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BR-MS, Skin, CT
5/26/06
| Question | Answer |
|---|---|
| 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 |