Ken Shared "UGC Picmaster Challenge" - 8 Picmonics

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UGC Picmaster Challenge

Li-Fraumeni's
Pathophysiology
Li-Fraumeni
autosomal dominant
TP53
Clinical
SBLA cancer syndrome
Sarcoma
Breast cancer
Leukemia
Adrenal tumors
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45 secs
Familial Hyperchylomicronemia (Type I)
Autosomal-Recessive
Lipoprotein Lipase Deficiency
ApoCII Deficiency
Elevated Chylomicrons
Complications
Acute Pancreatitis
Pruritic Xanthomas
Hepatosplenomegaly
Diagnosis
Creamy Supernatant
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Waldenstrom Macroglobulinemia
B-cell lymphoma with monoclonal IgM production
Generalized lymphadenopathy
Increased serum proteins with M spike (comprised of IgM)
Bleeding - viscous serum results in defective platelet aggregation
Cold Agglutinin Autoimmune Hemolytic Anemia (C-AIHA)
Raynaud phenomenon
Visual and neurologic deficits (e.g. retinal hemorrhage or stroke) - IgM (large pentamer) causes serum hyperciscosity
Treatment
Acute complications: plasmapheresis (removes IgM from the serum)
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1 min
Potter Sequence (Syndrome)
PATHOGENESIS
Oligohydramnios
clinical findings
Limb Deformities
Facial Anomalies
Pulmonary Hypoplasia Most Common Cause of Death
CAUSES
Autosomal Recessive Polycystic Kidney Disease (ARPKD)
Posterior Urethral Valves
Chronic Placental Insufficiency
Bilateral Renal Agenesis
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3 mins
Class II Anti-arrhythmic (Beta-blockers)
Indication
Supraventricular tachycardia
Ventricular rate control for atrial fibrillation and atrial flutter
Side Effects
COPD and Asthma Exacerbation
Impotence
Adverse CNS effects
Cardiovascular Effects of Beta-Blockers
Mask Hypoglycemia
Drug specific side effects
Propranolol
Vasospasm in Prinzmetal's Angina
Alpha-1-agonism if given alone for Pheochromocytoma and Cocaine
Metoprolol
Dyslipidemia
Overdose
Saline Infusion
Atropine
Glucagon
Mechanism of Action
Decreases SA and AV node activity (phase 4 depolarization)
Contraindicated in Wolf-Parkinson-White
Decreases mortality in CHF
Hypotension, Bradycardia, Heart Block, Bronchospasm (SE)
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Dubin Johnson Syndrome (with Audio)
Pathophysiology
defective hepatocyte secretion in to proper canal
Autosomal recessive- "hereditary conjugated hyperbilirubinemia"
Absent MRP2 (multidrug resistant protein)
Error is inside the hepatocyte.... NOT due to a clogged bile duct
Signs/symptoms
Liver changes from Brown to Black
Jaundiced (otherwise asymptomatic)
labs
Conjugated hyperbilirubinemia
Treatment
NO excess urinary coproporphyrins
no treatment necessary (benign disease)
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6 mins
Familial Adenomatous Polyposis
Pathophysiology
Autosomal Dominant
APC
Chromosome 5
Management
Begin screening at 10-12 years old
Prophylactic removal of colon
Gardner Syndrome
Gardner syndrome
Osteomas
Dental Abnormalities
Soft Tissue Tumors
Congenital hypertrophy of retinal pigment epithelium
Turcot Syndrome
Turcot Syndrome
Medulloblastoma
CNS tumor
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2 mins
Dantrolene
Complications
Hepatotoxicity
Excessive Tears
Tachycardia
Uticaria
Drowsy
Vision Changes
Muscle Weakness
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