Aliases & Classifications for Lipogranulomatosis

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Aliases & Descriptions for Lipogranulomatosis:

Name: Lipogranulomatosis 46 48
Erdheim-Chester Disease 66
 
Lipogranuloma 66

Classifications:



Summaries for Lipogranulomatosis

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MalaCards based summary: Lipogranulomatosis, also known as erdheim-chester disease, is related to farber lipogranulomatosis and arthritis, and has symptoms including polydipsia, infusion related reaction and vestibulitis. An important gene associated with Lipogranulomatosis is ASAH1 (N-Acylsphingosine Amidohydrolase 1), and among its related pathways are Sphingolipid metabolism and S-1P Stimulated Signaling. Affiliated tissues include lymph node, liver and breast.

Related Diseases for Lipogranulomatosis

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Diseases related to Lipogranulomatosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 14)
idRelated DiseaseScoreTop Affiliating Genes
1farber lipogranulomatosis12.5
2arthritis9.8
3atherosclerosis9.8
4hepatitis9.8
5skin disease9.8
6lymphoma9.8
7obstructive jaundice9.8
8sclerosing cholangitis9.8
9panniculitis9.8
10facial dermatosis9.8
11lipid metabolism disorder9.8
12hypersplenism9.8
13cholangitis9.8
14lissencephaly, isolated9.5ASAH1, ASAH2

Graphical network of diseases related to Lipogranulomatosis:



Diseases related to lipogranulomatosis

Symptoms for Lipogranulomatosis

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UMLS symptoms related to Lipogranulomatosis:


polydipsia, infusion related reaction, vestibulitis, procedural pain, incision site erythema, post-tussive vomiting, injection related reaction, incision site hyperaesthesia

Drugs & Therapeutics for Lipogranulomatosis

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Drugs for Lipogranulomatosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 6)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
ThalidomidePhase 2106050-35-15426
Synonyms:
( inverted question mark)-Thalidomide
(+)-Thalidomide
(+-)-N-(2,6-dioxo-3-Piperidyl)phthalimide
(+-)-Thalidomide
(+/-)-THALIDOMIDE
(?)-Thalidomide
(±)-N-(2,6-dioxo-3-piperidyl)phthalimide
(±)-thalidomide
.alpha.-(N-Phthalimido)glutarimide
.alpha.-N-Phthalylglutaramide
.alpha.-Phthalimidoglutarimide
1,3-dioxo-2-(2,6-dioxopiperidin-3-yl)isoindoline
14088-68-7
2,6-Dioxo-3-phthalimidopiperidine
2,6-dioxo-3-phthalimidopiperidine
3-Phthalimidoglutarimide
50-35-1
731-40-8
AB00052362
AC-917
AC1L1KBZ
AC1Q6FJA
AI3-50606
Algosediv
Asidon 3
Asmadion
Asmaval
BPBio1_000159
BRD-A93255169-001-06-9
BRN 0030233
BSPBio_000143
BSPBio_001156
BSPBio_003330
Bio1_000387
Bio1_000876
Bio1_001365
Bio2_000418
Bio2_000898
Bonbrain
Bonbrrin
C07910
C13H10N2O4
CCRIS 8148
CHEBI:105028
CHEMBL468
CID5426
CPD000058524
Calmore
Calmorex
Celgene Brand of Thalidomide
Contergan
Corronarobetin
D00754
D013792
DB01041
Distaval
Distaxal
Distoval
DivK1c_000051
E-217
EINECS 200-031-1
ENMD 0995
EU-0101224
Ectiluran
Enterosediv
Gastrinide
Glupan
Glutanon
Grippex
HMS1362J17
HMS1568H05
HMS1792J17
HMS1922E12
HMS1990J17
HMS2090O05
HMS2093G15
HMS500C13
HSDB 3586
Hippuzon
I06-0197
IDI1_000051
IDI1_002173
IN1061
Imida-Lab
Imida-lab
Imidan
Imidan (peyta)
Imidene
Isomin
K 17
K-17
KBio1_000051
KBio2_000496
KBio2_002322
KBio2_003064
KBio2_004890
KBio2_005632
KBio2_007458
KBio3_000911
KBio3_000912
KBio3_002550
KBio3_002802
KBioGR_000496
KBioGR_001474
KBioGR_002322
KBioSS_000496
KBioSS_002324
Kedavon
Kevadon
LS-109463
LS-140
Lopac0_001224
Lulamin
MLS000069353
MolPort-003-665-582
N-(2,6-Dioxo-3-piperidyl)phthalimide
N-(2,6-dioxo-3-piperidyl)phthalimide
N-Phthalimidoglutamic acid imide
N-Phthaloylglutamimide
N-Phthalyl-glutaminsaeure-imid
N-Phthalyl-glutaminsaeure-imid [German]
N-Phthalylglutamic acid imide
NCGC00015989-03
NCGC00015989-13
NCGC00024708-02
 
NCGC00024708-03
NCGC00024708-04
NCGC00024708-05
NCGC00024708-06
NCGC00024708-07
NCGC00024708-08
NCGC00024708-09
NCI60_023904
NCIOpen2_003188
NINDS_000051
NSC 527179
NSC-66847
NSC527179
NSC66847
NSC91729
NSC91730
Neaufatin
Neo
Neosedyn
Neosydyn
Nerosedyn
Neufatin
Neurodyn
Neurosedin
Neurosedym
Neurosedyn
Nevrodyn
Nibrol
Noctosediv
Noxodyn
Pangul
Pantosediv
Poly-Giron
Polygripan
Predni-Sediv
Prestwick0_000192
Prestwick1_000192
Prestwick2_000192
Prestwick3_000192
Prestwick_463
Pro-Bam M
Pro-ban M
Profarmil
Psycholiquid
Psychotablets
Quetimid
Quietoplex
S1193_Selleck
SAM002564245
SMR000058524
SPBio_000893
SPBio_002064
SPECTRUM1503607
ST51039042
Sandormin
Sedalis
Sedalis sedi-lab
Sedimide
Sedin
Sedisperil
Sedoval
Shin-naito S
Shinnibrol
Sleepan
Slipro
Softenil
Softenon
Spectrum2_000707
Spectrum3_001715
Spectrum4_001087
Spectrum5_001791
Synovir
T144_SIGMA
T150_SIGMA
T151_SIGMA
THALIDOMIDE (AIDS INITIATIVE)
Talargan
Talidomida
Talidomida [INN-Spanish]
Talidomide
Talidomide [DCIT]
Talimol
Talismol
Talizer
Telagan
Telargan
Telargean
Tensival
Thaled
Thaled (TN)
Thalidomide
Thalidomide (+ and -)
Thalidomide (JAN/USP/INN)
Thalidomide (soluble form)
Thalidomide Celgene
Thalidomide Pharmion
Thalidomide [USAN:INN:BAN]
Thalidomidum
Thalidomidum [INN-Latin]
Thalidomine USP26
Thalin
Thalinette
Thalomid
Thalomid (TM)
Thalomid (TN)
Thalomid, Thalidomide
Thalomide
Theophilcholine
UNII-4Z8R6ORS6L
UPCMLD-DP139
UPCMLD-DP139:001
Valgis
Valgraine
WLN: T56 BVNVJ C- DT6VMVTJ
WLN: T56 BVNVJ C- DT6VMVTJ -D
WLN: T56 BVNVJ C- DT6VMVTJ -L
Yodomin
alpha-(N-Phthalimido)glutarimide
alpha-N-Phthalylglutaramide
alpha-Phthalimidoglutarimide
alpha-phthalimidoglutarimide
cMAP_000022
thalidomide
α-(N-phthalimido)glutarimide
α-N-phthalylglutaramide
α-phthalimidoglutarimide
2
LenalidomidePhase 2691191732-72-6216326
Synonyms:
1-oxo-2-(2,6-Dioxopiperidin-3-yl)-4-aminoisoindoline
191732-72-6
3-(4-Amino-1-oxo-1,3-dihydro-2H-isoindol-2-yl)piperidine-2,6-dione
3-(4-Amino-1-oxoisoindolin-2-yl)piperidine-2,6-dione
3-(7-Amino-3-oxo-1H-isoindol-2-yl)-piperidine-2,6-dione
3-(7-amino-3-oxo-1H-isoindol-2-yl)piperidine-2,6-dione
346670-73-3
443912-14-9
AC-914
AC1L50II
AKOS005146276
ALBB-015321
Bio-0168
C467567
CC 5013
CC-5013
CC-5013, Revlimid, Lenalidomide
CC5013
CDC 501
CDC-501
CDC-5013
CHEMBL848
CID216326
Celgene brand of lenalidomide
D04687
 
DB00480
EC-000.2340
ENMD-0997
I06-0831
IMID-1
IMID-5013
IMiD3
IMiD3 cpd
IMid-1
LS-184040
Lenalidomide
Lenalidomide (USAN/INN)
Lenalidomide [USAN]
MolPort-003-848-370
NCGC00167491-01
NSC747972
Revamid
Revimid
Revlimid
Revlimid (Celgene)
Revlimid (TN)
S1029_Selleck
STK639603
Thalidomide analog CC-5013
UNII-F0P408N6V4
lenalidomide
3
TrametinibPhase 2115871700-17-311707110
Synonyms:
GSK 1120212
GSK1120212
JTP 74057
JTP-74057
 
MEK Inhibitor GSK1120212
Mekinist
Trametinib Dimethyl Sulfoxide
Trametinibum
trametinib
4
DabrafenibPhase 27544462760, 44516822
Synonyms:
BRAF inhibitor GSK2118436
Dabrafenib
 
GSK2118436
GSK2118436a
Tafinlar
dabrafenib
5
Dimethyl sulfoxidePhase 23367-68-5679
Synonyms:
(CH3)2SO
(methylsulfinyl)methane
DMSO
Dimethyl sulfoxide
Dimethyl sulfoxixde
Dimethyl sulfur oxide
Dimethyl sulphoxide
 
Dimethyl sulpoxide
Dimethyli sulfoxidum
Dimethylsulfoxid
Dimethylsulfoxyde
Dimetil sulfoxido
Methylsulfinylmethane
S(O)Me2
Sulfinylbis(methane)
6
CholineNutraceutical14762-49-7305
Synonyms:
(2-Hydroxyethyl)trimethyl ammonium
(2-Hydroxyethyl)trimethylammonium
(beta-hydroxyethyl)trimethylammonium
2-Hydroxy-N,N,N-trimethyl-ethanaminium
2-Hydroxy-N,N,N-trimethylethanaminium
Bilineurine
Biocolina
Biocoline
Choline
Choline cation
 
Choline ion
Cholinum
Hepacholine
Hormocline
Lipotril
N,N,N-Trimethylethanol-ammonium
N,N,N-Trimethylethanolammonium
N,N,N-trimethylethanol-ammonium
N-trimethylethanolamine
Neocolina
Paresan
trimethylethanolamine

Interventional clinical trials:

idNameStatusNCT IDPhase
1Dabrafenib and Trametinib in People With BRAF V600E Mutation Positive Lesions in Erdheim Chester DiseaseRecruitingNCT02281760Phase 2
2A Study of Lenalidomide for Adult Histiocyte DisordersRecruitingNCT02523040Phase 2
3Pilot Study of Tocilizumab in Patients With Erdheim-Chester DiseaseTerminatedNCT01727206Phase 2
4Long-term Outcome After Vemurafenib / BRAF Inhibitors Interruption in Erdheim-chester DiseaseRecruitingNCT02089724
5Clinical and Basic Investigations Into Erdheim Chester DiseaseRecruitingNCT01417520
6Biomarker for Farber DiseaseRecruitingNCT02298634
7International Rare Histiocytic Disorders Registry (IRHDR)RecruitingNCT02285582
8Uptake and Biodistribution of 18F-fluorocholine in Histiocytic Disorders by PET Imaging and Biopsy MeasurementRecruitingNCT02608619

Search NIH Clinical Center for Lipogranulomatosis

Genetic Tests for Lipogranulomatosis

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Anatomical Context for Lipogranulomatosis

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MalaCards organs/tissues related to Lipogranulomatosis:

34
Lymph node, Liver, Breast, Skin, Spleen, Bone, Bone marrow

Animal Models for Lipogranulomatosis or affiliated genes

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Publications for Lipogranulomatosis

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Articles related to Lipogranulomatosis:

(show top 50)    (show all 54)
idTitleAuthorsYear
1
Extensive facial sclerosing lipogranulomatosis as a complication of cosmetic acupuncture. (25768878)
2015
2
Coelomic Granulomatous Fat Necrosis (Lipogranulomatosis) in an Umbrella Cockatoo (Cacatua alba). (25843325)
2014
3
Lipogranulomatosis and hypersplenism induced by ruptured silicone breast implants. (24228267)
2013
4
Farber lipogranulomatosis with predominant joint involvement mimicking juvenile idiopathic arthritis. (23385296)
2013
5
Molecular analyses of novel ASAH1 mutations causing Farber lipogranulomatosis: analyses of exonic splicing enhancer inactivating mutation. (24355074)
2013
6
Farber lipogranulomatosis type 1--late presentation and early death in a Croatian boy with a novel homozygous ASAH1 mutation. (20609603)
2011
7
Disseminated lipogranulomatosis. (19242039)
2009
8
Surreptitious injection of mineral oil: a case report of sclerosing lipogranulomatosis. (18948786)
2008
9
Diffuse lipogranulomatosis involving soft tissues of the head and neck due to multiple self-injections of mineral oil. (18617587)
2008
10
Farber lipogranulomatosis: clinical and molecular genetic analysis reveals a novel mutation in an Indian family. (16951918)
2006
11
Farber disease [Farber lipogranulomatosis], acid ceramidase deficiency]. (9645089)
1998
12
A case of Farber lipogranulomatosis. (9539328)
1998
13
Bone marrow involvement and obstructive jaundice in Farber lipogranulomatosis: clinical and autopsy report of a new case. (8892023)
1996
14
Clofazimine in inflammatory facial dermatosis--granuloma faciale and lipogranulomatosis subcutanea (Rothmann-Makai) (8721503)
1996
15
Farber's lipogranulomatosis]. (8577050)
1995
16
The anesthetic implications of a patient with Farber's lipogranulomatosis. (8291711)
1994
17
Sclerosing lipogranulomatosis: a case report of scrotal injection of automobile transmission fluid and literature review of subcutaneous injection of oils. (8430154)
1993
18
An autopsy case of Farber's lipogranulomatosis in a Japanese boy with gastrointestinal involvement. (1557987)
1992
19
Farber's lipogranulomatosis. (3327826)
1987
20
Farber lipogranulomatosis: an unusual presentation in a black child. (2854742)
1986
21
Pulmonary lipogranulomatosis due to excessive consumption of apples. (3757573)
1986
22
Hepatic lipogranulomatosis simulating veno-occlusive disease of the liver. (3838237)
1985
23
"Banana bodies" in disseminated lipogranulomatosis (Farber's disease). (6414327)
1983
24
Idiopathic lipogranulomatosis of spleen and periaortic lymph nodes with noncaseating granulomas of liver. (7173854)
1982
25
Lipogranulomatosis circumscripta (panniculitis non suppurativa). (7147697)
1982
26
Lipogranulomatosis of the genitalia caused by topical application of "baby oil". (7351706)
1980
27
Subtle clues to diagnosis of skin diseases by electron microscopy. "Farber bodies" in disseminated lipogranulomatosis (Farber's disease). (7246981)
1980
28
Farber's lipogranulomatosis: ceramidase deficiency and prolonged survival in three relatives. (7441940)
1980
29
Farber's disease (disseminated lipogranulomatosis)--a pathological, histochemical and ultrastructural study--. (219658)
1979
30
A specific ultrastructural marker for disseminated lipogranulomatosis (Faber). (230790)
1979
31
Lipogranulomatosis of the mesentery. (948565)
1976
32
Familial lipogranulomatosis (Farber's disease). (1277575)
1976
33
Diagnosis of lipogranulomatosis (Farber disease) by use of cultured fibroblasts. (932904)
1976
34
Lipogranulomatosis of external genitalia. (4594721)
1974
35
Lipogranulomatosis subcutanea of Rothman-Makai. (4664563)
1972
36
Farber's disease (lipogranulomatosis). (5171854)
1971
37
Ceramides in a patient with lipogranulomatosis (Farber's disease) with chronic course. (5109101)
1971
38
Sclerosing lipogranulomatosis. (5569331)
1971
39
Fatal lipogranulomatosis with atrial pseudotumour, intrahepatic sclerosing cholangitis and lymphoreticular proliferation simulating malignant lymphoma. (5477029)
1970
40
Farber's lipogranulomatosis. Report of a case and demonstration of an excess of free ceramide and ganglioside. (5395479)
1969
41
Biochemical and histochemical studies of a case of Farber's lipogranulomatosis. (5656562)
1968
42
Sclerosing lipogranulomatosis: its several abdominal syndromes. (6026707)
1967
43
LIPOGRANULOMATOSIS SUBCUTANEA (ROTHMANN-MAKAI); AN APPRAISAL. (14170601)
1964
44
DISSEMINATED LIPOGRANULOMATOSIS: EARLY ROENTGENOGRAPHIC CHANGES. (14131673)
1964
45
Disseminated lipogranulomatosis: report of a case. (14443810)
1960
46
Disseminated lipogranulomatosis (Farber's disease). (13582627)
1958
47
Lipogranulomatosis; a new lipo-glycoprotein storage disease. (13481627)
1957
48
Sex differences in incidence and severity of lymph node lipogranulomatosis; a similarity to atherosclerosis. (13434569)
1957
49
Intestinal lipogranulomatosis (Whipple's disease). (14398057)
1954
50
A lipid metabolic disorder: disseminated lipogranulomatosis; a syndrome with similarity to, and important difference from, Niemann-Pick and Hand-SchA1ller-Christian disease. (12975849)
1952

Variations for Lipogranulomatosis

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Expression for genes affiliated with Lipogranulomatosis

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Search GEO for disease gene expression data for Lipogranulomatosis.

Pathways for genes affiliated with Lipogranulomatosis

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Pathways related to Lipogranulomatosis according to GeneCards Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
1
Show member pathways
9.1ASAH1, ASAH2
29.1ASAH1, ASAH2
39.1ASAH1, ASAH2
49.1ASAH1, ASAH2

GO Terms for genes affiliated with Lipogranulomatosis

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Biological processes related to Lipogranulomatosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1ceramide metabolic processGO:00066729.1ASAH1, ASAH2

Molecular functions related to Lipogranulomatosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1ceramidase activityGO:00170409.1ASAH1, ASAH2

Sources for Lipogranulomatosis

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet