Andrew Shared "CMBL Week 8" - 11 Picmonics
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CMBL Week 8
Osteogenesis Imperfecta
- Pathophysiology
- Autosomal Dominant
- Decreased Type I Collagen Production
- Symptoms
- Phenotypically Diverse
- Brittle Bone Disease
- Fractures from Minimal Trauma
- Confused with Child Abuse
- Hearing Loss
- Dental Imperfections
- Blue Sclera
1 min
Hurler Syndrome
- Pathophysiology
- Autosomal Recessive
- Mucopolysaccharidoses
- Alpha-L-Iduronidase Deficiency
- Increased Heparan Sulfate
- Increased Dermatan Sulfate
- Signs and Symptoms
- Hepatosplenomegaly
- Developmental Delay
- Gargoylism
- Corneal Clouding
- Airway Obstruction
2 mins
Hunter Syndrome
- Pathophysiology
- X-linked Recessive
- Mucopolysaccharidoses
- Iduronate Sulfatase Deficiency
- Increased Dermatan Sulfate
- Increased Heparan Sulfate
- Signs and Symptoms
- No Corneal Clouding
- Aggressive Behavior
1 min
Low Blood Pressure Response
- Characteristics
- Posterior Pituitary Releases Vasopressin
- Vasoconstriction
- Adrenal Cortex Releases Aldosterone
- Increased Na+ and H2O Reabsorption
1 min
High Blood Pressure Response
- Characteristics
- Atria Release Atrial Natriuretic Peptide
- Vasodilation
- Antagonizes Aldosterone
- Na+ and Water Excreted
1 min
Anion Gap Metabolic Acidosis
- Pathophysiology
- Increased Anion Gap
- MUDPILES
- Methanol
- Uremia
- Diabetic Ketoacidosis (DKA)
- Propylene Glycol
- Isoniazid or Iron
- Lactic Acid
- Ethylene Glycol
- Salicylates
1 min
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)
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
3 mins
Coagulation Cascade Overview
- Secondary Hemostasis
- Intrinsic Pathway
- Partial Thromboplastin Time (PTT)
- Heparin
- Extrinsic Pathway
- Prothrombin Time (PT)
- Warfarin
- Common Pathway
- Thrombin
- Convert Fibrinogen to Fibrin
- Stabilizes the Platelet Plug
2 mins
Clotting Overview
- Characteristics
- Endothelial Cells are Damaged
- Platelets Respond to Exposed Collagen and Stick
- Platelets Release Recruiting Factors
- More Platelets are Attracted
- Soluble Fibrinogen Turns to Insoluble Fibrin
- Fibrin Seals the Clot
- Clot Dissolves
- Liver Produces Clotting Factors
2 mins
Marfan Syndrome
- Mechanism
- Fibrillin-1 Mutation
- Symptoms
- Autosomal Dominant
- Tall
- Arachnodactyly
- Pectus Excavatum
- Hypermobile Joints
- Aortic Aneurysm And Dissection
- Mitral Valve Prolapse (MVP)
- Subluxation of Lens (Superior)
2 mins
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