儿科造血系统-kawasakidisease英文课件

1、Kawasaki Disease,站,Introduction,Kawasaki disease (KD) is a common vasculitic disorder usually seen in children below 5 years of age The leading cause of acquired heart disease in children,Kawaski T.,Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children: clinical observations of 50 cases. Jpn J Allergol.1967; 16 :178 222,China kawasaki disease,Epidemiologic study of admitted children with Kawasaki disease in Beijing from 1995

2、 to 1999 :The incidence of KD in Beijing is lower than that reported in Japan, similar to the incidence in the United States and higher than in those other Western countries.,Etiology,No one knows what causes Kawasaki disease. But it is thought to start from an infection or from exposure to some toxin. There is no firm evidence that the disease can spread from one person to another. Superantigen-producing bacteria.,Histroy,Kawasaki disease has 3 stages, as follows Acute stage (1-11 d) Subacute s

3、tage (11-30 d) Convalescent/chronic phase (30 d),Acute stage (1-11 d),1. High fever (temperature 39) 2. Nonexudative bilateral conjunctivitis (90%) 3. Polymorphous erythematous rash 4. Acral erythema and edema that impede ambulation 5. Strawberry tongue and lip fissures 6. Lymphadenopathy (75%), generally a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm,Acute stage,Nonexudative bilateral conjunctivitis (90%),Acute stage,Polymorphous erythematous rash,Acute stage,Ac

4、ral erythema and edema that impede ambulation,Acute stage,Strawberry tongue and lip fissures,Acute stage,Lymphadenopathy (75%), generally a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm,Acute stage (1-11 d),7. Hepatic, renal, and gastrointestinal dysfunction 8. Myocarditis and pericarditis 9. Irritability 10. Anterior uveitis (70%) 11. Perianal erythema (70%),Irritability,Subacute stage (11-30 d),Persistent irritability, anorexia, and conjunctival injection Decrea

5、sed temperature Thrombocytosis Acral desquamation Aneurysm forms,站,Aneurysm forms,Convalescent/chronic phase (30 d),Expansion of aneurysm Possible MI A tendency for smaller aneurysms to resolve on their own (60% of cases),Diagnostic criteria,1.Fever( 5 days) and refractory to appropriate antibiotic therapy 2.Polymorphous erythematous rash 3.Oropharyngeal changes, including diffuse hyperemia, strawberry tongue, and lip changes (eg, swelling, fissuring, erythema, bleeding),Diagnostic criteria,4.Pe

6、ripheral extremity changes, including erythema, edema, induration, and desquamation 5.Nonpurulent cervical lymphadenopathy 6.Nonexudative bilateral conjunctivitis,Diagnostic criteria,Patients with classic Kawasaki disease must have 5 of the former symptoms, with fever an absolute criterion.,Differentials,Staphylococcal infection (such as scalded skin syndrome, toxic shock syndrome) Streptococcal infection (such as scarlet fever, toxic shock-like syndrome). Throat carriage of group A streptococcu

7、s does not exclude the possibility of Kawasaki disease,Differentials,Measles and other viral exanthems Leptospirosis Rickettsial disease Stevens-Johnson syndrome Drug reaction Juvenile rheumatoid arthritis,Lab Studies,Mild-to-moderate normochromic anemia moderate-to-high WBC count ESR , C-reactive protein , and serum a-1-antitrypsin . Culture results are all negative,Lab Studies,ANA, RF,ASO normal Platelets Thrombocytosis （2-3w） associated with severe coronary artery disease and MI. Liver enzyme

8、s AST, ALT bilirubin ,Lab Studies,Cardiac enzymes （ CK，CK-MB, cardiac troponin, LDH） Radiography: rule out cardiomegaly or subclinical pneumonitis.,Imaging Studies,Echocardiography: rule out CAAs and myocarditis, valvulitis, or pericardial effusion. Diffuse dilatation of coronary lumina can be observed in 50% of patients by the 10th day of illness. Echocardiography should be repeated in the second or third week of illness and 1 month after all other laboratory results have normalized.,Imaging St

9、udies,Ultrasonography: Gall bladder ultrasonography （liver or gall bladder dysfunction ） scrotal ultrasound to evaluate for epididymitis.,Imaging Studies,MRA: defines CAA in patients with Kawasaki disease. noninvasive.,Other Tests,ECG acute infarction. Tachycardia, a prolonged PR interval, ST-T wave changes, decreased voltage of R waves may indicate myocarditis. Q waves or ST-T wave changes may indicate an MI.,Medical Care,The main goal of treatment is to prevent coronary artery disease and relieve symptoms.: Full doses of salicylates (aspirin); intravenous gammaglobulin are the mainstays of treatment.,Drug Category,IVIG (first line but not the sole therapy) Neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, blocks Fc receptors on macrophages suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination,IVIG,400 mg/kg/d IV as a single daily infusion

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