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50 Cards in this Set
- Front
- Back
Hereditary Dz: Achondroplasia
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inherited dwarfism, auto. dominant, mutation: FGFR3
Mutation inhibits cartillage synth > early growth plate dusion. Short extremities. Other stuff is normal |
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Osteogenesis Imperfecta
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aka brittle bone dz.
issues w/ sytn of Type I collagen. generalized osteopenia, thin sclera (blue) w/ blue hue, hypermobility. Also deafness Type I (blue sclera), Type III (blue >> white sclera), Type IV (normal sclera). These are dominant. Type II: Autosomal recessive, DEAD! |
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Osteopetrosis (Albers-Schonberg Dz)
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aka marble bone dz.
decreased osteoclast function. Thickened, brittle bones; reduced medullary cavity - myelophthisic process extramedullary hematopoeisis, cranial nerve compression > blindness, deafness. X-ray: osteosclerosis, broadened metaphysis (ERLENMEYER FLASK shaped deformity in long bones). |
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Two types of Osteopetrosis (Albers-Schonberg Dz)
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Autosomal Recessive: infants, children, early death due to anemia, infection, hemorrhage.
Autosomal Dominant (benign type): adults, fractures, mild anemia, cranial nerve impingement. Carbonic Anhydrase II deficiency: renal tubular acidosis and cerebral calcification. |
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Paget Disease (Osteitis Deformans)
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dz of bone remodelling (localized). Thickened but weak bones.
after 40 yo, european ancestory. Mono-ostotic, Polyostotic (85%). Skull common location, also Iliac crest. Hat doesn't fit anymore. Facial bones may produce lionlike facies. Micro: MOSAIC pattern, haphazard arrangement of cement lines |
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Clinical features of Paget dz
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Warmth of overlying skin due to hypervascularity. Fractures.
INCREASED RISK OF OSTEOGENIC SARCOMA. Complications: AV shunts within marrow > high cardiac failure. Lab: Elev Alk Phosphatase, Increased Urinary Hydroxyproline (bone breakdown marker) |
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What's the bone breakdown markder?
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hyroxyproline
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Osteoporosis
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most common bone dz in US.
postmenopausal female, elderly, high in Caucasian, lack of excercise. Genetic dz: OGI, Gaucher Dz immobilization, endocrinopathies (cushings, thyrotoxicosis), malnutrition (Vit C, D, Ca), corticosteroids. |
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What's a Colles fracture?
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distal radius fracture (fall on outstretched hands).
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Bone in osteoporosis
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bone formed normally but in decreased amounts
Labs in Osteoporosis are NORMAL. |
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Osteomalacia and Rickets
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Decreased mineralization of bone.
Def or abnormality of Vitamin D. (dietary def, malabsorption, low sunlight, renal and liver failure). |
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Rickets
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Children
Enchondral bone formation affected. Craniotabes, frontal bossing, Rachitic rosary, Pectus carinatum, bowing of the leg. |
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Osteomalacia
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Adults: vertebrae, hips and wrists.
Use Lab finding: ABNORMAL, low Ca, and Phosphorus, High Alk Phosphatase. |
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Osteomyelitis: Pyogenic
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hematogenous spread, affects meaphysis where feeding arteries come in.
Staph Aureus, E. Coli, Strep, Gonococci, HI Sickle cell pt: Salmonella IVDA and diabetics: Pseudomonas formation of Sequestrum (central necrotic bone), and involucrum (new bone formation surrounding sequestrum) Tx: drainage, antibiotics |
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Complications of osteomyelitis
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fracture, intraosseous (Broadie) abscess, SAA amyloid, SCC (rare), osteogenic sarcoma (rare)
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Tuberculous Osteomylitis
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1% of TB cases. Thoracic and Lumbar (Pott dz) vertebrae
Complications: compression fracture, Psoas abscess, Amyloidosis. |
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Osteitis fibrosa cystica (Von Recklinghausen dz of bone)
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different from Von Recklinghausen dz aka Neurofibromatosis I
------------------ Excess PTH, so Osteoclast activated. could be PT adenoma or hyperplasia. Excess bone resportion, Cystic spaces in trabecular bone. BROWN TUMOR (fibrosis and hemmorhage in the cysts) |
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Hypertrophic Osteoarthropathy
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end of long bone: periosteal new bone formation, digital clubbing.
painful swellings of wrist, fingers, angkles, knees or elbows. arthritis of ajdacent joints. Etiology: bronchogenic carcinoma, chronic lung dz, haeart dz, IBD |
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Benign Tumors: Osteoma
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freq. skull and facial bones.
Hyperostosis frontalis interna: osteoma extends into orbit or sinuses. Assoc. w/ Gardner Syndrome (a familial polyposis) |
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Osteoid Osteoma
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benign, painful growth of diaphysis (tibia or femur).
Young males. PAIN WORST AT NIGHT AND RELIEVED BY ASPIRIN. small lesions in Ctx of bone |
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Osteoblastoma
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larger than 2 Cm and involves vertebrae (othewise same as osteoid osteoma)
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Osteochondroma (exostosis)
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benign bony metaseal growth. have a Cap of cartillage**.
adolescent males. May be multiple, symmetrical: (osteochondromatosis) |
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Enchondroma
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benign, cartillage in medullary cavity of hand and feet. Found accidently
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Multiple Endchodromas
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Ollier Dz: non-herditary, pain and fracture, may transform to chondrosarcoma.
Maffucci syndrome: multiple endchoromas, hemangiomas, increased risk of malignant tranformation, ovarian carcinoma, and brain gliomas. |
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Malignant Tumors of Bone: Osteosarcoma (osteogenic sarcoma)
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Most common, primary malignant tumor of bone.
Higher in young males, pt's with familial retinoblastoma. occurs in METAPHYSIS, Codman's triangle, sunburst pattern. Most Common near the KNEE (distal femuar, proximal tibia) Histo: anaplastic cell producing osteoid (bone producing tumor) |
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Loci of Tumors
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Epiphysis: Giant Cell Tumor (middle age FEMALES)
Metaphysis: Osteosarcoma (young teen males) Diaphysis: Ewing's Sarcoma |
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Secondary Osteosarcoma
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elderly patients, assoc. w/ Paget Dz, irradiation and osteomyelitis.
Highly AGRESSIVE. |
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Chondrosarcoma
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malignant tumor of chondroblast
Males, Middle ages Involves pelvic bones, shoulder girdle, spine Enlarging mass Histo: atypical chondrocytes and blasts, often MULTIPLE nuclei in a lacuna. |
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Giant Cell Tumor (Osteoclastoma)
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Females, 20-50 yo.
SOAP Bubble appearance Gross: RED BROWN mass, around KNEE, at EPIPHYSIS locally agressive with high recurrence. |
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Ewing Sarcoma
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malignant tumor of UNDIFFERENTIATED cells in Marrow cavity.
most common in Teen males. t (11;22) EWS-FL11 fusion protein. Xray: "onion-skin" layer of new periosteal bone. Diaphysis Micro: HOMER Wright pseudorosettes, sheets of undifferentiated small round blue cells resembling lymphocytes. |
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Osteoarthritis (degenerative joint dz)
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most common form of arthritis, increases with age.
**Worsens with repetitive motion**. Xray: narrowing of joint spaces. common in wt bearing joints: knee, hips, spine.ASSYMETRIC Eburnation (polishing) of exposed bone. Has osteophytes (reactive bony outgrowth). |
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Heberden nodes and Bouchard nodes
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Heberden: osteophytes at distal interphalangeal joints (DIP)
Bouchard: osteophytes in proximal interphalangeal (PIP) joints. |
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RA
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RA factor: IgM auto Ab., synovial proliferation, small joints (hand, feet), SYMMETRICAL and migratory.
MORNING STIFFNESS that improves with activity. HLA-DR4 and DR1 x-ray: juxtaarticular osteoporosis PANNUS FORMATION: prolif of synovium and granulation tissue over the cartillage of the joint. Lab: Elev. ESR, RA factor pos. (titer correclates with severity). |
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Felty Syndrome
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RA + splenomegaly and neutropenia
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Caplan Syndrome
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RA and pneumonoconiosis
RA also associated with Sjogren Syndrome and Amyloidosis. |
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Ankylosing spondylitis
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young men, HLA-B27, sacroiliac joints and spine (bamboo spine), assoc. w/ inflammatory bowel dz
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Reiter Syndrome
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triad: conjunctivitis, urethritis, arthritis
can't see, pee or climb a tree HLA-B27, may follow a venereal dz |
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Enteropathic arthritis
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found in 20% Ulcerative Colitis pts, assoc. w/ HLA-B27
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Psoriatic arthritis
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mild, slow progressive arthritis, assoc with B27, 5-10% pts with psoriasis
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Gout
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hyperuricemia and deposition of monosodium urate crystals due to overproduction or underexcretion of uric acid.
Big toe involvement, aspiration: NEGATIVE BIREFRINGENT, NEEDLE SHAPED URIC ACID CRYSTALS. Tophi appears as chalky white deposits. |
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Secondary Gout
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only 10% cases:
Excessive cell breakdown in leukemia. Renal dz Lesch-Nyhan Syndrome: HPRT deficiency, X-linked, mental retardation, SELF-MUTILATING behavior. |
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Pseudogout (chondrocalcinosis)
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older >50 yo
deposition of Calcium pyrophosphate crystal. POSITIVE BIREFRINGENT, RHOMBOID-SHAPED CRYSTALS. Knee joint most common |
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Polymyositis
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autoimmune myopathy, adults, bilateral proximal muscle weakness
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Dermatomyositis
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children and adults.
SKIN RASH OF UPPER EYELIDS perifascicular fiber atrophy |
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inclusion body myositis
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older males > 50 yo
assymetrical distal muscle weakness EM: filamentous inclusion |
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Myesthenia gravis
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autoantibodies against ACh-R, facial muscles, ptosis and diplopia
WEAKNESS WORSENS with repeated contractions. |
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Eaton-Lambert syndrome
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a paraneoplastic syndrome of small cell lung cancer.
autoantibodies against Calcium channel. WEAKNESS IMPROVES with repeated contractions. involves proximal larger muscles. |
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Duchenne Muscular dystrophy
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X-linked, absence of dystrophin protein.
Sx by age 5, calf pseudohypertrophy, weakness of proximal shoulder and pelvic girdles Heart failure Lab: serum creatine kinase Dx by biopsy or PCR. |
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Becker muscular dystrophy
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not as severe as DMD
ALTERED dystrophin protein no cardiac involvement |
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Guillain-Barre syndrome
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follows viral illness.
ascending paralysis inflammation and demyelination of peripheral nerves 5% fatal (respir. failure) |