acute rhematic fever.ppt

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    Acute Rheumatic Fever

    And

    Rheumatic heart disease

    Agus Priyatno by 

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    Etiologi :

      1. Immunologic  Streptococcus beta hemolytic group A

      2. Predispositing Factor:  - Family history  - Socio-economic status  - Age 5 – 15 years pea! " years#

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    Pathology

    • Inflamatory lesion; heart,brain,joint,skin

    • Ashoff bodies ( in atrial myocardium):

      characteristic   !entral necrosis surrounded by lym"hocytes,  "lasma cell, and large mononuclear and giant  multinucleate cell

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    !linical manifestations

    #istory$tre"tococcal "haryngitis, % & ' eeks (ae *) beforeonset+

    !horea & - mos+Pallor, easy fatigability, e"ista.is, abdominal "ainPositie family history+

    Strep throat

    $heumatic %e&er

    Acute glomerulonephritis

    Strep s!in

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    Histologic section of typical RF

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    /ones criteria ( u"date %00)

    o 1ajor criteria :

     2 arthritis 2 carditis 2 erythema marginatum 2 subcutaneous nodules 2 $ydenham3s chorea+

    o 1inor criteria :

     2 arthalgia 2 feer

     2 eleated acute "hasereactan ( !4P, E$4)

     2 E!5 : P4 interal 6 :  not s"ecific

    "lus $u""orting eident of antecedent $tre" 7 grou" A infec+  2 culture (8) or ra"id stre"t antigent test  2 eleated or rising A$9

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    %+ Arthritis

      Affects of cases

      ?arge joints : knee, ankle, elbo, rist  ften 6 % joints, simultaneously or

    in succession, migratory+  $elling, heat, redness, seere "aint,  tenderness, motion @  ramatic res"onse to salicylate

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    Migratory arthritis

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    + !arditis

    2 '= > of cases

    2 iagnosis reBuires "resence of :

    %+ 9achycardia  + #eart murmur of alulitis ( 14 and A4 )  *+ Pericarditis( friction rub, "ericard

    effusion,chest "ain, E!5 changes)  C+ !ardiomegaly on chest D ray

      '+ !ongestif heart failure ( seere carditis)

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    Involve of the heart;  pericarditis

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    Involve of the heart;myocardium andendocardium

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    *+ Erythema marginatum

    2 @ %= > of cases2 on "ruritic erythematous rashes,

      neer on faces2 1ost "rominent on trunk and inner "ro.imal  "ortion2 isa""ear on e."osure to cold, seldom detected  on A! hos"ital

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    Erythemamarginatum

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    C. $ubcutaneous nodules 

    2  & %=> of cases, es" in recurrences2 #ard, "ainless, non "ruritic,freely moeable,  selling =, & cm2 Fssually symmetric on e.tensor surfaces  of joint, scal", along s"ine, last for eeks

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    Subcutaneousnodules

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    '+ $ydenham3s chorea+

    2 %'> of cases, more often in "re"ubertal girl2 Gegin ith emotional lability and "ersonality

      changes+2 $"ontaneous, "ur"oseless moement folloed  by motor eakness, slurred s"eech2 ysfunction of basal ganglia and cortical neural  com"onents

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    Involuntary grimaces

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    iagnosis of rheumatic feer

    Gased on

    major criteria  or  % minor criteria

    8 A$9

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    E.ce"tion to the /ones criteria

    %+ !horea may occur as the only manifestation  of 4H+ Indolent carditis may be the only manifestation

    *+ ccasionally "atients ith 4H recurrences maynot fulfill the /ones criteria

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    ifferential diagnosis of 4H

    • /uenile rheumatoid arthritis• !ollagen ascilar disease• irus associated acute arthritis• #ematologic disorder: sicklemia and leukemia

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    ote

    • 4H is a clinical syndrom for hich no s"esific  diagnostic test e.ist• o sym"tom, sign or lab test result is "athogno  monic, although seeral combinations of them  are diagnostic• nly carditis can cause "ermanent cardiac damage+

    $ign of mild carditis disa""ear ra"idly in eeks  but seere carditis may last for & - month+

      !horea and arthritis usually subside ithout"ermanent damage

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    1anagement of 4H

    • GenJathin "enicillin 5 =,- & %, 1 units i+m  for eradication and "ro"hyla.is+  Allergic to "enicillin : erythromisin C= mgKkgKday  in to to four doses for %= days+• Ged rest• Acetosal for mild cases• Prednison for seere cases• Anti inflammatory agents not needed for isolated

      chorea• 9reatment of congestie heart failure

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    4ecommended anti2inflammatory agents

    Arthritis  alone

      'ildcarditis

    'oderate carditis

     Se&ere carditis

    Prednison ( ( ( 2-) *!+

    Aspirin 1-2 *! ,- *! )-" *! 2-mo

    •Prednison should be tapered and aspirin started during the the %inal *ee!.

     Aspirin may be reduced to )( mg/!g/day

    0osages: Prednison: 2 mg/!g/day in di&ided doses  Aspirin : 1(( mg/!g/day in -) di&ided doses

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    Ged rest and indoor ambulation

    Arthritis 'ild 'oderate se&ere  alone carditis carditis carditis

    ed rest 1-2 *! ,- *! -) *! as longas 34F

    Indoor amb 1-2 *! ,- *! -) *! 2-, mo

    utdoor acti& 2 *! 2- *! 1-, mo 2-, mo  school#Full acti&ity 6-) *! 6)-1( *! 6,-) mo &ariable

    E$4: im"ortant for duration of restriction of actiitiesHull actiity: E$4 normal, e.ce" significant cardiac inolement

    1ild card: Luestionable cardiomegaly, moderate: definite but mild,$eere: marked cardiomegaly or !#H

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    Preention

    • Ideally "ro"hyla.is is indefinite• GenJathin "enicillin eery M days, min till  age % & ' ys

    • $ulfadiaJine =,' g %. daily ( b @ < kg)  % g %. daily (b 6 < kg)• Peniccilin . '= mgKday• Erythromycin . '= mgKday

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    4#EF1A9I! #EA49 I$EA$E

    Affecs; 2 1itral ale   2 9ricus"id ale rare

      2 Pulmonary ale neer

    $tenosis and regurgitation usually occurtogether+

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    1I94A? $9E$I$

    • "realent• most common alular inolement in adult

    • reBuires ' & %= ys from the initial attack• if 4H is "realent, 1$ occurs under age %' ys

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    Pathology

    • 9hickening of the leaflets and fusion of thecommisure

    • !alsification result oertime

    • ?A and right2sides heart chambers become dilated  and hi"ertro"hied• Pulmonary enous hy"ertension, "ulmonary congestion  and edema and fibrosis of the aleolar alls,  hy"ertro"hy of the "ulmonary aleolar, loss of lung  com"liance

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    Aortic and mitral valve

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    !linical manifestations

    • 1ild 1$ : asym"tomatic• 1ore seere: dys"nea ithKout e.ertion, ortho"neu,  nocturnal dys"nea and "al"itation+ 

    Physical E.aminations:

    • Increased 4 im"ulse along ?$G

    • Neak "eri"heral "ulse ith narro "ulse "ressure• Pulmonary hy"ertension: loud $% at a"e. and narro  s"lit $, accentuated P• 1ild diatolicK"resystolic murmur

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    Cardiac nding of MS

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    E!5 :2 4A, ?A#, 4# ( due to P# )

      2 atrial fibrilation is rare in children!D4 : 2 enlarge ?A and 4,2 1PA segment "rominent2 "ulmonary enous congestion

     Echocardiogra"hy :

    • accurate noninasie tool to detection of 1$• 12mode; diminished E to H slo"e, thickened  mitral leaflets, large ?A dimention

    • 9o : doming of thick mitral, a small mitral  orifice, dilated ?A, 1PA, 4 and 4A• o""ler: estimate of "ressure gradient;1itral  ale and "ulmonary ale+

     

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    9reatment of 1$• "ro"hylactic antibiotic• restriction of actiity de"ends on seerity• sym"tomatic "atient ( dys"nea on e.ertion,

      "ulmonary edema, "aro.ysmal dys"nea): balloon  or surgery

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    7ypical appearances o% ad&anced 'S on mediastinal

    organ and lung

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    1I94A? 4E5F45I9A9I1ost common in 4#

    Pathology:

    2 mitral ale leaflets are shortened because of  fibrosis+2 hen degree of 14 increases, dilatation of ?A  and ? result, mitral ring becomes dilated+

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    !linical manifestation:• asym"tomatic during chilhood• rare; fatigue, "al"itation

    Physical e.amination

    • heaing, hy"erdinamic a"ical im"uls in seere 14• $% normal or diminished+• $ may s"lit ( shortening of ? ejection, early

    aortic closure )

    • $* commonly is "resent and loud• Pansystolic murmur at the a"e., ith transmision  to the left a.illa 

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    Cardiac nding of MR

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    E!5:2 normal in mild cases

    2?# or ? dominance, ith or ithout ?A#2Atrial fibrilation is rare in children

    !D4:

    2?A and ? enlarged2Pulmonary congestion "attern in !#H

    Echocardiogra"hy:

    2to : dilated ?A and ?2color2flo ma""ing; regurgitant jet into the ?A2do""ler: asses the seerity of the regurgitation

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    9reatment:

    • Pro"hylactic antibiotic• o restriction of actiity in mild cases• $urgical: intractable !#H,

    "rogressie cardiomegaly,"ulmonary hy"ertension• If atrial fibrilation; digo.in• Afterload2reducing agent; maintaining the forard  stroke olume+

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    A49I! 4E5F45I9A9I

    ?ess common than 14+ 1ostly associated ithmitral ale disease+

    Pathology• semilunar cus"s are deformed and shortened• ale ring is dilated• commisures usually are fused

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    !linical manifestation

    • mild regurgitation: asym"tomatic• more seere; reduce e.ercise tolerance test

    Physical e.amination

    • "recordium may be hy"erdinamic, distolic thrill  at * ?I!$• $% decreased, $ may be normal or single• high "itched diastolic cresendo murmur at * ?I!$  or C ?I!$• systolic murmur at 4I!$ due to relatie A$• seere A$ : middiastolic murmur at a"e.

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    Cardiac nding of AR

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    E!5 : 2 normal in mild cases  2 seere ; ?#, ?A#

    !D4 : 2 cardiomegaly (?#)  2 dilated ascending aorta

    Echocardiogra"hy :

    • the ? dimension is increased• color2flo and do""ler to estimate the seere  of the regurgitation+

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    9reatment:

    • "ro"hylactic antibiotics• mild case : no restriction in actiity• surgical : in anginal "ain or dys"nea on  e.ertion, significant cardiomegaly

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    Streptococcal infectionAnd reccurentFor three prophylactic!"# Sulphadia$in%# &enicillin'# (en$atin penicillin

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    )ecline in ne* cases of RF  admitted to hospital

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    9hank Oou