XXI - Musculoskeletal System Flashcards
Known as “brittle bone disease”.
Osteogenesis Imperfecta (TOPNOTCH)Robbins Basic Pathology,8th ed., p802
This is a group of hereditary disorders caused by defective synthesis of type I collagen. Classic finding of a “blue sclerae” is seen in one type of this disorder.
Osteogenesis Imperfecta (TOPNOTCH)Robbins Basic Pathology,8th ed., p802
Characterized by , disproportionate shortening of the proximal extremities, bowing of the legs, and a lordotic (sway-backed) posture.
Achrondroplasia (TOPNOTCH)Robbins Basic Pathology,8th ed., p802
A group of rare genetic disorders characterized by reduced osteoclast-mediated bone resorption and therefore defective bone remodelling. Literally means “stone bone”.
Osteopetrosis(TOPNOTCH)Robbins Basic Pathology,8th ed., p802
The hallmark of this disease is loss of bone which tends to be most conspicuous in trabecular bone. The bony trabeculae are thinner and more widely separated than usual, resulting in an increased susceptibility to fractures.
Osteoporosis (TOPNOTCH)Robbins Basic Pathology,8th ed., p804
Characterized by repetitive episodes of frenzied, regional osteoclastic activity and bone resorption, followed by exuberant bone formation, and finally by an apparent exhaustion of cellular activity. Pathognomonic feature is a “mosaic pattern” of lamellar bone.
Paget Disease (Osteitis Deformans) (TOPNOTCH)Robbins Basic Pathology,8th ed., p806
The hallmark of this disease is increased osteoclastic activity, with bone resorption. This leads to characteristic radiographic changes best seen along the radial aspect of the middle phalanges of the second and third fingers.
Hyperparathyroidism (TOPNOTCH)Robbins Basic Pathology,8th ed., p808
Characterized by dead bone with empty lacunae is interspersed with areas of fat necrosis and insoluble calcium soaps. The cortex is usually not affected because of collateral blood supply.
Osteonecrosis (avascular necrosis)(TOPNOTCH)Robbins Basic Pathology,8th ed., p809
Inflammation of the bone and marrow cavity.
Osteomyelitis(TOPNOTCH)Robbins Basic Pathology,8th ed., p809
What do you call the dead entrapped bone seen in infected sites of bones affected with osteomyelitis?
Sequestrum(TOPNOTCH)Robbins Basic Pathology,8th ed., p809
What do you call the shell of living tissue around a segment of devitalized bone?
Involucrum (TOPNOTCH)Robbins Basic Pathology,8th ed., p809
Benign bone tumor characterized as exophytic growths attached to bone surface of the face and skull, which may histologically resemble normal bone.
Osteoma(TOPNOTCH)Robbins Basic Pathology,8th ed., p811
Benign bone tumor that are cortical tumors, histologically described as interlacing trabeculae of woven bone. Characteristic symptom is pain. Common locations include the metaphyses of the femur and tibia.
Osteoid osteoma(TOPNOTCH)Robbins Basic Pathology,8th ed., p811
These bone-forming lesions arise in vertebral transverse and spinous processes of the vertebral column, which may be histologically similar to osteoid osteoma.
Osteoblastoma(TOPNOTCH)Robbins Basic Pathology,8th ed., p811
A bone-producing malignant mesenchymal tumor, characterized as gritty, gray-white tumors, often exhibiting hemorrhage and cystic degeneration. The production of mineralized or unmineralized bone (osteoid) by malignant cells is essential for diagnosis.
Osteosarcomas(TOPNOTCH)Robbins Basic Pathology,8th ed., p811
These are relatively common benign hyaline cartilage-capped outgrowths attached by a bony stalk to the underlying skeleton.
Osteochondromas (TOPNOTCH)Robbins Basic Pathology,8th ed., p814
These are benign tumors of hyaline cartilage, described as gray-blue, translucent well-circumscribed nodules. May be seen in metaphyseal regions of tubular bones of the hands and feet. On x-ray, the unmineralized nodules of cartilage produce well-circumscribed oval lucencies surrounded by thin rims of radiodense bone (O-ring sign).
Enchondromas (TOPNOTCH)Robbins Basic Pathology,8th ed., p814
Chondromas seen on the bone surface.
Juxtacortical chondromas(TOPNOTCH)Robbins Basic Pathology,8th ed., p814
These lesions arise within the medullary cavity of the bone to form an expansile glistening mass that often erodes the cortex. They exhibit malignant hyaline and myxoid cartilage.
Conventional chondrosarcomas (TOPNOTCH)Robbins Basic Pathology,8th ed., p814
These tumors are characterized as viscous and gelatinous, with the matrix oozing from the cut surface. Spotty calcifications are typically present, with central necrosis creating cystic spaces.
Myxoid chondrosarcomas(TOPNOTCH)Robbins Basic Pathology,8th ed., p815
Presents as sharply demarcated radiolucencies surrounded by a thin zone of sclerosis. They are gray to yellow-brown, and microscopically are cellular lesions composed of cytologically benign fibroblasts and macrophages. The fibroblasts classically exhibit a “storiform (pinwheel) pattern”.
Fibrous Cortical Defect and Nonossifying Fibroma (TOPNOTCH)Robbins Basic Pathology,8th ed., p815
A benign tumor of the bone wherein all components of normal bone are present, but they fail to differentiate into mature structures. Curved trabeculae of woven bone mimick “Chinese characters” histologically.
Fibrous Dysplasia (TOPNOTCH)Robbins Basic Pathology,8th ed., p816
Primary malignant small round-cell tumor of bone and soft tissue, characterized by Sheets of small round cells with scant, cleared cytoplasm, circled about a central fibrillary space or “Homer-Wright rosettes”.
Ewing sarcoma (TOPNOTCH)Robbins Basic Pathology,8th ed., p817
A relatively uncommon benign tumor usually arising in individuals in their 20s to 40s. These are large and red-brown lesions with frequent cystic degeneration, composed of uniform oval mononuclear cells with frequent mitoses, with scattered osteoclast-type giant cells containing 100 or more nuclei.
Giant-cell tumors (GCTs)(TOPNOTCH)Robbins Basic Pathology,8th ed., p817
These are the most common malignant tumor of bone.
Metastatic tumors (TOPNOTCH)Robbins Basic Pathology,8th ed., p818
Pathways of metastasis to the bone.
1) direct extension, (2) lymphatic or hematogenous dissemination, (3) intraspinal seeding(TOPNOTCH)Robbins Basic Pathology,8th ed., p818
Characterized by fibrillation and cracking of the articular cartilage matrix, bone eburnation, and bony outgrowths/spurs (osteophytes). Full-thickness portions of the cartilage are lost, and the subchondral bone plate is exposed.
Osteoarthritis (TOPNOTCH)Robbins Basic Pathology,8th ed., p819
Characterized by a dense neutrophilic infiltrate permeating the synovium and synovial fluid. Long, slender, needle-shaped monosodium urate crystals are frequently found in the cytoplasm of the neutrophils as well as in small clusters in the synovium.
Acute gouty arthritis(TOPNOTCH)Robbins Basic Pathology,8th ed., p821
Results from repetitive precipitation of urate crystals during acute attacks. The synovium becomes hyperplastic, fibrotic, and thickened by inflammatory cells, forming a pannus that destroys the underlying cartilage.
Chronic tophaceous arthritis (TOPNOTCH)Robbins Basic Pathology,8th ed., p821
Formed by large aggregations of urate crystals surrounded by an intense inflammatory reaction of lymphocytes, macrophages, and foreign-body giant cells, attempting to engulf the masses of crystals. Pathognomonic feature of gout.
Tophi (TOPNOTCH)Robbins Basic Pathology,8th ed., p821
Refers to multiple different renal complications associated with urate deposition, variously forming medullary tophi, intratubular precipitations, or free uric acid crystals and renal calculi.
Gouty nephropathy(TOPNOTCH)Robbins Basic Pathology,8th ed., p821
Also known as chondrocalcinosis or-more formally-calcium pyrophosphate crystal deposition disease. The crystals are weakly birefringent, rhomboid in shape with blunted ends.
Pseudogout (TOPNOTCH)Robbins Basic Pathology,8th ed., p823
Main causative agent of suppurative arthritis in children under 2 years old.
Haemophilus influenzae(TOPNOTCH)Robbins Basic Pathology,8th ed., p824
Main causative agent of suppurative arthritis in older children and adults.
Staphylococcus aureus(TOPNOTCH)Robbins Basic Pathology,8th ed., p824
Main causative agent of suppurative arthritis in late adolescence and young adulthood.
Neisseria gonorrhea(TOPNOTCH)Robbins Basic Pathology,8th ed., p824
Individuals with sickle cell disease are prone to developing osteomyelitis and suppurative arthritis caused by this agent.
Salmonella(TOPNOTCH)Robbins Basic Pathology,8th ed., p824
Arthritis caused by infection with the spirochete Borrelia burgdorferi, transmitted by deer ticks of the Ixodes ricinus complex.
Lyme arthritis (TOPNOTCH)Robbins Basic Pathology,8th ed., p824
A small (<1.5 cm) cyst located near a joint capsule or tendon sheath, most commonly the wrist, which manifest as a firm to fluctuant pea-sized nodule.
Ganglion cyst(TOPNOTCH)Robbins Basic Pathology,8th ed., p825
The most severe and the most common form of muscular dystrophy. Characterized by marked variation in muscle fiber size, caused by concomitant myofiber hypertrophy and atrophy. The definitive diagnosis is based on the demonstration of abnormal staining for “dystrophin” in immunohistochemical preparations.
Duchenne muscular dystrophy (DMD) (TOPNOTCH)Robbins Basic Pathology,8th ed., p825
This can present as either acute or chronic proximal muscle weakness, and can precede the onset of other signs of thyroid dysfunction. Findings include myofiber necrosis, regeneration, and interstitial lymphocytes.
Thyrotoxic myopathy (TOPNOTCH)Robbins Basic Pathology,8th ed., p830
This can occur with binge drinking, where there is an acute toxic rhabdomyolysis with accompanying myoglobinuria that can cause renal failure. On histology, there is myocyte swelling and necrosis, myophagocytosis, and regeneration.
Ethanol myopathy (TOPNOTCH)Robbins Basic Pathology,8th ed., p830
An autoimmune disorder of the neuromuscular junction characterized by muscle weakness which first manifests in the extraocular muscles. Caused by circulating antibodies to the skeletal muscle acetylcholine receptors (AChRs).
Myasthenia gravis (TOPNOTCH)Robbins Basic Pathology,8th ed., p830