Melinda Shared "Q1 Biochemistry I Exam 5" - 37 Picmonics

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Q1 Biochemistry I Exam 5

Acute Intermittent Porphyria (AIP)
Pathophysiology
Autosomal Dominant
Inhibited Porphobilinogen Deaminase
Increased Serum Porphobilinogen
Increased Serum Delta-ALA
Increased Urinary Porphyrins
Signs & Symptoms
Precipitated By P450 Inducers
Painful Abdomen
Polyneuropathy
Psychological Disturbances
Port Wine-Colored Urine
Treatment
Glucose and Hemin
Considerations
Avoid Triggers
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Porphyria Cutanea Tarda (PCT)
Pathophysiology
Inhibited Uroporphyrinogen Decarboxylase (UROD)
Most Common Porphyria
20% Autosomal Dominant
80% Sporadic Type
Hepatitis C Association
Signs & Symptoms
Photosensitivity
Blistering of Skin
Tea Colored Urine
Diagnosis
Increased Uroporphyrins (Urinary & Serum)
Treatment
Avoid Sunlight
Phlebotomy
Hydroxychloroquine (Low Dose)
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Aspirin
Indications
Fever
Pain and Headache
Cardiovascular Disease (CAD)
Mechanism of Action
Irreversible inhibition of COX-1 and COX-2
Suppress Thromboxanes and Prostaglandins
NSAID
Side Effects
Reye's Syndrome
Tinnitus
Peptic Ulceration
Mixed Acidosis/Alkalosis
Nephropathy
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Ibuprofen (Advil, Motrin)
Mechanisms
NSAID
COX Inhibitor
Indications
General Pain
Fever
Inflammatory Conditions
Side Effects
GI Ulcers
Nephrotoxicity
Rash
Considerations
Avoid with Alcohol
Risk of Thrombotic Event
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Acetaminophen
Indications
Fever
Pain
Mechanism of Action
Reversibly inhibits COX
Acts primarily in CNS
Side Effect
Hepatic Necrosis
N-acetylcysteine Antidote
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Prednisone (Glucocorticoids)
Mechanism
Glucocorticoid
Indications
Inflammatory Conditions
Organ Transplant Rejection
Side Effects
Cataracts and Glaucoma
Cushing's Syndrome
Osteoporosis
Immunosuppression
Hyperglycemia
Ulcers
Considerations
Adrenal Insufficiency
Taper Gradually
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Montelukast (Singulair)
Mechanism
Leukotriene Receptor Blocker
Indications
Allergic Rhinitis
Asthma Prophylaxis
Exercise Induced Bronchospasm
Side Effects
Fatigue
Headache
Mood Changes
Suicidal Thoughts
Considerations
Increased Risk of Churg Strauss
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Von Willebrand Disease
Mixed platelet and coagulation disorder
Mechanism
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)
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1 min
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
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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
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Immune/Idiopathic Thrombocytopenic Purpura (ITP)
Anti-GPIIb/IIIa or Anti-GPIb/IX Antibodies (IgG)
Splenic MO phagocytose Ag-Ab complexes --> Splenomegaly
Acute: Children, post-viral
Elevated Thrombopoietin (TPO)
Sx: Easy Bruising/Bleeding, Petechiae, Epistaxis, Gingival Hemorrhage
Dx: Increased Megakaryocytes (BMBx), Large Platelets, Thrombocytopenia, Nml. PT & PTT
Tx: Steroids, Plt Infusions, IVIG | Rituximab, Splenectomy, TPOR Agonists
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Glanzmann Thrombasthenia
Pathophysiology
Autosomal Recessive or Acquired as an Autoimmune Disorder
Deficiency of Glycoprotein IIb/IIIa (Gp IIb/IIIa) Receptor
Defective Platelet-to-Platelet Aggregation
Symptoms
Mucous Membrane Bleeding (Gingival Bleeding)
Easy Bruising
Increased Bleeding Time
Normal Platelet Count
Considerations
Avoid NSAIDs
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Alteplase
Mechanism
Tissue Plasminogen Activator
Indications
Thrombosis
Side Effects
Bleeding
Contraindications
Intracranial Hemorrhage
Internal Hemorrhaging
Considerations
Minimize Bleeding
Monitor for Shock
Antidote
Aminocaproic Acid
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Heparin Mechanisms
Mechanism of Action
Short Half-life
Antithrombin Activation Cofactor
Decrease Thrombin, Factor Xa
Side Effects
Bleeding
Heparin Induced Thrombocytopenia (HIT)
Antidote
Protamine Sulfate
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Heparin Indications
Indications
Acute Coronary Syndrome (ACS)
Prophylaxis
Pulmonary Embolism
Atrial Fibrillation
Used during Pregnancy
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Heparin Induced Thrombocytopenia (HIT)
Pathophysiology
5-10 Days After Heparin Exposure
Heparin-Platelet Factor 4 Complex
Autoantibodies Form Against Complex
Heparin-Platelet Factor 4-Antibody Complex Binds Platelets
Platelet Aggregation
Procoagulant Release
Symptoms
Thrombocytopenia
Diagnosis
Serotonin Release Assay (SRA)
Treatment
Stop Heparin, Start Direct Thrombin Inhibitor
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Warfarin
Mechanism of Action
Interferes with Vitamin K-dependent Clotting Factors
Extrinsic Pathway
Bridge with Heparin
Indications
Chronic Anticoagulation
Side Effects
Bleeding
Necrosis
Cytochrome P450
Contraindications
Pregnancy
Antidote
FFP + Vitamin K
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Hemophilia A, B, C
X-linked Recessive
A: Low Factor 8
Hemarthrosis, Intramuscular Hematoma, Intracerebral Hemorrhage
Mgmt: DDAVP + aminocaproic acid (anti-fibrinolytic)
B: Low Factor 9
C: Low Factor 11 | Autosomal Recessive
Increased PTT (Intrinsic) ONLY
Complications: Hemosiderin deposition, fibrosis
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Crigler-Najjar Syndrome Type 1
Pathophysiology
Autosomal Recessive
Absent UDP-glucuronosyltransferase
Symptoms
Neonatal Jaundice
Kernicterus
Diagnosis
Normal Liver Function Tests
Elevated Unconjugated Bilirubin
Treatment
Phototherapy
Plasmapheresis
Calcium Phosphate and Orlistat
Death often by Age 2
Considerations
No Response to Phenobarbital
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3 mins
Gilbert's Syndrome
Pathophysiology
Decreased UDP-Glucuronosyltransferase Activity
Decreased Bilirubin Conjugation
Signs and Symptoms
Often Asymptomatic
Jaundice (Mild)
Fasting
Stress
Alcohol Intake
Labs
Increased Unconjugated Bilirubin
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