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me Shared "Immuno-L" - 32 Picmonics

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Immuno-L

Chronic Granulomatous Disease
Pathophysiology
Most Commonly X-linked Recessive
Defective NADPH Oxidase (Enzyme Complex)
Decreased Hydrogen Peroxide
Impaired Intracellular Killing in Phagocytes
Signs and Symptoms
Recurrent Purulent Skin and Lung Infections
Catalase Positive Organisms
Diagnosis
Negative Nitroblue Tetrazolium Test
DHR (Dihydrorhodamine) Flow Cytometry
Treatment
Antimicrobial Prophylaxis
TMP-SMX
Itraconazole
Interferon-Gamma
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3 mins
Chediak-Higashi Syndrome
Pathophysiology
Autosomal Recessive
Defective Lysosomal Trafficking Regulator Gene (LYST)
Defect In Microtubular Function
Decrease In Phagocytosis
Signs And Symptoms
Recurrent Pyogenic Infections
Staph Aureus And Streptococci
Partial Albinism
Peripheral Neuropathy
Pancytopenia
Diagnosis
Giant Granules In Granulocytes And Platelets
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2 mins
Leukocyte Adhesion Deficiency
Pathophysiology
Autosomal Recessive
Integrin CD18 Protein
Defect in LFA-1
Attached to ICAM
Signs and Symptoms
Neutrophilia
Bacterial Infections
Non-Pyogenic
Delayed Separation of Umbilical Cord
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2 mins
Wiskott Aldrich
Pathophysiology
X-linked Recessive
WASp Protein Mutation
Antigen Presentation Defect
Progressive Deletion of B and T Cells
Signs and Symptoms
Thrombocytopenia
Eczema
Infections
Decreased IgM
Increased IgE, IgA
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2 mins
Hyper IgM Syndrome
Pathophysiology
Deficient CD40 Ligand on Helper T-cells
Defective CD40 Receptor on B-cells
Inability to Class Switch
Signs and Symptoms
Pyogenic Abscess
Decreased IgG, IgA, IgE
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4 mins
Ataxia Telangiectasia
Pathophysiology
Autosomal Recessive
ATM Gene
Defect in DNA repair
Signs & Symptoms
Cerebellar defects
Spider Angiomas
Sensitive to Radiation
Defective Tumor Suppressor Gene
Increased risk of Cancer, Leukemias, and Lymphomas
Increased AFP levels
IgA deficiency
Repeated sinopulmonary infections
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2 mins
Severe Combined Immunodeficiency (SCID)
Both B and T Cell Deficiency
Recurrent Viral, Bacterial, Fungal, Protozoal Infections
Defective Interleukin (IL) 2 Receptor
X-Linked
Adenosine Deaminase Deficiency
Failure to Synthesize MHC II Antigens
Treat with Bone Marrow Transplant
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2 mins
Chronic Mucocutaneous Candidiasis
Mechanism and Characteristics
T Cell Dysfunction
Candida Albicans
Signs and Symptoms
Infections of Skin
Infections of Mucous Membrane
Susceptible to HPV Infections like Condyloma Acuminatum
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42 secs
Hyper IgE Syndrome
Job Syndrome (Autosomal Dominant Hyper IgE Syndrome)
Pathophysiology
Autosomal Dominant
STAT3 Mutation
Increased IgE
Abnormal Chemotaxis
Signs and Symptoms
Leonine Facies
Cold (Non-Inflamed) Staph Aureus Abscesses
Retained Primary Teeth
Eczema
Management
Prophylactic Antibiotics
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2 mins
IL-12 Receptor Deficiency
Pathophysiology
IL-12 Secreted by Macrophages
Decreased Th1 Response
Decreased IFN Gamma
Signs and Symptoms
Mycobacterial Infections
Salmonella
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1 min
Thymic Aplasia (DiGeorge Syndrome)
Pathophysiology
DiGeorge Syndrome
22q11 Deletion
Failure to Develop Third and Fourth Pharyngeal Pouches
Signs and Symptoms
Undeveloped Thymus
T Cell Deficiency
Recurrent Viral and Fungal Infections
Undeveloped Parathyroids
Hypocalcemia
Tetany
Aortic Defects
Congenital Heart Defects
Diagnosis
Absent Thymic Shadow on CXR
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2 mins
Common Variable Immunodeficiency
Pathophysiology
Defect in B Cell Maturation
Decreased Plasma Cells
Decreased Immunoglobulins
Signs & Symptoms
Sinopulmonary Infections
Increased Risk of Autoimmune Disease
Lymphoma
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1 min
Selective IgA Deficiency
Pathophysiology
Defect in Class Switching
Decreased IgA
Signs and Symptoms
Often Asymptomatic
Increase in Atopic & Autoimmune Diseases
Sinopulmonary Infections
Increased Risk Giardia Lamblia Infections
Inflammatory Bowel Disease
Celiac Disease
Considerations
Anaphylaxis From IgA Containing Blood Products
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2 mins
Bruton's Agammaglobulinemia
Pathophysiology
X-linked Recessive
Defective Bruton Tyrosine Kinase
Blocks B Cell Maturation
Signs & Symptoms
Decreased Immunoglobulins of All Classes
Bacterial Infections After Six Months
Maternal IgG Protects Before Six Months
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2 mins
Hypersensitivity Type IV
Delayed-Type Hypersensitivity
T Cell Mediated
Antibody Independant
Macrophages Activated
Examples
Transplant Rejection
PPD Skin Test
Contact Dermatitis
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1 min
Hypersensitivity Type III
Immune Complex
Antigen-antibody (IgG) complex activates complement
Neutrophils release lysozomal enzymes
Examples
Arthus Reaction
Serum Sickness
Systemic Lupus Erythematosus (SLE)
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1 min
Hypersensitivity Type II
Cytotoxic
IgM or IgG antibody-mediated
Membrane Attack Complex (MAC)
Mechanism
Opsonization
Complement Activation
NK Cells
Testing
Direct Coombs test
Indirect Coombs Test
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1 min
Hypersensitivity Type I
Previous Antigen Exposure
Immediate Reaction
IgE Cross-linking
Presensitized Mast Cells
Histamine Release
Vasodilation
Anaphylaxis
Allergies
Treatment
Epinephrine
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1 min
Hashimoto's Thyroiditis
Symptoms
Hypothyroidism with Bouts of Hyperthyroidism
Enlarged, Nontender Thyroid
Mechanism
Autoimmune
Anti-Thyroid Peroxidase (TPO)
Anti-Thyroglobulin
Lab Findings
Hurthle Cells
Lymphoid Follicles
Associations
HLA-DR5 and HLA-DR3
Increased Risk Of Non-Hodgkin Lymphoma
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2 mins
Pemphigus Vulgaris
Pathophysiology
Autoantibodies against desmosomes
Symptoms
Flaccid Bullae on Skin
Oral Mucosa Involvement
Intraepidermal Acantholysis
Fluid Loss
Infection
Nikolsky Positive
Diagnosis
Biopsy showing Autoantibodies
Treatment
IVIG and Plasmapheresis
Oral Steroids
Immunosuppressants
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2 mins

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