MCID: GLM004
MIFTS: 52

Gliomatosis Cerebri

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Gliomatosis Cerebri

MalaCards integrated aliases for Gliomatosis Cerebri:

Name: Gliomatosis Cerebri 12 76 53 59 55 15 73
Neoplasms, Neuroepithelial 44
Astrocytosis Cerebri 12

Characteristics:

Orphanet epidemiological data:

59
gliomatosis cerebri
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:6128
MeSH 44 D018302
NCIt 50 C4318
SNOMED-CT 68 26138003
Orphanet 59 ORPHA251582
UMLS via Orphanet 74 C0334576
ICD10 via Orphanet 34 C71.0
UMLS 73 C0334576

Summaries for Gliomatosis Cerebri

NIH Rare Diseases : 53 Gliomatosis cerebri is a type of malignant (cancerous) brain tumor called a glioblastoma that develops from a specific type of brain cell called an astrocyte. It involves at least three sections (lobes) within the main part of the brain (cerebrum). It may also affect both sides of the cerebrum as well as the nerve cells within the brain (grey matter). There are two types of gliomatosis cerebri. Type 1 (classic form) is characterized by scattered and widespread tumor cells and no apparent mass or tumor, while type 2 has similar characteristics in addition to a detectable mass or tumor. Signs and symptoms of gliomatosis cerebri may include personality changes, memory disturbance, headaches, and seizures. Because this type of cancer can be found in many areas of the brain, it can be challenging to treat. Treatment might include surgery to remove areas of the brain affected (when possible), radiation therapy, and chemotherapy.

MalaCards based summary : Gliomatosis Cerebri, also known as neoplasms, neuroepithelial, is related to glioma susceptibility 1 and glioma, and has symptoms including headache, seizures and sensory manifestations. An important gene associated with Gliomatosis Cerebri is GFAP (Glial Fibrillary Acidic Protein), and among its related pathways/superpathways are PI3K/AKT activation and Phospholipase-C Pathway. The drugs Temozolomide and Valproic Acid have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and pituitary, and related phenotypes are Increased cell death HMECs cells and digestive/alimentary

Wikipedia : 76 Gliomatosis cerebri (infiltrative diffuse astrocytosis) is a rare primary brain tumor. It is commonly... more...

Related Diseases for Gliomatosis Cerebri

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

(show top 50) (show all 185)
# Related Disease Score Top Affiliating Genes
1 glioma susceptibility 1 30.9 IDH1 TP53
2 glioma 28.7 EGFR IDH1 MDM2 PTEN TP53
3 glioblastoma 28.7 EGFR IDH1 MDM2 PTEN TP53
4 hydrocephalus 28.5 GFAP NES PTEN TP53
5 medulloblastoma 27.0 EGFR GFAP IDH1 NES PTEN TP53
6 glioblastoma multiforme 26.3 EGFR GFAP IDH1 MDM2 NES PTEN
7 extraventricular neurocytoma 10.7 GFAP IDH1
8 angiocentric glioma 10.7 GFAP IDH1
9 gemistocytic astrocytoma 10.6 GFAP PTEN
10 gangliocytoma 10.6 GFAP PTEN
11 intracranial chondrosarcoma 10.6 IDH1 TP53
12 cerebellar astrocytoma 10.6 IDH1 TP53
13 adult astrocytic tumour 10.6 IDH1 PTEN
14 frontal convexity meningioma 10.6 GFAP TP53
15 cerebellopontine angle tumor 10.6 GFAP IDH1
16 gliofibroma 10.6 GFAP TP53
17 cerebral convexity meningioma 10.6 GFAP TP53
18 protoplasmic astrocytoma 10.6 GFAP TP53
19 cellular ependymoma 10.6 GFAP TP53
20 cerebral neuroblastoma 10.5 GFAP TP53
21 spinal cancer 10.5 GFAP TP53
22 cerebral ventricle cancer 10.5 GFAP TP53
23 cerebrum cancer 10.5 GFAP TP53
24 chordoid glioma 10.5 GFAP TP53
25 supratentorial cancer 10.5 GFAP TP53
26 malignant ependymoma 10.5 GFAP TP53
27 female reproductive endometrioid cancer 10.5 PTEN TP53
28 synchronous bilateral breast carcinoma 10.5 PTEN TP53
29 papilloma of choroid plexus 10.5 GFAP TP53
30 choroid plexus cancer 10.5 GFAP TP53
31 anaplastic oligodendroglioma 10.5 EGFR GFAP
32 periosteal chondrosarcoma 10.5 IDH1 MDM2
33 necrotizing sialometaplasia 10.5 GFAP TP53
34 inflammatory mfh 10.5 GFAP MDM2
35 cystic teratoma 10.5 GFAP TP53
36 cerebellar liponeurocytoma 10.5 GFAP TP53
37 ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome 3 10.4 PTEN TP53
38 cowden syndrome 1 10.4 EGFR PTEN
39 bile duct adenocarcinoma 10.4 IDH1 TP53
40 brain ependymoma 10.4 EGFR TP53
41 ring chromosome 7 10.4 MDM2 TP53
42 zika virus infection 10.4 MDM2 TP53
43 juvenile pilocytic astrocytoma 10.4 GFAP TP53
44 actinic cheilitis 10.4 MDM2 TP53
45 clear cell ependymoma 10.4 GFAP TP53
46 sclerosing liposarcoma 10.4 MDM2 TP53
47 lip cancer 10.4 MDM2 TP53
48 peripheral osteosarcoma 10.4 IDH1 MDM2
49 breast squamous cell carcinoma 10.4 EGFR TP53
50 microglandular adenosis 10.3 EGFR TP53

Graphical network of the top 20 diseases related to Gliomatosis Cerebri:



Diseases related to Gliomatosis Cerebri

Symptoms & Phenotypes for Gliomatosis Cerebri

UMLS symptoms related to Gliomatosis Cerebri:


headache, seizures, sensory manifestations

GenomeRNAi Phenotypes related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased cell death HMECs cells GR00103-A-0 8.92 EGFR MDM2 PTEN TP53

MGI Mouse Phenotypes related to Gliomatosis Cerebri:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.65 TP53 EGFR GFAP MDM2 PTEN
2 muscle MP:0005369 9.55 PTEN TP53 EGFR GFAP MDM2
3 normal MP:0002873 9.43 TP53 EGFR GFAP INA MDM2 PTEN
4 pigmentation MP:0001186 8.92 TP53 EGFR MDM2 PTEN

Drugs & Therapeutics for Gliomatosis Cerebri

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

(show top 50) (show all 86)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Temozolomide Approved, Investigational Phase 3,Phase 2 85622-93-1 5394
2
Valproic Acid Approved, Investigational Phase 3,Phase 2 99-66-1 3121
3
Dacarbazine Approved, Investigational Phase 3 4342-03-4 5351166
4
chloroquine Approved, Investigational, Vet_approved Phase 3 54-05-7 2719
5
Cyproheptadine Approved Phase 3 129-03-3 2913
6
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
7 Dermatologic Agents Phase 3,Phase 1
8 Alkylating Agents Phase 3,Phase 2
9 Central Nervous System Depressants Phase 3,Phase 2
10 GABA Agents Phase 3,Phase 2
11 Neurotransmitter Agents Phase 3,Phase 2,Not Applicable
12 Tranquilizing Agents Phase 3,Phase 2
13 Anticonvulsants Phase 3,Phase 2
14 Psychotropic Drugs Phase 3,Phase 2
15 Antimanic Agents Phase 3,Phase 2
16 Antineoplastic Agents, Alkylating Phase 3,Phase 2
17 Anti-Infective Agents Phase 3,Phase 2
18 Chloroquine diphosphate Phase 3 50-63-5
19 Analgesics Phase 3
20 Analgesics, Non-Narcotic Phase 3
21 Anthelmintics Phase 3
22 Peripheral Nervous System Agents Phase 3,Phase 1
23 Pharmaceutical Solutions Phase 3
24 Antiparasitic Agents Phase 3
25 Antiprotozoal Agents Phase 3
26 Anti-Inflammatory Agents Phase 3,Phase 1
27 Anti-Inflammatory Agents, Non-Steroidal Phase 3
28 Antimalarials Phase 3
29 Antirheumatic Agents Phase 3
30
Histamine Phosphate Phase 3 51-74-1 65513
31 Serotonin Agents Phase 3
32 Serotonin Antagonists Phase 3
33 Gastrointestinal Agents Phase 3,Phase 1,Not Applicable
34 Histamine Antagonists Phase 3
35 Histamine H1 Antagonists Phase 3
36 Anti-Allergic Agents Phase 3
37 Antipruritics Phase 3
38
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
39
Gefitinib Approved, Investigational Phase 1, Phase 2 184475-35-2 123631
40
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
41
Iodine Approved, Investigational Phase 2 7553-56-2 807
42
Dexamethasone Approved, Investigational, Vet_approved Phase 2,Phase 1 50-02-2 5743
43
Acetaminophen Approved Phase 2 103-90-2 1983
44
Diphenhydramine Approved, Investigational Phase 2 147-24-0, 58-73-1 3100
45
Benzocaine Approved, Investigational Phase 2 1994-09-7, 94-09-7 2337
46
Promethazine Approved, Investigational Phase 2 60-87-7 4927
47
Crizotinib Approved Phase 1, Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
48
Bevacizumab Approved, Investigational Phase 2 216974-75-3
49 tannic acid Approved, Nutraceutical Phase 2
50
Doxil Approved June 1999 Phase 2 31703

Interventional clinical trials:

(show all 14)
# Name Status NCT ID Phase Drugs
1 International Cooperative Phase III Trial of the HIT-HGG Study Group (HIT-HGG-2013) Not yet recruiting NCT03243461 Phase 3 Temozolomide + Valproic Acid;Temozolomide + Chloroquine
2 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
3 Gefitinib and Radiation Therapy in Treating Children With Newly Diagnosed Gliomas Completed NCT00042991 Phase 1, Phase 2 gefitinib
4 Prolonged Exposure to Doxorubicin in Patients With Glioblastoma Multiforme and Diffuse Intrinsic Pontine Glioma Recruiting NCT02758366 Phase 2 Doxorubicin
5 Valproic Acid and Radiation Followed by Maintenance Valproic Acid and Bevacizumab in Children With High Grade Gliomas or Diffuse Intrinsic Pontine Glioma Active, not recruiting NCT00879437 Phase 2 Valproic acid;Bevacizumab
6 Iodine I 131 Monoclonal Antibody 3F8 in Treating Patients With Central Nervous System Cancer or Leptomeningeal Cancer Active, not recruiting NCT00445965 Phase 2
7 Crizotinib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Anaplastic Large Cell Lymphoma Active, not recruiting NCT00939770 Phase 1, Phase 2 crizotinib
8 Photodynamic Therapy With Porfimer Sodium in Treating Patients With Refractory Brain Tumors Unknown status NCT00002647 Phase 1 verteporfin
9 Imetelstat Sodium in Treating Young Patients With Refractory or Recurrent Solid Tumors or Lymphoma Completed NCT01273090 Phase 1 imetelstat sodium
10 Palbociclib Isethionate in Treating Younger Patients With Recurrent, Progressive, or Refractory Central Nervous System Tumors Recruiting NCT02255461 Phase 1 palbociclib isethionate
11 Oncolytic HSV-1716 in Treating Younger Patients With Refractory or Recurrent High Grade Glioma That Can Be Removed By Surgery Terminated NCT02031965 Phase 1 dexamethasone
12 Outcomes and Risk in People With Ependymoma Recruiting NCT03251989
13 18F-FDOPA PET/CT or PET/MRI in Measuring Tumors in Patients With Newly-Diagnosed or Recurrent Gliomas Terminated NCT02175745 Not Applicable 18F-fluoro-dihydroxyphenylalanine
14 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452 Not Applicable

Search NIH Clinical Center for Gliomatosis Cerebri

Cochrane evidence based reviews: neoplasms, neuroepithelial

Genetic Tests for Gliomatosis Cerebri

Anatomical Context for Gliomatosis Cerebri

MalaCards organs/tissues related to Gliomatosis Cerebri:

41
Brain, Spinal Cord, Pituitary, Breast

Publications for Gliomatosis Cerebri

Articles related to Gliomatosis Cerebri:

(show top 50) (show all 239)
# Title Authors Year
1
Lactoferrin- and RGD-comodified, temozolomide and vincristine-coloaded nanostructured lipid carriers for gliomatosis cerebri combination therapy. ( 29861635 )
2018
2
Incidence and survival of gliomatosis cerebri: a population-based cancer registration study. ( 29464663 )
2018
3
Gliomatosis Cerebri: A Rare Brain Tumor. ( 29459616 )
2018
4
An Adolescent Presenting With Seizures as a Symptom of Gliomatosis Cerebri. ( 28486262 )
2017
5
Molecular alterations in pediatric gliomatosis cerebri are similar to those in less invasive forms of pediatric diffuse glioma. ( 28421462 )
2017
6
Bevacizumab for Patients with Recurrent Gliomas Presenting with a Gliomatosis Cerebri Growth Pattern. ( 28353668 )
2017
7
Gliomatosis cerebri in a 10-year-old male patient. ( 28484570 )
2017
8
Gliomatosis cerebri: Prognosis based on current molecular markers. ( 28539209 )
2017
9
Gliomatosis Cerebri: Current Understanding and Controversies. ( 28824876 )
2017
10
Management and Survival Patterns of Patients with Gliomatosis Cerebri: A SEER-Based Analysis. ( 28366748 )
2017
11
Magnetic resonance imaging features of canine gliomatosis cerebri. ( 29110365 )
2017
12
Pediatric Gliomatosis Cerebri: A Review of 15 Years. ( 26239491 )
2016
13
Gliomatosis cerebri: no evidence for a separate brain tumor entity. ( 26493382 )
2016
14
Perioperative risk assessment of patients with gliomatosis cerebri. ( 27836704 )
2016
15
Radiology Case of the Month: Gliomatosis Cerebri. ( 27598892 )
2016
16
Gliomatosis cerebri: A consensus summary report from the First International Gliomatosis cerebri Group Meeting, March 26-27, 2015, Paris, France. ( 27466787 )
2016
17
Gliomatosis cerebri in children shares molecular characteristics with other pediatric gliomas. ( 26744350 )
2016
18
Gliomatosis Cerebri in the Brain of a Cat. ( 29056722 )
2016
19
Nanostructured lipid carriers based temozolomide and gene co-encapsulated nanomedicine for gliomatosis cerebri combination therapy. ( 26017099 )
2016
20
Gliomatosis cerebri: Case series of six cases with review of literature. ( 26752659 )
2015
21
Gliomatosis cerebri in a 12-year-old patient. ( 25662824 )
2015
22
"Fungimitosis": Invasive fulminant Aspergillus brain infection mimicking gliomatosis cerebri. ( 26053824 )
2015
23
Corticosteroid sensitivity in gliomatosis cerebri delays diagnosis. ( 25922538 )
2015
24
Gliomatosis cerebri having a poor performance status without recurrence after radiotherapy: a single institutional experience. ( 25569296 )
2015
25
Gliomatosis cerebri with spinal metastasis presenting with chronic meningitis in two boys. ( 26318497 )
2015
26
Gliomatosis cerebri mimicking encephalitis evaluated using fluorine-18 fluorodeoxyglucose: Positron emission tomography/computed tomography. ( 25589818 )
2015
27
Gliomatosis cerebri in L-2-hydroxyglutaric aciduria. ( 25997888 )
2015
28
Pediatric gliomatosis cerebri mimicking tubercular encephalitis. ( 26962350 )
2015
29
Gliomatosis Cerebri Type 1 with Extensive Involvement of the Spinal Cord and BRAF V600E Mutation. ( 24353007 )
2014
30
A comparison of clinical, magnetic resonance imaging and pathological findings in dogs with gliomatosis cerebri, focusing on cases with minimal magnetic resonance imaging changes(a8!) ( 24945683 )
2014
31
Clinical manifestations and imaging characteristics of gliomatosis cerebri with pathological confirmation. ( 24969874 )
2014
32
Gliomatosis cerebri mimicking viral encephalitis in a 4-year-old child. ( 24717581 )
2014
33
Procarbazine, CCNU, and Vincristine Chemotherapy in Gliomatosis Cerebri. ( 25408934 )
2014
34
Patterns of presentation and failure in patients with gliomatosis cerebri treated with partial-brain radiation therapy. ( 24845411 )
2014
35
Nocardia abscesses mimicking tumor progression in gliomatosis cerebri responding to temozolomide. ( 25189790 )
2014
36
Exclusive radiotherapy for gliomatosis cerebri: long-term follow-up at a single institution. ( 24474572 )
2014
37
Gliomatosis cerebri: a review. ( 24390814 )
2014
38
Gliomatosis cerebri mimicking acute viral encephalitis and with malignant transformation of partial lesions: A case report. ( 25120625 )
2014
39
Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases. ( 23306961 )
2013
40
Letter to the editor: gliomatosis cerebri. ( 23198839 )
2013
41
Bilateral ptosis as initial presentation of gliomatosis cerebri: case report. ( 23427031 )
2013
42
Infiltration of the Optic Chiasm, Nerve, and Disc by Gliomatosis Cerebri. ( 23287703 )
2013
43
Teaching NeuroImages: diffuse cerebral neurosarcoidosis mimicking gliomatosis cerebri. ( 23940026 )
2013
44
18F FDG PET/CT in a child with gliomatosis cerebri. ( 23122744 )
2013
45
Juvenile parkinsonism as an initial manifestation of gliomatosis cerebri. ( 24158273 )
2013
46
Uncal decompression in gliomatosis cerebri. ( 23618524 )
2013
47
Gliomatosis cerebri: clinical characteristics, management, and outcomes. ( 23341100 )
2013
48
Response: gliomatosis cerebri. ( 23495379 )
2013
49
Gliomatosis cerebri. ( 22230515 )
2012
50
IDH1 mutation, a genetic alteration associated with adult gliomatosis cerebri. ( 21481010 )
2012

Variations for Gliomatosis Cerebri

Expression for Gliomatosis Cerebri

Search GEO for disease gene expression data for Gliomatosis Cerebri.

Pathways for Gliomatosis Cerebri

Pathways related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

(show all 39)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.91 EGFR MDM2 PTEN TP53
2
Show member pathways
12.85 EGFR MDM2 PTEN TP53
3
Show member pathways
12.77 EGFR MDM2 PTEN TP53
4
Show member pathways
12.66 EGFR MDM2 PTEN TP53
5 12.62 EGFR MDM2 PTEN TP53
6
Show member pathways
12.55 EGFR MDM2 PTEN TP53
7
Show member pathways
12.5 EGFR MDM2 PTEN TP53
8
Show member pathways
12.31 EGFR PTEN TP53
9
Show member pathways
12.29 EGFR MDM2 TP53
10
Show member pathways
12.26 EGFR MDM2 TP53
11 12.21 EGFR MDM2 PTEN TP53
12
Show member pathways
12.19 MDM2 PTEN TP53
13 12.11 EGFR MDM2 TP53
14
Show member pathways
12.08 MDM2 PTEN TP53
15 11.99 MDM2 PTEN TP53
16
Show member pathways
11.93 EGFR MDM2 PTEN
17
Show member pathways
11.9 EGFR MDM2 PTEN
18
Show member pathways
11.84 MDM2 PTEN TP53
19 11.82 EGFR MDM2 PTEN TP53
20 11.8 EGFR GFAP TP53
21 11.77 MDM2 PTEN TP53
22 11.75 EGFR MDM2 PTEN TP53
23
Show member pathways
11.73 GFAP INA NES
24
Show member pathways
11.72 EGFR GFAP MDM2
25 11.7 MDM2 PTEN TP53
26 11.61 EGFR MDM2 PTEN
27 11.58 EGFR MDM2 PTEN TP53
28 11.5 EGFR MDM2 TP53
29 11.32 MDM2 TP53
30 11.31 MDM2 PTEN TP53
31 11.3 EGFR MDM2 PTEN TP53
32 11.23 PTEN TP53
33 11.17 EGFR TP53
34 11.16 MDM2 TP53
35 11.16 MDM2 PTEN TP53
36 11.13 MDM2 TP53
37 10.97 MDM2 TP53
38
Show member pathways
10.96 MDM2 TP53
39 10.74 EGFR IDH1 PTEN TP53

GO Terms for Gliomatosis Cerebri

Cellular components related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 9.33 GFAP INA NES
2 neurofilament GO:0005883 8.96 GFAP INA
3 intermediate filament cytoskeleton GO:0045111 8.8 GFAP INA NES

Biological processes related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 protein deubiquitination GO:0016579 9.63 MDM2 PTEN TP53
2 negative regulation of apoptotic process GO:0043066 9.56 EGFR MDM2 PTEN TP53
3 protein kinase B signaling GO:0043491 9.49 MDM2 PTEN
4 long-term synaptic potentiation GO:0060291 9.48 GFAP PTEN
5 response to steroid hormone GO:0048545 9.43 IDH1 MDM2
6 response to antibiotic GO:0046677 9.4 MDM2 TP53
7 cellular response to gamma radiation GO:0071480 9.37 MDM2 TP53
8 response to organic cyclic compound GO:0014070 9.33 EGFR IDH1 PTEN
9 positive regulation of protein export from nucleus GO:0046827 9.32 MDM2 TP53
10 cellular response to hypoxia GO:0071456 9.13 MDM2 PTEN TP53
11 cellular response to actinomycin D GO:0072717 8.62 MDM2 TP53

Molecular functions related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 enzyme binding GO:0019899 9.26 EGFR MDM2 PTEN TP53
2 disordered domain specific binding GO:0097718 9.16 MDM2 TP53
3 identical protein binding GO:0042802 9.1 EGFR GFAP IDH1 MDM2 PTEN TP53

Sources for Gliomatosis Cerebri

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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