Paolo Shared "Module 7 Midterm" - 26 Picmonics

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Module 7 Midterm

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
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
Rheumatoid Arthritis Assessment
Mechanism
Destruction of Connective Tissue and Synovial Membrane
Signs and Symptoms
Inflammation of Joints
Morning Stiffness
Nodules
Joint Deformities
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1 min
Rheumatoid Arthritis Interventions
Lifestyle Modifications
Heat and Cold Applications
Therapeutic Exercise
Balance Rest and Activity
Drug Therapy
NSAIDs
DMARDs
Glucocorticoids
Considerations
PT/OT
Reconstructive Joint Surgery
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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
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
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 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
Amyloidosis
Primary
Multiple Myeloma
Secondary
Tuberculosis (TB)
Rheumatoid Arthritis
Congo Red Stain
Apple Green Birefringence
Nephrotic
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2 mins
Granulomatosis With Polyangiitis (Wegeners) Characteristics
Pathophysiology
Granulomatosis With Polyangiitis
Vasculitis
Necrotizing
C ANCA
Lungs And Upper Airway
Crescentic Glomerulonephritis
Treatment
Cyclophosphamide
Corticosteroids
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1 min
Sarcoidosis Symptoms
Black Females
Symptoms
Asymptomatic
Uveitis
Interstitial Fibrosis
Erythema Nodosum
Hepatomegaly
Myocardial Sarcoidosis
Lupus Pernio
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1 min
Sarcoidosis Characteristics and Labs
Mechanism
Unknown Mechanism
Immune-mediated
Noncaseating granulomas
Vitamin D activation
Labs
Hypercalcemia
Elevated ACE
Asteroid bodies
X-Ray shows Bilateral hilar lymphadenopathy
Treatment
Corticosteroids
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1 min
HIV CD4+ 500
Signs and Symptoms
Candida albicans (Oral Thrush)
Kaposi's Sarcoma (HHV-8)
Human Papillomavirus (HPV)
Epstein-Barr Virus (Oral Hairy Leukoplakia)
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3 mins
HIV CD4+ 200
Signs and Symptoms
Herpes Simplex Virus (HSV)
JC Virus
Progressive Multifocal Leukoencephalopathy (PML)
Pneumocystis Pneumonia
(HIV) Dementia
Histoplasmosis
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3 mins
HIV CD4+ 100
Signs and Symptoms
Aspergillus fumigatus
Cryptococcus neoformans
Candida albicans (Esophagitis)
Mycobacterium avium complex (MAC)
Cytomegalovirus (CMV)
Epstein-Barr virus (EBV) B-cell lymphoma
Toxoplasma gondii
Cryptosporidium
Bartonella henselae
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5 mins
Cystic Fibrosis Mechanisms
Autosomal Recessive
CFTR chromosome 7
Cl- channel defect
Decreased chloride secretion
Increased Na and water reabsorption
Increased Na and Cl in sweat
Dehydration of mucous layers
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2 mins
Cystic Fibrosis Diagnosis and Treatment
Diagnosis
Sweat chloride test > 60 mmol/L
Meconium Ileus
Treatment
N-acetylcysteine
Antibiotic Prophylaxis
Pulmonary Maintenance
Lung transplant
Vitamin Replacement
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1 min
Cystic Fibrosis Symptoms and Complications
Recurrent Pulmonary Infections
Chronic Bronchitis
Nasal Polyps
Pancreatic Insufficiency
Intestinal Obstruction
Malabsorption and Diarrhea
Vitamin Deficiencies
Chronic Hepatic Disease
Infertility in Males
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1 min
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
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Pathophysiology
Defective PIG-A Gene
Deficiency of DAF (Decay Accelerating Factor CD55)
Symptoms
Dark Urine in the Morning
Intravascular Hemolysis
Thrombosis
Diagnosis
Decreased CD55 and CD59
Treatment
RBC Transfusion
Eculizumab
Bone Marrow Transplant is Curative
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3 mins

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