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Clinical Medicine: Ocular Manis of Skeletal and CT Disorders
Terms in this set (20)
This disease is caused by excess of bone destruction and organized bone formation and repair.
show abnormal morphology and are INCREASED in number. Results in problems remodeling. The etiology is unknown (postulated inflammatory, vascular, ______, neoplastic, lead toxicity, and ______). Usually affects what three things? Most persons are asymptomatic and diagnosis is incidental.
a) Paget's Disease (Osteitis Deformans)
b) Viral, Genetic
c) Axial skeleton, vertebrae, and skull
What is the most common of Paget's Disease (Osteitis Deformans)? Worse with
or activity but may also occur at rest. Skull pain (headache, warmth, tenderness, and enlargement of head -- compromised nerve foramen due to growth of skull).
a) Bone pain
Paget's Disease Symptoms: Neurological symptoms, sarcomatous transformation, cardiac failure, and pathologic fractures, deformities, bony enlargement, ______ of the limbs and spinal curvature. What are two other associated disorders?
b) Gout and Hyperuricemia
Paget's Disease Ocular Manifestations: Blindness and ___ due to increased pressure. What are three types of retinal changes that occur?
b) PPA, Mottling (RPE), and angioid streaks
Paget's Disease Diagnosis and Treatment: X-ray; markers of bone turnover (_____ _________ elevated as a result of osteoblast activity). Serum calcium normal and isotope bone scars.
Paget's Disease: Relieve pain symptoms (Analgesics and NSAIDS) and prevent fracture and deformities. Pharmacological agents to suppress osteoclastic activity are what two? Physical therapy and assistive devices (cane, walker).
This disease is caused by an AD mutation in the FBN1 gene that determines the structure of
, important part of CT and elastic fibers. Affects multiple parts of the body such as bones, joints, eyes, blood vessels, lungs and heart.
a) Marfan Syndrome
Symptoms of Marfan's Syndrome: _______ of arms and legs leading the arm span being longer than height, _________ (long fingers). Joint _________. Sternal deformity (pectus escavatum or carinatum). Scoliosis, super arched mouth which leads to very crowded front teeth, mandible malocclusion. Mitral Valve Prolapse. Aortic Dilation. Aortic Dissection/Rupture. _________ lung disease.
Ocular Manifestations of Marfan's Syndrome: ______ _____ (______ lens dislocation); early glaucoma and cataracts; iritis; secondary _________; retinal detachment. Aging is accelerated.
a) Ectopia Lentis (Superior)
Diagnosis and Treatment of Marfan's Syndrome: Family history; eye exam (slit lamp lens dislocation); _______ (look for aortic route dilation). Treatment includes: _____ ____, TGFBeta, and aortic surgery (control BP).
b) Beta Blockers
So...Marfan is caused by a defect in what?
This is a genetic disease that causes disruptions to the structure as well as COLLAGEN production. The genetic defect weakens connective tissue of the skin, bones, and other parts of the body. Different genetic defects (primarily AD and AR).
a) Ehler-Danlos Syndrome
Symptoms of Ehler-Danlos: skin ________ (smooth and easy to bruise or tear); joint _________ (sprains, dislocations, and subluxations). _____ ____ _____ Disease can also be present. Advanced premature osteoarthritis with chronic pain. Slow and poor wound healing with wide scarring. Muscle fatigue and pain. Mitral valve prolapse and aortic root dilation.
Think: Collagen is destroyed so cannot hold things in place
c) Chronic degenerative
Ocular Manifestations of Ehler-Danlos: High myopia, retinal detachments, _______, ______ ______, lens ________, angioid streaks, epicanthal folds, dry eyes, glaucoma, strabismus, and macular degeneration.
b) Blue Sclera
c) Lens Subluxation
Diagnosis and Treatment of Ehler's Danlos: Family history/pedigree, physical exam/history, ECHO, DEXA, and genetic testing. Treatment includes rehabilitation such as ____ therapy and PT/OT; NSAIDS and chronic pain management; orthopedics and possible role for braces; and genetic counseling.
What two ocular manifestations are found in both Ehler's Danlos and Marfan's Syndrome?
a) Lens subluxation
This is "brittle bone disease" and is a congenital disorder of bone fragility caused by mutations in the genes that code for Type 1 PROCOLLAGEN.
Type 1 collagen is a major component of the CT in bones, ligaments, teeth and sclera. There are several types of this disorder that vary in severity and characteristics.
a) Osteogenesis Imperfecta
Symptoms of osteogenesis imperfecta: _____ bones with redundant ____ formation. Fractures. Osteoperosis. Joint and ligament laxity. Thinning of the skin. Fragile and transparent teeth. Easy bruising. Low muscle mass. __________ (50% have this by age 40). Much more severe than PAGET's
Ocular Manifestations of Osteogensis Imperfecta: ______ sclera, lens dislocation, _______ of the vitreous, corneal opacity, keratoconus, optic ____ and ______, retinopathy, cataracts, and ocular proptosis.
c) Neuropathy and Atrophy
Osteogenesis Imperfecta Diagnosis and Treatment: X ray, ECHO, DEXA, skin biopsy, and genetic testing.
Tx: ______ (a bone-resorption inhibitor that reduces the incidence of fracture and increases bone mineral density while reducing pain and increasing energy levels). Hearing aids.
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