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PATHOLOGIES OF THE MUSCULOSKELETAL SYSTEM

I. Diseases of Bone Framework

Disease Etiology Clinical Features

OSTEOPOROSIS  a group of diseases charac by a ↓ in bone  Immobilization: confinement to bed > 6 months

mass resulting in vulnerability to loss of 30% of initial bone. Lack of exercise leads

fracture. Mostly a consequence of to ↑ in bone resorption and ↑ bone formation.

aging, part. in post-menopausal  Other: can occur in Cushings Syndrome,

women. hypogonadism, hyperthyroid, hypopituitary,

 MORPH: Cortex ↓ thickness pregnancy and lactation, malabsorption,

Trabeculae of cancellous bone ↓ in malnutrition, prolonged admin of heparin

number and size. Bone mineralization is  Clinical: bone pain, deformity, height loss,

normal and osteoid seams are normal fractures of femoral neck and vertebrae

width. Common in vertebrae, femoral  Diagnosis: XRay with thin cortices. Osteopenia or

neck, wrist. “little bone” seen in osteomalcia, OI, osteitis

 Aging: most imp. factor, esp > 40-50 fibrosa, renal osteodys, multiple myeloma. Serum

when mass decreases at a faster rate Ca2+ and PO4- normal.

and more pronounced in women. Cause  Estrogen replacement, exercise, and Ca2+ help ↓

of ↓ mass and ↑ osteoclastic resorption risks.

is unknown. Ca2+ absorption also ↓

with aging.

 ↓ Estrogenic activity post-menopause:

↑ loss of cortical bone in post-

menopausal and post-oophorectomy.

For men, androgen deficiency is imp.

RICKETS  Vit. D deficiency or PO4- depletion in  CLINICAL: pain, bone deform including rachitic

children. Can result from dietary rosary (swollen costochondral jxn of ribs), outward

deprivation, fat malabsorption, curvature of sternum (pigeon chest), shortened and

prolonged lack of sunlight, chronic renal deformed limbs with severe bowing of arms and

failure. PO4- depletion can occur from legs, freq. fxs.

Fanconi syndrome, heavy metal  XRAY: thickened, irreg. and Lobulated epiphyseal

poisoining, idiopathic PO4- leak. plate

 MORPH: defective mineralization of

osteoid wide osteoid seams and a

decrease in calcified bone.

OSTEOMALACIA  Vit. D deficiency or PO4- depletion in  CLINICAL: In dietary Vit D deficiency, serum levels

(Soft Bones) adults. Can result from dietary of Ca2+ and PO3 are low due to diminished

deprivation, fat malabsorption, absorption, esp. of Ca2+. Low Ca2+ stimulates

prolonged lack of sunlight, chronic renal Parathyroid glands Secondary HyperPTH

failure. PO4- depletion can occur from  XRAY: osteopenic patten with compression fxs,

Fanconi syndrome, heavy metal decreased in bone thickness.

poisoining, idiopathic PO4- leak.

 MORPH: defective mineralization of

osteoid wide osteoid seams and a

decrease in calcified bone.

OSTEITIS FIBROSA  “von Recklinghausen Disease”  XRay shows “moth-eaten” in phalanges and

CYSTICA  ↑ parathyroid hormone mobilizes Ca2+ clavicles.

(Hyperparathyroidism) from bone ↑ osteoclastic activity  Tumor like masses with giant cells, cysts,

subsequent fibrous replacement. hemorrhages, and fibrous tissue Brown Tumors

 ↑ resorption and fibrosis formation of reparative giant cell granulomas.

cysts and areas of hemorr.

RENAL  Changes include: osteitis fibrosa  May be osteosclerosis if severe

OSTEODYSTROPHY cystica, osteomalacia, osteoporosis



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Disease Etiology Clinical Features

PAGET DISEASE  disease of uncertain etiology assoc. with  AGE: 40-60, most common in Caucasians

(Osteitis Deformans) irreg. thickening and softening of  MICRO: Osteolytic stage resorption with

bones. increased osteoclasts Mixed Stage new bone

 ETIOLOGY: paramyxovirus infection formation with abundant osteoblasts. Bone

suggested, but not proven. Polyostotic haphazardly laid down mostly as woven bone, some

85% and monostotic 14% of cases converted to lamellar. Mosaic Pattern:

 STAGES: 1) Initial Osteolytic: bone minerlization lags and newly laid down bone well

resorption by osteoclasts 2) Mixed demarcated by osteoid seams or cement lines.

Osteolytic-osteoblastic: woven bone Marrow spaces filled with loose, vascular connective

with mosaic pattern 3) Burnt-out, tissue Osteosclerotic Stage predom. bone

quiescent Osteosclerotic formation and osteosclerosis

 MACRO: most common in spine, pelvis,  CLINICAL: Initially, bone pain, fxs, deformities.

femur, skull, tibia, humerus. Deafness common when skull affected. Vertebral

Spine  thickening and softening of compression with height distortion. Coarsening of

resulting in kyphosis, shortening of trunk facial bones leontiassi ossea (lion-like) Serum

and pressure on nerves in vertebral ALP markedly ↑. Can have high output cardiac

foramina. Skull  prog. ↑ in size due to failure in polyostotic cases. Osteosarcoma may

thickening, the base may be involved dev. in 1%

with pressure in nerves. Femur  ↑  XRAY: bone lysis and reformation

thickness with coax vera and anterior

bowing.

FIBROUS DYSPLASIA  benign replacement of bone by disorg  Albright Syndrome: 5% most commonly in

fibrous and osseus elements. Occurs in precocious puberty in females. Other endocrine

children or adults and may be problems include acromegaly, Cushings Syndrome,

monostotic. Polyostotic assocl with skin hyperthyroid. Skin Lesions: pigmented macules

pigmentation and endocrine problems. (Café au lait spots) usually over buttocks, back,

 Monostotic FD: most common (70%) sacrum.

and most common in prox femur, tibia,  XRAY: lucent ground glass appearance with well-

ribs, facial bones. Can lead to marginated borders and thin cortex

pathologic fx.  MICRO: benign fibroblastic tissue in a whorled

 Polyostotic FD: 25% usually in children pattern with haphazardly arranced trabeculae of

with pthologic fxs, limb deformities, woven bone which lack osteblastic rimming and

limb length discrepancies. Most common form configurations likened to Chinese charac.

in females. RARELY, sarcomas may arise.

 Serum ALP elevated. Ca2+ and PO4- normal.

NONOSSIFYING  non-neoplastic developmental lesion in  MICRO: cellular fibroblastic tissue with scattered

FIBROMA children most commonly in femur, tibia, lipid-laden Macros and multinucleated giant cells.

(Fibrous Cortical and fibula. Well-demarcated,  CLINICAL: extremely common, usually asx and

Defect) radiolucent defects of cortex with thin often disappears spontaneously within years. NO

intact layer of subperiosteal cortical TX to sarcomas.

bone.

HYPERTROPHIC  periosteal new bone formation, arthritis,  New bone formation distal ends of long bones,

OSTEOARTHROPATHY clubbing of digits usually in pts with metatarsals, metacarpals, and prox phalanges.

bronchial CA, pleural tumors,  Arthritis adjacent joints and is nonspecific with

pulmonary METS, mediastinal Hodgkins, edematous thickening of synovium and mild

Chronic Lung Infections, Chronic Liver lymphoplasmacytic infiltrate

Dz.  Clubbing edematous fibrovascular overgrowth in

 Clubbing alone may occur secondary to tips of digits and appears dusky to cyanotic.

same conditions but also with cyanotic  Resection of underlying CA reverses changes.

heart disease, infective endocarditis,

UC, Crohns, CA of Eso and Colon.









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