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BR-MS, Skin, CT

5/26/06

QuestionAnswer
Osteitis Fibrosa Cystica (von Recklinghausen's dz of bone) is caused by increased levels of: PTH d/t primary or secondary hyperparathyroidism; cystic spaces in bone are lined w/osteoclasts, brown d/t bleeds = "brown tumor of bone"
Achondroplasia is the most common cause of _ and is inherited in a _ fashion, it is d/t _: dwarfism (short limbs only); AD; premature sealing of narrow long bone epiphyses with the metaphasis
Osteogenesis imperfecta is a group of gene mutations causing: defective collagen synthesis & "brittle bone dz" (AD is mc); they have multiple fxs even at birth and blue sclera
Osteopetrosis is an _ in bone mass d/t _: increase; failure of osteoclast resorption of bone; it forms "marbelized bone;" most severe form is AD; fxs, narrow marrow (anemia), blindness, deafness d/t CN compression
Paget's disease of bone is most common in this population: elderly; causes skeletal deformities, pain d/t fx, high output cardiac failure d/t AV Shunts in bone; hearing loss d/t thickness of bone in ear; Osteosarcomas w/mosaic patterns
The most common cause of avascular necrosis is: steroid-induced vascular compression
Caisson Disease leads to necrosis via thrombosis and embolism; it is caused by gas emboli resulting from decompression syndrome (ex: rapid ascension from scuba diving)
Osteoporosis causes a decrease in: bone mass; d/t dec synth or inc resorption of bone (physical inactivity, inc thyroid levels, postmenopausal, inc cortisol or dec Ca levels); Fxs in spine and other wt bearing bones; Radiolucent on xray
Scurvy is d/t a lack of Vit C and defective _ and _ hydroxylation: proline, lysine (necessary for collagen synthesis); impaired bone formation, painful subperiosteal hemorrhage, osteoporosis, bleeding gums
Rickets and Osteomalacia are both related to a deficiency in Vit D (diet, sun, "renal osteodystrophy"); Craniotabes (thin skull), late fontanelle closure, dec height, Rachitic ROSARY from costochondral thickening, pigeon breast; Radiolucency in adults
Avascular necrosis is the death of: osteocytes and fat via: vascular compression, interruption (fx), thrombosis (sickle cell, caisson dz), injury; It results in Joint Pain, OA, esp head of femur (Legg-Calve-Perthes)
Legg-Calve-Perthes disease is AVN of the: head of femur
Pyogenic osteomyelitis infection is most commonly d/t: Staph aureus via hematogenous spread, adjacent injury, or open fx/surgery; Fever, pain, tender (metaphysis of distal femur, proximal tibia or humerus; forms Sequestrum and Involucrum
Sickle cell patients get osteomyelitis from: salmonella, although S. aureus is still #1
IVDA get osteomyelitis from: Pseudomonas
Tuberculosis osteomyelitis spreads to bone from elsewhere in the body; usu seen in hips, long bones, hands, feet, and spine (Pott's dz)
Tumors of bone and cartilage are rare; usu in lower limbs of young males; Metastasis is more common (80%) than 1* d/t prostate, breast and lung cancers
Osteochondroma is the most common _ tumor of bone benign; in metaphyses of long bones; men <25yo in distal femur or proximal tibia
The only way that osteochondromas undergo malignant transformation is when: they are the familial variant characterized by multiple lesions; these form OSTEOSARCOMAS
Giant cell tumor of bone is most common in: women age 20-55; it is benign w/ "SOAP Bubble" appearance on xray; spindley cells w/multinuclear giant cells occupy the distal femur or proximal tibia epiphyses
Osteomas are commonly found as _ tumors that protrude from the: benign; skull/face of men; it is mature/dense tissue
Osteoid Osteomas are identified on xray as a "_ _ _": Painful RadioLUCENT Nidus (surrounded by osteoblasts & vascular tissue); commonly in 20-30yo males (femur/tibia)
Osteosarcomas are bad boys malignant & in teenage boys; felt as "knee pain" w/METASTASIS to LUNG; growth under periostium forms "Codman's Triangle" on xray
Predisposing factors for osteosarcoma include (4): 1. Paget's dz of bone, 2. Li-Fraumeni syndrome (mutations of p53 on chrom 17), 3. Familial retinoblastoma, 4. Bone infarcts
Chondrosarcoma is a malignant _ tumor: cartilage; lobulated translucent tumor w/necrosis and calcification in mid-aged men in axial skeleton; Xray shows localized bone destruction
The most common bone sarcoma in children is: Ewing's
Ewing's sarcoma is a malignant _ tumor w/a gene translocation on: Cartilage; t(11;22); it forms "Homer-Wright Pseudorosettes;" 10-15yo boys (long bones, ribs, pelvis, scapula); Metastasis; Responds to chemo; painful, warm, swollen
Fibrous dysplasia forms "_ figures" on xray Chinese d/t replacement of bone w/disorganized fibrous tissue; may be a/w precosious puberty and cafe au lait spots
Metastatic bone cancer forms _ lesions unless arising from _ or _ lytic (lung, kidney); breast or prostate; a/w ecoptic PTHrP
Cafe au lai spots are seen in (3): Fibrous dysplasia of bone, Peutz-Jeghers syndrome, Neurofibromatosis type I
Two factors to consider in an acutely painful joint include: Infection and Urate deposition; they can be distinguished by: aspiration of joint fluid w/evaluation for WBCs, bacteria on gram stain or crystals
Osteoarthritis may affect _ joint: only one; ex: Distal InterphalangealJoints (DIPs) of hand; pain usu gets worse as day goes on
Rheumatoid arthritis is usu: bilateral and symmetric and does NOT affect DIP joints; pt is usu stiff in the morning, but relieved as day goes on
OA is the degeneration of: joint cartilage, followed by growth of surrounding bone; it is the MCC of arthritis; primary form has no risks, 2* is d/t trauma, metabolic disorder, or inflammation; KNEE is mc site; OSTEOPHYTES cause Heberden's (DIP) and Bouchard's (PIP) Nodes
RA is an _ _ reaction in _ and _; autoimmune inflammatory; joints, tendons; forms PANNUS of synovial hyperplasia over articular cartilage; Swan-neck and Boutonniere deformities (d/t inflammation, muscle atrophy, contracture); spares DIP joints
Rheumatoid Factor is an _ Ab to _ IgM; IgG; more common in women; a/w HLA-DR4
Ankylosing Spondylitis has a high a/w with _ HLA-B27 in Males; chronic low back pain, calcification of spinal ligaments, "Bamboo Spine;" also; apical lung fibrosis, aortic insufficiency, cauda equina syndrome
Psoriatic Arthritis may present similar to _, but has an _ association RA; HLA-B27; M=F; Asymmetric involvement of DIPs, PIPs, feet, ankles, knees; "PENCIL in CUP" deformity of proximal phalanges
Reiter's Syndrome has a classic triad of: Urethritis, Congunctivitis, Acute assymetric Arthritis; HLA-B27; 20-40yo males
Gout is an inflammatory rxn caused by: urate crystal deposition w/IgG opsonization & phagocytosis that triggers inflammation; can be precipitated by large meal or excess EtOH; PODAGRA of big toe, ear, Achilles; renal tubule damage; STRONG NEGATIVE BIREFRINGENCE under polarized light
Non-Gonoccocal Septic Arthritis is most commonly a/w these 2 bugs: Staph aureus and Strep species; MONOARTICULAR (knee); chills/fever, rash; G+ Stain of synovial fluid
Gonococcal Septic Arthritis is the most common arthritis in: sexually active adults; MONOARTICULAR; chills/fever, papular/pustular rash; G(-) Stain of synovium
Lyme Disease Arthritis is a late manifestation of infection with Borrelia burgdorferi; causes ERYTHEMA CHRONICUM MIGRANS (expanding bull's eye rash); KNEE, mycardial, pericardial and neurologic signs
Hypertrophic Osteoarthropathy, a sequelae of systemic dz) manifests as: clubbing of digits and periostitis; a/w Chronic lung dz, Cirrhosis, Inflammatory bowel dz, Congenital cyanotic heart disease
Lesch-Nyhan Syndrome is an X-linked deficiency of: Hypoxanthine-guanine phophoribosyl transferase (HGRPT) causing elevated URIC ACID levels, and manifests as MR, GOUT, Self-mutilation
Treatment of Lesch-Nyhan Syndrome Allopurinol; it blocks xanthine oxidase to prevent uric acid formation
Pseudogout is caused by deposits of: calcium pyrophosphate crystals; presentation is similar to gout, but crystals have weak POSITIVE birefringence under polarized light
Systemic Lupus Erythematosis immune complex deposit in vessels of most organs; Libman-Sacks endocarditis (mitral valve lesions on both sides); Wire-loop membranous GN; arthralgia/-itis; Malar rash; dsDNA Ab; anti-Sm; low C3 & C4; false+ syphilis; anti-cardiolipin Ab hypercoagulable
Marfan's syndrome is an AD disease d/t a deficiency of _, resulting in: fibrillin; skeletal, visual, cardiovascular defects; Arachnodactyly, Ectopia lentis (dislocation), mitral valve prolapse, proximal aortic aneurysm/dissection/valvular insufficiency
Ehlers-Danlos Syndrome is d/t a defect in collagen and _ formation, which causes: Elastin; hemorrhage; Hyperextensibility of Joints/Skin; fragility of tissue
Scleroderma (progressive systemic sclerosis) causes diffuse _ and _ of almost every organ d/t: fibrosis, degeneration; autoimmune reaction w/ Anti-scl-70 (ANA) Ab, or anti-centromere in CREST; Thick skin, fixed facies, claw-like sclerodactyly of hands, Raynauds, esophageal fibrosis
What does CREST stand for? Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Telangiectasia; more common in women; a/w anti-centromere Ab
Sjogren's Syndrome is an auto-immune reaction related to the following antibodies and clinical triad: anti-Ro (ssA), anti-La (ssB); Xerostomia, Xerophthalmia, presence of other CT/AI disease (esp RA);
Polymyositis is a unique autoimmune inflammatory disorder b/c it is a/w: malignancy; there is proximal weakness of extremities, high CK levels, and when skin is involved it's called dermato-
Mixed Connective Tissue Disease is rarely a/w renal involvement (as opposed to other CT dzs); anti-nRNP is highly specific; a/w Raynaud's, arthralgia, muscle inflammation, esophageal dysmotility
Sicca syndrome involves only xerostomia and xerophthalmia; an other autoimmune disease
Nerve damage that causes degeneration of the distal segment is d/t dispersion of Nissl substance as cell body undergoes chromatolysis
When a nerve is damaged, muscle continues to atrophy for _ weeks; in the PNS _ proliferate, in CNS _ cells proliferate 3; Schwann (help direct regenerating neuron); Astrocytes (forming scar that PROHIBITS regeneration)
If regenerating nerve fibers don't find the degenerating segment, what forms? a painful neuroma
Successful regeneration of a nerve occurs at a rate of _mm/_; allowing... 1mm/day; muscle to return to normal size
Muscles innervated by C5, C6 suprascapular, upper & lower subscapular, musculocutaneous, axillary
Which muscles are innervated by C8, T1? medial pectoral, medial brachial cutaneous, medial antebrachial cutaneous, ulnar
Erb-Duchenne Palsy is related to what kind of branchial plexus lesion? upper; C5-C6; it causes HYPER-ADDUCTION of arm in the "Waiter's tip" position (extended, adducted, forearm pronated)
Klumpke's palsy is a lesion the _ brachial plexus? lower (C7-T1); it causes HyperABDUCTION of arm; Claw hand d/t ulnar nerve; wrist/hand dysfxn; a/w Horner's syndrome
Horner's syndrome is a combo of: unilateral ptosis, miosis, anhydrosis d/t lesion in cervical sympathetic chain; seen w/ Pancoast's tumor and lower brachial plexus injuries
Claw hand is most noted d/t lesion of the ulnar nerve; commonly in kids w/Epiphyseal separation at medial epicondyle of humerus; there is Weak finger ADDuction, medial hand numbness, dysfxn of 4th, 5th digits
Radial nerve palsy causes a wristdrop deformity w/inability to extend the wrist or fingers; a/w fx of mid-humerus OR lead poisoning
The radial nerve is also involved in a more proximal pain syndrome: lateral epicondylitis (tennis elbow)
Carpal tunnel syndrome is d/t _ wrist motion that compresses the _ nerve: repetitive; median (C5-T1) via tendons in flexor retinaculum; Flexion = Pain; Extension = Pain relief; symptoms are often worse at night
The median nerve is also damaged in fractures to the: distal 1/3 of the humerus and elbow, causing total loss of thumb opposition; also via slashing the wrist
Medial Winging of the scapula is d/t injury to the: long thoracic nerve (C5,6,7); mastectomies can cause this injury; Pt has limited arm ABDuction/flexion; Serratus anterior paralysis; Medial scapula protrudes if pt pushes on wall
Lateral Winging of the Scapula is d/t lesion of this nerve: Accessory nerve, leading to trapezius paralysis
Anterior Shoulder dislocation can damate the _ nerve: axillary; usu d/t forced abduction and extension; loss of innervation to deltoid w/compromised shoulder flexion & extension; there is a palpable depression under acromion
A fracture to the surgical neck of the humerus from falling onto elbow can damage the _ nerve: axillary; similar to damage caused by shoulder dislocation: loss of innervation to deltoid, poor shoulder flexion/extension, palpable depression under acromion
Anterior Cruciate Ligament Tears are detected by a positive: anterior draw sign (lower leg pulled foward w/knee flexed); can manifest as Terrible Triad
The Terrible Triad includes: Torn Anterior Cruciate Lig, Medial Collateral Lig, medial meniscus
Clavicle fractures occur in the _ third of the bone with the following displacement: middle; proximal segment goes upward, distal segment goes downward; causes severe pain
Compartment syndrome occurs when hemorrhage fills the fascial separations in a limb, compressing neurovascular structures; usu d/t crush or fx injury;
Inversion sprain of the ankle is the... mc ankle injury and is caused by force inversion; stretches/tears lateral ligaments, esp ANTERIOR TALOFIBULAR
Scaphoid fracture may lead to avascular necrosis if untreated d/t retrograde perfusion; tenderness is in anatomical snuffbox
What makes up the anatomical snuffbox? extensor pollicis longus (dorsal), extensor pollicis brevis and abductor pollicus longus (palmar); scaphoid and trapezium (base)
Subacromial bursitis is the most common bursitis in the body; simply inflammation of the bursa
Tennis elbow is d/t a sprain of the radial collateral ligament (lateral epicondyle); pain occurs w/wrist extension and forearm supination; median nerve
Waddling gait is a limp d/t _ _ nerve injury superior gluteal; affects gluteus medius and minmus; pt cannot ABDuct the thigh; causes "Trendelenburg's sign"
Golf elbow involves: medial epicondyle
Trendelenberg's sign results in tilting of the pelvis to the side: opposite that of the injury when standing on the injured foot (also seen in hip dislocation or fx of neck of femur)
Smooth Muscle Fibers (maintain lumens of respiratory tract, GI tract and blood vessels) central nucleus, no bands, dense bodies (no z-lines), no T-tubules, Gap junctions, High regeneration, Ca from SR (released by IP3) and ECF, Ca binds Calmodulin
Cardiac Muscle Fibers Central nucleus, bands, Z-line (actin converges), T-tubules at Z-line, Intercalated discs btw cells, No regeneration, Ca from SR, ECF; Ca-induced Ca release, Ca binds Troponin
Skeletal Muscle Fibers Peripheral nucleus, Bands, Z-line, T-tubules at A-I junctions (triads), No cell-cell communication, NMJ, some regeneration, Ca from SR via depolarization of T-tubule, Ca binds Troponin
Type I Muscle Fibers sustained force; wt-bearing muscles; abundant lipid stores, few glycogen stores, aerobic/mitochondrial respiration, slow twitch, red color d/t blood supply
Type 2 Muscle Fibers sudden movment/directed action; few lipid, but many glycogen stores; anaerobic respiration (few mitochondria) easily fatigued); Fast Twitch; White coloration
Sarcomeres < Myofibril <Muscle fiber < Muscle Fasciculus Makes up a skeletal muscle
Which bands shorten with muscle contraction? H and I
Muscular pathology is more commonly d/t metabolic dyscrasias (induced or inherited) than neoplasms
The embryonal type of rhabdomyosarcoma is related to sarcoma botryoides, resulting in a "bunch of grapes" appearance in the vagina
Rhabdomyosarcoma most common soft tissue sarcoma of childhood; malignant and aggressive
Leiomyosarcoma uncommon malignant "cigar shaped" nuclei;
Leiomyoma most common tumor in women; benign; usu in uterus
Increased ion gap metabolic acidosis is usu d/t salicylate poisoning, alcohol intoxication, acute renal failure, diabetic ketoacidosis, aspirin ingestion
Normal anion gap metabolic acidosis is d/t diarrhea and renal tubular acidosis
Lactic acidosis causes inc anion gap Metabolic Acidosis d/t inc serum concentration from shock, sepsis, methanol, metformin, or liver failure; it can cause coma or death
Myasthenia Gravis is an autoimmune disease involving: antibodies againt the ACh receptor in NMJ; a/w HLA-DR3 and thymus disorders; weakness begins with ptosis, facial, ocular, limb proximal weakness; mc in women; Improves with EDROPHONIUM test
Duchenne's Muscular Dystrophy is inherited as an: X-linked recessive deficiency in DYSTROPHIN; causes lack of actin stabilization; Progressive proximal muscle weakness; PSEUDOHYPERTROPHY of calves; Gower's maneuver; Inc CK and Lactate DH; death via cardio/respiratory failure
Becker's Muscular Dystrophy is similar to Duchenne's but less severe
Mitochondiral Myopathy is transmitted through the... mtDNA from mother; "Ragged Red Fibers" are seen on muscle biopsy; Proximal muscle weakness;
McArdle's Type V glycogen storage disorder: deficiency in glycogen phosphorylase; affects Skeletal muscle only; weakness/cramping after exercise; no rise in lactate; No MR; Myoglobinuria in later life; OK prognosis; High [normal glycogen] in muscle
Pompe's Disease: Type II Glycogen Storage Disorder lysosomal a-glucosidase deficiency; Inborn defect; Affects Liver, Heart AND Muscle; Excess [glycogen] in abnormal cytosolic vacuoles; Normal blood sugar, CARDIOMEGALY & Death
Von Gierke's Disease: Type I Glycogen Storage Disorder glucose-6-phosphatase deficiency; Affects Liver, Kidney, Intestine; SEVERE fasting HYPOGLYCEMIA; Fatty liver/hepatomegaly; Hyperlacticacidemia & Hyperuricemia; Normal Glycogen structure, with INC STORAGE
Keloid Scarring is characterized by excessive, raised scarring that occurs after minor trauma; more common in African Americans; genetic predisposition
Xanthomas form from the accumulation of foam-filled histiocytes in the dermis; a/w HYPERLIPIDEMIA or Lymphoproliferative disorders; common on Achilles tendon and Extensor Tendons of fingers
Seborrheic Keratosis common, benign neoplasm in elderly; Raised papules and plaques that appear to be "pasted on"
Albinism is d/t a lack of melanin pigment production; Ocular type is limited to eyes (X-linked); Oculocutaneous type involves skin, eyes, hair (Auto Recessive; lack of tyrosinase enzyme needed to convert tyrosine to DOPA)
Albinism predisposes a person to actinic keratosis, basal cell carcinoma, squamous cell carcinoma and malignant melanoma
Hemangiomas are malformations of large vessels that form masses of blood-filled channels; "Port Wine Stain" birth marks are most common manifestation; Cavernous hemangiomas are a subset
Cavernous Hemangiomas are a/w von Hippel-Lindau disease; large caverous vascular spaces
Acanthosis nigricans is a thickening and hyperpigmentation of axilla, neck, groin; the malignant type is a/w underlying adenocarcinoma (usu lung)
Actinic Keratosis is a series of dysplastic changes that occur before the onset of squamous cell carcinoma; the buildup of keratin from sun exposure leads to a "Warty" appearance
Squamous cell carcinoma appears as microscopic islands of neoplastic cells with "Whorls of Keratin Pearls;" Malignant, rare to metastasize; seen as Ulcerated Scaling Nodules
Basal Cell Carcinoma appears as a pearl-like papule on sun-exposed areas; it is the most common skin malignancy; on histo is has a dark cluster with "Palisading Peripheral Cells;" rare to metastasize
Malignant Melanoma is an aggressive tumor arising from melanocytes; a/w sun exposure; has 2 growth patterns (benign radial and aggressive vertical); a/w S-100 tumor marker
Anticancer therapies for skin 5-FU, Dactinomycin, Methotrexate, Vincristine
Anticholinesterase Inhibitors Edrophonium, Neostigmine, Physostigmine
Antigout therapies allopurinol, colchicine, sulfinpyrazone
Muscle Relaxants Baclofen, Benzodiazepine, Dantrolene
Created by: bscaryp
 

 



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