Moyamoya

UMLS: C0026654
Basic Information
Severity Level:
Severe
Based on 10 similar ADEs (max similarity: 0.471), includes 10 severe case(s)
Definition:
A noninflammatory, progressive occlusion of the intracranial CAROTID ARTERIES and the formation of netlike collateral arteries arising from the CIRCLE OF WILLIS. Cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. It is characterized by endothelial HYPERPLASIA and FIBROSIS with thickening of arterial walls. This disease primarily affects children but can also occur in adults.
UMLS ID:
C0026654
MeSH ID:
Synonyms:
Moyamoya
Moyamoya Disease
Moyamoya Disease
Moyamoya Disease
Progressive Intracranial Arterial Occlusion
Progressive Intracranial Arterial Occlusion Syndrome
Disease (Or Disorder); Moyamoya
Disease Moya Moya
Disease Moyamoya
Moya Disease
Moya Moya Disease
Moya-Moya Disease
Classification Hierarchy
MeSH Tree Number breakdown for C0026654
C10
Nervous System Diseases
Category: Nervous System Diseases Parent: C (Diseases) Level: 0
C10.228
Central Nervous System Diseases
Category: Nervous System Diseases Parent: C10 Level: 1
C10.228.140
Brain Diseases
Category: Nervous System Diseases Parent: C10.228 Level: 2
C10.228.140.300
Cerebrovascular Disorders
Category: Nervous System Diseases Parent: C10.228.140 Level: 3
C10.228.140.300.200
Carotid Artery Diseases
Category: Nervous System Diseases Parent: C10.228.140.300 Level: 3
C10.228.140.300.200.600
Moyamoya Disease
Category: Nervous System Diseases Parent: C10.228.140.300.200 Level: 4 Tree Number: C10.228.140.300.200.600
C10
Nervous System Diseases
Category: Nervous System Diseases Parent: C (Diseases) Level: 0
C10.228
Central Nervous System Diseases
Category: Nervous System Diseases Parent: C10 Level: 1
C10.228.140
Brain Diseases
Category: Nervous System Diseases Parent: C10.228 Level: 2
C10.228.140.300
Cerebrovascular Disorders
Category: Nervous System Diseases Parent: C10.228.140 Level: 3
C10.228.140.300.510
Intracranial Arterial Diseases
Category: Nervous System Diseases Parent: C10.228.140.300 Level: 3
C10.228.140.300.510.200
Cerebral Arterial Diseases
Category: Nervous System Diseases Parent: C10.228.140.300.510 Level: 4
C10.228.140.300.510.200.737
Moyamoya Disease
Category: Nervous System Diseases Parent: C10.228.140.300.510.200 Level: 5 Tree Number: C10.228.140.300.510.200.737
C14
Cardiovascular Diseases
Category: Cardiovascular Diseases Parent: C (Diseases) Level: 0
C14.907
Vascular Diseases
Category: Cardiovascular Diseases Parent: C14 Level: 1
C14.907.137
Arterial Occlusive Diseases
Category: Cardiovascular Diseases Parent: C14.907 Level: 2
C14.907.137.615
Moyamoya Disease
Category: Cardiovascular Diseases Parent: C14.907.137 Level: 3 Tree Number: C14.907.137.615
C14
Cardiovascular Diseases
Category: Cardiovascular Diseases Parent: C (Diseases) Level: 0
C14.907
Vascular Diseases
Category: Cardiovascular Diseases Parent: C14 Level: 1
C14.907.253
Cerebrovascular Disorders
Category: Cardiovascular Diseases Parent: C14.907 Level: 2 Tree Number: C14.907.253
C14
Cardiovascular Diseases
Category: Cardiovascular Diseases Parent: C (Diseases) Level: 0
C14.907
Vascular Diseases
Category: Cardiovascular Diseases Parent: C14 Level: 1
C14.907.253
Cerebrovascular Disorders
Category: Cardiovascular Diseases Parent: C14.907 Level: 2 Tree Number: C14.907.253
ADE Molecular Mechanism Mapping

Drug-ADE-Human Protein Triplets

Molecular mechanism triplets showing drug-adverse event-protein relationships

0 Total Triplets
0 High Confidence
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Personalized Drug-Protein-ADE Network

Comprehensive Information for Personalized ADE

Comprehensive Information and Reliability for Drug-ADE Association

Drug Confidence Filter
DTA Confidence Level
Layout Style
Show Labels
Total Drugs
58
2 High | 11 Medium | 45 Low Confidence
Total Proteins
0
Target proteins associated with this ADE
Drug-Protein-ADE association
0
0 Known | 0 High | 0 Middle | 0 Low Confidence
Confidence Profile
0%
Known/High/Middle-confidence interactions

This interactive network visualization shows the relationships between the top 50 drugs (ranked by case number) out of 58 total drugs associated with this adverse event, their known and potential off-target proteins, and the associated adverse effect. The connections indicate binding interactions and mechanistic pathways.

Adverse Drug Event (ADE)
Drug
Protein
Drug-Center
Human Protein-Center
confidence level

Drug Confidence Level Distribution

Drug Type Distribution

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Pharmacological Target Class Distribution

No main class data available

Top 8 Function Target Sub Classes

No sub class data available

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The Introduction of Drug-AE Confidence Score Grade
Priority Level Score Range Description Evidence Characteristics
High Score ≥ 6.67
High-priority associations requiring immediate attention.
Strong evidence across multiple dimensions with high statistical significance, robust association measures, and substantial case numbers. Warrant priority pharmacovigilance action. High P-value scores (significant Adjust-P), Strong ROR/PRR values, Substantial case numbers, Complete dimensional coverage, Minimal data filtering needed.
Medium 3.33 ≤ Score < 6.67
Medium-priority associations requiring careful evaluation.
Moderate evidence quality with acceptable statistical significance and association strength. May need additional validation or monitoring before definitive action. Moderate P-value significance, Reasonable ROR/PRR measures, Adequate case numbers, Partial dimensional coverage, Some data quality considerations.
Low Score < 3.33
Low-priority associations with limited evidence strength.
May represent weak signals, insufficient data, or associations requiring extensive additional investigation before any regulatory or clinical consideration. Weak P-value significance, Low association measures, Limited case numbers, Incomplete dimensional data, Significant data filtering applied.
PersDTA overview
The Introduction of Drug-AE Severity Score Grade
Severity Level Score Range Description Typical outcomes
Minimal 0 ≤ Score ≤ 0.387
Low severity ADRs requiring minimal intervention.
Events that need medical attention to prevent permanent damage but without hospitalization or life-threatening consequences. Primarily RI (Required Intervention) outcomes, Outpatient management, Preventive measures, Early intervention prevents escalation.
Mild 0.387 < Score ≤ 0.861
Mild severity events requiring hospitalization.
Mix of intervention-required and hospitalization cases with manageable clinical outcomes and good recovery potential. Mix of RI and HO outcomes, Initial or prolonged hospitalization, Active medical management, Generally favorable prognosis.
Moderate 0.861 < Score ≤ 1.500
Moderate ADRs resulting in permanent disability.
Higher proportion of hospitalization and disability cases requiring intensive medical management and long-term care planning. Increased HO and DS outcomes, Permanent disability, Extended hospitalization, Rehabilitation required, Long-term functional impairment.
Severe 1.500 < Score ≤ 2.52
High severity life-threatening events with significant morbidity.
Notable presence of disability and life-threatening outcomes requiring emergency intervention and intensive care management. Prominent DS and LT outcomes, Life-threatening events, Emergency intervention required, ICU admission, High risk of permanent consequences.
Critical 2.524 < Score ≤ 5.000
Critical life-threatening or fatal ADRs with maximum clinical impact.
High proportion of life-threatening events and deaths with very strong drug-event associations requiring immediate regulatory action. Significant LT and DE outcomes, Fatal events, Emergency life-support measures, Immediate drug discontinuation, Regulatory safety alerts.
The Introduction of Drug-AE-Protein Confidence Score Grade
Confidence Level Relationships Description Evidence Types
Known/Valid Direct DTA relationships The drug-ADE, drug-target, and target-ADE associations are all derived from the same literature source. Experimental validation, Clinical evidence
High DT + TA + DA Two of the three associations (drug-ADE, drug-target, and target-ADE) are derived from the same literature source, while the third is validated by other literature. Multiple corroborating sources
Medium DT + TA + DA Two of the three associations (drug-ADE, drug-target, and target-ADE) are validated by separate literature sources. Partial evidence chain
Low TA + DT Only target-ADE and drug-target associations are validated by literature sources. Associative evidence only
Personalized ADE

Route and Formulation-specified Associations

Pharmaceutic Granularity for Route and Formulation specification

Administration Routes (Total: 68 cases across 6 routes)

Dosage Forms (Total: 51 cases across 6 forms)

Drug Route Analysis
Drug Form Analysis

Drug Route Analysis

Route Drug Name Case Number Confidence Score Confidence Level Severity Score Severity Level Details
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Drug Form Analysis

Form Drug Name Case Number Confidence Score Confidence Level Severity Score Severity Level Details
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Disease-stratified Associations

Personalized Granularity for Disease and Indication Stratification

Indication Name
Drug Name
Case Number
Confidence Score
Confidence Level
Severity Score
Severity Level
Details