James Shared "Block 7" - 36 Picmonics

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Block 7

Sickle Cell Anemia (Mechanism)
Intrinsic Normocytic Hemolytic Anemia
Point Mutation
Autosomal Recessive
African-American
Dehydration or Decreased O2
Newborns Asymptomatic
Heterozygote Malarial Resistance
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2 mins
Sickle Cell Anemia (Signs and Complications)
Signs
Crew-cut on Skull X-ray
Complications
Auto-splenectomy
Painful Crisis
Aplastic Crisis
Splenic-sequestration Crisis
Hyper-hemolytic Syndrome
Renal Papillary Necrosis
Salmonella osteomyelitis
Pulmonary Hypertension
Hyposthenuria
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2 mins
Sickle Cell Anemia (Management)
Long Term Treatment
Hydroxyurea
Bone Marrow Transplant
Folate
Acute Treatment
Exchange Transfusion
Prophylaxis
Penicillin Prophylaxis
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1 min
Beta Thalassemia
Mechanism
Microcytic, Hypochromic Anemia
Mediterranean Populations
Decreased Beta-Globin
Beta-Thalassemia Minor
Increased HbA2
No Intervention
Beta-thalassemia Major
Blood Transfusions
Hemochromatosis
Crew-cut on Skull X-Ray
Diagnosis
Electrophoresis
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2 mins
Hemochromatosis Pathophysiology
Pathophysiology
Hemosiderin Deposition
Autosomal Recessive
HLA-A3
Secondary to Transfusion Therapy
Increase Iron
Increase Ferritin
Increased Transferrin Saturation
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2 mins
Iron Deficiency Anemia
Causes
Malnutrition/Malabsorption
Hemorrhage
Signs
Microcytic, Hypochromic Anemia
Decreased Heme Synthesis
Labs
Decreased Reticulocytes
Decreased Ferritin
Increased Red Cell Distribution Width (RDW)
Poikilocytosis
Anisocytosis
Increased Central Pallor
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2 mins
Ganciclovir
Indications
Cytomegalovirus (CMV)
Immunocompromised
Mechanism
Guanosine Analog
Disrupts DNA Synthesis
Inhibits Viral DNA Polymerase
Side Effects
Hematologic Effects
Nephrotoxicity
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1 min
Cromolyn (Mast Cell Stabilizers)
Mechanism
Mast Cell Stabilizer
Indications
Asthma Prophylaxis
Bronchial Inflammation
Allergic Rhinitis
Side Effects
Cough
Bronchospasm
Consideration
Fixed Schedule Administration
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2 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
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
Cell-mediated Adaptive Immune Response
Characteristics
Macrophages and Dendritic Cells Present Antigens
T Cell Develops Antigen Specificity
Regulatory T Cells Suppress Response
Memory T Cells Cause Faster Secondary Response
Cytotoxic T Cells Lyse Infected Cells
Infected Cells Present Antigen On Surface
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2 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
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
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
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
Humoral Adaptive Immunity
Characteristics
B Cell Binds Free Floating Antigen
B Cell Presents Antigen to Helper T Cell
Helper T Cells Help B Cells Develop Antigen Specificity
Plasma B Cells Secrete Antibodies
Memory B Cells Cause Faster Secondary Response
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2 mins
Hyper IgE Syndrome
Pathophysiology
Job's Syndrome
Abnormal Chemotaxis
Decreased IFN Gamma
Signs and Symptoms
Leonine Facies
Cold Non-Inflamed Staph Aureus Abscesses
Retained Primary Teeth
Eczema
Red Hair
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1 min
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
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
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

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