MCID: WHP001
MIFTS: 53

Whipple Disease malady

Neuronal diseases, Gastrointestinal diseases, Infectious diseases categories

Summaries for Whipple Disease

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42NIH Rare Diseases, 43NINDS, 32MalaCards
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NIH Rare Diseases:42 Whipple disease is a multi-system infectious bacterial disease that interferes with the body's ability to metabolize fats. the disorder usually occurs in the gastrointestinal system, but may affect any part of the body including the heart, lungs, brain, joints, and eyes. in the gastrointestinal system it interferes with the body's ability to absorb certain nutrients. this leads to a condition known as malabsorption. whipple disease causes weight loss, incomplete breakdown of carbohydrates or fats, and malfunctions of the immune system. it is caused by infection from bacteria called tropheryma whippelii. when recognized and treated, whipple disease can usually be cured. untreated, the disease may be fatal. last updated: 8/1/2012

MalaCards: Whipple Disease, also known as intestinal lipodystrophy, is related to arthritis and uveitis, and has symptoms including hypothyroidy, insulin resistance and galactorrhea. An important gene associated with Whipple Disease is IL16 (interleukin 16), and among its related pathways are Immune response Antigen presentation by MHC class II and Tuberculosis. The compounds nickel and ecori have been mentioned in the context of this disorder. Affiliated tissues include eye, lung and brain.

NINDS:43 Whipple's disease is a multi-system infectious bacterial disease that interferes with the body's ability to metabolize fats. The disorder can affect any system in the body, including the central nervous system, but usually occurs in the gastrointestinal system. Gastrointestinal symptoms may include diarrhea, weight loss, fatigue, weakness, and abdominal bleeding and pain. Neurological symptoms may include abnormalities of eye and facial muscle movements, confusion, seizures, ataxia, memory loss, and vision impairment. Fever, cough, and joint soreness may also be present.

Aliases & Classifications for Whipple Disease

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 48Orphanet, 60UMLS, 39NCIt, 27ICD9CM, 56SNOMED-CT, 34MeSH, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

48
intestinal lipodystrophy:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

whipple disease 8 42 48 60
intestinal lipodystrophy 8 42 43 48
intestinal lipophagic granulomatosis 42 48 60
secondary non-tropical sprue 42 48
whipple's disease 8 43
tropheryma whippelii infection 42


External Ids:

Disease Ontology8 DOID:8476
NCIt39 C85228
ICD9CM27 040.2
MeSH34 D008061
SNOMED-CT56 41545003
MESH via Orphanet35 C531849, D008061
ICD10 via Orphanet26 K90.8+, M14.8*
SNOMED-CT via Orphanet57 41545003
UMLS via Orphanet61 C0023788, C2930851

Related Diseases for Whipple Disease

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Whipple Disease:



Diseases related to whipple disease

Clinical Features for Whipple Disease

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48Orphanet
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Symptoms:

48 (show all 45)
  • hypothyroidy
  • insulin resistance
  • galactorrhea
  • hydrocephaly
  • cranial nerves palsy
  • peripheral neuropathy
  • cranial hypertension
  • encephalitis
  • ataxia/incoordination/trouble of the equilibrium
  • pyramidal syndrome
  • seizures/epilepsy/absences/spasms/status epilepticus
  • muscle weakness/flaccidity
  • myositis
  • anaemia
  • hyponatremia
  • thirst
  • humour troubles/anxiety/depression/apathy/euphoria/irritability
  • impotence/painful erection/priapism/erection troubles
  • edema of the legs/lower limbs
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis
  • asthenia/fatigue/weakness
  • fever/chilling
  • wasted (excluding lipodystrophy)/poorly muscled build/cachexy
  • arthritis/synovitis/synovial proliferation
  • articular/joint pain/arthralgia
  • anorexia
  • psychic/psychomotor regression/dementia/intellectual decline
  • myoclonus/fasciculations
  • mediastinal/hilar adenopathies
  • malabsorption/chronic diarrhea/steatorrhea
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • myocardium anomalies/myocarditis
  • cough
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • thoracic/chest pain
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • diffuse/generalised skin hyperpigmentation/melanoderma
  • proptosis/exophthalmos
  • myalgia/muscular pain
  • sleep and vigilance disorders
  • hypotension
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • acute abdominal pain/colic

Drugs & Therapeutics for Whipple Disease

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Whipple Disease

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Anatomical Context for Whipple Disease

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Sources:
32MalaCards
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MalaCards organs/tissues related to Whipple Disease:

32
Eye, Lung, Brain, Heart, Skin, Liver, Spinal cord, Bone, B cells

Animal Models for Whipple Disease or affiliated genes

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Publications for Whipple Disease

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50PubMed
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Articles related to Whipple Disease:

(show top 50)    (show all 90)
idTitleAuthorsYear
1
A severe Whipple disease with an immune reconstitution inflammatory syndrome: An additional case of thalidomide efficiency. (24289960)
2013
2
Rheumatic and musculoskeletal features of Whipple disease: a report of 29 cases. (24187107)
2013
3
Resolution of Whipple Disease-Induced Pulmonary Hypertension Following Antibiotic Therapy. (23344104)
2013
4
Treated Whipple disease with erythema nodosum leprosum-like lesions: cutaneous PAS-positive macrophages slowly decrease with time and are associated with lymphangiectases: a case report. (22123268)
2012
5
Whipple disease with crystalline keratopathy and chronic uveitis. (22965603)
2012
6
Whipple disease mimicking adult-onset Still's disease and treated by anakinra: diagnosis using PCR. (22639877)
2012
7
Connecting the dots: the many systemic manifestations of whipple disease. (22347837)
2012
8
MR imaging of central nervous system Whipple disease: a 15-year review. (20395395)
2010
9
Whipple disease of the central nervous system: an unusual occurrence in association with acquired immune deficiency syndrome. (19612977)
2010
10
The amnesic syndrome of primary Whipple disease of the brain. (20299864)
2010
11
Intestinal Mycobacterium avium complex infection initially misdiagnosed and mistreated as Whipple disease. (20138615)
2010
12
Primary cerebral Whipple disease presenting as KlA1ver-Bucy syndrome. (19139312)
2009
13
A paradoxical Tropheryma whipplei western blot differentiates patients with whipple disease from asymptomatic carriers. (19635029)
2009
14
Whipple disease: unusual presentation of a protean and sometimes confusing disease. (18538828)
2009
15
Postoperative panophthalmitis caused by Whipple disease. (19402983)
2009
16
Reversible pulmonary hypertension in Whipple disease: a case report with clinicopathological implications, and literature review. (21686934)
2009
17
Renal amyloidosis in Whipple disease: a case report. (19918433)
2009
18
Whipple disease. (18301263)
2008
19
Neurologically presenting Whipple disease: case report and review of the literature. (18381382)
2008
20
Acquired resistance to trimethoprim-sulfamethoxazole during Whipple disease and expression of the causative target gene. (18500934)
2008
21
Isolated CNS Whipple disease with a variant of oculofacial-skeletal myorhythmia (OFSM). (17846404)
2007
22
Isolated CNS Whipple disease: acute onset and relapsing-remitting course. (17577831)
2007
23
Hypersomnia in Whipple disease: case report. (17057900)
2006
24
Whipple disease: a case report and review of the literature. (15974820)
2005
25
Proton magnetic resonance spectroscopy and diffusion-weighted imaging of central nervous system whipple disease. (15891498)
2005
26
Whipple disease confined to the central nervous system presenting as a solitary frontal tumor. Case report. (15309928)
2004
27
Whipple disease revisited: radiographic features of a patient with 35 years of undiagnosed arthritis. (17043469)
2004
28
Whipple disease associated with giardiasis. (12964113)
2003
29
Treatment of Whipple disease. (14973431)
2003
30
Whipple disease. (14565900)
2003
31
Neurologic presentation of Whipple disease: report of 12 cases and review of the literature. (12441901)
2002
32
An unusual spinal presentation of Whipple disease. (11337349)
2001
33
Culture and immunological detection of Tropheryma whippelii from the duodenum of a patient with Whipple disease. (11209175)
2001
34
Successful treatment of Whipple disease diagnosed 36 years after symptom onset. (11605693)
2001
35
Whipple disease and arthritis. (11148719)
2001
36
Spinal cord involvement in CNS Whipple disease: an ongoing experience in the magnetic resonance imaging era. (11733340)
2001
37
Cognitive and behavioral abnormalities in a case of central nervous system Whipple disease. (10714668)
2000
38
Treatment of refractory Whipple disease with interferon-gamma. (9867729)
1998
39
Diagnostic utility of the polymerase chain reaction in 2 cases of suspected Whipple disease. (9554687)
1998
40
Whipple disease presenting as posterior uveitis without prominent gastrointestinal symptoms. (9683160)
1998
41
Cardiac Whipple disease: identification of Whipple bacillus by electron microscopy of a patient before death. (9027272)
1997
42
Detection of a clonal BCL2 gene rearrangement in tissues from a patient with Whipple disease. (8633834)
1996
43
Whipple disease of the mediastinum. (1692443)
1990
44
Oculo-facial-skeletal myorhythmia in Whipple disease: treatment with ceftriaxone. (1689977)
1990
45
Computed tomography in Whipple disease. (6195202)
1983
46
Whipple disease of the nervous system. (6178057)
1982
47
Inferior vena cava obstruction in Whipple disease. Association with retroperitoneal lymphadenopathy. (59825)
1976
48
Letter: Whipple disease. (58083)
1976
49
Whipple disease. Occurrence in a 76-year-old man with a 20-year prodrome of arthritis. (48564)
1975
50
Whipple disease: light and electron microscopy study. (4138040)
1974

Genetic Variations for Whipple Disease

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Expression for genes affiliated with Whipple Disease

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Whipple Disease

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Pathways for genes affiliated with Whipple Disease

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12EMD Millipore, 29KEGG, 51QIAGEN, 53Reactome
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Compounds for genes affiliated with Whipple Disease

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44Novoseek, 24HMDB
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Compounds related to Whipple Disease according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1nickel44 2410.7HLA-DQB1, HSPD1
2ecori449.4HLA-B, HLA-DQB1
3rantes449.1IL16, HLA-B, HSPD1
4ribonucleic acid448.8HSPD1, HLA-B, PRL

GO Terms for genes affiliated with Whipple Disease

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16Gene Ontology
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Cellular components related to Whipple Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1MHC class II protein complexGO:0426139.5HLA-DQB1, HLA-DRB1
2trans-Golgi network membraneGO:0325889.5HLA-DRB1, HLA-DQB1
3clathrin-coated endocytic vesicle membraneGO:0306699.4HLA-DRB1, HLA-DQB1
4transport vesicle membraneGO:0306589.2HLA-DRB1, HLA-DQB1
5integral to lumenal side of endoplasmic reticulum membraneGO:0715569.0HLA-DRB1, HLA-B, HLA-DQB1
6ER to Golgi transport vesicle membraneGO:0125079.0HLA-DRB1, HLA-B, HLA-DQB1

Biological processes related to Whipple Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1immunoglobulin production involved in immunoglobulin mediated immune responseGO:0023819.6HLA-DRB1, HLA-DQB1
2humoral immune response mediated by circulating immunoglobulinGO:0024559.4HLA-DRB1, HLA-DQB1
3interferon-gamma-mediated signaling pathwayGO:0603339.0HLA-DRB1, HLA-B, HLA-DQB1
4detection of bacteriumGO:0160459.0HLA-DRB1, HLA-B
5cytokine-mediated signaling pathwayGO:0192218.9HLA-DRB1, HLA-B, HLA-DQB1
6immune responseGO:0069558.6HLA-DQB1, HLA-B, HLA-DRB1, IL16

Molecular functions related to Whipple Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1peptide antigen bindingGO:0426059.3HLA-DRB1, HLA-B
2MHC class II receptor activityGO:0323959.2HLA-DRB1, HLA-DQB1

Products for genes affiliated with Whipple Disease

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  • Antibodies
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  • Lysates
  • Antibodies

Sources for Whipple Disease

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet