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 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

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

Slide3305/05/1999Dr.Said Alavi 33