Transcript 幻灯片 1
Kawasaki disease is a rare condition. It is most common in children under five years old and most cases occur in children aged between nine months and two years. Cases occur in all ethnic origins, it is more common in children of Asian origin and more common in boys than girls. Origin In 1967, a Japanese allergist, Professor Kawasaki, first described an outbreak of a peculiar illness in young children in Japan. One of the patients was his son. Several years later, a few outbreaks were reported in other countries and the development of coronary vasculitis were reported. Kawasaki disease occurs year-round, but a greater number of cases are reported in the winter and spring. Annual incidence rates in the United States and Canada range from about 6 to 11 cases per 100,000 children less than five years, the incidence rates in North America are highest in children of Asian ethnicity (especially those of Japanese or Korean background). In Japan, the yearly incidence rate was 90 cases per 100,000 children less than five years. A variety of infectious agents have been proposed, however, an etiologic agent has not been documented. The immune system is known to be involved during the acute stage of Kawasaki disease. In response to an unknown triggering process, marked immunoregulatory abnormalities are observed. Antibodies may then target the vascular endothelium, resulting in a cascade of events leading to vascular damage. A variety of infectious agents have been proposed, however, an etiologic agent has not been documented. The immune system is known to be involved during the acute stage of Kawasaki disease. In response to an unknown triggering process, marked immunoregulatory abnormalities are observed. Antibodies may then target the vascular endothelium, resulting in a cascade of events leading to vascular damage. Acute phase Subacute phase Convalescent phase Acute Phase The first set of symptoms commonly last up to 10 or 11 days. They include: Sudden onset of fever (high temperature ) Conjunctivitis Changes in the mouth: a red throat, spotty tongue or cracked lips A blotchy pink rash Enlarged lymph node in the neck Swelling and redness of the hands and feet Subacute Phase Gradual subsiding of fever Marked thromb Changes in the mouth: a red throat, spotty tongue or cracked lips A blotchy pink rash Enlarged lymph node in the neck Swelling and redness of the hands and feet Convalescent Phase Coronary artery dilation or aneurysm. Convalescent Phase Echocardiogram documented coronary artery dilation or aneurysm. Convalescent Phase Angiographically documented coronary artery dilation or aneurysm. Elevated leukocyte count with a predominance of neutrophils or a normal leukocyte count with a left shift is typical on the acute phase of illness. Elevated ESR is almost universally present in acute phase. Thrombocytosis in the later stages is common. Ig: In acute phase low levels of IgG are reported. In subacute phase elevated levels of IgG, IgM, IgE, IgA have been reported. Echocardiography may provide the presence and type of coronary artery disease in patients with Kawasaki disease. PRINCIPAL SYMPTOMS Fever: persisting 5 days or more (inclusive of those cases in whom the fever has subsided before the 5th day in response to therapy) PRINCIPAL SYMPTOMS Bilateral conjunctival congestion Characters: Bilateral painless bulbar conjunctival injection without exudate PRINCIPAL SYMPTOMS Changes of lips and oral cavity: Reddening of lips, Strawberry tongue, Diffuse injection of oral and pharyngeal mucosa. PRINCIPAL SYMPTOMS Polymorphous exanthema PRINCIPAL SYMPTOMS Changes of peripheral extremities: Initial stage: Reddening of palms and soles, Indurative edema Convalescent stage: Membranous desquamation from fingertips PRINCIPAL SYMPTOMS Acute nonpurulent cervical lymphadenopathy ≥ 1.5 cm in diameter OTHER SIGNIFICANT SYMPTOMS OR FINDINGS Cardiovascular : Auscultation (heart murmur, gallop rhythm, distant heart sounds) ECG changes (prolonged PR/QT intervals, abnormal Q wave, low-voltage QRS complexes, ST-T changes, arrhythmias), Chest X-ray findings (cardiomegaly) 2-D echo findings (pericardial effusion, coronary aneurysms) Aneurysm of peripheral arteries other than coronary (axillary etc.) OTHER SIGNIFICANT SYMPTOMS OR FINDINGS GI tract : Diarrhea, Vomiting, Abdominal pain, Hydrops of gall bladder, Paralytic ileus, Mild jaundice, Slight increase of serum transaminase Urine : Proteinuria, Increase of leukocytes in urine sediment OTHER SIGNIFICANT SYMPTOMS OR FINDINGS Blood :Leukocytosis with shift to the left, Thrombocytosis, Increased ESR, Positive CRP, Hypoalbuminemia, Increased α2-globulin, Slight decrease in erythrocyte and hemoglobin levels Respiratory :Cough, Rhinorrhea, Abnormal shadow on chest X-ray OTHER SIGNIFICANT SYMPTOMS OR FINDINGS Skin :Redness and crust at the site of BCG inoculation, Small pustules, Transverse furrows of the finger nails Joint :Pain, Swelling Neurological :CSF pleocytosis, Convulsion, Unconsciousness, Facial palsy, Paralysis of the extremities REMARKS For item 5 under principal symptoms, the convalescent stage is considered important. Non-purulent cervical lymphadenopathy is less frequently encountered (approximately 65%) than other principal symptoms during the acute phase. Male : Female ratio : 1.3-1.5 : 1, patients under 5 years of age : 80-85%, fatality rate : 0.1% REMARKS Recurrence rate : 2-3%, proportion of siblings cases : 1-2% Approximately 10 percent of the total cases do not fulfill five of the six principal symptoms, in which other diseases can be excluded and Kawasaki disease is suspected. In some of these patients coronary artery aneurysms (including so-called coronary artery ectasia) have been confirmed. Staphylococcal and streptococcal diseases Leptospirosis A variety of viral infections Drug reactions Steven-Johnson Syndrome JRA Therapies include intravenous immunoglobulin, ASA, and antithrombotics. Treatment of Kawasaki's disease in the first 10 days of illness with a single 2g/kg dose of IVIG and with aspirin at 80 - 100 mg/kg/day reduces the prevalence of coronary artery abnormalities. This non-specific but highly effective therapy is well accepted. High dose of aspirn is continued until signs of acute phase (7~10 days) ,it should be reduced to 5~10 mg/kg/d when the platelet count increase and should be discontinued once the platelet count normalize. Corticosteroids is disputed. We prefer not use corticosteroids in the pre-IVIG period and should be used in recurrent or severe cases.