OMS
MCID: OPS006
MIFTS: 47

Opsoclonus-Myoclonus Syndrome (OMS)

Categories: Endocrine diseases, Eye diseases, Infectious diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Opsoclonus-Myoclonus Syndrome

MalaCards integrated aliases for Opsoclonus-Myoclonus Syndrome:

Name: Opsoclonus-Myoclonus Syndrome 52 58 54 17 71
Kinsbourne Syndrome 52 53 58 71
Paraneoplastic Opsoclonus-Myoclonus-Ataxia Syndrome 52 58
Opsoclonus-Myoclonus-Ataxia Syndrome 52 58
Paraneoplastic Opsoclonus-Myoclonus 52 58
Dancing Eye-Dancing Feet Syndrome 52 58
Ataxo-Opso-Myoclonus Syndrome 52 58
Dancing Eye Syndrome 52 58
Poma Syndrome 52 58
Oma Syndrome 52 58
Oms 52 58
Dancing Eyes-Dancing Feet Syndrome 53
Opsoclonus Myoclonus Syndrome 52
Opsoclonus Myoclonus 53

Characteristics:

Orphanet epidemiological data:

58
opsoclonus-myoclonus syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe),<1/1000000 (United Kingdom); Age of onset: Childhood,Infancy; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare eye diseases


External Ids:

MESH via Orphanet 44 D053578
ICD10 via Orphanet 33 G25.3
UMLS via Orphanet 72 C0393626 C1096154 C1721017
Orphanet 58 ORPHA1183
UMLS 71 C0393626 C1096154

Summaries for Opsoclonus-Myoclonus Syndrome

NIH Rare Diseases : 52 Opsoclonus-myoclonus syndrome (OMS) is a rare disorder that affects the nervous system. Symptoms include rapid, multi-directional eye movements (opsoclonus), quick, involuntary muscle jerks (myoclonus ), uncoordinated movement (ataxia ), irritability, and sleep disturbance. The onset of OMS is usually abrupt and often severe. The disease may become chronic. OMS typically occurs in association with tumors (neuroblastomas ), or following a viral or bacterial infection. Treatment may include corticosteroids or ACTH (adrenocorticotropic hormone ). When there is a tumor present, treatment may include chemotherapy , surgery, and/or radiation. In some cases, when the underlying cause of OMS is treated, symptoms improve.

MalaCards based summary : Opsoclonus-Myoclonus Syndrome, also known as kinsbourne syndrome, is related to viral encephalitis and malaria, and has symptoms including myoclonus and ophthalmoplegia. An important gene associated with Opsoclonus-Myoclonus Syndrome is POMC (Proopiomelanocortin), and among its related pathways/superpathways are ERK Signaling and PEDF Induced Signaling. The drugs rituximab and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include eye, lung and brain, and related phenotypes are hematopoietic system and immune system

NINDS : 53 Opsoclonus myoclonus is a rare neurological disorder characterized by an unsteady, trembling gait, myoclonus (brief, shock-like muscle spasms), and opsoclonus (irregular, rapid eye movements). Other symptoms may include difficulty speaking, poorly articulated speech, or an inability to speak. A decrease in muscle tone, lethargy, irritability, and malaise (a vague feeling of bodily discomfort) may also be present. Opsoclonus myoclonus may occur in association with tumors or viral infections. It is often seen in children with tumors.

Wikipedia : 74 Opsoclonus myoclonus syndrome (OMS), also known as opsoclonus-myoclonus-ataxia (OMA), is a rare... more...

Related Diseases for Opsoclonus-Myoclonus Syndrome

Diseases related to Opsoclonus-Myoclonus Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 218)
# Related Disease Score Top Affiliating Genes
1 viral encephalitis 29.9 TNFSF13B CXCL9 CXCL10
2 malaria 28.9 TNFSF13B CXCR3 CXCL9 CXCL11 CXCL10
3 autoimmune disease 28.5 TNFSF13B TNFSF13 GPI CXCR3 CXCL10
4 neuroblastoma 11.9
5 myoclonic encephalopathy of infants 11.8
6 myoclonus 11.3
7 ataxia and polyneuropathy, adult-onset 11.0
8 myoclonus and ataxia 10.6
9 encephalitis 10.6
10 small cell cancer of the lung 10.5
11 dengue virus 10.5
12 teratoma 10.5
13 teratoma, ovarian 10.5
14 west nile virus 10.5
15 gangliocytoma 10.5
16 ganglioneuroblastoma 10.5
17 ganglioneuroma 10.5
18 ovarian germ cell teratoma 10.5
19 tremor 10.5
20 lung cancer 10.4
21 small cell carcinoma 10.4
22 encephalopathy 10.4
23 ovarian cancer 10.3
24 melanoma, cutaneous malignant 10 10.3
25 lymphoma 10.3
26 melanoma 10.3
27 mutism 10.3
28 rare movement disorder 10.3
29 heart block, congenital 10.3
30 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.3
31 neuroblastoma 1 10.3
32 suppression of tumorigenicity 12 10.3
33 human immunodeficiency virus type 1 10.3
34 neuroblastoma 3 10.3
35 chikungunya 10.3
36 exanthem 10.3
37 horner's syndrome 10.3
38 benign mesothelioma 10.3
39 differentiating neuroblastoma 10.3
40 malignant pleural mesothelioma 10.3
41 lupus erythematosus 10.3
42 breast cancer 10.3
43 lymphoma, hodgkin, classic 10.3
44 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.3
45 gastroenteritis 10.3
46 breast adenocarcinoma 10.3
47 movement disease 10.3
48 dystonia 10.3
49 adrenal neuroblastoma 10.3
50 acute disseminated encephalomyelitis 10.3

Graphical network of the top 20 diseases related to Opsoclonus-Myoclonus Syndrome:



Diseases related to Opsoclonus-Myoclonus Syndrome

Symptoms & Phenotypes for Opsoclonus-Myoclonus Syndrome

UMLS symptoms related to Opsoclonus-Myoclonus Syndrome:


myoclonus, ophthalmoplegia

MGI Mouse Phenotypes related to Opsoclonus-Myoclonus Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.43 CXCL10 CXCR3 GPI POMC TNFSF13 TNFSF13B
2 immune system MP:0005387 9.1 CXCL10 CXCR3 GPI POMC TNFSF13 TNFSF13B

Drugs & Therapeutics for Opsoclonus-Myoclonus Syndrome

Drugs for Opsoclonus-Myoclonus Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 53)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 3 174722-31-7 10201696
2
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
3
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
4
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
5 Immunoglobulins, Intravenous Phase 3
6 Rho(D) Immune Globulin Phase 3
7 Corticotropin-Releasing Hormone Phase 3
8 gamma-Globulins Phase 3
9 Gastrointestinal Agents Phase 3
10 Antineoplastic Agents, Immunological Phase 3
11 Hormones Phase 3
12 Antineoplastic Agents, Hormonal Phase 3
13
protease inhibitors Phase 3
14 Alkylating Agents Phase 3
15 Antiemetics Phase 3
16 Immunosuppressive Agents Phase 3
17 Hormone Antagonists Phase 3
18 HIV Protease Inhibitors Phase 3
19 glucocorticoids Phase 3
20 BB 1101 Phase 3
21 Anti-Inflammatory Agents Phase 3
22 Antirheumatic Agents Phase 3
23 Immunologic Factors Phase 3
24
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
25
Carmustine Approved, Investigational Phase 2 154-93-8 2578
26
Etoposide Approved Phase 2 33419-42-0 36462
27
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
28
Cytarabine Approved, Experimental, Investigational Phase 2 147-94-4, 65-46-3 6253
29
Melphalan Approved Phase 2 148-82-3 460612 4053
30
Phenylalanine Approved, Investigational, Nutraceutical Phase 2 63-91-2 6140
31
Cortisone Experimental Phase 2 53-06-5 222786
32 Adrenocorticotropic Hormone Phase 2
33 Neurotransmitter Agents Phase 2
34 Endorphins Phase 2
35 Melanocyte-Stimulating Hormones Phase 2
36 beta-Endorphin Phase 2
37 Etoposide phosphate Phase 2
38 Anti-Infective Agents Phase 2
39 Nitrogen Mustard Compounds Phase 2
40 Dermatologic Agents Phase 2
41 Antiviral Agents Phase 2
42 Thymoglobulin Phase 2
43 Podophyllotoxin Phase 2 518-28-5
44 Tubulin Modulators Phase 2
45 Keratolytic Agents Phase 2
46 Antimetabolites Phase 2
47 Antimitotic Agents Phase 2
48 Antilymphocyte Serum Phase 2
49
L-Alanine Nutraceutical Phase 2 56-41-7 5950
50 Antibodies Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Pilot Study Randomized Trial of Intravenous Gammaglobulin Therapy for Patients With Neuroblastoma Associated Opsoclonus-Myoclonus-Ataxia Syndrome Treated With Chemotherapy and Prednisone Unknown status NCT00033293 Phase 3 cyclophosphamide;prednisone;Corticotropin-Releasing Hormone
2 Multinational European Trial for Children With the Opsoclonus Myoclonus Syndrome / Dancing Eye Syndrome Recruiting NCT01868269 Phase 3 Dexamethasone acetate;dexamethasone and cyclophosphamide;dexamethasone and rituximab
3 Use of Rituximab in Opsoclonus-Myoclonus in Children With Neuroblastoma Unknown status NCT00202930 Phase 2 anti-CD20 (Rituximab)
4 A Phase I Clinical Trial of Rituximab for Pediatric Opsoclonus-Myoclonus Syndrome Completed NCT00244361 Phase 1, Phase 2 rituximab
5 High-Dose Immunosuppressive Therapy Using Carmustine, Etoposide, Cytarabine, and Melphalan (BEAM) + Thymoglobulin Followed by Syngeneic or Autologous Hematopoietic Cell Transplantation for Patients With Autoimmune Neurologic Diseases Recruiting NCT00716066 Phase 2 Carmustine;Cytarabine;Etoposide;Melphalan;Prednisone
6 An Open Label, Multi-centered, Randomized Phase 2 Study to Evaluate the Safety, Tolerability and Bioactivity of Subcutaneous ACTH GeL in PAtients With Scleritis: The ATLAS Study Recruiting NCT03465111 Phase 2 ACTH (adrenocorticotropic hormone) gel
7 Open Label Study of Safety and Tolerability of Rituximab in Neuromyelitis Optica, Recurrent Transverse Myelitis and Recurrent Bilateral Simultaneous Optic Neuritis Completed NCT00501748 Phase 1 Rituximab
8 Cytokines as Biomarkers and Therapeutic Targets in Paraneoplastic Opsoclonus-Myoclonus Syndrome (OMS) Completed NCT00806182
9 Immunotherapy of the Paraneoplastic Syndromes Completed NCT00378326 Tacrolimus
10 Neuroblastoma Biology Studies Recruiting NCT00904241

Search NIH Clinical Center for Opsoclonus-Myoclonus Syndrome

Genetic Tests for Opsoclonus-Myoclonus Syndrome

Anatomical Context for Opsoclonus-Myoclonus Syndrome

MalaCards organs/tissues related to Opsoclonus-Myoclonus Syndrome:

40
Eye, Lung, Brain, Breast, B Cells, T Cells, Prostate

Publications for Opsoclonus-Myoclonus Syndrome

Articles related to Opsoclonus-Myoclonus Syndrome:

(show top 50) (show all 370)
# Title Authors PMID Year
1
Neurometabolic effects of ACTH on free amino compounds in opsoclonus-myoclonus syndrome. 54 61
18991196 2008
2
Monoaminergic effects of high-dose corticotropin in corticotropin-responsive pediatric opsoclonus-myoclonus. 54 61
9613746 1998
3
Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient. 61
32472953 2020
4
Serum IgG-induced microglial activation enhances neuronal cytolysis via the NO/sGC/PKG pathway in children with opsoclonus-myoclonus syndrome and neuroblastoma. 61
32546235 2020
5
Anti-Ri-associated paraneoplastic ophthalmoplegia-ataxia syndrome in a woman with breast cancer: a case report and review of the literature. 61
32527295 2020
6
Opsoclonus-myoclonus syndrome associated with herpes simplex virus infection: a case report. 61
32116082 2020
7
Pediatric whole body MRI detects causative ovarian teratoma in opsoclonus myoclonus syndrome. 61
31890069 2020
8
[Opsoclonus-myoclonus syndrome: Experience in a tertiary hospital in the last 12 years]. 61
32070664 2020
9
Benign opsoclonus myoclonus syndrome and prostate cancer. 61
28958399 2020
10
Treatment-refractory paraneoplastic opsoclonus-myoclonus syndrome in a patient with small-cell carcinoma of the lung. 61
30791855 2020
11
Paraneoplastic opsoclonus-myoclonus syndrome in a patient with oesophageal adenocarcinoma. 61
31899013 2019
12
[Opsoclonus myoclonus syndrome secondary to abdominal neuroblastoma. Presentation of a pediatric clinical case]. 61
31758904 2019
13
Paraneoplastic opsoclonus-myoclonus syndrome as a presentation of high grade serous ovarian cancer. 61
31828196 2019
14
[Neurological complications associated with dengue virus infection]. 61
31310001 2019
15
Diagnostic value of whole-body MRI in Opsoclonus-myoclonus syndrome: a clinical case series (3 case reports). 61
31429709 2019
16
An upfront immunomodulatory therapy protocol for pediatric opsoclonus-myoclonus syndrome. 61
31033188 2019
17
Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India. 61
31359941 2019
18
Update on opsoclonus-myoclonus syndrome in adults. 61
30483882 2019
19
Corrigendum: Treatment-refractory paraneoplastic opsoclonus-myoclonus syndrome in a patient with small cell carcinoma of the lung. 61
30907284 2019
20
IGF-1 alleviates serum IgG-induced neuronal cytolysis through PI3K signaling in children with opsoclonus-myoclonus syndrome and neuroblastoma. 61
30718793 2019
21
A novel exaggerated "spino-bulbo-spinal like" reflex of lower brainstem origin. 61
30316728 2019
22
Paraneoplastic Opsoclonus-Myoclonus Syndrome Preceding a Mucosal Malignant Melanoma. 61
30281137 2019
23
Paraneoplastic opsoclonus-myoclonus syndrome as a rare presentation of breast cancer. 61
30788093 2019
24
Opsoclonus in a child with neuroborreliosis: Case report and review of the literature. 61
30655046 2019
25
Paraneoplastic Opsoclonus Myoclonus in a Patient with Pancreatic Adenocarcinoma. 61
31139479 2019
26
Immune-mediated Cerebellar Ataxias: Practical Guidelines and Therapeutic Challenges. 61
30221603 2019
27
Paraneoplastic opsoclonus-myoclonus syndrome secondary to melanoma metastasis form occult primary cancer. 61
31543788 2019
28
Neurovascular compression syndrome of the brain stem with opsoclonus-myoclonus syndrome combined with vestibular paroxysmia and autonomic symptoms. 61
31534075 2019
29
A case of adult-onset poststreptococcal opsoclonus-myoclonus syndrome. 61
29713906 2018
30
NMDA receptor antibody in teratoma-related opsoclonus-myoclonus syndrome. 61
30340968 2018
31
Opsoclonus Myoclonus Syndrome: A Rare Manifestation of Dengue Infection in a Child. 61
30937088 2018
32
Opsoclonus myoclonus syndrome in children: Paraneoplastic versus parainfectious. 61
30232990 2018
33
Paraneoplastic movement disorders. 61
29561731 2018
34
Acute transverse myelitis following an opsoclonus-myoclonus syndrome: An unusual presentation. 61
29773357 2018
35
Evaluation of Responsiveness to Reduced-Dose Rituximab in Corticotropin/Intravenous Immunoglobulin/Rituximab Combination Immunotherapy for Opsoclonus-Myoclonus Syndrome. 61
30197220 2018
36
Multifactorial analysis of opsoclonus-myoclonus syndrome etiology ("Tumor" vs. "No tumor") in a cohort of 356 US children. 61
29727049 2018
37
Relation of intrathecal oligoclonal band production to inflammatory mediator and immunotherapy response in 208 children with OMS. 61
29685330 2018
38
Effect of low-dose cyclophosphamide, ACTH, and IVIG combination immunotherapy on neuroinflammation in pediatric-onset OMS: A retrospective pilot study. 61
29555260 2018
39
Exaggerated startle in post-infectious opsoclonus myoclonus syndrome. 61
29729591 2018
40
Opsoclonus-myoclonus syndrome following long-term use of cyclosporine. 61
28905654 2018
41
Rituximab, IVIg, and Tetracosactide (ACTH1-24) Combination Immunotherapy ("RITE-CI") for Pediatric Opsoclonus-Myoclonus Syndrome: Immunomarkers and Clinical Observations. 61
29258131 2018
42
Opsoclonus-Myoclonus Syndrome-Additional Clinical Considerations. 61
29522119 2018
43
Opsoclonus-Myoclonus Syndrome-Reply. 61
29522060 2018
44
Genomic Profiles of Neuroblastoma Associated With Opsoclonus Myoclonus Syndrome. 61
29135842 2018
45
Kinsbourne syndrome associated with cryptococcosis infection. 61
29174170 2018
46
Autoimmune and paraneoplastic movement disorders: An update. 61
29406902 2018
47
Immune-mediated ataxias. 61
29891069 2018
48
Opsoclonus-Myoclonus Syndrome Associated With West-Nile Virus Infection: Case Report and Review of the Literature. 61
30386288 2018
49
A case report of sphenoid sinusitis causing opsoclonus myoclonus syndrome. 61
30596202 2018
50
Corrigendum to "Dexamethasone, Intravenous Immunoglobulin, and Rituximab Combination Immunotherapy for Pediatric Opsoclonus-Myoclonus Syndrome" [Pediatric Neurology 73 (2017) 48-56]. 61
29169865 2018

Variations for Opsoclonus-Myoclonus Syndrome

Expression for Opsoclonus-Myoclonus Syndrome

Search GEO for disease gene expression data for Opsoclonus-Myoclonus Syndrome.

Pathways for Opsoclonus-Myoclonus Syndrome

Pathways related to Opsoclonus-Myoclonus Syndrome according to GeneCards Suite gene sharing:

(show all 14)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.41 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10
2
Show member pathways
13.22 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10
3
Show member pathways
13.19 POMC CXCR3 CXCL9 CXCL11 CXCL10
4
Show member pathways
12.95 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10
5
Show member pathways
12.53 CXCR3 CXCL9 CXCL11 CXCL10
6
Show member pathways
12.5 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10
7
Show member pathways
12.48 TNFSF13B TNFSF13 CXCL9 CXCL11
8
Show member pathways
11.89 CXCR3 CXCL9 CXCL11 CXCL10
9 11.62 TNFSF13B TNFSF13 CXCL10
10
Show member pathways
11.45 TNFSF13B TNFSF13
11
Show member pathways
11.39 TNFSF13B TNFSF13
12 11.06 CXCL9 CXCL10
13 10.46 TNFSF13B TNFSF13 CXCL9 CXCL11 CXCL10
14 10.46 CXCR3 CXCL9 CXCL11 CXCL10

GO Terms for Opsoclonus-Myoclonus Syndrome

Cellular components related to Opsoclonus-Myoclonus Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell GO:0005623 9.56 CXCR3 CXCL9 CXCL11 CXCL10
2 extracellular region GO:0005576 9.5 TNFSF13B TNFSF13 POMC GPI CXCL9 CXCL11
3 external side of plasma membrane GO:0009897 9.33 CXCR3 CXCL9 CXCL10
4 extracellular space GO:0005615 9.17 TNFSF13B TNFSF13 POMC GPI CXCL9 CXCL11

Biological processes related to Opsoclonus-Myoclonus Syndrome according to GeneCards Suite gene sharing:

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 9.92 POMC CXCR3 CXCL9 CXCL11 CXCL10
2 inflammatory response GO:0006954 9.8 CXCR3 CXCL9 CXCL11 CXCL10
3 regulation of cell proliferation GO:0042127 9.73 CXCL9 CXCL11 CXCL10
4 cell-cell signaling GO:0007267 9.73 POMC CXCL9 CXCL11 CXCL10
5 cellular response to lipopolysaccharide GO:0071222 9.72 CXCL9 CXCL11 CXCL10
6 defense response GO:0006952 9.71 CXCL9 CXCL11 CXCL10
7 chemotaxis GO:0006935 9.71 CXCR3 CXCL9 CXCL11 CXCL10
8 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.69 CXCL9 CXCL11 CXCL10
9 neutrophil chemotaxis GO:0030593 9.65 CXCL9 CXCL11 CXCL10
10 antimicrobial humoral immune response mediated by antimicrobial peptide GO:0061844 9.63 CXCL9 CXCL11 CXCL10
11 signal transduction GO:0007165 9.56 TNFSF13B TNFSF13 POMC GPI CXCR3 CXCL9
12 positive regulation of release of sequestered calcium ion into cytosol GO:0051281 9.54 CXCL9 CXCL11 CXCL10
13 leukocyte chemotaxis GO:0030595 9.5 CXCL9 CXCL11 CXCL10
14 chemokine-mediated signaling pathway GO:0070098 9.46 CXCR3 CXCL9 CXCL11 CXCL10
15 T cell chemotaxis GO:0010818 9.43 CXCR3 CXCL11 CXCL10
16 immune response GO:0006955 9.1 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10

Molecular functions related to Opsoclonus-Myoclonus Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 signaling receptor binding GO:0005102 9.62 TNFSF13B TNFSF13 POMC CXCL10
2 chemokine activity GO:0008009 9.43 CXCL9 CXCL11 CXCL10
3 tumor necrosis factor receptor binding GO:0005164 9.37 TNFSF13B TNFSF13
4 CXCR chemokine receptor binding GO:0045236 9.33 CXCL9 CXCL11 CXCL10
5 CXCR3 chemokine receptor binding GO:0048248 9.13 CXCL9 CXCL11 CXCL10
6 cytokine activity GO:0005125 9.1 TNFSF13B TNFSF13 GPI CXCL9 CXCL11 CXCL10

Sources for Opsoclonus-Myoclonus Syndrome

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72 UMLS via Orphanet
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