Exam 2 - CT

Question 1

synthesize and secrete the organic matrix and regulate its mineralization (build bone).

Answer 1

Osteoblasts

Question 2

are former osteoblasts entombed within a mineralized matrix and function as “mechanoreceptors” detecting strain and signal bone remodeling.

Answer 2

Osteocytes

Question 3

are involved in resorption of bone.

Answer 3

Osteoclasts

Question 4

a member of (TNF) receptor family, is expressed on the cell membranes of preosteoclasts and mature osteoclasts

Answer 4

RANK

Question 5

expressed by osteoblasts and marrow stromal cells. RANK stimulation by this leads to activation of the transcription factor NF-κB, which drives the expression of genes that stimulate osteoclast formation, fusion.

Answer 5

RANK Ligand (RANKL)

Question 6

RANKL can be blocked by this “decoy” receptor

Answer 6

Osteoprotegrin (OPG)

Question 7

(1) skeletal changes that include long, thin extremities, frequently associated with loose joints; (2) reduced vision as the result of dislocations of the lenses (ectopia lentis); and (3) aortic aneurysms.

Mutation in fibrillin-1 gene

Answer 7

Marfan Syndrome

Question 8

hyperextensible skin and hypermobile joints, but the category includes rare patients with other distinctive features 40% to 50% of patients.

Answer 8

Ehlers-Danlos syndrome (EDS)

Question 9

category of heritable disorders involving skin that is specifically characterized by blistering as a result of friction

Answer 9

Epidermolysis Bullosa (EB)

Question 10

severe decrease in bone mass that makes bones brittle. The disorder is frequently associated with blue sclerae, dental abnormalities (dentinogenesis imperfecta), progressive hearing loss, and a positive family history. Most patients have mutations in one of the two genes coding for type I collagen

Answer 10

Osteogenesis imperfecta (OI)

Question 11

disorder of cartilage and bone development primarily affecting the ends of the long bones in the arms and legs. There are two types which can be distinguished by their pattern of inheritance (Autosomal dominant and Autosomal Recessive)

Answer 11

Multiple Epiphyseal Dysplasia

Question 12

dwarfing condition which leads to abnormal epiphyseal development in the upper and lower extremities

Answer 12

Spondyloepiphyseal Dysplasia Congenital (SEDC)

Question 13

a type II collagen disorder , patients have marked shortening of the trunk, lumbar lordosis, and kyphoscoliosis. The phenotype of this disorder varies widely, ranging from severe with poor survival to mild.

Answer 13

Kniest dysplasia

Question 14

disease caused by a deficiency of vitamin C (ascorbic acid) and manifests as collagen defects, hemorrhagic diathesis, abnormalities in bone maturation, epiphyseal disease, lifting of the periosteum, hemarthroses, irritability, decreased appetite, delayed development, and pseudoparalysis related to bone pain.

Answer 14

Scurvy

Question 15

refers to deficient mineralization at the growth plate, as well as architectural disruption of this structure.

Answer 15

Rickets

Question 16

impaired mineralization of the bone matrix.

Answer 16

Osteomalacia

Question 17

acquired disorder characterized by reduced bone mass leading to increased risk of fracture. Bone loss occurs when the dynamic balance between bone formation by osteoblasts and bone resorption by osteoclasts tilts in favor of resorption.

Answer 17

Osteoporosis

Question 18

joint failure, a disease in which all structures of the joint have undergone pathologic change. Characterized by hyaline articular cartilage loss, present in a focal and, initially, nonuniform manner

Answer 18

Osteoarthritis

Question 19

synovial hypertrophy and synovitis of hands and feet, articular deterioration, and chronic joint inflammation along with the potential for extra-articular manifestations.

+/-anti-citrullinated protein antibodies (ACPAs) specific to this disease 90% (anti-CCP Antibody)

Answer 19

Rheumatoid Arthritis

Question 20

as persistent arthritis of at least 6 weeks duration in one or more joints in a person aged 16 or younger. A new nomenclature, Juvenile idiopathic arthritis (JIA) is being increasingly used to provide better definition, there are five subtypes of JIA by definition

Answer 20

Juvenile rheumatoid arthritis (JRA)

Question 21

a family of disorders characterized by inflammation around the entheses (sites of ligament insertion into bone), syndesmophytes, dactylitis, an association of the human leukocyte antigen (HLA) –B27, spinal fusion and radiographic sacroiliitis.

Answer 21

Ankylosing Spondylitis

Question 22

form of seronegative spondyloarthritis clinically associated with inflammatory back pain, migratory oligoarthritis, and extra-articular symptoms that typically follow a gastrointestinal or urogenital infection (Chlamydia trachomatis) by a minimum of 1 to a maximum of 3-6 weeks.

Answer 22

Reactive Arthritis (Reiter Syndrome)

Question 23

to disease that occurs in response to the presence of monosodium urate crystals in joints, bones, and soft tissues

Answer 23

Gout

Question 24

known as chondrocalcinosis, calcium pyrophosphate crystal deposition (CPPD) or as defined by EULAR as CPP crystal arthritis. Typically occurs in persons older than 50 years.

Answer 24

Pseudogout

Question 25

is a chronic multisystem disorder It is characterized by the presence of antinuclear antibodies plus 3 or more other criteria per the American College of Rheumatology.

Answer 25

Systemic lupus erythematosus (SLE)

Question 26

idiopathic inflammatory myopathy, characterized by the shared features of proximal skeletal muscle weakness and evidence of muscle inflammation

Answer 26

Polymyositis

Question 27

: An idiopathic inflammatory myopathy, characterized by the shared features of proximal skeletal muscle weakness and evidence of muscle inflammation just as polymyositis but characteristic rash is an additional criterion (heliotrope rash, shawl sign and grotton’s papules).

Answer 27

Dermatomyositis

Question 28

an inflammatory rheumatologic syndrome that manifests as pain and morning stiffness involving the neck, shoulder girdle and/or pelvic girdle in individuals older than age 50 years.

Answer 28

Polymyalgia Rheumatica

Question 29

is a multisystem, autoimmune disease, characterized by a functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs and production of autoantibodies

Answer 29

Scleroderma

Question 30

a slowly progressive autoimmune rheumatic disorder in which the exocrine glands are the primary target tissue. An alternative name is keratoconjunctivitis sicca.

Answer 30

Sjӧgren Syndrome

Question 31

Acute inflammatory disease of small blood vessels that occurs in the setting of ongoing antigen load (such as infection or drug) and an established humoral immune response (autoimmune).

Answer 31

Immune Complex Vasculitis

Question 32

systemic necrotizing vasculitis that typically affects medium-sized muscular arteries . May present with fever, fatigue, weight loss and lower extremity nodules.

Answer 32

Polyarteritis nodosa (PAN)