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MCID: MKL001
MIFTS: 46

Mikulicz Disease

Categories: Bone diseases, Endocrine diseases, Eye diseases, Immune diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Mikulicz Disease

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MalaCards integrated aliases for Mikulicz Disease:

Name: Mikulicz Disease 12 52 58 15 71
Igg4-Related Dacryoadenitis and Sialadenitis 12 52 58
Chronic Dacryoadenitis and Sialadenitis 52 58
Mikulicz's Disease 12 52
Mikulicz Syndrome 74 52
Mikulicz' Disease 43

Characteristics:

Orphanet epidemiological data:

58
igg4-related dacryoadenitis and sialadenitis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Eye diseases Bone diseases Immune diseases Endocrine diseases
See all MalaCards categories (disease lists)
ICD10: 32 33
Orphanet: 58  
Rare eye diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:12900
MeSH 43 D008882
NCIt 49 C34819
SNOMED-CT 67 7826003
ICD10 32 K11.8
MESH via Orphanet 44 D008882
ICD10 via Orphanet 33 K11.8
UMLS via Orphanet 72 C0026103
Orphanet 58 ORPHA79078
UMLS 71 C0026103
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Summaries for Mikulicz Disease

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NIH Rare Diseases : 52 IgG4-related dacryoadenitis and sialoadenitis (formerly called Mikulicz disease) is an IgG4-related disease characterized by inflammation of the lacrimal glands (which produce tears), parotid glands, and submandibular glands (two of the major salivary glands ). In some cases, it also affects other glands or organs . The condition is usually painless, mainly causing mouth and eye dryness, and swelling over the affected glands. When other organs are affected, it can be accompanied by complications such as autoimmune pancreatitis , retroperitoneal fibrosis , and tubulointerstitial nephritis . The underlying cause of IgG4-related disease is still not known. Treatment involves corticosteroids , which are usually effective. Medicines that suppress the immune system (immunosuppressants) may also be used in cases that do not respond to corticosteroids. IgG4-related dacryoadenitis and sialoadenitis was previously considered a subtype of Sjogren syndrome , but it is now known to be a distinct condition.

MalaCards based summary : Mikulicz Disease, also known as igg4-related dacryoadenitis and sialadenitis, is related to cytokine deficiency and keratoconjunctivitis, and has symptoms including lumps of the face An important gene associated with Mikulicz Disease is IL21 (Interleukin 21), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Prednisolone phosphate and Leflunomide have been mentioned in the context of this disorder. Affiliated tissues include eye, salivary gland and bone, and related phenotypes are hematopoietic system and digestive/alimentary

Disease Ontology : 12 An autoimmune hypersensitivity disease that is characterized by chronic enlargement of the glands of the head and neck, has symptom lumps of the face and neck, dry eyes, dry mouth, and intermittent fever, and is often associated with an underlying autoimmune disorder, hematologic malignancy, tuberculosis infection, or syphilis infection.

Wikipedia : 74 Benign lymphoepithelial lesion is a type of benign enlargement of the parotid and/or lacrimal glands.... more...

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Related Diseases for Mikulicz Disease

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

(show top 50) (show all 563)
# Related Disease Score Top Affiliating Genes
1 cytokine deficiency 31.0 IL5 IL13
2 keratoconjunctivitis 30.2 IL5 IL4 IL13
3 keratoconjunctivitis sicca 30.1 IL4 CD4 CCR6
4 dacryoadenitis 30.0 KNTC1 IL4 IL21 IL13 IL10 CD4
5 uveitis 29.9 IL4 IL10 CXCR5
6 churg-strauss syndrome 29.9 IL5 IL10 CD40LG
7 xerophthalmia 29.9 CRP CD40LG CD4
8 interstitial nephritis 29.9 CRP CD40LG CCR6
9 juvenile arthritis 29.9 IL10 CRP
10 autoimmune pancreatitis 29.8 IL10 CD40LG CD4
11 rhinitis 29.8 IL5 IL4 IL13 IL10
12 hypereosinophilic syndrome 29.8 IL5 IL4 IL13 IL10
13 allergic rhinitis 29.7 IL5 IL4 IL13 IL10
14 vasculitis 29.6 IL10 CRP CD40LG
15 cholangitis 29.6 IL10 CRP CD40LG
16 polyclonal hypergammaglobulinemia 29.6 CRP CD40LG CD4
17 mononeuritis multiplex 29.5 CRP CD40LG CD4
18 thrombocytopenia due to platelet alloimmunization 29.5 IL10 CD40LG CD4 CCR6
19 salivary gland disease 29.5 IL10 CD40LG CD4 CCR6
20 miliary tuberculosis 29.5 CRP CD40LG CD4
21 castleman disease 29.5 IL5 IL4 IL10 CRP
22 purpura 29.4 IL4 IL10 CRP CD40LG
23 sarcoidosis 1 29.4 IL13 CRP CD40LG CD4 CCR6
24 lymphadenitis 29.4 IL10 CRP CD4 CCR6
25 lymphoma, mucosa-associated lymphoid type 29.3 CXCR5 CD40LG CD4 CCR6
26 multicentric castleman disease 29.1 IL5 IL21 IL10 CRP CD4
27 pancytopenia 29.1 CRP CD40LG CD4
28 crohn's disease 29.0 IL4 IL10 CRP CD4 CCR6
29 lymphoma 29.0 IL4 IL10 CXCR5 CD40LG CCR6
30 igg4-related disease 29.0 SLAMF7 KNTC1 IL4 CRP CD4 CCR6
31 mycobacterium tuberculosis 1 29.0 IL4 IL10 CRP CD4 CCR6
32 parotitis 28.9 SPINK1 CRP CD40LG CD4
33 exanthem 28.9 IL10 CRP CD40LG CD4 CCR6
34 lymphoma, non-hodgkin, familial 28.5 IL4 IL10 CXCR5 CD40LG CD4 CCR6
35 bronchiolitis 28.5 IL5 IL4 IL13 IL10 CRP CD40LG
36 connective tissue disease 28.5 IL4 IL10 CRP CD40LG CD4 CCR6
37 acquired immunodeficiency syndrome 28.3 IL10 CXCR5 CRP CD40LG CD4 CCR6
38 autoimmune disease 28.0 IL5 IL4 IL21 IL13 IL10 CRP
39 myeloma, multiple 27.9 SLAMF7 IL4 IL10 CRP CD40LG CD4
40 rheumatoid arthritis 27.5 IL4 IL13 IL10 CXCR5 CRP CD40LG
41 aztreonam allergy 10.4 IL5 IL13
42 angioimmunoblastic lymphadenopathy with dysproteinemia 10.4 IL5 IL13
43 respiratory syncytial virus infectious disease 10.4 IL5 IL13
44 cefaclor allergy 10.4 IL5 IL13
45 cefuroxime allergy 10.4 IL5 IL13
46 ceftriaxone allergy 10.4 IL5 IL13
47 loeffler syndrome 10.4 IL5 IL13
48 atopic keratoconjunctivitis 10.4 IL5 IL4
49 eosinophilic gastritis 10.4 IL5 IL13
50 tropical endomyocardial fibrosis 10.4 IL4 IL10

Graphical network of the top 20 diseases related to Mikulicz Disease:



Diseases related to Mikulicz Disease
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Symptoms & Phenotypes for Mikulicz Disease

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Symptoms:

12
  • • lumps of the face

MGI Mouse Phenotypes related to Mikulicz Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.9 CCR6 CD4 CD40LG CXCR5 IL10 IL13
2 digestive/alimentary MP:0005381 9.73 CD4 IL10 IL13 IL4 IL5 SPINK1
3 homeostasis/metabolism MP:0005376 9.7 CCR6 CD4 CD40LG CRP IL10 IL13
4 immune system MP:0005387 9.4 CCR6 CD4 CD40LG CRP CXCR5 IL10
Sources

Drugs & Therapeutics for Mikulicz Disease

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

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
2
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
3
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
5
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
6 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
7
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
8
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
9 Hormones Phase 4
10 Immunologic Factors Phase 4
11 Antineoplastic Agents, Hormonal Phase 4
12 Methylprednisolone Acetate Phase 4
13 glucocorticoids Phase 4
14 Hormone Antagonists Phase 4
15 Immunosuppressive Agents Phase 4
16 Liver Extracts Phase 4
17 Anti-Inflammatory Agents Phase 4
18 Immunoglobulins
19 Antibodies
20 Immunoglobulins, Intravenous
21 Immunoglobulin G

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Study of Safety and Efficacy of Leflunomide for Maintenance of Remission in IgG4 Related Disease Completed NCT02703194 Phase 4 Prednisone;Leflunomide
2 A Survey of Patients With Elevated Serum IGG4 With Unknown IGG4-related Disease to Whom Biopsy and Performance of Subsequent Specific Staining for Clinical, Laboratory and Pathologic Correlation. Completed NCT02229448

Search NIH Clinical Center for Mikulicz Disease

Cochrane evidence based reviews: mikulicz' disease

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Genetic Tests for Mikulicz Disease

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Anatomical Context for Mikulicz Disease

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MalaCards organs/tissues related to Mikulicz Disease:

40
Eye, Salivary Gland, Bone, Liver, Bone Marrow, Lung, Skin
Sources

Publications for Mikulicz Disease

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Articles related to Mikulicz Disease:

(show top 50) (show all 91)
# Title Authors PMID Year
1
Cutting Edge: A Critical Role of Lesional T Follicular Helper Cells in the Pathogenesis of IgG4-Related Disease. 61 52
28916523 2017
2
Serum immunoglobulin G4 in Sjögren's syndrome: a pilot study. 61
32062697 2020
3
The diagnostic utility of submandibular gland sonography and labial salivary gland biopsy in IgG4-related dacryoadenitis and sialadenitis: Its potential application to the diagnostic criteria. 61
30696319 2020
4
Clinical features of immunoglobulin G4-related disease with central nervous system involvement: an analysis of 15 cases. 61
32083541 2020
5
Mikulicz Disease. 61
31913425 2020
6
Interleukin 5-producing ST2+ memory Th2 cells in IgG4-related dacryoadenitis and sialadenitis. 61
30354922 2019
7
Potential utility of core needle biopsy in the diagnosis of IgG4-related dacryoadenitis and sialadenitis. 61
29656682 2019
8
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients. 61
30701685 2019
9
IgG4-related respiratory disease. 61
30474465 2019
10
Stage classification of IgG4-related dacryoadenitis and sialadenitis by the serum cytokine environment. 61
29385874 2018
11
Early Therapeutic Intervention for IgG4-related Dacryoadenitis and Sialadenitis: The Balance Between Risk of Observation Only and Therapeutic Adverse Effects. 61
30173184 2018
12
Estimation of proton density fat fraction of the salivary gland. 61
29376736 2018
13
Genes and pathways associated with the occurrence of malignancy in benign lymphoepithelial lesions. 61
29207199 2018
14
Mikulicz Disease Mimicking Intraorbital Tumors. 61
28987847 2018
15
[Non-Hodgkin's lymphoma mimicking Mikulicz disease: a case report]. 61
27987516 2016
16
Gallium SPECT/CT in evaluation of IgG4-related disease: A case report and literature review. 61
27631251 2016
17
IgG4-related disease. 61
26259069 2016
18
IgG4-related hypophysitis presenting as diabetes insipidus with tubulo-interstital nephritis and mediastinal lymphadenopathy. 61
27398219 2016
19
[Review of ear and nose and throat involvement in IgG4-RD]. 61
26911076 2015
20
[112th Scientific Meeting of the Japanese Society of Internal Medicine: Symposium: Recent Progress in IgG4-related Disease: IgG4-related Dacryoadenitis and Sialadenitis]. 61
30160920 2015
21
[IgG4-related Dacryoadenitis and Sialadenitis]. 61
30160881 2015
22
Effectiveness of imaging modalities for screening IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) and for differentiating it from Sjögren's syndrome (SS), with an emphasis on sonography. 61
26298875 2015
23
IgG4-related Mikulicz Disease in a 76-year-old Chinese Male: A Case Report. 61
24432837 2015
24
IgG4 related disease of the head and neck. 61
25804380 2015
25
Diagnostic criteria for IgG4-related ophthalmic disease. 61
25392273 2015
26
Identification of relapse predictors in IgG4-related disease using multivariate analysis of clinical data at the first visit and initial treatment. 61
24907151 2015
27
IgG4-related skin disease. 61
25065694 2014
28
The role of cytotoxic T cells in IgG4-related dacryoadenitis and sialadenitis, the so-called Mikulicz's disease. 61
24593171 2014
29
¹⁸F-FDG PET-CT usefulness in extra-pancreatic involvement in IgG4 related diseases. 61
24630373 2014
30
Mechanisms and assessment of IgG4-related disease: lessons for the rheumatologist. 61
24296677 2014
31
IgG4-related multiorgan disease: report of the first autopsy case. 61
23645656 2013
32
Rare diagnosis of IgG4-related systemic disease by lip biopsy in an international Sjögren syndrome registry. 61
23146570 2013
33
IgG4-related disease in the head and neck. 61
23068303 2012
34
"Quasi-symmetric" FDG avidity of bilateral salivary glands in Mikulicz disease yielding false-positive staging in lung cancer. 61
22996254 2012
35
IgG4-related disease of the head and neck: CT and MR imaging manifestations. 61
23150850 2012
36
Autoimmune pancreatitis that developed over a 3-month observation period for IgG4-related dacryoadenitis and sialadenitis. 61
26182321 2012
37
Utility of FDG PET/CT in IgG4-related systemic disease. 61
22119099 2012
38
Th2 and regulatory immune reactions contribute to IgG4 production and the initiation of Mikulicz disease. 61
21898360 2012
39
[Usefulness of measuring serum IgG4 level as diagnostic and treatment marker in IgG4-related disease]. 61
22374440 2012
40
Jan Mikulicz-Radecki (1850-1905): his impact on modern medicine. 61
25872240 2012
41
[A case of IgG4-related disease with marked thickening of the bronchial wall]. 61
22352056 2011
42
Cytohistological features of rhinoscleroma. 61
22234117 2011
43
Connective tissue diseases: Immune responses in Mikulicz disease differ from those in SS. 61
21971284 2011
44
Membranous nephropathy associated with IgG4-related disease. 61
21658826 2011
45
IgG4-associated sialadenitis. 61
21124091 2011
46
Clinicopathologic similarities between Mikulicz disease and Küttner tumor. 61
20015799 2010
47
[Clinical analysis of 11 cases of Mikulicz disease in the salivary glands]. 61
21176583 2010
48
[The first experience of using rituximab in Mikulicz disease]. 61
20731115 2010
49
Highly active state of autoimmune pancreatitis with mikulicz disease. 61
19904224 2010
50
[Autoimmune pancreatitis]. 61
19860211 2009
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Variations for Mikulicz Disease

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Expression for Mikulicz Disease

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Search GEO for disease gene expression data for Mikulicz Disease.
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Pathways for Mikulicz Disease

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Pathways related to Mikulicz Disease according to GeneCards Suite gene sharing:

(show all 32)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 65
Show member pathways
 13.86 SLAMF7 IL5 IL4 IL13 IL10 CRP
2
ERK Signaling 63
Show member pathways
 13.66 IL5 IL4 IL21 IL13 IL10 CXCR5
3
PEDF Induced Signaling 63
Show member pathways
 13.5 IL5 IL4 IL21 IL13 IL10 CXCR5
4
Cytokine Signaling in Immune system 65
Show member pathways
 13.22 IL5 IL4 IL13 IL10 CD40LG CD4
5
Akt Signaling 63
Show member pathways
 13.2 IL5 IL4 IL21 IL13 IL10 CXCR5
6
PAK Pathway 63
Show member pathways
 13.04 IL5 IL4 IL21 IL13 IL10 CXCR5
7
Allograft rejection 1 36
Show member pathways
 12.6 IL5 IL4 IL21 IL13 IL10 CXCR5
8
Immune response NFAT in immune response 22
Show member pathways
 12.51 IL4 IL10 CD40LG CD4
9
T cell receptor signaling pathway 36
Show member pathways
 12.51 IL5 IL4 IL10 CD40LG CD4
10
Th17 cell differentiation 36
Show member pathways
 12.45 IL5 IL4 IL21 IL13 IL10 CD4
11
NF-kappaB Signaling 4
12.44 IL4 IL10 CXCR5 CD40LG
12
JAK-STAT signaling pathway 36
Show member pathways
 12.35 IL5 IL4 IL21 IL13 IL10
13
Immune response IFN alpha/beta signaling pathway 22
Show member pathways
 12.15 IL5 IL4 IL13 IL10
14
Hematopoietic Stem Cells and Lineage-specific Markers 64
11.91 IL5 IL4 IL10 CD4
15
Interleukin-4 and 13 signaling 65
11.88 IL4 IL13 IL10
16
IL27-mediated signaling events 1
Show member pathways
 11.84 IL21 IL10 CD4
17
Hematopoietic cell lineage 36
11.79 IL5 IL4 CD4
18
Th17 Differentiation 64
Show member pathways
 11.75 IL4 IL21 CXCR5 CD4
19
Glucocorticoid receptor regulatory network 1
11.7 IL5 IL4 IL13
20
Calcineurin-regulated NFAT-dependent transcription in lymphocytes 1
Show member pathways
 11.66 IL5 IL4 CD40LG
21
Lung fibrosis 1
11.57 IL5 IL4 IL13
22
IL4-mediated signaling events 1
11.56 IL5 IL4 IL10 CD40LG
23
Interleukin-10 signaling 65
11.46 IL4 IL13 IL10
24
Inflammatory Response Pathway 1
11.28 IL5 IL4 CD40LG
25
Development and heterogeneity of the ILC family 1
11.26 IL5 IL4 IL13
26
Th2 Differentiation 64
11.22 IL5 IL4 IL13 IL10 CD4
27
OX40 Pathway 63
11.08 IL5 IL4 IL13 IL10
28
Innate Lymphoid Cells Differentiation 64
11.07 IL5 IL4 IL13 IL10 CXCR5 CD4
29
G-protein signaling_Rap2B regulation pathway 22
10.9 IL5 IL4 IL21 IL13 IL10 CD40LG
30
IL-4 Signaling and its Primary Biological Effects in Different Immune Cell Types 64
10.88 IL4 IL21
31
Cytokines and Inflammatory Response 1
10.82 IL5 IL4 IL13 IL10 CD4
32
IL-9 Signaling and its Primary Biological Effects in Different Immune Cell Types 64
10.73 IL5 IL4 IL13
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GO Terms for Mikulicz Disease

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Cellular components related to Mikulicz Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.76 SPINK1 IL5 IL4 IL21 IL13 IL10
2 external side of plasma membrane GO:0009897 9.35 IL13 CXCR5 CD40LG CD4 CCR6
3 extracellular space GO:0005615 9.23 SPINK1 IL5 IL4 IL21 IL13 IL10

Biological processes related to Mikulicz Disease according to GeneCards Suite gene sharing:

(show all 19)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.01 IL5 IL4 IL21 IL10 CXCR5 CD40LG
2 inflammatory response GO:0006954 9.87 IL5 IL13 CRP CD40LG
3 negative regulation of inflammatory response GO:0050728 9.75 IL4 IL13 IL10
4 B cell differentiation GO:0030183 9.67 IL4 IL10 CD40LG
5 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.65 IL4 IL21 IL13
6 cytokine-mediated signaling pathway GO:0019221 9.65 IL5 IL4 IL13 IL10 CD4
7 positive regulation of macrophage activation GO:0043032 9.58 IL13 IL10
8 macrophage activation GO:0042116 9.56 IL4 IL13
9 positive regulation of interleukin-10 secretion GO:2001181 9.55 IL4 IL13
10 positive regulation of mast cell degranulation GO:0043306 9.54 IL4 IL13
11 negative regulation of tumor necrosis factor biosynthetic process GO:0042536 9.52 IL4 IL10
12 positive regulation of MHC class II biosynthetic process GO:0045348 9.51 IL4 IL10
13 negative regulation of endothelial cell apoptotic process GO:2000352 9.5 IL4 IL13 IL10
14 type 2 immune response GO:0042092 9.46 IL4 IL10
15 positive regulation of T cell proliferation GO:0042102 9.46 IL4 IL21 CD40LG CD4
16 regulation of isotype switching GO:0045191 9.43 IL4 IL10
17 negative regulation of complement-dependent cytotoxicity GO:1903660 9.37 IL4 IL13
18 immune response GO:0006955 9.28 IL5 IL4 IL21 IL13 IL10 CXCR5
19 positive regulation of B cell proliferation GO:0030890 9.26 IL5 IL4 IL21 IL13

Molecular functions related to Mikulicz Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine receptor binding GO:0005126 9.13 IL4 IL21 IL13
2 cytokine activity GO:0005125 9.1 IL5 IL4 IL21 IL13 IL10 CD40LG
Sources

Sources for Mikulicz Disease

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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