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Textbook of Systemic Vasculitis Aman Sharma

SECTION 1: INTRODUCTION
CHAPTER 1:Epidemiology of Systemic Vasculitis
- INTRODUCTION
- LARGE-VESSEL VASCULITIS
  - Giant Cell Arteritis
  - Takayasu's Arteritis
- MEDIUM-VESSEL VASCULITIS
  - Kawasaki's Disease
    - Other Factors
  - Polyarteritis Nodosa
    - Ethnic Differences
- SMALL-VESSEL VASCULITIS ANCA-ASSOCIATED VASCULITIS
- SMALL-VESSEL VASCULITIS-IMMUNE COMPLEX
CHAPTER 2:Genetics of Vasculitis
- INTRODUCTION
- LARGE-VESSEL VASCULITIS
- MEDIUM-VESSEL VASCULITIS
  - Kawasaki Disease
  - Polyarteritis Nodosa
- SMALL-VESSEL VASCULITIS
  - ANCA Associated Vasculitis
  - Henoch-Schönlein Purpura
CHAPTER 3:Classification of Vasculitis
- AMERICAN COLLEGE OF RHEUMATOLOGY CRITERIA
- CHAPEL HILL CONSENSUS CONFERENCE NOMENCLATURE SYSTEM
- EUROPEAN MEDICAL AGENCY ALGORITHM
- CLASSIFICATION OF CHILDHOOD VASCULITIS
SECTION 2: BASIC SCIENCES
CHAPTER 4:Neutrophils in ANCA-associated Vasculitis
- INTRODUCTION
- THE ANCA ANTIGENS PR3 AND MPO: KEYS IN THE PATHOPHYSIOLOGY OF ANCA-ASSOCIATED VASCULITIDES
- NEUTROPHIL ACTIVATION IN ANCA-ASSOCIATED VASCULITIDES: ROLE OF ANCA
  - Animal Models to Study the Pathogenicity of ANCA In Vivo
- CONCLUSION
CHAPTER 5:Antineutrophil Cytoplasmic Antibodies
- ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES
- ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS
- PRODUCTION OF ANCA
- INTERACTION BETWEEN ANCAS AND THEIR TARGET ANTIGENS
- PATHOPHYSIOLOGY OF AAV
- GENETIC ASSOCIATION OF AAV
- ENVIRONMENTAL FACTORS
- PATHOGENIC B-CELL RESPONSE AND PRODUCTION OF ANCA
- ABERRANT T-CELL RESPONSE AND GRANULOMA FORMATION
- MONOCYTE ACTIVATION AND AAV
- ANCA-MEDIATED INTERACTION BETWEEN NEUTROPHILS AND ENDOTHELIAL CELLS
- ANCA IN THE DIAGNOSIS OF VASCULITIS
- SHOULD ANCA TITERS MAY BE USED FOR THE MANAGEMENT OF ANCA-VASCULITIS?
CHAPTER 6:Animal Models of ANCA Vasculitis
- INTRODUCTION
- PASSIVE TRANSFER MODEL
- BONE MARROW TRANSPLANT MODEL
- MOUSE MODEL OF AUTOIMMUNE VASCULITIS
- RAT MODEL OF EXPERIMENTAL AUTOIMMUNE VASCULITIS
- RODENT MODELS OF PR3-ANCA DISEASE
- INSIGHTS FROM ANIMAL MODELS
- CONCLUSION
CHAPTER 7:Pathogenesis of Vasculitis
- INTRODUCTION
- CLASSIFICATION AND NOMENCLATURE
- PATHOGENESIS OF LARGE-VESSEL VASCULITIS
  - Giant Cell Arteritis
    - Pathology
    - Pathogenesis
  - Takayasu's Arteritis
    - Pathology
    - Pathogenesis
- PATHOGENESIS OF MEDIUM-VESSEL VASCULITIS
  - Polyarteritis Nodosa
    - Pathology
    - Pathogenesis
  - Kawasaki Disease
    - Pathology
    - Pathogenesis
- PATHOGENESIS OF IMMUNE-COMPLEX-MEDIATED SMALL-VESSEL VASCULITIS
  - Pathology
  - Pathogenesis
- ANCA-ASSOCIATED SMALL VESSEL VASCULITIS
  - Pathology
  - Pathogenesis
    - Genes and Environment
CHAPTER 8:Pathology of Cutaneous Vasculitis
- INTRODUCTION
- LARGE-VESSEL VASCULITIS
- MEDIUM-VESSEL VASCULITIS
- SMALL-VESSEL VASCULITIS
  - Leukocytoclastic Vasculitis
  - Granulomatous Vasculitis
    - ANCA-Associated Vasculitis
    - Others
CHAPTER 9:Pathology of Pulmonary Vasculitis
- INTRODUCTION
- PRIMARY VASCULITIDES
- SECONDARY VASCULITIDES
  - Infection Associated Vasculitis
CHAPTER 10:Pathology of Renal Vasculitis
- SMALL-VESSEL VASCULITIS
- MEDIUM-VESSEL VASCULITIS
- LARGE-VESSEL VASCULITIS
  - Takayasu Arteritis (Figs 10.10 to 10.14)
    - Gross Pathology 3
    - Light Microscopy
  - Giant Cell Arteritis
CHAPTER 11:Pathology of Central Nervous System and Peripheral Nerves
- NONINFECTIOUS CENTRAL NERVOUS SYSTEM VASCULITIDES
  - Takayasu's Arteritis
  - Giant Cell Arteritis
- PRIMARY ANGIITIS OF CENTRAL NERVOUS SYSTEM (PACNS)
- CENTRAL NERVOUS SYSTEM VASCULITIS DUE TO INFECTIONS
SECTION 3: IMAGING MODALITIES
CHAPTER 12:Imaging in Vasculitis
- INTRODUCTION
- ULTRASONOGRAPHY
- COMPUTED TOMOGRAPHY
- MAGNETIC RESONANCE IMAGING
- DIGITAL SUBTRACTION ANGIOGRAPHY
- IMAGING: WHAT TO LOOK FOR?
- LARGE-VESSEL VASCULITIS
  - Takayasu's Arteritis
  - Giant Cell Arteritis
- MEDIUM-VESSEL VASCULITIS
  - Polyarteritis Nodosa
- KAWASAKI DISEASE
- SMALL-VESSEL VASCULITIS
  - Rheumatoid Vasculitis
  - Systemic Lupus Vasculitis
- VARIABLE-VESSEL VASCULITIS
  - Behçet's Disease Vasculitis
  - Cogan's Syndrome Vasculitis
- SINGLE-ORGAN VASCULITIS
- VASCULITIS ASSOCIATED WITH SYSTEMIC DISEASE
- INFECTION-RELATED VASCULITIS
CHAPTER 13:Positron Emission Tomography and Vasculitides
- PRINCIPLES OF PET IMAGING
- 18F-FDG PET/CT IN VASCULITIDES
- FUTURE PROMISING TECHNICAL IMPROVEMENTS
  - Novel Radiotracers
  - Combined PET-MR
- ACKNOWLEDGMENTS
SECTION 4: CLINICAL MANIFESTATIONS ACCORDING TO ORGAN INVOLVEMENT
CHAPTER 14:Cutaneous Vasculitis
- INTRODUCTION
- EPIDEMIOLOGY
- CLASSIFICATION AND NOMENCLATURE OF VASCULITIS
- PATHOGENESIS
  - Pathogenesis of Pauci Immune SVV
  - Pathogenesis of Immune Complex Vasculitis
- APPROACH TO A PATIENT WITH VASCULITIS (FLOW CHART 14.1)
- CUTANEOUS LEUKOCYTOCLASTIC ANGIITIS (CLA, TABLE 14.3)
- IgA VASCULITIS
- URTICARIAL VASCULITIS
- CRYOGLOBULINEMIC VASCULITIS
- ANCA-ASSOCIATED VASCULITIS
- POLYARTERITIS NODOSA AND CUTANEOUS ARTERITIS
- TREATMENT
CHAPTER 15:Retinal Vasculitis
- INTRODUCTION
  - Primary Retinal Vasculitis
  - Secondary Retinal Vasculitis
- CLINICAL FEATURES
- DIAGNOSTIC WORK-UP
- TUBERCULOSIS
- HERPES VIRUSES
- CYTOMEGALOVIRUS
- TOXOPLASMOSIS
- SYPHILIS
- BEHÇET’S DISEASE
- SARCOIDOSIS
- SYSTEMIC VASCULITIS GRANULOMATOSIS WITH POLYANGIITIS (WEGENER’S GRANULOMATOSIS), POLYARTERITIS NODOSA, AND TAKAYASU’S ARTERITIS
- FROSTED BRANCH ANGIITIS
  - Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis
  - Complications of Retinal Vasculitis
CHAPTER 16:Cardiac Manifestations
- INTRODUCTION
- PATHOGENESIS
- SPECIFIC VASCULITIS
  - Giant Cell Arteritis
- POLYARTERITIS NODOSA
- GRANULOMATOSIS WITH POLYANGIITIS (WEGENER’S)
- EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS SYNDROME)
- MICROSCOPIC POLYANGIITIS
- CRYOGLOBULINEMIC VASCULITIS
- KAWASAKI’S DISEASE
- TAKAYASU ARTERITIS
- BEHÇET’S DISEASE
CHAPTER 17:Pulmonary Manifestations
- INTRODUCTION
- CLINICAL MANIFESTATIONS
- GRANULOMATOSIS WITH POLYANGIITIS (WEGENER’S)
  - Clinical Features
  - Diagnosis
- BEHÇET’S DISEASE
- EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG–STRAUSS SYNDROME)
- CONNECTIVE TISSUE DISORDERS
- MISCELLANEOUS VASCULITIDES WITH PULMONARY MANIFESTATIONS
- PULMONARY RENAL SYNDROME
- MANAGEMENT OF PULMONARY VASCULITIS
CHAPTER 18:Renal Manifestations
- INTRODUCTION
- LARGE-VESSEL VASCULITIS
  - Takayasu Arteritis
  - Giant Cell Arteritis
- MEDIUM-VESSEL VASCULITIS
  - Polyarteritis Nodosa
- SMALL-VESSEL VASCULITIS
  - ANCA-Associated Vasculitis
- OTHER VASCULITIS
CHAPTER 19:Neurological Manifestations
- INTRODUCTION
- CENTRAL NERVOUS SYSTEM SYNDROMES
- PERIPHERAL NERVOUS SYSTEM SYNDROMES
CHAPTER 20:Vasculitic Neuropathy
- INTRODUCTION
- CLASSIFICATION
- EPIDEMIOLOGY
- PATHOGENESIS AND PATHOLOGY
- CLINICAL FEATURES
- TYPES OF VASCULITIC NEUROPATHIES
- DIAGNOSIS
- DIFFERENTIAL DIAGNOSIS
- MANAGEMENT
- CONCLUSION
CHAPTER 21:Gastrointestinal Manifestations
- INTRODUCTION
- GASTROINTESTINAL TRACT INVOLVEMENT IN LARGE-VESSEL VASCULITIS
  - Takayasu Arteritis
  - Giant Cell Arteritis
- GASTROINTESTINAL TRACT INVOLVEMENT IN MEDIUM-VESSEL VASCULITIS
  - Polyarteritis Nodosa (Table 21.1)
  - Kawasaki's Disease
- GASTROINTESTINAL TRACT INVOLVEMENT IN SMALL-VESSEL VASCULITIS
- IgA VASCULITIS (HENOCH-SCHÖNLEIN PURPURA)
- VASCULITIS ASSOCIATED WITH SYSTEMIC DISEASE
- BEHÇET’S DISEASE VASCULITIS
- LOCALIZED VASCULITIS OF GASTROINTESTINAL TRACT
CHAPTER 22:Head and Neck Manifestations
- INTRODUCTION
- OTOLARYNGOLOGIST PERSPECTIVE
- HEAD AND NECK MANIFESTATIONS IN GPA
- OTOLOGIC MANIFESTATION
- RHINOLOGIC MANIFESTATIONS
- OTOLARYNGOLOGIST’S ROLE
- LARYNGEAL AND TRACHEAL MANIFESTATION
- ORAL MANIFESTATIONS
  - Salivary Gland Involvement
- DIFFERENTIAL DIAGNOSIS
- EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS VASCULITIS)
- KAWASAKI DISEASE
- POLYARTERITIS NODOSA
- GIANT CELL ARTERITIS
- HEAD AND NECK MANIFESTATIONS
SECTION 5: PRINCIPLES OF MANAGEMENT
CHAPTER 23:Approach to Childhood Vasculitis
- INTRODUCTION
- PREVALENCE
- CLINICAL FEATURES
CHAPTER 24:Approach to Adult with Systemic Vasculitis
- INTRODUCTION
CHAPTER 25:Assessment of Disease Activity and Damage
- INTRODUCTION
- TOOLS FOR ASSESSMENT
  - Histopathology
- RADIOLOGICAL ASSESSMENT
- COMPUTERIZED TOMOGRAPHY
- ULTRASONOGRAPHY
- POSITRON EMISSION TOMOGRAPHY
- SEROLOGICAL MARKERS IN DISEASE ASSESSMENT
- CLINICAL INDICES OF DISEASE ASSESSMENT
- DISEASE ACTIVITY
- INSTRUMENTS FOR ASSESSING DISEASE ACTIVITY
- DAMAGE
- VASCULITIS DAMAGE INDEX
- QUALITY OF LIFE ASSESSMENT
- PATIENT REPORTED OUTCOMES
CHAPTER 26:Newer and Future Treatment of Vasculitis
- INTRODUCTION
- CURRENT OUTCOMES
- NEWER THERAPIES
- OTHER FORMS OF VASCULITIS
  - Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg Strauss Syndrome)
  - Large Vessel Vasculitis
- FUTURE THERAPIES FOR VASCULITIS
- CONCLUSION
- ACKNOWLEDGMENT
- DISCLOSURES
SECTION 6: INDIVIDUAL VASCULITIS SYNDROMES
CHAPTER 27:Giant Cell Arteritis and Polymyalgia Rheumatica
- INTRODUCTION
  - Polymyalgia Rheumatica
  - Giant Cell Arteritis
- EPIDEMIOLOGY
  - Polymyalgia Rheumatica
  - Giant Cell Arteritis
- DIAGNOSIS
  - Polymyalgia Rheumatica
  - Giant Cell Arteritis
- CLINICAL FEATURES
  - Polymyalgia Rheumatica
  - Giant Cell Arteritis
- INVESTIGATIONS
  - Polymyalgia Rheumatica
  - Giant Cell Arteritis
- DIAGNOSTIC APPROACH
  - Polymyalgia Rheumatica
  - Giant Cell Arteritis
- MANAGEMENT
  - Polymyalgia Rheumatica
  - Giant Cell Arteritis
CHAPTER 28:Takayasu's Arteritis
- INTRODUCTION
- HISTORY
- EPIDEMIOLOGY
- CLASSIFICATION CRITERIA AND DIAGNOSIS
- PATHOLOGY
- PATHOGENESIS
- CLINICAL FEATURES
- DIAGNOSIS
- IMAGING
- DIFFERENTIAL DIAGNOSIS
- TREATMENT
- CORTICOSTEROIDS
- CONVENTIONAL IMMUNOSUPPRESSIVES
- DEFINITION OF REFRACTORY DISEASE IN TA
- BIOLOGIC AGENTS
- ENDOVASCULAR AND SURGICAL INTERVENTIONS
- DISEASE ACTIVITY ASSESSMENT AND MONITORING
- FUTURE DIRECTIONS
CHAPTER 29:Kawasaki Disease
- INTRODUCTION
- EPIDEMIOLOGY
- ETIOPATHOGENESIS
- CLINICAL FEATURES
- CLINICAL CRITERIA FOR THE DIAGNOSIS OF KD
- INVESTIGATIONS
- INCOMPLETE AND ATYPICAL KD
- DIFFERENTIAL DIAGNOSIS
- TREATMENT
- CONCLUSION
CHAPTER 30:Polyarteritis Nodosa
- INTRODUCTION
- CLASSIFICATION CRITERIA AND DIAGNOSIS
- EPIDEMIOLOGY
- ETIOLOGY AND PRECIPITATING FACTORS
  - HBV-related PAN
  - Other Etiologies
- HISTOLOGY
- CLINICAL FEATURES
- LABORATORY TESTS
- ANGIOGRAPHY
- OUTCOME AND PROGNOSIS OF PAN
  - Deaths
- TREATMENT OF PAN
  - Vasculitis Severity
- HOW SHOULD PAN BE TREATED?
CHAPTER 31:Granulomatosis with Polyangiitis (Wegener's Granulomatosis)
- INTRODUCTION
- CLASSIFICATION AND NOMENCLATURE
- EPIDEMIOLOGY
- PATHOGENESIS
- CLINICAL FEATURES
- UPPER AIRWAYS AND NOSE
- ORAL MANIFESTATIONS
- PULMONARY INVOLVEMENT
- RENAL MANIFESTATIONS
- FIBEROPTIC ENDOSCOPY AND BRONCHOSCOPY
- MEASUREMENT OF DISEASE ACTIVITY
- MORBIDITY
CHAPTER 32:Microscopic Polyangiitis
- INTRODUCTION
  - Laboratory Investigations
- EPIDEMIOLOGY
- DIAGNOSIS
- CLINICAL FEATURES
- INVESTIGATIONS
- DIAGNOSTIC APPROACH
- MANAGEMENT
- PROGNOSIS
CHAPTER 33:Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
- INTRODUCTION
- EPIDEMIOLOGY
- PATHOGENESIS
- DIAGNOSIS
- CLINICAL FEATURES
  - Investigations
  - Diagnostic Approach
- MANAGEMENT
- CONCLUSION
CHAPTER 34:Immunoglobulin A Vasculitis
- INTRODUCTION
CHAPTER 35:Primary Angiitis of Central Nervous System
- INTRODUCTION
- IMAGING
- BIOPSY AND PATHOLOGY
- MIMICS
- DIAGNOSING PACNS
- TREATMENT
CHAPTER 36:Behçet's Disease
- INTRODUCTION
- EPIDEMIOLOGY
- DIAGNOSIS
- CLINICAL FEATURES
- INVESTIGATIONS
- DIAGNOSTIC APPROACH
- MANAGEMENT
  - Mucocutaneous and Musculoskeletal Involvement
  - Major Organ Disease
CHAPTER 37:Cryoglobulinemic Vasculitis
- INTRODUCTION
- EPIDEMIOLOGY
- DIAGNOSIS
- CLINICAL FEATURES
- DIAGNOSTIC APPROACH
- MANAGEMENT
- THERAPEUTIC GUIDELINES FOR HCV-MC
  - Follow-up
- CONCLUSION
CHAPTER 38:Relapsing Polychondritis
- INTRODUCTION
- EPIDEMIOLOGY
- ETIOPATHOGENESIS
- CLINICAL FEATURES
- AURICULAR AND VESTIBULAR INVOLVEMENT
- JOINT INVOLVEMENT
- NASAL INVOLVEMENT
- RESPIRATORY TRACT INVOLVEMENT
- CARDIOVASCULAR INVOLVEMENT
- OCULAR INVOLVEMENT
- NEUROLOGIC INVOLVEMENT
- RENAL INVOLVEMENT
- DERMATOLOGICAL INVOLVEMENT
- OTHER SYMPTOMS
- PROGNOSIS
- ASSOCIATED CONDITIONS
- DIAGNOSTIC CRITERIA
- DIFFERENTIAL DIAGNOSIS
- INVESTIGATIONS
- ASSESSMENT OF DISEASE ACTIVITY
- MANAGEMENT
- MANAGEMENT OF COMPLICATIONS AND SEQUELAE
CHAPTER 39:IgG4 Disease
- INTRODUCTION
  - How Concept of IgG4-RSD Had Evolved?
- EPIDEMIOLOGY
- PATHOGENESIS
- DIAGNOSIS
- HISTOPATHOLOGICAL FEATURES OF IGG4-RELATED DISEASE
- QUANTITATIVE ASSESSMENT OF THE IGG4 STAIN
- IMAGE CHARACTERISTICS SUGGESTIVE OF IGG4-RELATED DISEASE ON 18 F-FDG PET/CT
  - Clinical Features
    - Synchronous Lesions
    - Metachronous Lesions
  - Diagnostic Approach
    - Comprehensive Clinical Diagnostic Criteria for IgG4-RD
- PANCREAS
- AORTITIS
  - Pituitary Gland and Dura Mater Involvement
    - Infundibulohypophysitis (Inflammation of the Pituitary Gland)
    - IgG4-related Pachymeningitis (Inflammation of the Dura Mater)
- PERIPHERAL NERVES
- CONCLUSION
CHAPTER 40:Infection-associated Vasculitis
- INTRODUCTION
- VIRAL INFECTIONS AND VASCULITIS
- HIV-ASSOCIATED VASCULITIS
- PARVOVIRUS B19 ASSOCIATED VASCULITIS
- CYTOMEGALOVIRUS
- EPSTEIN-BARR VIRUS
- VARICELLA-ZOSTER VIRUS
- BACTERIAL INFECTIONS AND VASCULITIS
CHAPTER 41:Vasculitis Mimics
- INTRODUCTION
- WHEN TO SUSPECT VASCULITIC MIMICS?
- INFECTIONS
- DISORDERS OF THE CONNECTIVE TISSUE MATRIX
- DRUG-INDUCED VASCULITIS
- MALIGNANCY-ASSOCIATED VASCULITIS
- ATHEROTHROMBOTIC VASCULAR DISORDERS
- MISCELLANEOUS
- DIAGNOSTIC APPROACH AND MANAGEMENT
- CONCLUSION
CHAPTER 42:Secondary Vasculitis
- INTRODUCTION
- RHEUMATOID VASCULITIS
- LUPUS VASCULITIS
- PARANEOPLASTIC VASCULITIS
- INFECTION-ASSOCIATED VASCULITIS
  - Management
- CONCLUSION
CHAPTER 43:Cogan's Syndrome
- INTRODUCTION
- EPIDEMIOLOGY
  - Typical Cogan's Syndrome
  - Atypical Cogan's Syndrome
- CLINICAL FEATURES
- OCULAR MANIFESTATIONS
- AUDIOVESTIBULAR MANIFESTATIONS
- SYSTEMIC MANIFESTATIONS
- DIAGNOSTIC APPROACH AND INVESTIGATIONS
- TREATMENT
CHAPTER 44:Malignancy-associated Vasculitis
- INTRODUCTION
- ASSOCIATION OF VASCULITIS AND MALIGNANCY
- RISK FACTORS AND PATHOGENESIS OF MALIGNANCY-ASSOCIATED VASCULITIS
- CLINICAL PRESENTATION AND PROGNOSIS
- SPECIFIC MALIGNANCY-ASSOCIATED VASCULITIS
- ANCA-ASSOCIATED VASCULITIS AND MALIGNANCY
- CONCLUSION: WHEN AND HOW TO RULE OUT UNDERLYING MALIGNANCY IN A PATIENT OF VASCULITIS?
CHAPTER 45:Pregnancy and Vasculitis
- INTRODUCTION
- FERTILITY CONCERNS
- CONCEPTION AND PRECONCEPTION COUNSELING
- PREGNANCY OUTCOMES
- LARGE-VESSEL VASCULITIS
  - Takayasu's Arteritis
  - Giant Cell Arteritis
- MEDIUM-VESSEL VASCULITIS
  - Polyarteritis Nodosa
  - Kawasaki Disease
- SMALL-VESSEL VASCULITIS
- VARIABLE-VESSEL VASCULITIS
CHAPTER 46:Prevention and Management of Infections
- INTRODUCTION
- DEFINING IMMUNOCOMPROMISED HOST
- COMMON MEDICATIONS IN THE TREATMENT OF VASCULITIS THAT CAUSE IMMUNOSUPPRESSION
- PREVENTION OF INFECTIONS IN THE PATIENT TREATED FOR VASCULITIS
- TREATMENT OF VASCULITIS IN SPECIFIC INFECTIONS
- CLINICAL FEATURES AND EVALUATION OF INFECTIONS IN AN IMMUNOCOMPROMISED HOST
- MANAGEMENT

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