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10 Cards in this Set

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Temporal Arteritis (Giant Cell ateritis) Overview, symptoms, findings and tx
Most common vasculitis and affects large and medium arteries, usually branches of carotid artery. Focal granulomatous inflamation. Affects elderly females
Symptoms: Unilateral headache, jaw claudication, impaired vision. (occlusion of opthalmic artery)
Findings: Associated with an increase ESR. Half of patients have systemic involvement and polymyalgia rhematica.
Tx: High-dose steroids.
Takayasu's arteritis
Affects medium and large arteries
Known as "pulseless disease"- granulomatous thickening of aortic arch and/or proximal great vessels.
Associated w/ increased ESR.
Primarily effects Asian females < 40 yrs old.
Symptoms: Fever, Arthritis, Night sweats, Myalgia, Skin nodules, Ocular disturbances, Weak pulses in upper extremities. (FAN MY SKIN On Wednesday.)
Wegener's granulomatosis
Overview, Symptoms, Findings, and tx
Affects small vessels
Characterized by triad of focal necrotzing vasculitis, necrotizing granulomas in the lungs and upper airway, and necrotizing glomerulonephritis.
Symptoms: Perforation of nasal septum, chronic sinuitis, otitis media, mastoiditis, cough, dyspnea, hemoptysis, hematuria
Findings: c-ANCA is a strong marker of disease; chest x-ray may reveal large nodular densities; hematuria and red cell cast
Tx: Cyclophoshamide and corticosteroids
Churg-Strauss syndrome
Effects small vessles
Granulomatous vasculitis w/eosinophilia. Involves lung, heart, skin, kidneys, nerves. Often seen atopic patients. p-ANCA.
Encephalopaphy:overview, gen. ss, various causes and types
A diffuse disease of the brain that affect brain function or structure.
General SS: main feature is altered mental state, nystagmus, myoclonus, tremor, fatigue, seizures. Symptoms vary w/the severity and type of encephalopathy
Causes: main causes are liver disease, kidney disease and lack of O2 to brain. Also maybe caused by certain drugs, infections, metabolism disturbances, brain tumors
Types: Hepatic, hypertensive, Wernike's, hypoxic, Uremic, etc
Hepatic Encephalopathy
SS: Disturbances in consciousness (beh. changes, stupor, confusion, coma)
Asterixis, Seizures, limb rigidity, hypereflexia and electroencephalogram changes.
Path: Loss of hepatocellular function and blood shunting. Alter metabolic milieu bathes CNS. Excessive amonia may be cause of damage. Little morphological change in the brain.
Hypertensive Encephalopathy
SS: Rapidly evolving severe htn. Diffuse cerebral dysfunction: headaches, nausea, confusion, convulsions which sometimes lead to coma.
Focal neurological signs are common.
Path: Associated w/malignant htn (diastolic > 130 and systolic >200)
Muscular dystrophy: overview and types
Inherited disorder characterized by progressive muscular weakness and wasting w/subsequent replacement by fibrous and fatty connective tissue
Often begins in childhood.
It is a mutation of the gene coding for dystrophin. Dystrophin connects the cytoskeleton of muscle fibers to the ecm thru the cell membrane
Types: Duchennes, Beckers, Mytonic, Emery-Dreifuss dystrophy, Lymb-girdle MD
Duchenne's MD: Overview and histo, SS and P
Most common (1/35000 males)
May be inherited in a recessive X linked fashion or aquired.
SS: Usually normal at birth, weak by age 5, in wheel-chair by 10-12 and die in early 20's.
Weakness begins in pelvic girdle and extends to shoulder girdle. Enlarged calves. Positive Gowers' sign.
Pathological changes in heart. Some cognitive impairment
Histo: Variation in myofiber size, Increased # of internalized nuclei, degeneration, necrosis and phagocytosis of muscle fibers, proliferation of endomysial connective tissue. Almost no to no dystrophin found in mucles.
Path: Frameshift or point mutations/deletion of Xp21:gene encoding for the dystrophin protein
Beckers MD
Same histo as Duchenne's. Milder course w/later onset. There is some level of the dystrophin protein present.
It is also an inherited disease