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Rheumatology > Robbins > Flashcards

Robbins Flashcards

1
Q

What is the inorganic mineral component of bone?

A

Calcium hydroxyapatite

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2
Q

What is the role of calcium hydroxyapatite in bone?

A

Strength + hardness to bone

Storehouse of 99% of bodys calcium + 85% phosphorus + 65% magnesium

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3
Q

What cells express RANK?

A

Preosteoclasts + mature osteoclsts

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4
Q

RANK Ligand is expressed by what cells?

A

Osteoblasts and marrow stromal cells

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5
Q

Stimulation of RANK by its Ligand leads to?

A

Activation of transcription of factor NF-kB, which drives expression of genes to stimulate osteoclast formation + diff + function + survival

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6
Q

What up upregulates RANKL?

A

Osteoclast stimulating factors

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7
Q

Overall function of RANK + Ligand?

A

Up-reguate OSTEOCLAST activity

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8
Q

what is OPGs function?

A

Osteoprotegerin–> Blocks RANKL from binding to RANK

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9
Q

What competitively binds to RANKL to prevent bone resorption?

A

OPG

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10
Q

Problems in migration of mesenchymal cells and formation of condensations is called?

A

Dysostoses –> results from Homeobox mutations

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11
Q

Mutations interfering with bone or cartilage formation/ growth and/or maintenance of normal matrix?

A

Dysplasia (not precancerous)

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12
Q

“Brittle bone disease?”

A

Osteogenesis imperfecta

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13
Q

Genetic disorder caused by defective synthesis of alpha 1 +2 chains of type I collagen?

A

Osteogenesis imperfecta

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14
Q

What is the role of type I collagen?

A

it is a main component of Matrix all over the body

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15
Q

Fundemental abnormality is Too little bone–> extreme skeletal fragility?

A

Osteogenesis imperfect

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16
Q

What is a pathooneumonic characteristic of Osteogenesis imperfecta Type I patients?

A

BLUE sclera

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17
Q

What is pg of pt with pathopneumonic BLUE SCLERA?

A

OI–> caused by decreased collagen content causing transparency allowing underlying choroid to be seen

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18
Q

What are some deficits attributed to OI?

A

Hearing loss–> conduction defect in middle ear and inner ear bones
Small misshapen teeth–> dentin deficiency

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19
Q

BLUE SCLERA + bone disease?

A

Osteogenesis Imperfecta

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20
Q

What is the most common form of dwarfism?

A

Achondroplasia

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21
Q

What is Pg of achondroplasia (dwarfism)?

A

Activating point mutation in FGFR3

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22
Q

What is the function of FGFR3?

A

inhibits the proliferation and function of growth plate chondrocytes–> normal epiphyseal plate is suppressed + long bone is Severely stunted

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23
Q

Disproportionate shortening of proximal extremities+ bowing legs+ frontal bossing+ midface hypoplasia?

A

Dwarfism (achondroplasia)

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24
Q

Genetic disorders characterized by defective OSTEOCLAST-mediated bone resorprtion?

A

Osteopetrosis

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25
Q

What is “bone like stone disorder?”

A

Osteopetrosis–> bone are dense, solid, and stone like.

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26
Q

What are some of the abnormalities in Ostepetrosis which inhibit Osteoclast activity to resorb bone?

A

Carbonic anhydrase II deficiency
Proton pump deficiency
Chloride channel defect

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27
Q

What disease makes pt susceptible to fractures + cranial nerve palsies due to compression from shrunken foramina?

A

Osteopetrosis

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28
Q

Causes recurrent infection due to reduced BM size + activity and hepatosplenomegaly due to extramedullar hematopoiesis?

A

Osteopetrosis

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29
Q

Osteoclasts are derived from what precursors?

A

Marrow MONOCYTE precursors

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30
Q

Tx for Osteopetrosis?

A

Hemato[oietic stem cell transplant to repopulate progenitor cells capable of differentiating into OSTEOCLASTS

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31
Q

Defective osteoclast function causing architecturally weak and bowing bone from defective bone resorption?

A

Osteopetrosis

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32
Q

Mutation in type I collagen (alpha 1+2 chains)?

A

Osteogenesis imperfecta

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33
Q

Acquired condition characterized by reduced bone mass?

A

Osteoporosis

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34
Q

What are main causes of primary Osteoporosis?

A

Aging –> senile osteoporosis

Postmenopausal–> decreased Estrogen exacerbates bone loss

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35
Q

What are two main locations most susceptible to bone loss in osteoporosis?

A

Spine + Femoral neck

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36
Q

Causes thinned cortices + dilated haversian canals + loss of interconnections?

A

Osteoporosis

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37
Q

What is osteolytic activity and mineral content of bone in Osteoporosis?

A

Osteolytic activity is present (not increased)

Mineral content NORMAL

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38
Q

Osteoporosis occurs when?

A

the balance btwn Bone formation (osteoblasts) and Bone resorption (osteoclasts) is tilted in favor of RESORPTION

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39
Q

Age related causes of Osteoporosis?

A

Osteoblast activity progressively diminishes + extracellular matrix growth factors diminish w. time

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40
Q

What is the osteoblast vs. osteoclast activity level in senile osteoporosis?

A

Osteoblasts–> diminished function

Osteoclasts–> NORMAL function

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41
Q

Decline in estrogen is associated with?

A

accelerated cortical bone and trabecular bone LOSS

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42
Q

Over 30-40 yo, can result in what % of cortical vs trabecular bone loss?

A

Cortical -> 35 % loss

Trabecular–> 50% loss

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43
Q

What is the Pg of Estrogen decline and bone loss?

A

Decreased estrogen–> increased Cytokine production (IL-1 + TNF + IL-6) –> Stimulate RANK and suppress OPG production

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44
Q

Estrogen therapy benefits and consequences for postmenopausal women?

A

May ameliorate bone loss

BUT Increases RISK for CV issues

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45
Q

Causes increased IL-1 + IL-6 + TNF levels leading to increased RANK actions and increased Osteolytic activity?

A

Menopause–> DECREASED ESTROGEN

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46
Q

Decreased replicative activity of osteoprogenitor cells and decreased osteoblast activity + decreased matrix growth factors?

A

Aging related Osteoporosis

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47
Q

How does physical activity affect bone?

A

Mechanical force stimulates bone remodeling–> Resistant exercises (weight lifting) are more affective at increasing bone mass

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48
Q

What are some secondary causes of osteoporosis?

A

Prolonged glucocorticoid use–> increases bone resorption and reduces bone synthesis
Cigarettes + alcohol–> reduce bone mass

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49
Q

MCC complications of osteoporosis?

A

Thoracic or lumbar VB fractures leading to Kyphoscoliosis

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50
Q

What are MCC consequences of Femoral neck or pelvis or spinal fractures?

A

PE or pneumonia

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51
Q

What are two tests used for determining Osteoporosis?

A

Dual energy absorptiometry and quantitative CT

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52
Q

What are some Tx of osteoporosis that target reducing Osteoclast activity?

A

Antiresorptives–> Bisphosphonates + calcitonin+ estrogen + Denosumab

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53
Q

Main anabolic agent targeted to stimulate osteoblastic activity when treating osteoporosis?

A

Parathyroid hormone or analogues

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54
Q

Bone disease resulting in Gain in bone mass but bone is disordered and weak so they become enlarged and misshapen?

A

Pagets (Osteitis Deformans)

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55
Q

What are the 3 characteristic stages of Osteitis Defomans?

A

Osteolytic stage
Exuberant bone formation stage
Exhaustion stage (osteosclerotic)

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56
Q

What populations have a high prevalence of Pagets disease?

A

Europe
Australia
New Zealand
USA

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57
Q

Newly formed bone remodeled into abnormal lamellar bone with MOSAIC pattern (Jigsaw puzzle)?

A

Pagets disease

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58
Q

What is the Pg of Osteitis Deformans?

A

Inflammatory process caused by underlying PARAMYXOVIRUS infection in osteoclasts

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59
Q

What is the role of Paramyxovirus infection in Osteitis deformans?

A

Virus causing production of IL-1 + IL-6 + M-CSF–> all of which Activate Osteoclasts

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60
Q

Disease can be monostotic (lytic lesion in Tibia, femur, humerus…) or Polystotic (lesions Proximal femur or axial skeleton)?

A

Osteitis Deformans

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61
Q

Elevated serum alkaline phosphatase and increased Urinary Hydorxyproline reflects what?

A

Continuous or excess BONE Turnover

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62
Q

What is a consequence of Polyostotic Pagets disease?

A

Hypervascularity resulting in High-output CHF

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63
Q

Causes Brittle bone “Chalk stick fractures?”

A

Osteitis Deformans

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64
Q

Pagets disease dreaded complications and possible treatment?

A

Comp–> Sarcoma

Tx: Bisphosphonates to stop bone resorption

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65
Q

Impaired mineralization resulting in unmineralized matrix?

A

Osteomalacia or Rickets

Hypovitaminosis D

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66
Q

What is the difference btwn Osteoporosis and Osteomalacia?

A

Osteoporosis–> Bone has NORMAL mineral content but decreased mass
Osteomalacia–> Abnormal mineral content

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67
Q

Roles of PTH?

A

Osteoclast activation-> increases RANKL express
Increased Renal Ca+ resorption
Increased Phosphate excretion
Increased 1,25(OH)2 Vit D

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68
Q

What is the main action of PTH?

A

Elevation of serum Calcium

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69
Q

How does Chronic renal failure cause Osteodystrophy?

A

Inadequate 1,25(OH) D production

Hyperphosphatemia–> suppresses alpha 1 hydroxylase

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70
Q

What is the hallmark of Excess PTH?

A

Increased Osteoclastic activity

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71
Q

Causes bone resorption in subperiosteal regions most pronounced @ Lateral Middle Phalanges of 2nd + 3rd fingers?

A

Excess PTH

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72
Q

What is seen microscopically in excess PTH?

A

Osteoclasts boring into center of bony trabeculae

And expanding Haversian canals

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73
Q

What is Osteitis fibrosa cystica?

A

Cystic changes caused by excess PTH

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74
Q

What contributes to osteonecrosis?

A 
Vascular compression 
Steroid use
Thrombembolic disease (Caissons)
SCD 
Vasculitis
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75
Q

What is Osteomyelitis and MCC?

A

Inflammation of bone marrow

MCC–> pyogenic bacteria + TB

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76
Q

What is the overall MCC of pyogenic Osteomyelitis?

A

S. aureus

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77
Q

What organisms are important causes of Neonatal osteomyelitis?

A

E. coli + group B strep

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78
Q

What is the MCC pathogen in Osteomyelitis + SCD?

A

Salmonella

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79
Q

Radiologic finding reveals: destructive lytic focus surrounded by edema and sclerotic rim?

A

Osteomyelitis

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80
Q

What are some complications of Osteomyelitis?

A

Pathologic fractures
2nd Amyloidosis
Endocarditis
SCC

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81
Q

MCC of infection in the vertebral bodies causing collapse and deformities from posterior dislocation?

A

POTT disease–> TB Osteomyelitis

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82
Q

Pt with Pott disease and psoas muscle abscess?

A

TB osteomyelitis

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83
Q

What is the most common primary bone tumor?

A

Osteosarcoma

84
Q

Tumor arising from Knee in adolescent MALE child?

A

Osteosarcoma

85
Q

MId to late adult with tumor involving trunk, limb girdle, or proximal long bones?

A

Chondrosarcoma

86
Q

What conditions are associated with Osteosarcoma?

A

Li-Fraumeni (TP53 mutation)
Retinoblastoma (Rb mutation)
Bone infarcts
Paget disease

87
Q

Benign slow growing, solitary, hard lesion of Head or Neck in middle aged man?

A

Osteoma

88
Q

Teenage male w. < 2cm lesion arising from beneath periosteum or within cortex in proximal Femur/ Tibia?

A

Osteoid Osteoma

89
Q

Most commonly presents w/ localized pain that is severe at Night and is relieved by Aspirin?

A

Osteoid Osteoma

90
Q

Teenager with lesion in vertebral column that are >2cm and have non-localized pain that does NOT respond to aspirin?

A

Osteoblastoma

91
Q

Bone-producing malignant mesenchymal tumor?

A

Osteosarcoma

92
Q

Morphology: Gray-white exhibiting hemorrhage and cystic degeneration. With Large and varying shaped cells + bizarre tumor giant cells?

A

Osteosarcoma

93
Q

What is essential for the Diagnosis of Osteosarcoma?

A

Production of mineralized or unmineralized bone by malignant cells

94
Q

What is the MCC mutation in Osteosarcoma?

A

RB gene mutation

95
Q

CXR shows a TRIANGULAR shadow btwn cortex and raised periosteum?

A

Codman triangle–> Osteosarcoma

96
Q

MC location of Osteosarcoma metastasis?

A

LUNG

97
Q

Child w. multiple bony lesions and known EXT1 or EXT2 mutations?

A

Multiple hereditary Osteochondromas

98
Q

What do EXT1 or EXT2 genes encode?

A

Glycosyltransferases essential for polymerizing heparin sulfate–> IMPORTANT for cartilage

99
Q

What tumor grows around Knee or metaphysis of long bones and stops growing once normal growth of bone has Halted?

A

Osteochondroma

100
Q

What is a benign neoplasm of hyaline cartilage arising within medulla called?

A

Endochondroma

101
Q

Benign neoplasm of hyaline cartilage arising from bone surface is called?

A

Juxtacortical chondromas

102
Q

Chondromas most often affecte what bones?

A

Short tubular bones of hands and feet

103
Q

Disease characterized by multiple chondromas involving ONE side of the body?

A

Ollier disease

104
Q

Disease characterized by multiple chondromas associated w/ soft tissue spindle cells hemangiomas?

A

Maffucci syndrome–> associated w. Ovarian carcinomas and Brain glioma

105
Q

What mutation is found in both Ollier + Maffucci syndromes and also AML and gliomas?

A

IDH1 and IDH2 enzyme mutation

IDH= isocitrate dehydrogenase

106
Q

X-ray showing well-circumscribed oval lucencies surrounded by thin rims of bone (O-ring sign)?

A

Chondroma

107
Q

Rapidly growing painful Lesions commonly arising from Pelvis, Shoulder, and ribs?

A

Chondrosarcoma

108
Q

Chondrosarcomas larger that what size are more aggressive and where do they metastasize?

A

> 10cm

LUNGS + Skeleton

109
Q

Lesion showing benign Fibroblasts and activated macrophages exhibiting a STORIFORM (pinwheel) pattern + hemorrhage?

A

Fibrous cortical defects

110
Q

Benign tumor in which all components of bone are present but fail to differentiate into mature structures?

A

Fibrous dysplasia

111
Q

Polyostotic disease associated w. cafe au lait skin and endocrine abnormalities precocious puberty?

A

McCune-Albright syndrome –> Fibrous dysplasia

112
Q

What is the mutation present in all forms of Fibrous dysplasia?

A

GNAS gene mutation-> Constitutively active Gs protein

113
Q

20-30 yo with tumor located in RIBS or JAW bones?

A

Fibrous dysplasia

114
Q

Large masses that expand and distort bones. Cells exhibit curved trabeculae of woven bone (CHINESE characters)?

A

Fibrous dysplasia

115
Q

X-ray showing Ground Glass appearance of well defined bone lesions?

A

Fibrous dysplasia

116
Q

2nd MC tumor of undifferentiated neural cells occurring in White pt 10-15 yo?

A

Ewing Sarcoma

117
Q

What is the MC chromosomal abnormality associated with Ewing Sarcoma?

A

Translocation (11;22)

118
Q

Cells having scant glycogen-rich cytoplasm and presence of Tumor cells circled about a central fibrillary space indicating neural differentiation?

A

Homer-Wright rosettes= Ewing Sarcoma

119
Q

Painful enlarging mass in Diaphyses of Femur or Pelvic flat bones and characteristic Onion-skin pattern of bone deposition?

A

Ewing Sarcoma

120
Q

Tumor of Osteoblast precursors located in the Epiphysis of Femur or Tibia (knee), “Soap bubble” appearance on X-ray?

A

Giant cell tumor

121
Q

What are the 4 MC metastases to bone in Adult pts?

A

Prostate
Breast
Kidney
Lung

122
Q

What are MC sources of bony metastases in Children?

A 
Neuroblastoma
WIlms tumor
Osteosarcoma 
Ewing sarcoma
Rhabdomyosarcoma
123
Q

What sources of bony metastases cause Lytic lesions due to secretion of PGs + IL + PTH related proteins that stimulate Osteoclasts?

A

Kidney
Lung
Melanoma

124
Q

What bony metastases elicit an Osteoblastic response?

A

Prostate adenocarcinoma

125
Q

Curvilinear trabeculae of woven bones surrounded by benign Fibroblasts?

A

Fibrous dysplasia

126
Q

Islands of woven bone, typically involving the proximal Femur or Tibia?

A

Osteoid Osteoma

127
Q

Cartilage capped outgrowth at Epiphyseal growth plate?

A

Osteochondroma

128
Q

NON inflammatory Degeneration of articular cartilage?

A

Osteoarthritis

129
Q

Fibrillation and cracking of matrix occurs as superficial layers of cartilage are degraded and Osteophyte growth occur in what disease?

A

Osteoarthritis

130
Q

50-60 yo with deep aching pain that is exacerbated by use, morning stiffness would have what pathoneumonic JOINT involvement?

A

Osteoarthritis–> PIP + DIP

131
Q

What is a Heberden node?

A

Osteophytes located in the DIP joints of Women with Osteoarthritis

132
Q

Disease characterized by CD4 mediated inflammation and Anti-antibodies against Cyclic citrullinated peptides (CCPs)?

A

Rheumatoid arthritis

133
Q

What are some of targets for Anti-Abs in RA?

A 
Citrullinated fibrinogen 
Type II collagen
alpha enolase 
vimentin 
Immune complex deposition may occur
134
Q

What is IgM that binds to the Fc portion of self IgG and form immune complexes that deposit in joints?

A

RA–> Rheumatoid factor

135
Q

Symmetric arthritis affecting MCP and PIP joints?

A

RA

136
Q

Disease characterized by chronic papillary synovitis w. synovial cell hyperplasia, CD4+ cells, plasma cells, macrophage, and PMN infiltrates?

A

RA

137
Q

What is Proliferation of synovial lining cells mixed with inflammatory cells, granulation tissue, and fibrous connective tissue?

A

Pannus of RA

138
Q

Radiology showing joint effusions and juxtaarticular osteopenia with erosions and narrowing of joint spaces and loss of articular cartilage?

A

RA

139
Q

Characterized by central focus of fibrinoid necrosis surrounded by a palisade of macrophages and rimmed granulation tissue and lymphocytes?

A

Rheumatoid nodules

140
Q

What are some complications of RA?

A 
acute necrotizing vasculitis 
Fibrinous pleuritis 
Pericarditis Interstitial fibrosis 
Ocular changes
Sjogren syndrome
141
Q

Pathological changes in ligamentous attachments to bone, absences of rheumatoid factor, and involvement of Sacroiliac joints?

A

Seronegative spondyloarthropathies

*Ankylosing spondylitis

142
Q

Monosodium urate crystals precipitate from supersaturated body fluids and induce inflammatory reaction?

A

Gout

143
Q

What is a tophi?

A

Large crystalline aggregates surrounded by inflammatory lymphocytes, macrophages, and foreign body giant cells trying to engulf them

144
Q

Tophi are pathopneumonic for?

A

Gout

145
Q

What is a major organ complication associated with urate deposition?

A

Gouty Nephropathy

146
Q

What enzyme deficiency is associated with overproduction of Uric acid?

A

HGPRT deficiency– > Lesch-Nyhan syndrome

147
Q

What is the Pg of Gout?

A

Excess uric acid-> urate crystals-> activation of complement or phagocytksed-> induce IL-1 production-> PMNs recruited-> LTB4 and protease released causing damage

148
Q

What HLA subtype is associated with RA and DB?

A

HLA- DR4

149
Q

“Cant see, Cant pee, Cant climb tree?”

A

Reactive arthritis triad–> conjunctivitis + urethritis+ arthritis

150
Q

Joints with dislodged pieces of cartilage and subchondral bone forming loose bodies (“joint mice”) causing pain and restricting movement?

A

Osteoarthritis

151
Q

What is pseudpgout?

A

Chondorcalcinosis–> Deposition of Calcium pyrophosphate crystals

152
Q

What are the crystals in psuedogout made out of?

A

Calcium pyrophosphate

153
Q

Describe the morphology of pseudogout crystals?

A

Rhomboid shaped

weak birefringent–> Blue

154
Q

Pseudogout most commonly affect what joints?

A

Menisci, IV discs, Articular sufaces >50yo

155
Q

MCC of suppurative arthritis in <2yo?

A

H. flu

156
Q

MCC of suppurative arthritis in children?

A

S. aureus

157
Q

MCC of suppurative arthritis in young adults (sexually active women)?

A

Gonococcus

158
Q

MCC of suppurative arthritis in SCD pt?

A

Salmonella

159
Q

Pt w. “sudden onset f pain, redness, swelling, restricted movement, and fever?”

A

Suppurative arthritis

160
Q

What soft tissue tumor has high prevalence in children?

A

Rhabdomyosarcoma

161
Q

Which soft tissue tumors have high prevalence in young adults and late adults?

A

Synovial sarcoma – young

Liposarcoma + pleomorphic fibroblastic sarcomas –> late

162
Q

Most common benign tumor in adults presenting as slowly enlarging, painless, mobile mass?

A

Lipomas–> non-differentiated adipocytes

163
Q

Rapidly growing solitary mass of Fibroblastic proliferation occurring in forearm, chest, or back following trauma?

A

Nodular fasciitis

164
Q

Dupuytrens contracture and peyronies disease are examples of locally aggressive fibroblast proliferation associated with what genetics?

A

Superficial Fibromatoses (trisomy 3, 8)

165
Q

Desmoid tumors arise in abdominal wall and muscles of trunk and are associated with what disease/ genetics?

A

Gardeners syndrome
APC or B catenin mutations

Proliferation of MYOfibroblasts

166
Q

Malignant spindle cells arranged in “herringbone pattern?”

A

Fibrosarcoma

167
Q

Histological appearance shows poorly differentiated sarcoma with bizarre multinucleate cells and storiform architecture?

A

Pleomorphic fibroblastic sarcoma

168
Q

What sarcoma is associated with a t(2;13)?

A

Rhabdomyosarcoma

169
Q

Histology showing sarcoma with spindle cells having “Cigar shaped nuclei?”

A

Leiomyosarcoma

170
Q

Axonal degeneration is associated with 2nd myelin loss?

A

Wallerian degeneration

* followed by regeneration and myelination of new axon

171
Q

Rapidly progressive demyelinating disorder affecting motor axons that result in Ascending weakness that lead to respiratory muscle failure?

A

Guillian Barre

172
Q

What is the Pg of Guillian Barre?

A 
Autoimmune response to infections 
Campylobacter jejuni 
EBV
CMV
HIV
173
Q

Tx of Guillian barre?

A

Plasmapheresis or IV Ig

174
Q

Symmetrical demyelination of motor and sensory neurons caused by Autoimmune attacks, associated with SLE and HIV following a chronic relapsing or progressive course?

A

Chronic inflammatory demyelinating polyneuropathy

175
Q

Tx for Chronic inflammatory demyelinating polyneuropathy?

A

Plasmapheresis or IV Ig

176
Q

Autonomic neuropathy and distal symmetric sensorimotor polyneuropathy is MC in what pathology?

A

Diabetes

177
Q

Mononeuritis multiplex with a painful asymmetric mixed sensory and motor peripheral neuropathy is seen in what pathology?

A

Vasculitis–> Polyarteritis nodosa, Churg- Strauss, Wegners

178
Q

MCC of peripheral neuropathy?

A

Diabetes

179
Q

Autoimmune antibodies blocking function of postsynaptic ACh receptors at motor end plate resulting in degradation and depletion of receptors?

A

Myasthenia Gravis

180
Q

What are the 2 MC conditions associated with Myasthenia Gravis?

A

60%–> Thymic hyperplasia

20%–> Thymoma

181
Q

Pt presenting with Ptosis and diplopia is most often associated with what neuromuscular disorder?

A

Myasthenia Gravis

182
Q

Treatment for Myasthenia Gravis?

A

AChE inhibitors
immunosuppressives
Plasmapheresis
Thymectomy

183
Q

Caused by autoantibodies against Presynaptic Ca+ channels reducing the release of ACh?

A

Lamber-Eaton Syndrome

184
Q

Repetitive use of electrophysiologic stimulation of muscles increases/ decreases severity in what diseases?

A

Increases–> myasthenia gravis

decreases–> Lambert-Eaton syndrome

185
Q

Eaton-Lambert syndrome is associated with what pathology and what is the treatment?

A

Small cell carcinoma LUNG
Tx: Plasmapheresis + immunosuppression

** AChE inhibitor do NOT work

186
Q

What two infectious organisms are associated with neural transmission defects?

A

Clostridium botulinum–> inhibits ACh release

Clostridium tetani–> increased ACh release

187
Q

What is the difference btwn type I & type II muscle fibers?

A

Type I–> slow twitch “aerobic”

Type II–> Fast twitch “anaerobic”

188
Q

Prolonged disuse and Glucocorticoid use affect predominately which muscle fibers?

A

Atrophy of Type II

189
Q

Characterized by ongoing myofiber necrosis and regeneration and progressive replacement of muscle tissue with fibrosis and fat?

A

DMD and BMD

190
Q

X linked recessive disease caused by Frameshift mutation causing Absent dystrophin protein?

A

DMD

191
Q

BMD Pg?

A

Frameshift mutation of X chromosome== ABNORMAL dystrophin protein

192
Q

Where is the dystrophin gene found?

A

Short arm of X chromosome

193
Q

What is the role of dystrophin protein?

A

Stabilization during contraction

Possible signaling

194
Q

Child presenting with clumsiness, muscle weakness, and pseudo-hypertrophy of calf muscle?

A

DMD or BMD

195
Q

What are some of the complications of DMD?

A

Heart failure
Arrhythmias
increased CK levels due to muscle breakdown
Respiratory failure or pneumonia = Death

196
Q

Characterized by “sustained involuntary contraction of group of muscles” causing stiffness and difficulty releasing grip?

A

Myotonic dystrophy

197
Q

What is the genetics of myotonic dystrophy?

A

TRINUCLEOTIDE repeat disorder–> AD mutation in Dystrophia myotonica protein kinase (DMPK)

198
Q

Caused by CTG repeats in the 3’ untranslated end of DMPK mRNA?

A

Myotonic dystrophy

199
Q

MC primary malignant bone tumor: knee joint?

A

Osteosarcoma

200
Q

Adults + cartilage + trunk and limbs?

A

Chondrosarcoma

201
Q

Peripheral primitive neruoectodermal tumor, children and young adults, Diaphysis of bone?

A

Ewing’s sarcoma

202
Q

Immunological disorders with LUPUS?

A

anti-dsDNA (histones, RNA proteins, nucleolar Ags)
Anti-Sm
antiphospholipid antibodies
Lupus anti-coagulant

203
Q

Anti-SS-A (RO) + Anti-SS-B (La) are specific for what disease?

A

Sjogren syndrome

204
Q

Anti-scl-70 (topo I) ?

A

Systemic sclerosis

205
Q

Anti-centromere antibodies?

A

Scleroderma (CREST syndrome)

206
Q

Anti-Jo 1 antibodies?

A

Inflammatory myopathies

207
Q

Characterized with calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia?

A

Crest syndrom (systemic sclerosis)

Rheumatology (4 decks)

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