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BIIC
Hemolytic Uremic Syndrome (HUS)
Children
Pathophysiology
Preceded by Acute Diarrheal Illness
Endothelial Damage
Classic Triad of Symptoms
Microangiopathic Hemolytic Anemia (MAHA)
Thrombocytopenia
Renal Failure
Labs
Helmet cells
LDH
Treatment
Dialysis
Supportive
2 mins
Thrombotic Thrombocytopenic Purpura (TTP)
Inhibition or deficiency of ADAMTS 13
vWF Multimers are Not Degraded
Microthrombi (and Emboli)
Symptoms
FAT RN Pentad
Fever
Anemia
Thrombocytopenia
Renal Dysfunction
Neurologic Abnormalities (Altered Mental status)
Treatment
Plasmapheresis
Corticosteroids
Splenectomy
2 mins
Disseminated Intravascular Coagulation (DIC)
Pathophysiology
Bleeding State
Activation of Clotting Factors
Deficiency of Clotting Factors
Causes
Sepsis
Trauma
Obstetric Complications
Acute Pancreatitis
Malignancy
Nephrotic Syndrome
Transfusion
2 mins
Bernard-Soulier Syndrome
Mechanism
Autosomal Recessive
Deficiency of Glycoprotein Ib (GpIb) Receptor
Symptoms
Mucosal Membrane Bleeding
Menorrhagia
Easy Bruising
Labs
Large Platelets
Increased Bleeding Time
No Platelet Agglutination with Ristocetin
3 mins
Bleeding Time Lab Values
2-7 Minutes
41 secs
Glanzmann Thrombasthenia
Pathophysiology
Autosomal Recessive or Acquired as an Autoimmune Disorder
Deficiency of Glycoprotein IIb/IIIa (Gp IIb/IIIa) Receptor
Autosomal Dominant Condition Leading to Reduced vWF
Defect in platelet plug formation
Labs
Increased Bleeding Time
PTT increased
Factor VIII decreased
Normal platelet count
Treatment
DDAVP (synthetic vasopressin)
1 min
Protein C or S Deficiency
Pathophysiology
Autosomal Dominant
Inability to Inactivate Factor Va and VIIIa
Symptoms
Hypercoagulable State
Recurrent DVTs or DVTs at Young Age
Treatment
Begin Heparin
Slowly Bridge to Warfarin
Hemorrhagic Skin Necrosis
2 mins
Polycythemia Vera Disease
Pathophysiology
JAK2 Mutation leading to increased red blood cells
Tyrosine Kinase
Symptoms
Erythromelalgia
Pruritus
Often After Hot Shower
Headache
Treatment
Phlebotomy
Aspirin
1 min
Polycythemia Vera Labs
Lab Findings (The 4 H's)
Hypervolemia
Histaminemia
Hyperviscosity
Hyperuricemia
Diagnosis
Elevated Hemoglobin or Hematocrit
Positive Jak2 Mutation
1 min
Factor V Leiden Thrombophilia
Pathophysiology
Most Common Genetic Clotting Disorder in Caucasians
Genetic Point Mutation
Glutamine Replaces Arginine
Mutant Factor V
Resistant to Degradation by Activated Protein C
Symptoms
Hypercoagulability
Recurrent DVT (Increased Risk of Thromboembolism)
Considerations
Avoid Oral Contraceptives
Caution During Pregnancy
2 mins
Systemic Lupus Erythematosus (SLE) Diagnosis and Clinical Features
IM DAMN SHARP acronym
Immunoglobulins
Malar rash
Discoid rash
Antinuclear antibody
Mucositis
Neurologic disorders
Serositis
Hematologic disorders
Arthritis
Renal disorders
Photosensitivity
2 mins
Systemic Lupus Erythematosus (SLE) Mechanism, Treatment and Complications
Mechanism and Characteristics
Systemic Autoimmune Disease
9:1 female to male ratio
Treatment
Glucocorticoids
NSAIDS
Hydroxychloroquine
Cyclophosphamide
Complications
Lupus nephritis
Libman-Sacks Endocarditis
Secondary Antiphospholipid Antibody Syndrome
Other
Drug-induced lupus
2 mins
Stages of Hypothermia
Mild
32 to 35°C (90 to 95°F)
Shivering
Moderate
28 to 32°C (82 to 90°F)
Obvious Motor Impairment
Slowed Thinking
Severe
< 28°C (< 82°F)
Shivering Stops
Paradoxical Undressing
Arrhythmias
3 mins
Hypothermia Interventions
Remove From Environment
Remove Wet Clothing
Passive Rewarming
Warm Clothing
Active Rewarming
Heated Blankets
Warm IV Solutions
Heated Oxygen
Warm Gastric Lavage
Considerations
Warm Trunk BEFORE Extremities
2 mins
Primary Immune Thrombocytopenia
Characteristics
Following Infection or Vaccination
Antiplatelet Antibodies
Clinical Features
Often Asymptomatic
Bleeding
Petechiae
Diagnosis
Thrombocytopenia
Increased Megakaryocytes
Management
Corticosteroids
Intravenous Immunoglobulin (IVIG)
Rituximab
Splenectomy
4 mins
Antiphospholipid Syndrome
Characteristics
Antiphospholipid Antibodies
Hypercoagulability
Clinical Features
Recurrent Thromboembolisms
Recurrent Miscarriages
Diagnosis
Anticardiolipin Antibodies
Anti-Beta-2-Glycoprotein Antibodies
Lupus Anticoagulant
Management
Anticoagulation
2 mins
Deep Vein Thrombosis (DVT) Characteristics
Mechanism
Virchow's Triad
Venous Stasis
Endothelial Damage
Hypercoagulability
Symptoms
Tenderness
Homan's Sign
Warmth
Redness
Swelling
Asymptomatic
1 min
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