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132 Cards in this Set
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These kill cells infected with viruses - they don't require an antigen to be presented to them in order to function
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Natural Killer (NK) Cells
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Line blood vessels in organs like the liver and spleen where they capture microorganisms as they bass by in the blood
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Monocytes
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Composed of 30 proteins - function in an ordered and integrated fashion (cascade) to defend agains infection and produce inflammation
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Complement System
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B cells cannot mature into plasma cells
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Common Variable Immunodeficiency (CVID)
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At what age does CVID often manifest
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Often during the second or third decades
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Failure of B-cell precursors to mature into B-lymphocytes and ultimately plasma cells
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X-linked Agammaglobulinemia or Bruton's Disease
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Due to mutation in btk gene that encodes for Bruton tyrosine kinase, required for B-cell development
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XLA or Bruton's Disease
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Most common way kids present with this is th have recurrent bacterial infections with encapsulated pyogenic bacteria such as Pseudomonas, Strep. pneumo, or H. flu
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XLA or Bruton's Disease
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History of recurrent infections involving ears, sinuses, bronchi, and lungs
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CVID
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Infections at or near the surfaces of mucus membranes
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XLA or Bruton's Disease
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Consider in any boy with recurrent or severe bacterial infections, particularly if he has small or absent tonsils and lymph nodes
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XLA or Bruton's Disase
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What are the most common IgG subclass deficiencies in children
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IgG2 and IgG4
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When does IgG subclass deficiency resolve spontaneously
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18 months to 6 years
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IgG1 and IgG3 protect against
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Diphtheria and tetanus
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IgG2 protects against
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Pneumococcus and H. influenzae
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IgG subclass deficiency seen in adults
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IgG3
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Most common Primary Immunodeficiency
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IgA deficiency
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Most common presentation with IgA deficiency
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80% asymptomatic - recurrent sinopulmonary infections most common manifestation
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Autoimmune diseases associated with Selective IgA deficiency (3)
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Rheumatoid arthritis, SLE, Immune Thrombocytopenic Purpura
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Suspect whern there are chronic or recurrent infections, allergies, autoimmune diseases, chronic diarrhea or some combo of these
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Selective IgA deficiency
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Labs show very low IgG levels, IgA may be low, IgM usually normal occurring between 3 and 6 months
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Transient hypogammaglobulinemia of infancy
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3rd and 4th pouch syndrome
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DiGeorge Syndrome
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CATCH 22 with DiGeorge Syndrome
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Cardiac defects, Abnormal facies, Thymic hypoplasia, Cleft palate, Hypocalcemia
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Most patients with this have a deletion on chromosome number 22
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DiGeorge Syndrome
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Most common cardiac defect with DiGeorge Syndrome
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Tetrology of Fallot
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This analysis has been used to make the diagnosis of DiGeorge syndrome mroe precise and more common
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FISH (Fluorescent in Situ Hybridization)
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Usually the determining factor with how well someone will do with DiGeorge Syndrome
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The severity of the heart defect
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T-cell deficient response to candida - 50% have endocrine abnormalities
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Chronic Mucocutaneous Candidiasis
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The most serious Primary Immunodeficiency - combined humoral and cellular dysfunction
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Severe Combined Immunodeficiency
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illnesses involved with SCID (4)
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Pneumonia, Sepsis, Meningitis, Thrush
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An average of 1500 lymphocytes
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Seen in SCID- no T-cells
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What is the best age to get a bone marrow transplant
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Given in the first 3 months of life have a 96% success rate
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Which vaccine should family members of someone with SCID not get
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OPV - oral polio vaccine
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Condition characterized by neurologic abnormalities resulting in an unsteady gait with dilated blood vessels of the eyes and skin
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Ataxia Telangiectasia
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Generally the first presenting symptom with Ataxia Telangiectasia
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Ataxia
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Later neurological symptoms of A-T
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Nystagmus, Oculomotor apraxia, and dysarthria
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A-T patients are at increased risk of these cancers
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Leukemia and Lymphoma
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95% of patients with this have elevated levels of serum alpha-1-fetoprotein
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Ataxia Telangiectasia
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A boy with the triad of Thrombocytopenia, Eczema, and Susceptibility to bacterial infections
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Wiskott-Aldrich Syndrome
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Only disorder where small platelets are found and are diagnostic
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Wiskott-Aldrich Syndrome
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The absence of this enzyme leads to an accumulation of toxic metabolic by-products within lymphocytes that cause the cells to die
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Adenosine Deaminase deficiency (ADA)
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What is associated with ADA deficiency
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Chondro-osseous dysplasia
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Skin boils, severe episodes of pneumonia, and very high serum IgE levels
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Hyper IgE syndrome (Job syndrome)
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Abnormality characterized by abnormalities related to the immune system, bones, and connective tissue
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Hyper IgE syndrome
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Many patients with this condition and pneumonia deny feeling sick
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Hyper IgE syndrome
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Inability to switch immunoglobulin production to IgG, IgA, or IgE
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Hyper IgM syndrome
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Impaired neutrophil migration and adhesion - lack of pus formation
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Leukocyte Adhesion Deficiency (LAD)
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Delayed separation of the umbilical cord with omphalitis
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Leukocyte Adhesion Deficiency
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Inability of phagocytic cells to make hydrogen peroxide
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Chronic Granulomatous Disease (CGD)
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Associated with an excessive accumulation of immune cells into aggregates
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Chronic Granulomatous Disease (CGD)
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Most common presentation of CGD in infancy
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Skin or bone infections with Serratia marcescens
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Most accurate test for CGD - measures the production of hydrogen peroxide in phagocytic cells
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Dihydrorodamine flow cytometry
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A visual test of CGD in which phagocytes producing oxidants turn blue and are scored manually usuing a microscope
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Nitroblue tetrazolium reduction assay
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Suspect with someone who has Aspergillis, Nocardia, Burkholderia cepacia pneumonia; staph liver abscess; bone infection with Serratia marcescens
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Chronic Granulomatous Disease (CGD)
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Most common prophylactic antibiotic used with CGD
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Trimethoprim-sulfamethoxazole
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Suspect if history of recurrent or overwhelming infection with meningococci
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Complement Deficiency
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Tests the integrity of the whole complement cascade
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CH50 assay
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See hereditary angioedema with this deficiency
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C1 inhibitor deficiency
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Most common form of anemia in children
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Iron Deficiency Anemia
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Common cause of iron deficiency in young childrend
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Drinking too much cow's milk
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Can get this if infants are fed only goat's milk
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Folate deficiency
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Can get this if breast feeding mother is a strict vegetarian
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B12 deficiency
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Mentzer index < 13
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Thalassemia
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Mentzer index > 13
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Iron Deficiency
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An overload of iron
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Hemosiderosis (Hemochromatosis)
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Most common intial symtom of Sickle cell disease
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Dactylitis
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"Fifth disease," slapped cheek appearance; aplastic crisis
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Human parvovirus
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How do you treat Diamond-Blackfan anemia
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Oral corticosteroids
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Triphalangeal thumbs
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Diamond-Blackfan anemia
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Slowly developing anemia characterized by gradual onset of pallor - often healthier than expected given low hemoglobin level
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Transient Erythroblastemia of childhood
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Low MCV, high RDW
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Iron Deficiency Anemia
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Low MCV, normal RDW
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Alpha and beta thalassemia
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MCV normal, RDW high
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Congenital hemolytic anemia
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Tear drop cells
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Bone marrow failure
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Target cells
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Hemoglobinopathy, liver disease
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Nucleated RBCs
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Thalassemia trait or disease
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Blister, bite or burr cells
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G-6PD deficiency
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Bizarre RBC shapes
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RBC membrane defects, thalassemia
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Physiologic anemia of infancy reached when; in premies?
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Full term at 2-3 months; premies at 4-7 months
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Lab test to distinguish between fetal and maternal red blood cells
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Apt test
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Lab test for fetal cells in Mom's circulation
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Kleihauer-Betke test
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3 processes of anemia in the neonate
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Hemorrhagic (most common), hemolytic, hypoplastic
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Develops in extremely jaundiced infants, especially those with severe Rh incompatibility; characterized initially by the loss of the Moro (startle) reflex, poor feeding, and decreased activity
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Kernicterus
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Where do red thrombi tend to occur?
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In the atria (atrial fibrillation) and veins (DVT)
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Where do white thrombi tend to occur?
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Arteries - due to damaged vessel wall
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Do you use fibrinolytics with a STEMI?
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Yes - otherwise you will die
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Do you use fibrinolytics with atrial fibrillation?
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No!
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Do you use fibrinolytics with a NSTEMI?
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No!
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Enoxaparin
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Low molecular weight heparin
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Dalteparin
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Low molecular weight heparin
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Ardeparin
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Low molecular weight heparin
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Tinzaparin
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Low molecular weight heparin
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How does Unfractionated Heparin work?
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It acclerates the reaction between Antithrombin and Thrombin, Factor Xa
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Why does Low molecular weight heparin not work on thrombin?
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You need at least 18 saccharide units; not big enough
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How is unfractionated heparin usually administered?
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IV - LMWH usually administered Subcutaneously
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The anticoagulant of choice during pregnancy?
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Heparin
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Immune response to heparin; delayed, sustained, severe
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Type II Heparin Induced Thrombocytopenia (HIT)
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What triggers the immune response in Type II HIT?
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Platelet factor 4/heparin complex
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Adverse effect of being on heparin for 3-6 months
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Osteoporosis
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Antidote for Heparin in case of life threatening bleeding
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Protamine sulfate
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Synthetic pentasaccharide that is a selective factor Xa inhibitor
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Fondaparinux
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When is Fondaparinux contraindicated?
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With severe renal impairment
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Drugs to use with an acute pulmonary embolism or DVT?
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Fondaparinux with warfarin
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Anticoagulant isolated from the salivary glands of a leech
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Hirudin
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IV Drugs like Hirudin (Thrombin inhibitors)
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Lepirudin, Bivalirudin
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FDA approved oral alternative to warfarin
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Dabigatran - Thrombin inhibitor
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Subcutaneous Thrombin Inhibitor
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Desirudin
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Use this instead if patient has HIT with heparin usage
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Lepirudin (thrombin inhibitor)
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Use this instead if patient has HIT while undergoing percutaneous coronary intervention (PCI)
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Bivalirudin (thrombin inhibitor)
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Use this for prophylaxis of deep vein thrombosis in patients undergoing hip replacement
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Desirudin
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Use this oral thrombin inhibitor with atrial fibrillation
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Dabigatran
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How does Warfarin work?
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It is a Vitamin K antagonist thus inhibits Factors II, VII, IX, and X in addition to Protein C and S
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Why does it take so long to get a peak anticoagulant effect of warfarin?
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Long half life of preexisting clotting factors
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Release of cholesterol microemboli from atheromatous plaques causing Purple Toe syndrome
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Adverse effect of Warfarin
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What dose of Aspirin should one use?
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Small; < 160-325
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Streptokinase
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Thrombolytic
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Acylated complex of human plasminogen and bacterial streptokinase
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Anistreplase
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Alteplase
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Recombinant t-PA; thrombolytic
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Reteplase
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Recombinant t-PA; thrombolytic
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Tenecteplase
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Recombinant t-PA; thrombolytic
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Ticlopidine, Clopidogrel, Prasugrel
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ADP Receptor Antagonists
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Abciximab, Eptifibatide, Tirofiban
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GP IIb/IIIa Receptor Antagonists
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Which deficiency causes nerve demyelination and damage?
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Vitamin B12
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Two drugs that can be used to treat Chronic Iron Toxicity
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Deferoxamine (IM or IV) or Deferasirox (oral)
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Borrelia burgdorferi
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Lyme Disease
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Smallest of the spirochetes
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Leptospira
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Prevent by avoiding contact with animal urine
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Leptospira
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Tick that is the vector for Rickettsia rickettsii
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Dermacentor (dog tick)
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Tick that is the vector for Anaplasma phagocytophilia (and Borrelia)
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Ixodes (deer tick)
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Tick that is the vector for Ehrlichia chafeensis
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Amblyomma (lone-star tick)
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Anaplasma phagocytophilum
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Human Granulocytic anaplasmosis
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Ehrlichia chafeensis
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Human Monocytic ehrlichiosis
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