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Definition
AD form of dwarfism abnl cartilage synthesis mutation of FGFR3 (receptor) |
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cartilaginous masses w/n medullary cavity of bone ollier dx: non-hereditary, multiple, most commonly hands and feet present with pain and fx -> may undergo malignant transformation Maffuci syndrome: familial; enchondromas and hemangiomas of skin
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Definition
focal fibrous replacement of bone that begins in medullary cavity and remains encased in cortical bone. esp male teenagers affects long bones, ribs, skull, facial bones -> pathologic fx McCune-Albright syndrome: assoc of polyostotic fibrous dysplasia, cafe-au-lait spots, and sexual precocity in W
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Term
hyperparathyroidism aka osteitis fibrosa cystica |
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Definition
osteoclasts resorb bone, kidney wastes Ca++ more common in primary hyperparathyroidism presents c bone pain and fracture (fx) fibrous replacement of marrow -> cystic spaces in bone and "brown tumors" |
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Term
| hypertrophic osteoarthropathy |
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Definition
etiology: intrathoracic carcinoma, cyanotic congenital heart dx, IBD presents as idiopathic painful swelling of wrists, fingers, ankles, knees or elbows periosteal inflammation, new bones form at end of long bones, MC and MTs arthritis often seen with digital clubbing |
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Definition
bony metaphyseal projections capped with cartilage Gardner syndrome: exostoses and colonic polyps -> may become carcinomas |
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Definition
defect in type I collagen charac by - fragile bones
- blue sclera
- lax ligaments
Type 1: AD - mild to mod dx Type 2: AR - stillborn infant - generalized crumpled bones Type 3: AR - prog severe deformity; white sclera Type 4: AD - var severity; nl sclera
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Definition
Vitamin D deficiency due to: - chronic renal insufficiency
- intestinal malabsorption
- dietary deficiency
osteoid produced in nl amounts, but cannot calcify properly -> diffuse radiolucency on bone films lo Ca++ and P, high alk phos Rickets: children, prior to closure of epiphyses -> "rachitic rosary", bowing of legs and fxs
Osteomalacia: impaired mineralization of nl osteoid matrix; fx and deformities
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Definition
spread by direct inoculation of bone or hematologenous seeding Staph aureus Strep Haem. influenzae Salmonella (sickle cell dx) Pseudomonas (IV drug users and diabetics) Mycobac tuberculosis (Pott dx - spine) presents with fever, localized pain, erythema and swelling |
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Definition
osteoclasts unable to resorb bone inc density of cortex with narrowing of erythropoeitic medullary cavities presents with - brittle bones
- anemia
- blindness
- deafness
- hydrocephalus
- CN palsies
AR: affects children -> early death (via anemia and infxn) AD: affects adults -> fx |
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Definition
dec in bone mass (esp postmenopausal F) RF: estrogen deficiency, low density of orig bone, lack of exercise all bones affected (XR: generalized radiolucency) weight-bearing bones predisposed to fx |
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Term
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Definition
excessive bone resorption c replacement by soft, poorly mineralized matrix (woven) -> XR: enlarged, radioluscent bones presents c pain, deformity, fxs Lab: high high! alk phos polyostotic: skull, pelvis, femur, vertebrae alternates osteolytic -> osteoblastic phase may cause bone hypervascularity c inc warmth of overlying skin |
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Definition
similar to osteoid osteoma BUT large, painless often involves vertebrae may be malignant |
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benign affects diaphysis of long bones (often tibia or femur) causes pain worse at night and relieved by ASA XR: central radiolucency surr by sclerotic rim Pathology: brown nodule surrounded by dense sclerotic cortical bone |
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Definition
benign freq involves skull hyperostosis frontalis interna: osteoma extends into orbit or sinuses pathology: dense nl bone |
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Definition
malignant produces osteoid and bone most common bone tumor M>F, usua 2nd-3rd decade assoc c Paget dx in older pts presents c localized pain, swelling, weight loss, anemia XR: Codman triangle (periosteal elevatoin) + bone destruxn Pathology: large, necrotic, hemorrhagic mass poor prog, met to lungs common |
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Definition
benign, rare affects young M firm mass w/n metaphyseal marrow cavity of the tibia or femur contains fibrous and myxomatous tissue |
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Definition
malignant tumor 30-60 yrs M>F de-novo or from pre-existing enchondroma slower growing than osteosarcomas presents c pain and swelling involves spine, pelvic bones and upper extremities |
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Definition
solitary cartilaginous growth with spongiosa of bone solitary growths similar to those in multiple form (ollier) |
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Definition
benign epiphyseal growth may be solitary lesions identical to those in multiple form |
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Definition
malignant , rare usual adolescents, often M from mesenchymal cells presents as pain, tenderness and early widespread dissemination commonly affects pelvis, metaphysis of long tubular bones t(11;22) |
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malignant, uncommon ages 20-50 arises in epiphyseal region of long bones present as bulky mass c pain and tenderness XR: expanding area of radiolucency without sclerotic rim |
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Definition
mostly young M & +HLA-B27 assoc c IBD involves SI joint + spine |
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Definition
polyarticular RA assoc with HLA-B27 with splenomegaly + leukopenia |
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Definition
hyperuricemia -> deposition of monosodium urate crystals (needle-shape, neg birefringent) in jts -> recurrent bouts of arthritis primary gout: 90%: due to inborn error of purine metabolism, usua from unknown enzyme deficiency (rare: Lesh-Nyhan) secondary gout: unrelated to purine metabolism (example: leukemia) |
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Definition
peak 1-3 yrs F>M often preceded by acute febrile illness sxs: periarticular swelling, LA, hepatosplenomegaly, neg RF var course; resolution possible |
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Definition
degenerative joint dx incidence inc with age (80% 70 y.o. in one jt) aging or wear and tear most important mech insidious onset with joint stiffness, dec ROM, and effusions XR: narrowing of joint space due to loss of cartilage and osteosclerosis joints: vertebrae, hips, knees, DIP jts of fingers pathology: subchondral cysts, eburnation, osteophytes, Heberden's nodes (DIP),Bouchard's nodes (PIP) |
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Definition
calcium pyrophosphate crystal deposition assoc with multiple dx (Wilson dx, hypothyroidism, DM) pos birefringence, rhomboid appearance |
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Definition
similar to RA, but no RF assoc with HLA-B27 |
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Term
| Rheumatoid Arthritis (RA) |
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Definition
progressive arthritis F>M; 20-60 yrs autoimmune rxn with formation of circulating Ab (Rheumatoid Factor) sxs: low grade fever, malaise, fatigue, AM stiffness PE: joint swelling, redness, warmth SF: inc cells (PMNs) + poor mucin inc ESR and hypergammaglobulinemia XR: erosions and osteoporosis starts in small jts of hands & feet; symmetrical |
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Definition
tender, red swollen ("hot knee") monoarticular - high PMN count in joint fluid Staphylococcus Streptococcus Gonococci Reiter syndrome: arthritis, uveitis, and conjunctivitis - possibly due to Chlamydia (can't pee, can't see, can't climb a tree)
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