capsule endoscopy in children with primary intestinal lymphangiectasia. (16996365)	Rivet C.... Lachaux A.	2006
29	Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia. (16292099)	Laharie D.... Amouretti M.	2005
30	High-resolution magnifying video endoscopy in primary intestinal lymphangiectasia: a new role for endoscopy? (15933946)	Cammarota G.... Gasbarrini G.	2005
31	Primary intestinal lymphangiectasia: a response to medium-chain triglyceride formula. (16188830)	Koo N.H.... Hwang J.S.	2005
32	Primary Intestinal Lymphangiectasia. (14723832)	Bliss C.M.... Schroy III P.C.	2004
33	Group G streptococcal empyema complicating primary intestinal lymphangiectasia. (12960729)	Hallevy C.... Almog Y.	2003
34	Operative ascitic drainage in a patient with primary intestinal lymphangiectasia. (12648135)	White S.M.	2003
35	Primary intestinal lymphangiectasia successfully treated with octreotide. (11227670)	Kuroiwa G.... Niitsu Y.	2001
36	Primary intestinal lymphangiectasia: a rare disease in the differential diagnosis of acute abdomen. (11698774)	UA9uralp S.... Mizrak B.	2001
37	Primary intestinal lymphangiectasia, nephrotic syndrome and clomid-induced triplet pregnancy. (19861780)	Al-Mushaikih A.A.	2001
38	Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts. (10896925)	Bouhnik Y.... Matuchansky C.	2000
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