儿科造血系统2-kawasakidisease英文

1、Kawasaki Disease,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,

2、 including diffuse hyperemia, strawberry tongue, and lip changes (eg, swelling, fissuring, erythema, bleeding),Diagnostic criteria,4.Peripheral extremity changes, including erythema, edema, induration, and desquamation 5.Nonpurulent cervical lymphadenopathy 6.Nonexudative bilateral conjunctivitis,Di

3、agnostic 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). Thro

4、at carriage of group A streptococcus 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-t

5、o-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 enzymes AST, ALT bilirubin ,Lab Studies,Cardiac enzymes （ CK，CK-MB, car

6、diac 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. Echocardiograp

7、hy should be repeated in the second or third week of illness and 1 month after all other laboratory results have normalized.,Imaging Studies,Ultrasonography: Gall bladder ultrasonography （liver or gall bladder dysfunction ） scrotal ultrasound to evaluate for epididymitis.,Imaging Studies,MRA: define

8、s 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 preve

9、nt 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 proinf

10、lammatory 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 for 4 d-5dAlternatively,; 2 g/kg IV infused over 12 h once as single dose,Drug Ca

11、tegory,Aspirin decrease inflammation, inhibit platelet aggregation improve complications of venous stases and thrombosis. Irreversibly inactivates cyclooxygenase, ultimately preventing thromboxane A2 production in platelets.,Drug Category,Aspirin 80-100 mg/kg/d PO divided qid for 2 wk initially; the

12、n 5-10 mg/kg PO qd for 6-8 wk until sedimentation rate and platelet count are within the reference range, typically used for 6-12 wk,Drug Category,Corticosteroid Not recommended to use only. Prescript only when the therapeutic effect of IVIG is not satisfied. 2mg/kg, 24 weeks,Complications,Cardiovas

13、cular Significant heart failure or myocardial dysfunction (unlikely to occur once fever is resolved) Diffuse coronary artery ectasia and aneurysm formation, giant aneurysm (internal luminal diameter 8 mm) MI,Complications,Cardiovascular Myocarditis (common but rarely causes CHF) Valvulitis, usually

14、mitral (only occurs in 1% of patients and rarely requires valve replacement) Pericarditis with small pericardial effusions (occurs in 25% of patients with acute illness) Systemic artery aneurysms Rupture of CAA with hemopericardium,Other complications,Extreme irritability, especially in younger infa

15、nts Aseptic meningitis Arthritis Mild hepatic dysfunction, rarely jaundice 巨嗜细胞活化过度综合症：死亡原因之一,Other complications,Gallbladder hydrops (diagnosed by means of ultrasonography but usually resolves without surgical intervention) Diarrhea Pneumonitis Otitis media,Other complications,Erythema and induration at the site of BCG inoculation (reported in Japan) Periphe