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Premium member Presentation Transcript PowerPoint Presentation: Prof. Dr. Saad S Al Ani Senior Pediatric Consultant Head of Pediatric Department Khorfakkan Hospital, Sharjah, UAE firstname.lastname@example.org Kawasaki diseaseIntroduction: Introduction Kawasaki disease, formerly known as mucocutaneous lymph node syndrome or infantile polyarteritis nodosa , is an acute febrile vasculitis of childhood first described by Dr. Tomisaku Kawasaki in Japan in 1967.: Idiopathic multisystem disease characterized by vasculitis of small & medium blood vessels, including coronary arteries DefinitionCont.: Cont. Worldwide, with Asians at highest risk. Approximately 20% of untreated patients develop coronary artery abnormalities Leading cause of acquired heart disease in children in the United States and Japan.Etiology: Etiology The cause of the illness remains unknown , but clinical and epidemiologic features strongly support an infectious origin. ? genetically predisposed hostsEpidemiology: Epidemiology 3,000 cases are diagnosed annually in the United States. The incidence in Asian children is substantially higher than in other racial groups , . In Japan , more than 170,000 cases have been reported since the 1960s.Cont.: Cont. The illness occurs predominantly in young children ; 80% of patients are younger than 5 yr only occasionally are teenagers and adults affected.Pathogenesis: Pathogenesis Severe vasculitis Edema of endothelial and smooth muscle cells Intense inflammatory infiltration of the vascular wall Weakens, resulting in dilatation or aneurysm formation Thrombi Fibrosis, intimal proliferation, stenosisPathogenesis (cont.): Pathogenesis (cont.) An inflammatory infiltrate in * myocardium * upper respiratory tract * pancreas *Kidney * biliary tractClinical Manifestations: Clinical Manifestations Kawasaki disease is generally divided into three clinical phases: 1.The acute febrile phase 2. The subacute phase 3. The convalescent phasePowerPoint Presentation: 1.The acute febrile phase - which usually lasts 1-2 wk - is characterized by fever and the other acute signs of illness.PowerPoint Presentation: 2. The subacute phase - Begins when fever and other acute signs have abated, but irritability, anorexia, and conjunctival injection may persist. - is associated with: * desquamation * thrombocytosis * development of coronary aneurysms * highest risk of sudden death. - This phase generally lasts until about the 4th wk.PowerPoint Presentation: 3.The convalescent phase -begins when all clinical signs of illness have disappeared - continues until the erythrocyte sedimentation rate (ESR) returns to normal, approximately 6-8 wk after the onset of illness.Diagnostic Criteria: Diagnostic Criteria - Fever for 5 or more days - Presence of 4 of the following : Bilateral conjunctival injection Changes in the oropharyngeal mucous membranes Changes of the peripheral extremities Rash Cervical adenopathy - Illness can’t be explained by other disease Lab Features : Lab Features WBC ESR, positive CRP Anemia Mild transaminases albumin Sterile pyuria, aseptic meningitis platelets by day 10-14Differential Diagnosis: Differential Diagnosis Measles Scarlet fever Drug reactions Viral exanthems Toxic Shock Syndrome Stevens-Johnson Syndrome Systemic Onset Juvenile Rheumatoid Arthritis Staph scalded skin syndrome1.Acute stage: Intravenous immunoglobulin 2 g/kg over 10-12 hr with aspirin 80-100 mg/kg/24 hr divided every 6 hr orally until 14th illness day 1.Acute stage TreatmentPowerPoint Presentation: Aspirin 3-5 mg/kg once daily orally until 6-8 wk after illness onset 2.Convalescent stage3.Long-term therapy for those with coronary abnormalities: 3.Long-term therapy for those with coronary abnormalities Aspirin 3-5 mg/kg once daily orally ± dipyridamole 4-6 mg/kg/24 hr divided in two or three doses orally (most experts add warfarin for those patients at particularly high risk of thrombosis)4.Acute coronary thrombosis: 4.Acute coronary thrombosis Prompt fibrinolytic therapy with tissue plasminogen activator , streptokinase , or urokinase under supervision of a pediatric cardiologistComplications : Complications Recovery is complete and without apparent long-term effects for patients who do not develop coronary disease Recurrent illness occurs in only 1-3% of cases Prognosis: Prognosis The prognosis for patients with coronary abnormalities depends on the severity of coronary disease. ,In Japan, fatality rates are now less than 0.1%. 50% of coronary artery aneurysms resolve echocardiographically by 1-2 yr after the illnessReferences: References Akagi T, Ogawa S, Ino T, et al: Catheter interventional treatment in Kawasaki disease: A report from the Japanese Pediatric Interventional Cardiology Investigation Group. J Pediatr 2000;137:181-6. Medline Similar articles American Heart Association Council on Cardiovascular Disease in the Young, Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease: Diagnostic guidelines for Kawasaki disease. Circulation 2001;103:335-6. Brown TJ, Crawford SE, Cornwall M, et al: CD8 T cells and macrophages infiltrate coronary artery aneurysms in acute Kawasaki disease. J Infect Dis 2001;184:940-3. Medline Similar articles Chang RKR: Hospitalizations for Kawasaki disease among children in the United States, 1988-1997. Pediatrics 2002;109:e87. Han RK, Silverman ED, Newman A, et al: Management and outcome of persistent or recurrent fever after initial intravenous gammaglobulin therapy in acute Kawasaki disease. Arch Pediatr Adolesc Med 2000;154:694-9. Medline Similar articles Rowley AH, Shulman ST, Mask CA, et al: IgA plasma cell infiltration of proximal respiratory tract, pancreas, kidney, and coronary artery in acute Kawasaki disease. J Infect Dis 2000;182:1183-91. Medline Similar articles Stockheim JA, Innocentini N, Shulman ST: Kawasaki disease in older children and adolescents. J Pediatr 2000;137:250-2. Medline Similar articlesPowerPoint Presentation: Thank you You do not have the permission to view this presentation. In order to view it, please contact the author of the presentation.