Dr. Said Alavi MD DCH DNB FCPS - Department of Pediatrics and Neonatology at Saqr Hospital in Ras Al Khaimah, United Arab Emirates
Dr. Said Alavi is a highly skilled and experienced physician
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About Dr. Said Alavi MD DCH DNB FCPS - Department of Pediatrics and Neonatology at Saqr Hospital in Ras Al Khaimah, United Arab Emirates
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Slide1Dr.Said AlaviMD,DCH,DNB,FCPS Dr.Said Alavi MD,DCH,DNB,FCPS Dept. of Pediatrics and Neonatology Dept. of Pediatrics and Neonatology Saqr Hospital,Ras Al Khaimah Saqr Hospital,Ras Al Khaimah UNITED ARAB EMIRATES UNITED ARAB EMIRATES E-mail: drsaid@emirates.net.ae E-mail: drsaid@emirates.net.ae
Slide205/05/1999Dr.Said Alavi 2 Objectives Objectives Objectives Objectives l Etiology l Epidemiology l Pathogenesis l Pathologic lesions l Clinical manifestations & Laboratory findings l Diagnosis & Differential diagnosis l Treatment & Prevention l Prognosis l References
Slide305/05/1999Dr.Said Alavi 3 Etiology Etiology l Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection l It is a delayed non-suppurative sequelae to URTI with GABH streptococci. l It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS
Slide405/05/1999Dr.Said Alavi 4 Epidemiology Epidemiology l Ages 5-15 yrs are most susceptible l Rare <3 yrs l Girls>boys l Common in 3rd world countries l Environmental factors-- over crowding, poor sanitation, poverty, l Incidence more during fall ,winter & early spring
Slide505/05/1999Dr.Said Alavi 5 Pathogenesis Pathogenesis l Delayed immune response to infection with group.A beta hemolytic streptococci. l After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain
Slide605/05/1999Dr.Said Alavi 6 l Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24 l Pharyngitis - produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis l Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit antigenicity Group A Beta Hemolytic Streptococcus
Slide705/05/1999Dr.Said Alavi 7 Diagrammatic structure of the group A beta hemolytic streptococcus Capsule Cell wall Protein antigens Group carbohydrate Peptidoglycan Cyto.membrane Cytoplasm …………………………………………… ……... Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain
Slide805/05/1999Dr.Said Alavi 8 Pathologic Lesions Pathologic Lesions l Fibrinoid degeneration of connective tissue,inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of A shcoff nodules , resulting in- - Pancarditis in the heart - Arthritis in the joints - Ashcoff nodules in the subcutaneous tissue - Basal gangliar lesions resulting in chorea
Slide905/05/1999Dr.Said Alavi 9 Rheumatic Carditis Histology (40X) Rheumatic Carditis Histology (40X)
Slide1005/05/1999Dr.Said Alavi 10 Histology of Myocardium in Rheumatic Carditis ( 200X) Histology of Myocardium in Rheumatic Carditis ( 200X)
Slide1105/05/1999Dr.Said Alavi 11 Clinical Features Clinical Features l Flitting & fleeting migratory polyarthritis, involving major joints l Commonly involved joints- knee,ankle,elbow & wrist l Occur in 80%,involved joints are exquisitely tender l In children below 5 yrs arthritis usually mild but carditis more prominent l Arthritis do not progress to chronic disease 1.Arthritis
Slide1205/05/1999Dr.Said Alavi 12 Clinical Features (Contd) Clinical Features (Contd) l Manifest as pancarditis (endocarditis, myocarditis and pericarditis),occur in 40- 50% of cases l Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ l Valvulitis occur in acute phase l Chronic phase- fibrosis,calcification & stenosis of heart valves (fishmouth valves) 2.Carditis
Slide1305/05/1999Dr.Said Alavi 13 Rheumatic heart disease . Abnormal mitral valve. Thick, fused chordae
Slide1405/05/1999Dr.Said Alavi 14 Another view of thick and fused mitral valves in Rheumatic heart disease
Slide1505/05/1999Dr.Said Alavi 15 Clinical Features (Contd) Clinical Features (Contd) l Occur in 5-10% of cases l Mainly in girls of 1-15 yrs age l May appear even 6/12 after the attack of rheumatic fever l Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face l Clinical signs- pronator sign, jack in the box sign , milking sign of hands 3.Sydenham Chorea
Slide1605/05/1999Dr.Said Alavi 16 Clinical Features (Contd) Clinical Features (Contd) l Occur in <5%. l Unique,transient,serpiginous-looking lesions of 1-2 inches in size l Pale center with red irregular margin l More on trunks & limbs & non-itchy l Worsens with application of heat l Often associated with chronic carditis 4.Erythema Marginatum
Slide1705/05/1999Dr.Said Alavi 17 Clinical Features (Contd) Clinical Features (Contd) l Occur in 10% l Painless,pea-sized,palpable nodules l Mainly over extensor surfaces of joints,spine,scapulae & scalp l Associated with strong seropositivity l Always associated with severe carditis 5.Subcutaneous nodules
Slide1805/05/1999Dr.Said Alavi 18 Clinical Features (Contd) Clinical Features (Contd) Other features (Minor features) l Fever-(upto 101 degree F) l Arthralgia l Pallor l Anorexia l Loss of weight
Slide1905/05/1999Dr.Said Alavi 19 Laboratory Findings Laboratory Findings l High ESR l Anemia, leucocytosis l Elevated C-reactive protien l ASO titre >200 Todd units. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) l Anti-DNAse B test l Throat culture-GABHstreptococci
Slide2005/05/1999Dr.Said Alavi 20 Laboratory Findings (Contd) Laboratory Findings (Contd) l ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression, T inversion l 2D Echo cardiography- valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility
Slide2105/05/1999Dr.Said Alavi 21 Diagnosis Diagnosis l Rheumatic fever is mainly a clinical diagnosis l No single diagnostic sign or specific laboratory test available for diagnosis l Diagnosis based on MODIFIED JONES CRITERIA
Slide2205/05/1999Dr.Said Alavi 22 Recommendations of the American Heart Association
Slide2305/05/1999Dr.Said Alavi 23 Exceptions to Jones Criteria Exceptions to Jones Criteria Chorea alone, if other causes have been excluded Insidious or late-onset carditis with no other explanation Patients with documented RHD or prior rheumatic fever,one major criterion,or of fever,arthralgia or high CRP suggests recurrence
Slide2405/05/1999Dr.Said Alavi 24 Differential Diagnosis Differential Diagnosis l Juvenile rheumatiod arthritis l Septic arthritis l Sickle-cell arthropathy l Kawasaki disease l Myocarditis l Scarlet fever l Leukemia
Slide2505/05/1999Dr.Said Alavi 25 Treatment Treatment l Step I - primary prevention (eradication of streptococci) l Step II - anti inflammatory treatment (aspirin,steroids) l Step III - supportive management & management of complications l Step IV - secondary prevention (prevention of recurrent attacks)
Slide2605/05/1999Dr.Said Alavi 26 STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G 600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association
Slide2705/05/1999Dr.Said Alavi 27 Step II: Anti inflammatory treatment Clinical condition Drugs
Slide2805/05/1999Dr.Said Alavi 28 l Bed rest l Treatment of congestive cardiac failure: - digitalis,diuretics l Treatment of chorea: - diazepam or haloperidol l Rest to joints & supportive splinting 3.Step III: Supportive management & management of complications
Slide2905/05/1999Dr.Said Alavi 29 STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of American Heart Association
Slide3005/05/1999Dr.Said Alavi 30 Duration of Secondary Rheumatic Fever Prophylaxis Category Duration Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease * ) age 40 y, sometimes lifelong prophylaxis Rheumatic fever with carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*) Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer *Clinical or echocardiographic evidence. Recommendations of American Heart Association
Slide3105/05/1999Dr.Said Alavi 31 Prognosis Prognosis l Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines l Good prognosis for older age group & if no carditis during the initial attack l Bad prognosis for younger children & those with carditis with valvar lesions
Slide3205/05/1999Dr.Said Alavi 32 References References Hoffman JIE: Rheumatic Fever . Rudolph's Pediatrics; 20th Ed: 1518 - 1521,1996. Stollerman GH: Rheumatic Fever . Harrison's Principles Of Internal Medicine; 13th Ed: 1046 - 1052,1995. Special Writing Group of the Committee on Rheumatic Fever,endocarditis & Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association: Guidelines for the Diagnosis of Rheumatic Fever. In Jones Criteria, 1992 Update JAMA 268:2029,1992 Todd J: Rheumatic Fever . Nelson's Textbook Of Pediatrics; 15th Ed: 754 - 760, 1996. Warren R, Perez M, Wilking A: Pediatric Rheumatic Diseases . Pediatric Clinics of North America; 41: 783 - 818,1994. World Health Organization Study Group: Rheumatic Fever & Rheumatic Heart Disease,technical Report Series No. 764.Geneva,world Health Organization, 1988
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