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Pathology 2 - Block 3 - Part 2

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davidwurbel7's version from 2016-07-31 23:45

Non-Neoplastic Disorders of Bone

Question Answer
Genetic disorder in which there is a problem with synthesis of type I collagenOsteogenesis Imperfecta
Osteogenesis Imperfecta has a problem with the synthesis of thisType I Collagen
Genetic disorder in which long bones do not form properlyAchondroplasia
Genetic disorder in which the bone marrow is replaced by boneOsteopetyosis
The organic component of the boneOsteoid
Osteoid is made by these cellsOsteoblast
Osteoblast synthesis thisType I Collagen
The inorganic component of bone is thisCalcium Hydroxyapatite
A patient with pathological fractures, blue sclera and deafnessOsteogenesis Imperfecta
Osteogenesis Imperfecta might be mistaken for thisChild Abuse
This is due to thinning of scleral collagen allows reflection of underlying choroidal veins in a patient with osteogenesis imperfectaBlue Sclera
A patient with osteogenesis imperfecta may have fractures of the ossicles leading to thisDeafness
Cause of dwarfism due to mutation in FGFR3Achondroplasia
Impaired formation of long bones. Impaired proliferation of cartilage at growth plateAchondroplasia
Achondroplasia is due to an activation mutation in this geneFGFR3
This inhibits cartilage synthesis at growth plate leading to decreased growth of long bonesOverexpression of FGFR3
A connective tissue matrix is laid down first and then ossifiedIntramembranous Bone Formation
Flat bones and small bones are formed in this mannerIntramembranous Bone Formation
Cartilage is formed at the growth plate. Distal cartilage of the growth plate is then ossifiedEnchondral Bone Formation
Long bones are formed in this mannerEnchondral Bone Formation
Dwarfism due low production of HGHPituitary Dwarfism
Dwarfism due to hypothyroidismCretinism
Condition in which bones becomes dense, thick. The marrow cavity is replaced by bone. The thickened bones are brittle and fracture easilyOsteopetrosis
This condition is due to decrease activity of osteoclasts in the boneOsteopetrosis
Osteopetrosis is caused by the decrease activity of these cellsOsteoclasts
Osteopetrosis is due to a mutation encoding for thisCarbonic Anhydrase II
Clinical findings include pathologic fractures, Pancytopenia (anemia, neutropenia and thrombocytopenia), Leukoerythroblastic blood picture with extramedullary hematopoiesis: hepatosplenomegaly - all due to replacement of marrow cavity by boneOsteopetrosis
Cranial nerve compression may result in blindness, deafness and facial nerve palsies due to thisCranial Foramen Stenosis
A patient with osteopetrosis may develop hydrocephalus due to thisForamen Magnum Stenosis
Treatment of osteopetrosis is this which may also reverse many of the skeletal deformitiesBone Marrow Transplant
A virus infects osteoclastic progenitors or osteoclasts in a genetically predisposed individual and stimulates osteoclastic activity, thereby leading to excessive resorption of bone. Over a period of years, the bone develops a characteristic mosaic pattern, produced by chaotically juxtaposed units of lamellar bone that form irregular cement lines. The adjacent marrow is often fibrotic, and there is a mixture of osteoclasts and osteoblasts on the surface of the bonePaget's Disease of Bone
Osteolytic stage, Mixed osteolytic-osteoblastic stage and Osteosclerotic - End result is thick, sclerotic bone that fractures easilyPaget Disease Stages
Paget Disease stage in which there is hyper activity of the osteoclasts breaking down the bone. Osteoblasts standby can continue normal functionOsteolytic Stage
Paget Disease stage in which there is hyper activity of the osteoclasts breaking down the bone. Osteoblasts begin to lay down in a haphazard manner producing a mosaic pattern produced by chaotically juxtaposed units of lamellar bone that form irregular cement linesMixed Osteolytic-Osteoblastic Stage
Paget Disease stage in which there is little osteoclast activity. Osteoblast continue to lay down bone haphazardly resulting in thick, sclerotic bone that fractures easilyOsteosclerotic Stage
MCC of isolated elevated alkaline phosphatase in patients >40 years oldPagets Disease
Only one bone is affected by Pagets diseaseMonostotic Pagets Disease
Multiple bones are affected by Pagets diseasePolystotic Pagets Disease
A complication of Pagets disease of the bone due to AV connections in vascular boneHigh Output Cardiac Failure
The high output cardiac failure seen in Pagets disease is due to thisAV Malformations
A person with Pagets disease of the bone is at increased risk for thisOsteogenic Sarcoma
Pagets disease of the bone is thought to be caused by infection by thisParamyxovirus
The bones most commonly affected by Pagets disease of the bonePelvis, Skull and Femur
This enzyme is used by osteoblasts in order to lay down new boneAlkaline Phosphatase
Treatment of Pagets disease which inhibits osteoclast functionCalcitonin
Treatment of Pagets disease which induces apoptosis of the osteoclastsBiphosphonates
Ischemic necrosis with resultant bone infarctionOsteonecrosis (Avascular Necrosis)
Fracture leading to bleeding into the joint capsule leading compression of the blood vesselsVascular Compression/Interruption
Long term therapy on this, commonly used for treatment of autoimmune diseases, can result in avascular necrosisCorticosteroids
Common sites of avascular necrosis include these bonesFemoral head and Scaphoid
This genetic condition may cause microinfarcts in the vessels of the bone leading to avascular necrosisSickle cell Disease
This condition ususally seen in commercial divers due to rapid ascension to the surfaceCaisson Disease (Decompression Sickness)
This type of bone infarction is usually asymptomaticMedullary Infarction
This type of bone infarction is symptomatic usually leading to osteoarthritisSubchondral Infaction
A Wedge shaped area of necrosis with viable overlying articular cartilage leading to collapse of the cartilage, predisposes that patient to thisOsteoarthritis
The gold standard for diagnosis of avascular necrosis of the hip is thisMRI
Condition in which there is a loss of both organic bone matrix and minerals resulting a decrease in bone mass and densityOsteoporosis
A decrease in thickness of cortical and trabecular boneOsteoporosis
Osteoporosis tends to affect this part of the boneTrabecular Bone
The most common type of fracture seen in patients with osteoporosis is thisCompression Fracture of the Spine
The second most common type of fracture seen in patients with osteoporosis is thisFemoral Neck Fracture
Diagnosis of osteoporosis is with this testDual Energy X-ray Absorptiometry (DEXA)
Osteoporosis seen in women after menopausePost-Menopausal Osteoporosis
The cause of post-menopausal osteoporosis is a deficiency of thisEstrogen
Stimulates OPG production. Decreases production of M-CSF. Decreases response of osteoclasts to RANK ligandEstrogen
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(Insert 1.13-1.16)
Question Answer
These increase RANK-L and decrease OPG promoting bone resorptionProstaglandins and parathyroid hormone
This dampens M-CSF and stimulates OPG decreasing osteoclastogenesisEstrogen
This diminishes RANK-L and enhances OPG expression decreasing osteoclast activityTGF-beta
Clinical findings include Bone pain, loss of height and KyphosisOsteoporosis
Kyphosis secondary to osteoporosis has this potential complicationRespiratory Compromise
Prevention of this is weight bearing exercises, calcium, vitamin D supplements and estrogen replacement therapyPostmenopausal Osteoporosis
Unopposed estrogen when used in HRT increases the risk of thisEndometrial Cancer
The risk of endometrial cancer can be reduced by the addition of this to the HRT regimeProgesterone
Treatment for osteoporosis that induces apoptosis of osteoclastsBiphosphonates
Treatment for osteoporosis that inhibits osteoclastsCalcitonin
The cause of this condition is either primary or secondary hyperparathyroidismOsteitis Fibrosa Cystica
Hyperparathyroidism due to problems with the parathyroid gland itself resulting in an increase in the level of PTHPrimary Hyperparathyroidism
Hyperparathyroidism due to prolonged hypocalcemia resulting in an increase in the level of PTHSecondary Hyperparathyroidism
“Brown tumor” of hyperparathyroidism is the other name of this conditionOsteitis Fibrosa Cystica
The mandible and proximal phalanx of the 2nd and 3rd digit are most often affected by thisOsteitis Fibrosa Cystica
The brown color seen in Osteitis Fibrosa Cystica is due to the deposition of thisHemosiderin
The presence of hemosiderin in the osteolytic lesion is due to thisHemorrhage
Decreased mineralization of newly formed bone. Usually caused by deficiency or abnormal metabolism of vitamin DRickets and osteomalacia
Vitamin D deficiency in adults leads to thisOsteomalacia
Vitamin D deficiency in children leads to thisRickets
Osteomalacia secondary to chronic renal disease. Chronic renal failure causes hypocalcemia due to lack of conversion of inactive vitamin D into active vitamin D. Hypocalcemia results in secondary hyperparathyroidism. PTH secretion stimulates osteoclast activityRenal Osteodystrophy
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Question Answer
The most common cause of pyogenic osteomyelitisStaphylococcus aureus
The most common cause of pyogenic osteomyelitis in sexually active young adultsN gonorrhoeae
The most common cause of pyogenic osteomyelitis in sickle cell diseaseSalmonella
The most common cause of pyogenic osteomyelitis in IV drug abusers, diabetics and puncture of foot through rubber footwearPseudomonas
The most common cause of pyogenic osteomyelitis in cat or dog bitesPasturella multocida
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Question Answer
Bones affected by pyogenic osteomyelitis in infant and childrenLong Bones
Bones affected by pyogenic osteomyelitis in adultsSmall Bones
The region of the long bone most affected by pyogenic osteomyelitis in children is thisMetaphysis
The region of the small bone most affected by pyogenic osteomyelitis in adults is thisEpiphysis
Necrotic bone due to interruption of blood supply caused by an enlargement of a subperiosteal abscess is calledSequestrum
Necrosis of bone seen in pyogenic osteomyelitis is due to shearing of blood vessel caused by thisSubperiosteal Abscess
The viable bone surrounding a sequestrum is called thisInvolucrum
Communication tract of a subperiosteal abscess with the surface of the skinSinus Tract
Clinical findings include rapid onset with C/O feeling ill. Most frequent manifestation as fever and severe pain over the affected area and reluctance to use affected extremities. On examination: Localized area of tenderness, Erythema, Swelling and Calor in the areaPyogenic Osteomyelitis
Complications include acute flareups, chronic osteomyelitis also may be complicated by pathologic fracture, secondary amyloidosis, endocarditis, sepsis, development of squamous cell carcinoma if the infection creates a sinus tract, and rarely osteosarcomaPyogenic Osteomyelitis
This test is best for the detection of early pyogenic osteomyelitis. The effected area will show localized increase uptake of tracersRadionuclide Bone Scan
Complication of chronic pyogenic osteomyelitis that develops at the orifice of sinus tractSquamous Cell Carcinoma
Complication of chronic pyogenic osteomyelitis in which infective material extends into the adjacent jointPyogenic Arthritis
Complication of chronic pyogenic osteomyelitis in which infective material seeds the blood streamSepticemia
Occurs secondary to tuberculous infection located elsewhere. Active tuberculosis of lung. Characteristically occurs in vertebraeTuberculosis Osteomyelitis
Clinical findings include back pain, Stiffness, Deformity (kyphosis), Neurological abnormalities (Pott’s paraplegia), Fever, night sweats and weight lossTuberculous Osteomyelitis
Characterized by development of bony tissue in skeletal muscle or soft tissue usually following a blunt trauma most often in the quadriceps or brachialis muscleMyositis Ossificans
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Labs
Question Answer
Calcium: Normal PTH: Normal PO4: Normal Vitamin D: Normal Alkaline Phosophase: NormalOsteoporosis
Calcium: Low PTH: Elevated PO4: Low Vitamin D: Low Alkaline Phosophase: ElevatedOsteomalacia
Calcium: Low PTH: Normal PO4: Normal Vitamin D: Normal Alkaline Phosophase: ElevatedOsteopetrosis
Calcium: Elevated PTH: Elevated PO4: Low Vitamin D: Normal Alkaline Phosophase: ElevatedOsteitis Fibrosa Cystica
Calcium: Normal PTH: Normal PO4: Normal Vitamin D: Normal Alkaline Phosophase: ElevatedPaget's Disease
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