Pics 2: hemoglobinopathy Flashcards by Victoria DeVito

Q

Thalassemia summary and map

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Alpha thal slide
silent carrier
trait

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HbH disease slide

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Hydrops fetalis slide

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HbE summary

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Hb Lepore summary

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Hb Constant spring summary

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Thalassmias and Hbopathy summary slide overview

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Q

HbH disease
symptoms
genotypes

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Q

Hydrops fetalis
Pathophys of manifestations

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Alpha thal
Genotype:phenotype associations

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B thal major: pathophys and clinical features

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B thal genotype:phenotype assocations

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Q

TDT vs NTDT full body complications

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Iron chelator agents
Effects on iron overload/organs
side effects/monitoring

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Q

Schematic of thalassmias and their classifications

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Thalassemia: newer therapies and their MOA

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Gene therapies in Thalassemia

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Q

Summary table for thalassemias vs hemoglobinopathies

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Q

Hb electrophoresis and HPLC examples

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Sickle cell trait and SCD phenotypes

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SCD complications by spectrum of hemolysis/endothelial dysfunction to viscosity/vasoocclusion

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Pathophys of RBC sickling

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Management algorithm for suspected acute chest syndrome

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Sickle cell disease full body complications
acute and chronic

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Mgmt of TIA or ischemic stroke in sickle cell disease
by timeline of presentation

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Peds: TCD and stroke prevention
primary vs secondary
trials

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Acute sickle hepatopathies: mgmt

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General overview of SCD treatments

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Benefits of HU in SCD by physiology

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HU therapy toxicities

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Acute emergency transfusion indications
Simple vs RCE

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Chronic transfusions
Targets
Indications for primary and secondary prevention

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Transfusion around elective surgery
Low/Int/High risk surgery

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Hemoglobin structure and globins, location of production over lifespan

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Spectrum of thalassemias by transfusion dependence

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Overview of SCD stuff to know

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Thalassemia: Classification TDT or NTDT and management

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Complications TDT vs NTDT chart/table Ash SAP

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Pathophys flowchart of beta thal

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Iron chelators table ASH SAP

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Q

Lab findings various Sickle Cell disease syndromes
severity, Hb fractions, Hb level and MCV

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Important clinical trials in SCD

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Acute exacerbation of anemia in SCD
Differential Dx

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Q

Immunizations for patients with SCD

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