kRheumatology/Musculoskeletal/Immunology System: Kaplan Companion to First Aid for USMLE Step 2 CK Flashcards

Terms Definitions
Q001. Ankylosing Spondylitis - What is it; Risk factors
A001. Chronic inflammatory disease of spine and pelvis; sacroilitis => fusion of affected joints; 20's - 30's; seronegative; HLA-B27; risk factors – male, family History
Q002. Ankylosing Spondylitis - History/PE
A002. History - hip and low back pain, worse with inactivity, worse in mornings, better with activity, thru day; PE - hip pain and stiffness; limited chest expansion; loss of lumbar lordosis; positive Schober test - decreased spine flexion; may have anterior uveitis,; 3rd degree heart block; aortic insufficiency => CHF
Q003. Ankylosing Spondylitis - Dx
A003. Dx based on clinical & XR; HLA-B27; ESR - may be elevated; RF & ANA - negative; spine and pelvic XR - "bamboo spine"
Q004. Ankylosing Spondylitis - Tx
A004. NSAIDs; exercise; physical therapy
Q005. Reiter's - What is it
A005. "Can't see, can't pee, can't climb a tree"; conjunctivitis; uveitis; urethritis; arthritis; may also have - keratoderma blennorrhagicum, circinate balanitis; after infection with – campylobacter, Shigella, salmonella, chlamydia, ureaplasma; seronegative; HLA-B27; young men
Q006. Psoriatic Arthritis - What is it
A006. Chronic inflammatory arthritis; psoriasis of skin; psoriatic nail lesions; DIP joints; sausage-shaped digits; "mushroom caps"; HLA-B27; seronegative
Q007. Rheumatoid Arthritis - What is it
A007. Chronic autoimmune diseases; peripheral joints - symmetrically inflamed; progressive destruction of articular tissue; erosion of - cartilage, bones, tendons; systemic Sxs; atlanto-axial subluxation; ruptured Baker's cyst; MC females 35-50; HLA-DR4; T cell activation - why HIV improves pre-existing
Q008. Rheumatoid Arthritis - History/PE
A008. Insidious onset of morning stiffness > 1 hr; painful, warm swelling of multiple symmetric joints > 6 wks; MC - wrist, MCP, PIP, any joint may be involved; axial rare except upper cervical spine; ulnar deviation; MCP joint hypertrophy, swan-neck, boutonniere; subq, painless Baker's cysts
Q009. Rheumatoid Arthritis - Dx
A009. RF (anti-Fc IgG Ab); ESR; XR; synovial fluid aspiration - slightly turbid, decreased viscosity, WBC 3,000-50,000
Q010. Rheumatoid Arthritis - Tx
A010. NSAIDs; COX-2 inhib; severe – corticosteroids, methotrexate, hydroxychloroquine sulfate, gold, azathioprine; operative may be necessary.
Q011. Felty's Triad - What is it
A011. RA; splenomegaly; neutropenia
Q012. Juvenile Rheumatoid Arthritis - What is it
A012. Nonmigratory, nonsuppurative, mono- and poly-arthropathy; bony destruction; < 16 y/o; lasts > 6 wks; 95% - disease resolves by puberty
Q013. Juvenile Rheumatoid Arthritis - History/PE
A013. 3 patterns; all patterns may have – fever, nodules, erythematous rashes, pericarditis, fatigue; pauciarticular - MC form, < 4 joints, weight-bearing joints; ANA type - MC subtype, asymm involvement of lg. joint, iridocyclitis – insidious, if not treated => blind; RF type - poor prognosis, HLA-B27 - boys also have spondyloarthropathies,; polyarticular - > 5 joints, similar to adult RA, symmetric, may develop iridocyclitis; Still's disease (systemic) - least common, daily high spiking fever, evanescent salmon-colored rash, hepatosplenomegaly, serositis
Q014. Juvenile Rheumatoid Arthritis - Dx
A014. No diag tests; XR; RF - positive in 15%; ANA - may be positive; RBC decreased; elevated – ESR, WBC, platelets; normal ESR doesn't exclude Dx
Q015. Juvenile Rheumatoid Arthritis - Tx
A015. NSAIDs; corticosteroids; ROM and strength exercises; methotrexate; monitor iridocyclitis
Q016. Scleroderma - What is it
A016. Progressive systemic sclerosis; multisys disease; thickening of skin from; accumulation of connective tissue; MCC of death - pulmonary; CREST - better prognosis; MC - females 30-50
Q017. Scleroderma - History/PE
A017. All have thick skin & Raynaud's; Lung - pulmonary fibrosis => restrictive lung disease and cor pulmonale; GI - esophageal dismotility, achalasia, decreased motility of small intestines, dilation of large intestines, large diverticula; renal - malignant HTN => ARF; CREST - limited form
Q018. CREST - What is it
A018. Calcinosis; Raynaud's; esoph dysmotility; sclerodactyly; telangiectasias
Q019. Scleroderma - Dx
A019. RF; ANA; eosinophilia; anticentromere Ab - CREST; anti-Scl-70 - systemic
Q020. Scleroderma - Tx
A020. Systemic glucocorticoids; penicillamine - skin changes; Ca2+ channel blockers - Raynaud's; ACE inhibitor - renal disease, HTN
Q021. SLE - What is it
A021. Multisys autoimmune; women - especially Black; Ab-mediated cellular attack; deposits of Ag-Ab complexes; lupus-like syndrome - drug-induced lupus, Hydralazine, penicillamine, Procainamide, INH, methyldopa; rash only, anti-histone Ab, resolves when drug discont.
Q022. SLE - History/PE
A022. DOPAMINE RASH; Discoid rash; Oral ulcers; Photosensitivity; Arthritis; Malar rash; Immunologic criteria; Neuro Sxs - lupus cerebritis, seizures; Elevated ESR; Renal disease; ANA positive; Serositis; Hematologic abnormalities; Sxs exacerbated - sun exposure, pregnancy
Q023. SLE - Dx
A023. Anemia; leukopenia; TCP; ANA; anti-dsDNA; anti-Sm Ab; active attacks - decreased C3 and C4; antihistone Ab - drug-induced; anti-Ro (SSA) - neonatal lupus, neonatal congenital heart block if pregnant, screen; antiphospholipid Ab – hypercoagulability, thromboembolic disease, recurrent spontan abortions, stillbirths; Tx - LMWH
Q024. SLE - Tx
A024. Arthritis - NSAIDs; major organ involvement - steroids; flare-ups - steroids; antimalarials - rash; nephritis – cytotoxics, cyclophosphamide, azathioprine
Q025. SLE - Complications
A025. Infections; progressive impairment of – brain, heart, lungs, kidney; increased risk - spontaneous abortion (antiphospholipid Ab); neonates - increased risk of congenital complete heart block (anti-Ro/SSA)
Q026. Temporal Arteritis - What is it
A026. Giant cell arteritis; women 2x's > men; > 50 y/o; subacute granulomatous inflamm; large vessels – aorta, external carotid - especially temporal, vertebral a; blindness - occlusion of central retinal; polymyalgia rheumatica - 50%
Q027. Temporal Arteritis - History/PE
A027. Headache; temporal tenderness; scalp pain; jaw claudication; fever; monocular blindness - transient or permanent; weight loss; malaise; myalgia; arthralgia
Q028. Temporal Arteritis - Dx
A028. ESR; ophthalmologic evaluation; Biopsy - temporal artery
Q029. Temporal Arteritis - Tx
A029. If suspect TA - start steroids before Biopsy; hi-dose prednisone - 1-2 mos. before taper; monitor eye exams
Q030. Henoch-Schönlein Purpura - What is it
A030. Immune-mediated vasculitis; IgA immune complexes; small arteries - GI tract; skin; joints; kidney; 2-11 y/o; degree of renal involvement; det. prognosis
Q031. Henoch-Schönlein Purpura - History/PE
A031. Palpable purpura - buttocks and legs; asymmetric, migratory; periarticular swelling; abdominal pain; preceding URI
Q032. Henoch-Schönlein Purpura - Tx
A032. Usually self-limited; Tx supportive; steroids - Sxs
Q033. Henoch-Schönlein Purpura - Complications
A033. GI bleeding; intussusception; glomerulonephritis
Q034. Polymyalgia Rheumatica - What is it
A034. Elderly females; close association with; temporal arteritis
Q035. Polymyalgia Rheumatica - History/PE
A035. Pain and stiffness – shoulder, pelvic girdle; especially severe in morning or after inactivity; minimal joint swelling; fever, malaise, weight loss; hard to - get out of chair, lift arms above head; muscles not weak; pain limits muscle effort
Q036. Polymyalgia Rheumatica - Dx
A036. Clinical; anemia; ESR high
Q037. Polymyalgia Rheumatica - Tx
A037. Low-dose prednisone
Q038. Inflammatory Myopathies - What Are They
A038. Inflammatory muscle diseases; progressive muscle weakness; polymyositis; dermatomyositis; inclusion body myositis
Q039. Inflammatory Myopathies - History/PE
A039. Difficulty with proximal muscles - lifting objects, combing hair, getting up from chair; difficulty with distal later - writing; dermatomyositis - heliotrope rash, Grottron's papules
Q040. Inflammatory Myopathies - Dx
A040. Most sensitive test - CK - elevated; aldolase - elevated; anti-jo-1 autoAb; EMG; muscle Biopsy - confirms
Q041. Inflammatory Myopathies - Tx
A041. Steroids - polymyositis; dermatomyositis; inclusion body myositis - resistant to immunosuppressives
Q042. Fibromyalgia - What is it
A042. Connective tissue d/o; myalgia; weakness; fatigue; no inflammation; associated with – depression, anxiety, IBS; MC women > 50
Q043. Fibromyalgia - History/PE
A043. Pain when palpate at least 11 of 18 tender points; palpate "trigger point"=> pain, body aches; fatigue; sleep disorders
Q044. Fibromyalgia - Dx
A044. Dx of exclusion; myofascial pain syndrome - < 11 tender points or nonfibromyalgia-associated tender points
Q045. Fibromyalgia - Tx
A045. Stretching; heat application; hydrotherapy; transcutan electrical; nerve stimulation; patient education; stress reduction; low-dose antidepressants
Q046. Sjogren's - What is it
A046. Chronic autoimmune disease; lymphocytes infiltrate exocrine glands; can become progressive => systemic => lymphoproliferative (malignant lymphome)
Q047. Sjogren's - History/PE
A047. Xerostomia; dry eyes; keratoconjunctivitis sicca; "sandy feeling under eyes"; dental caries; parotid enlargement
Q048. Sjogren's - Dx
A048. Schirmer's test - decreased tear production; rose bengal stain - corneal ulcers; anti-Ro (SSA) & anti-La (SSB); Biopsy - lymphocytes infiltrate salivary gland
Q049. Sjogren's - Tx
A049. None; symptomatic Tx - artificial tears
Q050. What drugs can induce SLE?
A050. Procainamide; Hydralazine; Isoniazid; Chlorpromazine; Methyldopa; Quinidine
Q051. What are the features of SLE?
A051. Fever, anorexia, malaise, weight loss; Butterfly rash; Nail fold infarcts; Splinter hemorrhages; Raynaud's Phenomenon
Q052. What is the diagnostic criteria for SLE?
A052. Requires 4 of 11 criteria:; Malar rash; Discoid rash; Photosensitivity; Oral ulcers; Arthritis; Serositis; Renal disorder; Seizures, psychosis; Hematologic; Immunologic; Positive ANA
Q053. How do you treat SLE?
A053. Refer to rheumatologist; NSAIDS; Antimalarial drugs; Steroids; Immunosuppressive drugs; Anticoagulation
Q054. What is antiphospholipid antibody syndrome?
A054. Recurrent venous or arterial occlusions; Recurrent fetal loss; Thrombocytopenia; Antiphospholipid antibodies; No other features of SLE
Q055. What is scleroderma?
A055. diffuse fibrosis of the skin and internal organs
Q056. What are the two forms of scleroderma?
A056. Limited - CREST; Diffuse - Renal failure, intersitial lung disease, cardiac disease
Q057. What is CREST syndrome?
A057. Calcinosis; Raynaud's syndrome; Esophageal dysmotility; Sclerodactyly; Telangiectasia
Q058. What are the clinical findings of scleroderma?
A058. Polyarthralgia, Raynaud's phenomenon; Thickened skin, with loss of normal folds; telangiectasia, pigmentation, depigmentation; Dysphagia; Restrictive lung disease; Pericarditis, heart block; Renal disease
Q059. What labs do you see with scleroderma?
A059. mild anemia; normal ESR; positive ANA; SCL-70; Anticentromere antibody (50% w/ CREST)
Q060. How do you treat scleroderma?
A060. Severe Raynaud's phenomenon - nifedipine, losartan; Esophageal reflux - H2 blockers, PPI, antacids; Bacterial overgrowth and pseudoobstruction - octreotide; Malabsorption d/t bacterial overgrowth - tetracycline
Q061. True or False: Prednisone is commonly used in the treatment of scleroderma.
A061. False
Q062. What is Sjogren's Syndrome?
A062. chronic dysfuction of exocrine glands
Q063. What are the clinical findings of Sjogren's Syndrome?
A063. keratoconjunctivitis; burning, itching, FB sensation in eye; Dry mouth; Loss of taste and smell; Parotid enlargement; Desiccation of nose, throat, larynx, vagina, skin; Dysphagia; Pancreatitis
Q064. What labs do you see with Sjogren's syndrome?
A064. Mild anemia; Leukopenia; Eosinophilia; Polyclonal hypergammaglobulinemia; Positive RF; ANA; Anti-SS-a and Anti-SS-b; Thyroid-associated autoimmunity; Schirmer test; Lip biopsy; Parotid gland biopsy
Q065. What does a Schirmer test measure?
A065. quantity of tears secreted
Q066. How do you treat Sjogren's syndrome?
A066. Supportive and symptomatic
Q067. What drug can relieve xerostomia?
A067. Pilocarpine
Q068. People with Sjogren's are at risk for developing what?
A068. lymphoma
Q069. What is polymyalgia rheumatica?
A069. pain and stiffness in neck, shoulder, and pelvic girdle
Q070. What are the characteristics of polymyalgia rheumatica?
A070. pain and stiffness in neck, shoulder, and pelvic girdle; symmetrical symptoms; more prolonged in the morning; low-grade fever, fatigue, weight loss
Q071. Does PMR have muscle weakness?
A071. No
Q072. What labs do you see with polymyalgia rheumatica?
A072. ESR and CRP elevated; Anemia and mild thrombocytosis
Q073. How do you treat polymyalgia rheumatica?
A073. Prednisone 10-20 mg/day for 6 months-2 years
Q074. Know about Giant Cell Arteritis.
A074. Symptoms; Treatment
Q075. What is polyarteritis nodosa?
A075. medium-sized necrotizing arteritis
Q076. What can cause polyarteritis nodosa?
A076. Idiopathic; Hep B or C; Drugs
Q077. What drugs can cause polyarteritis nodosa?
A077. PTU; Hydralazine; Allopurinol; Penicillamine; Sulfasalazine
Q078. What are the signs and symptoms of polyarteritis nodosa?
A078. fever, malaise, weight loss; pain in extremities; Foot drop; livedo reticularis; HTN
Q079. What labs do you see in polyarteritis nodosa?
A079. anemia; elevated ESR; Leukocytosis; Hep B or C
Q080. How do you confirm a diagnosis of polyarteritis nodosa?
A080. tissue biopsy
Q081. How do you treat polyarteritis nodosa?
A081. corticosteroids (up to 60mg/day); immunosuppressive agents (cyclophosphamide); refer to rheumatologist
Q082. What is a complication of polyarteritis nodosa?
A082. mesenteric vasculitis
Q083. What is Reiter's syndrome?
A083. Reactive arthritis
Q084. What causes Reiter's syndrome?
A084. dysentery - Shigella, Salmonella, Yersinia, Campylobacter; STD - Chlamydia
Q085. What are the clinical findings of Reiter's syndrome?
A085. Urethritis; Conjunctivitis; Mucocutaneous lesions; Aseptic arthritis; Fever, weight loss; Aortic regurgitation
Q086. With Reiter's syndrome, what do you see on x-ray?
A086. permanent or progressive joint disease (sacroiliac or peripheral joints)
Q087. How do you treat Reiter's syndrome?
A087. NSAIDS; antibiotics at time of infection; tetracycline; sulfasalazine; methotrexate; anti-TNF agents
Q088. True or False: Once symptoms of Reiter's syndrome develop, antibiotics can reduce the severity of the disease.
A088. False
Q089. Sjogren's si/sx
A089. triad - keratoconjuctivitis sicca, xerostomia, arthritis; also, pancreatitis, fibrinous pericarditis
Q090. Sjogren's labs
A090. + ANA, antiRo/antiLa abs, 70% RF +; a/w HLA-DR3
Q091. Behcet's si/sx
A091. 20 yo; oral/genital ulcers; uveitis, arthritis, other derm disease
Q092. Dermatomyositis si/sx
A092. young kids, old peeps; symmetric weakness of proximal muscles (can see dysphonia/dysphagia) look for trouble getting out of chair. skin involvement = heliotrope rash around eyes + periorbital edema
Q093. Dermatomyositis labs
A093. high ESR, high CPK, abnormal EMG, muscle biopsy --> inflammation + ANA
Q094. Kawasaki's si/sx
A094. <5 yo; truncal rash, fever >104x(>5d), conjunctival injection, cervical LAD, strawberry tongue, skin desquamation. -> coronary vessel vasculitis, aneurysms, MI
Q095. Kawasaki Tx
A095. NOT STEROIDS; aspirin + IVIG
Q096. Polyarteritis nodosa si/sx
A096. Associated with hepatitis B + cryoglobulinemia; fever, abdominal pain, weight loss, renal disorder, peripheral neuropathies.
Q097. Polyarteritis nodosa labs
A097. high ESR, leukocytosis, anemia, hematuria/proteinuria. Biopsy of medium-sized vessels --> vasculitis
Q098. Scleroderma si/sx
A098. C - calcinosis; R - raynaud's; E - esophageal dysmotility; S - sclerodactyly; T- telangectasia; heartburn, mask-like leathery facies.
Q099. Scleroderma labs
A099. Positive ANA; positive anticentromere Abs = CREST; positive antitopoisomerase = scleroderma
Q100. What drugs can cause secondary SLE?
A100. procainamide; hydralazine; dilantin (phenytoin); sulfanomide; INH
Q101. What is the dx lab in secondary SLE?
A101. Positive antihistone Abs
Q102. What are the dx labs in SLE?
A102. Positive ANA screen; positive antiSm or anti-ds-DNA
Q103. What are other lab findings in SLE?
A103. false positive for VDRL or RPR for syphilis; PTT falsely elevated; (SLE actually thrombogenic)
Q104. Takayasu's arteritis si/sx
A104. East Asian women 15-30yo; "pulseless" in 1 or both arms. carotid involve --> neuro sign, stroke; CHF
Q105. Wegener's granulomatosis si/sx
A105. old peep; nose bleeds, nasal perforation, hemoptysis, dyspnea, hematuria, ARF
Q106. What's the diff between Wegener's and Goodpasture?
A106. Weg = old peep, +ANCA; Goodpasture = young peep, positive antiglomerular antibody
Q107. Polymyalgia Rheumatica si/sx
A107. Women >50yo; Pectoral and pelvic girdles, neck involvement; a/w temporal arteritis
Q108. Polymyalgia Rheumatica labs
A108. Really high ESR; normal muscle biopsy/EMG
Q109. Rheumatoid factor
A109. It is an antibody against the Fc portion of IgG = RF & IgG form immune complexes. which is itself an antibody, IgM or IgA type.
Q110. RF or RhF positive in:; is often determined in patients suspected in any form of arthritis
A110. High levels RF: generally > 20 IU/mL; rheumatoid arthritis: present in 80%; Sjögren's syndrome: present in 60%
Q111. false (+) RF or RhF; Blood test performed in patients with suspected rheumatoid arthritis (RA)
A111. Chronic hepatitis; Any chronic viral infection; Leukemia; Dermatomyositis; Infectious mononucleosis; Scleroderma; Systemic lupus erythematosus (SLE)
Q112. Sjögren's syndrome; * antibodies to Ro(SSA) or La(SSB) antigens, or both; * > 40 years old at the time of diagnosis & Woman >> Man
A112. an autoimmune disorder in which immune cells attack and destroy the exocrine glands that produce tears and saliva with Schirmer test(+); A lip biopsy can reveal lymphocytes clustered around salivary gland
Q113. Spondyloarthropathies
A113. Group of related INFLAMMATORY JOINT Ds associated with the MHC class I molecule HLA-B27
Q114. Seronegative spondylarthropathy
A114. Spondylarthropathy & (-)rheumatoid factor (RhF)
Q115. Non-specific spondylarthropathy
A115. Indicator of other rheumatological disease (in particular rheumatoid arthritis)
Q116. Subgroups (with increased HLA-B27 frequency)
A116. ankylosing spondylitis Caucasians (AS, 92%),; ankylosing spondylitis African-Americans (AS, 50%),; reactive arthritis (Reiter's syndrome) (RS, 60-80%),; enteropathic arthritis associated with inflammatory bowel disease (IBD, 60%),; Psoriatic arthritis (60%),; isolated acute anterior uveitis (AAU, iritis or iridocyclitis, 50%), and; undifferentiated SpA (USpA, 20-25%).
Q117. Buergers
A117. thromboangiitis obliterans;; idiopathic, segmental, thrombosing vasculitis of intermed/small peripheral arteries and veins; seen in heavy smokers (tx- stop smoking)
Q118. Buerger's findings
A118. intermittent claudication, superficial nodular phlebitis, cold sensitivity, pain
Q119. Takayasu's arteritis
A119. pulseless disease;; granulomatous thickening of aortic arch and/or proximal great vessels;; associated with elevated ESR; primarily affects Asian females < 40 y/o
Q120. Takayasu's sxs
A120. fever,; arthritis,; night sweats,; myalgia,; skin nodules,; ocular disturbances,; weak pulses in upper extremities
Q121. Temporal arteritis (giant cell)
A121. most common vasculitis that affects medium and small arteries, usually branches of carotid; focal, granulomatous; unilateral HA, jaw claudication, impaired vision (occlusion of opthalmic artery- blindness); half of patients have systemic involvement and polymyalgia rheumatica; elevated ESR
Q122. Temporal arteritis treatment
A122. steroids
Q123. young male smoker
A123. buergers
Q124. young asian female with weak pulses in upper extremities
A124. takayasus
Q125. PAN
A125. necrotizing immune complex inflammation of medium sized muscular arteries typically involving renal and visceral vessels; sxs: weight loss, malaise, fever, abdominal pain, melena, HA, myalgia, HTN, neuro dysfunction, cutaneous eruptions
Q126. PAN association
A126. hep B;; multiple aneurysms and constrictions on arteriogram; not associated with ANCA (P maybe)
Q127. Tx of PAN
A127. corticosteroids, cyclophosphamide (hemorrhagic cystitis)
Q128. Wegener's granulomatosis
A128. traid of focal necrotizing vasculitis,; necrotizing granulomas in the lung and upper airway and necrotizing GN
Q129. Wegener's sxs
A129. perforation of nasal septum,; chronic sinusitis,; otitis media,; mastoiditis,; cough,; dyspnea,; hemoptysis,; hematuria; looks like goodpastures but upper respiratory tract is also involved
Q130. findings in wegeners
A130. CANCA,; CXR- may reveal large nodular densities,; hematuria and red cell casts; tx: cyclophosphamide, corticosteroids
Q131. Churg Strauss
A131. granulamatous vasculitis with eosinophilia;; involves lung, heart, skin, kidneys, and nerves; often seen in atopic pts; tx: steroids
Q132. Kawasaki's disease
A132. acute, self limiting disease of infants and kids;; acute necrotizing vasculitis of small/medium sized vessels; fever, congested conjunctiva, changes in lips/oral mucosa,; LAD, may develop coronary aneurysms,; skin rash,; strawberry tongue; tx: high dose aspirin
Q133. HSP
A133. most common childhood vasculitis;; skin rash (palpable purpura) below legs,; arthralgia,; intestinal hemorrhage,; abdominal pain,; melena
Q134. telangiectasia
A134. AV malformation in small vessels;; looks like dilated capillary; hereditary hemorrhagic telangiectasia-AD inheritance; presents with nosebleeds and skin discolorations
Q135. What is arthrocentesis?
A135. Arthrocentesis is a needle aspirate of synovial fluid for microscopic analysis. The cell count is the most accurate way of telling immediately if there is an infection versus inflammation. Counts from 0 to 2,000 are normal; from 2,000 to 20,000 are from inflammatory disorders such as gout. Counts above 50,000 are from infections. Counts between 20,000 and 50,000 are indeterminate.
Q136. Anti-Scl-70
A136. Scleroderma
Q137. Anti- Microsomal; (2)
A137. 1. Hashimoto's; 2. Autoimmune Hepatitis
Q138. Anti-b islet cell
A138. IDDM; type I diabetes
Q139. Anti-Acetylcholine receptor
A139. Myasthenia gravis
Q140. Anti-Adrenal
A140. Addison's
Q141. Anti-Basement membrane
A141. Goodpasture's
Q142. Anti-Centromere
Q143. Anti-ds DNA
A143. SLE [specific]
Q144. Anti-Epithelial
A144. Pemphigus vulgaris
Q145. Anti-Gliadin
A145. Celiac disease
Q146. Anti-Histone
A146. Drug induced lupus
Q147. Anti-IgM
A147. Rheumatoid arthritis
Q148. Anti-Intrinsic Factor
A148. Pernicious anemia
Q149. Anti-Mitochondrial
A149. Primary biliary cirrhosis
Q150. Anti-Neutrophil
A150. Vasculitis
Q151. c-ANCA
A151. Wegener's granulomatosis
Q152. p-ANCA
A152. Polyarteritis nodosa
Q153. Anti-Nuclear
A153. SLE
Q154. Anti-Thyroglobulin
A154. Hashimoto's
Q155. Anti-Thyroid peroxidase
A155. Hashimoto's
Q156. Anti-TSH receptor; (2)
A156. Graves' or Hashimoto's
Q157. Anti-Jo-1
A157. Polymyositis;; dermatomyositis
Q158. Anti-parietal cell
A158. Pernicious anemia
Q159. Anti-platelets
A159. ITP
Q160. Anti-smith
A160. SLE
Q161. B27
A161. PAIR; Psoriasis,; Ankylosing spondylitis,; Inflammatory bowel diseas,; Reiter's syndrome
Q162. B8
A162. Graves Disease, Celiac Sprue
Q163. DR2
A163. Mulitple sclerosis,; hay fever,; SLE,; Goodpastures
Q164. DR3
A164. DM Type 1
Q165. DR4
A165. Rheumatoid Arthritis, DM Type 1
Q166. DR5
A166. Pernicious anemia = B12 deficiency,; Hashimoto's thyroiditis
Q167. DR7
A167. Steroid-response nephrotic syndrome
Q168. Compartment Syndrome - What is it
A168. Increased pressure in a confined space; compromises nerve, muscle & soft tissue perfusion; MC - ant. compartment of lower leg & forearm; causes – fractures, crush injuries, burns, ischemic- reperfusion after an injury, casts
Q169. Compartment Syndrome - History/PE
A169. The 6 P's:; Paresthesias; Palpation - tense compartment; Pallor; Poikilothermia; Pulselessness; Paralysis; Pain - out of proportion with passive motion of fingers & toes; Volkmann's contracture
Q170. Compartment Syndrome - Dx
A170. Measure compartment pressure - > 30 mmHg not good; delta pressure - diastolic minus compartment; should be > 30 to be OK
Q171. Compartment Syndrome - Tx
A171. Surgical emergency; immediate fasciotomy of all compartments; do in < 6 hrs.
Q172. Low Back Pain - What is it
A172. Paraspinous; strains - muscle injury; sprain - ligament injury
Q173. Low Back Pain - History/PE
A173. If malignancy - pain worse at night; pain not relieved by rest; pain not relieved by changing positions; if point tenderness over vertebral body – osteomyelitis, fracture, malignancy; cauda equina syndrome - bladder or bowel dysfunction, saddle-area anesthesia, impotence, surgical emergency
Q174. Low Back Pain - L4 Associated Deficits:; Motor; Reflex; Sensory
A174. Motor - foot dorsiflexion, tibialis anterior; reflex - patellar; sensory - medial aspect of leg
Q175. Low Back Pain - L5 Associated Deficits:; Motor; Reflex; Sensory
A175. Motor - big toe dorsiflexion, extensor hallucis longus; reflex - none; sensory - medial forefoot & lateral aspect of leg
Q176. Low Back Pain - S1 Associated Deficits:; Motor; Reflex; Sensory
A176. Motor - foot eversion; peroneus longus/brevis; reflex - achilles; sensory - lateral foot
Q177. Low Back Pain - Dx
A177. Mainly clinical; XR; MRI; electrodiagnostic studies - nerve conduction velocity test
Q178. Low Back Pain - Tx
A178. Sprains & strains - NSAIDs; physical therapy; continue activities as tolerated; rest > 1-3 days unnecessary. 90% recover spontaneously in 6 weeks; surgery - if correctable spinal disease, cauda equina syndrome - surgical emergency: immediate decompression, laminectomy
Q179. Herniated Disk - What is it
A179. Nucleus pulposus herniates posteriorly => nerve root or cord compression; neck/back pain; sensory & motor deficits; causes - degenerative changes, trauma, neck/back strain, neck/back sprain; middle-aged & older men after strenuous activity; L4-L5 & L5-S1
Q180. Herniated Disk - History/PE
A180. Several months of aching pain => sudden onset of severe, electricity-like LBP; pain exacerbated by straining; sciatica: tingling - lower extremities, numbness, muscle weakness, atrophy, contractions, spasms, pain increased by - passive straight leg, crossed straight leg raises, large midline herniations => cauda equina syndrome
Q181. Herniated Disk - Dx
A181. MRI
Q182. Herniated Disk - Tx
A182. Most cases - bed rest; NSAIDs; physical therapy; localized heat; resolved in 2-3 weeks; if no neuro deficit - bed rest not advised, early mobilization; muscle relaxant, NSAIDs; diskectomy - if persistent or disabling Sxs
Q183. Spinal Stenosis - What is it
A183. Stenosis of cervical or lumbar spinal canal => compression of nerve roots; usually from degenerative joint disease; middle-aged or elderly
Q184. Spinal Stenosis - History/PE
A184. Neck pain; back pain - radiates to butt & legs, leg numbness, leg weakness, "spaghetti legs", "walks like a drunken sailor"; leg cramping - at rest, standing, walking; sitting gives relief; leaning forward gives relief (flexing at hips decreased pain)
Q185. Spinal Stenosis - Dx
A185. XR; MRI or CT
Q186. Spinal Stenosis - Tx
A186. Mild to moderate - NSAIDs; abdominal muscle strengthening; advanced - epidural steroid injection; surgical laminectomy - short-term, will recur
Q187. Developmental Dysplasia of Hip; What is it
A187. Congenital hip dislocation => dislocated femoral heads due to - lax musculature; excessive uterine packing (breech) => poor development of acetabulum, hip; will progress if not corrected; MC - 1st born breech females
Q188. Developmental Dysplasia of Hip; History/PE
A188. Barlow's - hip adducted; Ortolani's - thighs abducted; Allis' (Galeazzi's) sign - knees unequal when hip & knees flexed, dislocated side is lower; asymmetrical skin folds; limited abduction of affected hip
Q189. Developmental Dysplasia of Hip; Dx
A189. Early detection; evaluate clinical; US - if after 10 weeks old; XR - unreliable until 4 mos. old, neonatal femoral head radiolucent
Q190. Developmental Dysplasia of Hip; Tx
A190. Start Tx early; < 6 mos. - Pavlik harness; 6-15 mos. - spica cast; 15-24 mos. - open reduction; if no Tx started by 24 mos. - significant defect
Q191. Developmental Dysplasia of Hip; Complications
A191. Complications - joint contractures; AVN of femoral head
Q192. Limp - What is it
A192. 1 of the MC musculoskeletal disorder of kids; MC cause - trauma
Q193. Limp - History/PE
A193. May be associated with pain or fever; ask about - history of trauma, recent infections, contact with TB- positive patients; young kids & toddlers - infected joint; adolescent & teens – JRA, slipped capital femoral epiphyses (SCFE), Legg-Calve-Perthes (LCP); disruption in normal gait – Trendelenburg, antalgic gait; infection – erythema, edema, limited ROM; trauma or tumor - point tenderness; always evaluate for – fever, signs of systemic infection, neuro involvement – reflexes, muscle atrophy, changes in sensation, bowel & bladder function
Q194. Limp - Differential Dx
A194. STARTSS HOTT; Septic joint; Tumor; Avascular necrosis (LCP); RA/JRA; TB; Sickle cell disease; SCFE; HSP; Osteomyelitis; Trauma; Toxic synovitis
Q195. Limp - Dx
A195. Thorough H&P; XR; CBC; ESR; CRP; bone scan; nerve conduction studies; joint aspirate & culture - if suspect septic joint
Q196. Limp - Tx
A196. Depends on cause
Q197. Legg-Calve-Perthes - What is it
A197. AVN of femoral head; UNK etiology; boys 4-10; can be bilateral
Q198. Legg-Calve-Perthes - History/PE
A198. Usually asymptomatic at first => painless limp or => pain, referred to knee; limited abduction; limited internal rotation; atrophy
Q199. Legg-Calve-Perthes - Tx
A199. Self-limited; observation; if disease extensive or ROM impaired – brace, hip abduction with Petrie cast, osteotomy; prognosis dependent on – age, ROM, extent of involvement, joint stability
Q200. Slipped Capital; Femoral Epiphysis - What is it
A200. Separation of proximal femoral epiphysis through growth plate => fem head displaced; medial & posterior to fem neck; can be bilateral; obese Black 11-13 y/o boys; if < 11 y/o, may be associated with endocrinopathies; may be due to imbalance between GH & sex hormones
Q201. Slipped Capital; Femoral Epiphysis - History/PE
A201. Thigh or knee pain; painful limp; acute or insidious; acute - restricted ROM, inability to bear weight. limited internal rotation; limited abduction; hip tenderness; flexion => obligatory external rotation
Q202. Slipped Capital; Femoral Epiphysis - Dx
A202. TSH; XR both hips – AP, frog-leg lat
Q203. Slipped Capital; Femoral Epiphysis - Tx
A203. Start promptly; no weight on limb until; surgical stabilized with screws; acute slip - gentle closed reduction
Q204. Slipped Capital; Femoral Epiphysis - Complications
A204. Chondrolysis; AVN of fem head; premature hip OA => hip arthroplasty
Q205. Shoulder Dislocation - Mechanics
A205. MC - anterior dislocation, axillary artery & nerve, hold arm in external. rotation; posterior dislocations, radial artery, seizures & electrocutions, hold arm in int. rotation
Q206. Shoulder Dislocation - Tx
A206. Closed reduction; followed by sling & swath; recurrent - surgery
Q207. Hip Dislocation - Mechanics
A207. MC - posterior dislocation, compress sciatic nerve., can cause AVN, posterior directed force on, internal rotated, flexed, adducted hip, "dashboard injury"; anterior dislocation - obturator nerve compromises.
Q208. Hip Dislocation - Tx
A208. Closed reduction followed by abduction; pillow/bracing; CT after reduction
Q209. Colles' Fracture - Mechanics
A209. MC wrist fracture; distal radius; fall onto outstretched hand => dorsally displaced/angle Fracture; common in elderly (osteoporosis), kids
Q210. Colles' Fracture - Tx
A210. Closed reduction; then long arm cast; intra-articular - open reduction
Q211. Scaphoid (Carpal Navicular) Fracture; Mechanics
A211. MC fractured carpal bone; can be 1-2 weeks for XR to show; assume if tenderness in anatomical snuff box; proximal 3rd scaphoid fractures can => AVN
Q212. Scaphoid (Carpal Navicular) Fracture; Tx
A212. Thumb spica cast; open reduction - displacement; nonunion common
Q213. Boxer's Fracture - Mechanics
A213. Fracture of 5th metacarpal neck; forward trauma of closed fist
Q214. Boxer's Fracture - Tx
A214. Closed reduction & ulnar gutter splint; excessive angulation - percutan pinning; skin broken - assume infection by human oral pathogens; "fight bite"; surgical irrigation; debridement; IV Antibiotics to cover Eikenella
Q215. Humerus Fracture - Mechanics
A215. Direct trauma; radial nerve. wrist drop; loss of thumb abduction
Q216. Humerus Fracture - Tx
A216. Hanging arm cast or coaptation splint & sling; functional bracing
Q217. Nightstick Fracture - Mechanics
A217. Ulna shaft fracture; from self-defense - arm against blunt object
Q218. Nightstick Fracture - Tx
A218. ORIF; open reduction & int. fixation
Q219. Monteggia's Fracture - Mechanics
A219. Diaphyseal Fracture; proximal ulnar; subluxation of radial head
Q220. Monteggia's Fracture - Tx
A220. ORIF - shaft fracture; closed reduction - radial head
Q221. Galeazzi's Fracture - Mechanics
A221. Diaphyseal Fracture; radius; dislocation of distal radioulnar joint; from direct blow to radius
Q222. Galeazzi's Fracture - Tx
A222. ORIF - radius; cast forearm in supination
Q223. Hip Fracture - Mechanics
A223. MC in osteoporotic women who fall; shortened, ext rotated leg; at risk for DVTs; displaced fem neck fractures - high risk of AVN, fracture nonunion
Q224. Hip Fracture - Tx
A224. ORIF; parallel pinning of femoral neck; anticoagulant; > 80 y/o - may need hip hemiarthroplasty
Q225. Femur Fracture - Mechanics
A225. Direct trauma (MVA); fat emboli; fever; scleral & axillary petechiae; confusion; dyspnea; hypoxia
Q226. Femur Fracture - Tx
A226. Intramedullary nailing; open fractures - thorough irrigation; debridement
Q227. Tibial Fracture - Mechanics
A227. Direct trauma; car bumper & pedestrian injury; compartment syndrome
Q228. Tibial Fracture - Tx
A228. Casting vs. intramedullary nailing
Q229. Open Fracture - Mechanics
A229. Orthopedic emergency; must go to OR in < 6 hrs. (risk of infection)
Q230. Open Fracture - Tx
A230. OR emergently to repair; Antibiotics
Q231. Achilles Tendon Rupture - Mechanics
A231. MC in unfit men in sports; hear sudden "pop"; sounds like rifle shot; limited plantar flexion; pos Thompson test - pressure on gastrocnemius; doesn't => ft plantar flexion
Q232. Achilles Tendon Rupture - Tx
A232. Long-leg cast for 6 wks
Q233. ACL Injury - Mechanics
A233. From forced hyperflexion; positive anterior drawer sign; Lachman's test; rule out meniscal or MCL injury
Q234. ACL Injury - Tx
A234. Surgery; graft from patellar or hamstring tendons
Q235. PCL Injury - Mechanics
A235. From forced hyperextension; positive posterior drawer test
Q236. PCL Injury - Tx
A236. Operative PCL repair - for highly competitive athlete
Q237. Meniscal Tears - Mechanics
A237. Clicking or locking; joint line tenderness; positive McMurray's test
Q238. Meniscal Tears,; MCL/LCL Injuries - Tx
A238. Conservative; unless associated with Sxs or ligament injuries
Q239. Clavicular Fracture - What is it
A239. MC fractured long bone in kids; birth-related (lg infants); brachial n. palsies; usually middle 3rd of clavicle; proximal end displaced superiorly; from pull of sternocleidomast
Q240. Clavicular Fracture - Tx
A240. Figure-of-8 sling vs. arm sling
Q241. Greenstick Fracture - What is it
A241. Incomplete fracture; cortex of one side of bone
Q242. Greenstick Fracture - Tx
A242. Reduction with casting; order films at 7-10 days
Q243. Nursemaid's Elbow - What is it
A243. Radial head subluxation; from being pulled or lifted by hand; kid will not bend elbow
Q244. Nursemaid's Elbow - Tx
A244. Manual reduction - gentle supination of elbow; at 90 degrees of flexion; no immobilization necessary.
Q245. Torus Fracture - What is it
A245. Buckling of cortex of long bone; secondary to trauma; usually distal radius or ulna
Q246. Torus Fracture - Tx
A246. Cast immobilization; 3-5 wks - dep. on age
Q247. Supracondylar Humerus Fracture - What is it
A247. 5-8 y/o; proximal to brachial a. risk of Volkmann's contracture
Q248. Supracondylar Humerus Fracture - Tx
A248. Cast immobilization; closed reduction; percutan pinning - if significant displaced
Q249. Osgood-Schlatter Disease - What is it
A249. Overuse apophysitis of tibial tubercle; localized pain; especially with quadriceps Ctx; active young boys
Q250. Osgood-Schlatter Disease - Tx
A250. Decreased activity 1-2 yrs; neoprene brace
Q251. Salter-Harris Fractures - What are they
A251. Fractures of growth plate of kids; classified by fracture location; I - physis; II - metaphysis & physis; III - epiphysis & physis; IV - epi-, meta- & physis; V - crush injury of physis
Q252. Salter-Harris Fractures - Tx
A252. I & II - nonoperatively; others & unstable fractures-operatively; prevents leg-length inequality
Q253. Osteosarcoma - What is it
A253. 2nd MC primary malignant; bone tumor; metaphyses - distal femur; proximal tibia; proximal humerus; can metas to lungs; men; 20s-30s; Paget's can precede development of secondary osteosarcoma
Q254. Osteosarcoma - History/PE
A254. Progressive pain => intractable pain; worse at night; constitutional Sxs; at site of tumor - erythema; enlargement
Q255. Osteosarcoma - Dx
A255. MRI; CT; XR - Codman's triangle, sunburst pattern
Q256. Osteosarcoma - Tx
A256. Limb-sparing surgical proc; pre- and postop chemo – methotrexate, doxorubicin, cisplatin, ifosfamide; amputation - may be necessary if big
Q257. Paget's (Osteitis Deformans) - What is it
A257. Osteoclasts accelerate bone turnover in local areas => hyper osteoblastic repair => abnorm structure that weakens bone; may be associated with paramyxovirus infection; mainly disease of elderly
Q258. Paget's (Osteitis Deformans) - History/PE
A258. Often asymptomatic; if Sx occur, develop insidiously; deep bone pain; bone softening => tibial bowing, kyphosis, freq fractures; increased in cranial diameter - (frontal bossing); deafness - CN8 compressed
Q259. Paget's (Osteitis Deformans) - Dx
A259. Alkaline phosphatase; increased urinary hydroxyproline; serum Ca2+ & phosphate- normal; XR - bony cortex very expanded, jigsaw/mosaic bone pattern, thick trabeculae
Q260. Paget's (Osteitis Deformans) - Tx
A260. NSAIDs; calcitonin; alendronate
Q261. Paget's (Osteitis Deformans) - Complications
A261. Fracture; hi-output cardiac failure; arthritis; deafness; secondary osteosarcoma; vertebral collapse => spinal cord compression
Q262. Gout - What is it
A262. Recurrent attacks - acute monoarticular arthritis; monosodium urate crystals; middle-aged, obese men, Pacific Islanders; hyperuricemia - secondary to uric acid underexcretion; other causes - Lesch-Nyhan, diuretics (furosemide, HCTZ), trauma, surgery, infections, steroid withdrawal, cyclosporine, malignancy, excessive red meat or red wine, hemoglobinopathies
Q263. Pseudogout - What is it; Dx; Tx
A263. MC - elderly or preexisting joint damage; if < 50 y/o - metabolic abnorm, hyperparathyroidism, hypophosphatasia, hypomagnesemia, hemochromatosis; knee - #1, also ankle, wrist, shoulder; Dx - fluid aspiration, Positive birefringent rhomboid crystals; XR - chondrocalcinosis; Tx - same as gout; low dose of colchicine - prevents recurrence
Q264. Gout - History/PE
A264. 1st episode - awakened from sleep; sudden onset of joint pain; podagra; usually 1st MTP joint, knee, ankle, DIP & PIP; joint – erythematous, swollen, tender; as becomes chronic - multiple joints involved; urate crystals deposited in connective tissue (tophi), kidneys
Q265. Gout - Dx
A265. Joint-fluid aspirate - needle-shaped, negative birefringent crystals; yeLLow when paraLLel to condenser; increased WBC; advanced - "rat bite" = punched-out erosions - of long- standing tophus, "overhanging margin"
Q266. Gout - Tx
A266. Acute - decrease inflammation; NSAIDs (indomethacin); IV colchicine or steroids - if elderly maintenance - decrease uric acid level:; probenecid if underexcrete, inhibits reabsorb of uric acid; allopurinol if overproduce or have RF or have kidney stones, can precipitate acute attack
Q267. Osteoarthritis - What is it; Risk Factors
A267. Degen joint disease; deterioration of articular cartilage of moveable, weight- bearing joints; MCC- idiopathic; secondary - any diseases that causes stress or trauma to joint; knee OA - MCC of chronic disability in elderly in West; risk factors – obesity, family History, history of joint trauma; especially intra-articular fractures, repetitive stress
Q268. Osteoarthritis - History/PE
A268. History - joint stiffness - insidious onset; pain - insidious onset; worse by activity & weight-bearing; relieved by rest; crepitus; decreased ROM; PE - weight-bearing joint involved; DIP - heberden nodes; PIP - bouchard nodes; MTP joint of 1st toe; cervical spine
Q269. Osteoarthritis - Dx
A269. Based on clinical & XR; XR - irregular joint space narrowing, osteophytes, subchondral sclerosis, subchondral bone cysts; ESR normal; synovial fluid aspiration - straw-colored fluid, normal viscosity, WBC < 3000
Q270. Osteoarthritis - Tx
A270. Weight reduction; physical therapy; NSAIDs; intra-articular corticosteroid injections; elective joint replacement - total hip/knee arthroplasty
Q271. Duchenne Muscular Dystrophy - What is it; What is Becker's
A271. XLR; defect of dystrophin - cytoskeletal protein; MC muscular dystrophy; most lethal; ages 2-6; Becker – milder, XLR, abnormal-sized dystrophin
Q272. Duchenne Muscular Dystrophy - History/PE
A272. History - axial and proximal before distal muscles; progressive clumsiness; fatigability; hard to stand or walk; hard to walk on toes; waddling gait; Gower's maneuver; PE - pseudohypertrophy of gastrocnemius; possible MR
Q273. Duchenne Muscular Dystrophy - Dx
A273. CK - always high; EMG; muscle Biopsy; DNA; diagnostic - immunostain for dystrophin
Q274. Duchenne Muscular Dystrophy - Tx
A274. No cure; Tx supportive; physical therapy; achilles tendon release; wheelchair by 13 y/o; death in 20s
Q275. what is the defect in Bruton's agammaglobulinemia?
A275. X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of Igs
Q276. this disorder occurs in boys and is associated with recurrent bacterial infections after 6 months of age, when levels of maternal IgG antibody decline
A276. Brutons agammaglobulinemia (B cells)
Q277. this immune deficiency presents with tetany owing to hypocalcemia and recurrent viral and fungal infections
A277. DiGeorge syndrome/thymic aplasia (T cells)
Q278. this immune deficiency is associated with congenital defects of heart and great vessels
A278. DiGeorge syndrome
Q279. what ist eh chromosomal abnormality associated with DiGeorge syndrome?
A279. 22q11 deletion
Q280. this is a defect in early stem cell differentiation and presents with recurrent viral, bacterial, fungal, and protozoal infections
A280. SCID - B and T cell deficiency
Q281. failure to synthesize MHC II antigens, defective IL-2 receptors, and adenosine deaminase deficiency can all lead to what immune deficiency?
A281. SCID
Q282. what does IL-12 receptor deficiency (decreased activation of T cells) present with?
A282. disseminated mycobacterial infections
Q283. defect in CD40 ligand on CD4 T helper cells leads to inability to class switch; presents early in life with severe pyogenic infections
A283. hyper-IgM syndrome (decreased activation of B cells)
Q284. this disorder is characterized by high levels of IgM and very low levels of IgG, IgA, and IgE
A284. hyper-IgM syndrome
Q285. this syndrome is characterized by an X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria
A285. Wiskott-Aldrich syndrome (decreased activation of B cells)
Q286. what is the triad of symptoms associated with Wiskott- Aldrich syndrome?
A286. infections, thrombocytopenic purpura, eczema (WIPE)
Q287. this syndrome is associated with elevated IgA levels, normal IgE levels, and low IgM levels
A287. Wiskott-Aldrich syndrome
Q288. this syndrome presents with recurrent 'cold' (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high IgE levels
A288. Job's syndrome (decreased activation of macrophages)
Q289. this syndrome is characterized by failure of gamma- interferon production by helper T cells; neutrophils fail to respond to chemotactic stimuli
A289. JOb's syndrome
Q290. what is the defect in leukocyte adhesion deficiency syndrome?
A290. defect in LFA-1 adhesion proteins on phagocytes
Q291. this syndrome presents early with severe pyogenic and fungal infections and delayed separation of umbilicus
A291. leukocyte adhesion deficiency syndrome
Q292. how is Chediak-Higashi inherited?
A292. autosomal recessive
Q293. this disease is marked by a defect in microtubular function and lysosomal emptying of phagocytic cells
A293. Chediak-Higashi disease
Q294. this disease presents with recurrent pyogenic infections by staph and strep, partial albinism, and peripheral neuropathy
A294. Chediak-Higashi disease
Q295. defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes
A295. chronic granulomatous disease
Q296. how is the diagnosis of chronic granulomatous disease confirmed?
A296. negative nitroblue tetrazolium dye reduction test
Q297. this disease presents with marked susceptibility to opportunistic infections with bacteria, especially S. aureus, E. coli, and Aspergillus
A297. chronic granulomatous disease
Q298. what is the defect in chronic mucocutaneous candidiasis present?
A298. idiopathic dysfunction of T cells specifically against candida albicans
Q299. what is the most comon selective immunoglobulin deficiency? how does it present?
A299. selective IgA deficiency - presents with sinus and lung infections;; milk allergies and diarrhea are common
Q300. ataxia-telangiectasia is an idiopathic dysfunction of what type of cells?
A300. B cells
Q301. defect in DNA repair enzymes with associated IgA deficiency; presents with cerebellar problems and spider angiomas
A301. ataxia-telangiectasia
Q302. deficiency of what leads to hereditary angioedema?
A302. C1 esterase inhibitor
Q303. deficiency of which complement protein leads to severe, recurrent, pyogenic sinus and RT infections?
A303. C3
Q304. deficiency of what complement proteins leads to Neisseria bacteremia?
A304. C6-C8
Q305. deficiency of what leads to paroxysmal nocturnal hemoglobinuria?
A305. decay-accelerating factor
Q306. patients with what deficiency have an increased susceptibility to recurrent bacterial infections, especially with encapsulated bacteria
A306. C3 deficiency;; not detected until later in life
Q307. what is the most important immunological protective mechanism against blood-borne encapsulated organisms?
A307. IgG-mediated opsonization in the spleen
Q308. Warthin-Finkeldy giant cell is pathognomonic for what?
A308. measles or the live attenuated measles vaccine
Q309. Lyme arthritis is associated with what HLA?
A309. HLA-DR4
Q310. patients with Wiskott-Aldrich syndrome have a 12% chance of developing what?
A310. non-Hodgkin's lymphoma
Q311. test of choice to determine presence of circulating anti-Rh antibody?
A311. indirect Coombs test to measure IgG anti-Rh antibody
Q312. low levels of all antibody classes
A312. common variable immunodeficiency
Q313. spleen is important for removing what type of organisms? list 3
A313. strep pneumo,; H. flu,; Neisseria
Q314. what is defective in leukocyte adhesion deficiency?
A314. integrins - function both in adhesion of leukocytes to other cells and in the phagocytosis of complement-coated material
Q315. HLA types associated with SLE?
A315. HLA-DR2 and HLA-DR3
Q316. most likely sequelae of rheumatic fever?
A316. mitral valve disease
Q317. antitopoisomerase antibodies?
A317. scleroderma/systemic fibrosis - likely to develop diffuse systemic fibrosis & death from consequences of systemic disease such as pulmonary fibrosis or malignant hypertension
Q318. what is responsible for strong binding between monocytes, T lymphocytes, macrophages, neutrophils, and dendritic cells, and injured epithelium?
A318. LFA-1; interacts with ICAM-1
Q319. an increased level of what cytokine would decrease the likelihood of a delayed-type hypersensitivity reaction?
A319. IL-10
Q320. what 2 molecules exert the most powerful chemotactic effect on neutrophils?
A320. C5a and C8
Q321. what are the best markers for identification of B cells?
A321. CD19, CD20, CD21
Q322. IgG subclass deficiency is associated with a deficiency with what other substrate?
A322. IgA
Q323. what is C-reactive protein a marker of?
A323. non-specific inflammation - one of the most commonly measured acute-phase reactants
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