Many parents are confused when they suddenly hear about Kawasaki disease, saying they have never heard of the disease. What are the manifestations of Kawasaki disease? What are the risks? The following is a brief introduction to this disease.
I. What is Kawasaki disease?
Kawasaki disease, also known as cutaneous mucosal lymph node syndrome, was first reported by Dr. Tomisaku Kawasaki in Japan in 1967 and named after him. The clinical manifestations are often fever, rash, non-purulent lymph node enlargement in the neck, congestion of the conjunctiva of the eye, diffuse congestion of the oral mucosa, prune tongue, palmoplantar erythema, and hard edema of the hands and feet. It is an acute febrile exanthematous pediatric disease with systemic vasculitis as the main pathology. The disease often occurs in infants and children, but 80-85% of patients are within 5 years of age, preferably in infants aged 6 to 18 months. More boys, male: female 1.3 to 1.5:1.
Second, how is Kawasaki disease caused?
The cause of the disease is not yet clear. The disease is somewhat epidemic and landlord, with clinical manifestations such as fever and rash, presumably related to infection. It is generally believed that multiple pathogens may be involved, including EBV, retrovirus, or streptococcal or propionibacterium infections. Mycoplasma, rickettsiae, and dust mites have also been proposed as the etiologic agent of the disease, which has not been confirmed. Environmental pollution or chemical allergies have also been considered as possible causes of the disease.
What are the manifestations of Kawasaki disease?
1. Fever: Initially high fever of 39℃ or more, in some cases 40℃ or more, generally lasting about 11 days without treatment, with a long fever course of up to 3-4 weeks, a few can be longer; after regular treatment, it can generally fall within 1 to 2 days.
Antibiotic treatment is ineffective.
2. Extremity changes: erythema of palms and soles in the acute stage; hard puffiness of hands and feet. In the subacute stage (after 2 to 3 weeks), the nails of the fingers and toes peel around the nail, and in severe cases, the finger and toe nails may also fall off.
3. Enlarged lymph nodes in the neck: >1,5cm in diameter, usually unilateral non-suppurative enlargement.
4.Changes in the mouth and lips: erythema, lip chapping, diffuse congestion of the mucosa of the poplar tongue and oropharynx, without purulent discharge and pseudomembrane formation.
5, polymorphic erythema: soon after fever (about 1 to 4 days), a maculopapular rash or polymorphic erythema-like rash, occasionally prickly rash-like rash, mostly on the trunk, but no herpes and crust, about a week or so to subside. The most common of these is the redness and molting of the perianal skin within the first week of fever.
6. Non-exudative bilateral bulbar conjunctival congestion: it appears in 3-4 days after the onset of the disease, without purulent discharge, and dissipates after the fever subsides.
7. Cardiovascular system manifestations: congestive heart failure, myocarditis, pericarditis, and valvular regurgitation may appear in the first 1-6 weeks of the disease. Coronary artery damage mostly occurs in 2 to 4 weeks, manifesting as coronary artery dilation, aneurysm or stenosis, and a few may develop myocardial infarction. Aneurysms may also occur in other non-coronary arteries of moderate size. Raynaud’s phenomenon and gangrene may occur in the distal extremities.
8. Others: Arthritis and arthralgia; diarrhea, vomiting, abdominal pain, liver dysfunction, gallbladder fluid; peripheral neurosensitivity, aseptic meningitis, sensory hearing loss; urethritis, urethral stomatitis; erythema and sclerosis at the site of BCG inoculation. Degenerative rash in the groin area.
IV. Examination performance.
1, blood routine: the total number of white blood cells and granulocyte percentage increase in the acute stage, and the nucleus shifts left. Platelets start to increase in the second week. Mild anemia is seen in more than half of the patients. The blood is hypercoagulable.
2. Blood sedimentation increases significantly, up to 100 mm or more in the first hour.
3.C-reactive protein is increased.
4.Blood culture is negative.
5.Anti-streptococcal hemolysin O titer is normal. Rheumatoid factor and antinuclear body are negative. Serum complement is normal or slightly elevated. Albumin is decreased. IgG, IgA and IgA are increased.
6.The urine sediment can be seen as leukocytosis and/or proteinuria.
7.Electrocardiogram may show various changes, with ST-segment and T-wave abnormalities, and may also show prolonged P-R and Q-R intervals, abnormal Q waves and rhythm disturbances.
8.Cardiac ultrasound is suitable for cardiac examination and long-term follow-up in half of the disease can detect various cardiovascular lesions such as pericardial effusion, left ventricular enlargement, mitral valve insufficiency and coronary artery dilatation or aneurysm formation. It is best to check once a week during the acute and subacute phases of the disease, which is the most reliable non-invasive examination method for monitoring coronary aneurysms.
9. In cases presenting with aseptic meningitis, lymphocytes in the cerebrospinal fluid may be as high as 50 to 70/mm3. In some cases, a slightly higher serum bilirubin or glutamine is seen. Bacterial culture and viral isolation are negative results.
V. Treatment of Kawasaki disease.
1, human blood gammaglobulin: recent studies have confirmed that early intravenous gammaglobulin plus oral aspirin treatment can reduce the incidence of coronary artery aneurysms in Kawasaki disease. The method is to inject 400mg/kg of gammaglobulin intravenously for 2-4 hours for 4-5 days; or to add 50-100mg/kg?d of oral aspirin daily for 3-4 times for 4 days, and then reduce it to 5mg/kg?d in a single dose.
2. Aspirin: Early oral aspirin can control the acute inflammatory process and reduce coronary artery lesions. Dose of 30-100mg/kg per day, divided into 3-4 times. Take it for 14 days, after the fever subsides, reduce it to 3~5mg/kg per day, and take it in one dose to achieve the anti-platelet aggregation effect.
3.Corticosteroids: Unless complicated by severe myocarditis or persistent hyperthermia in severe cases, prednisone and aspirin can be used in combination.
4. Anticoagulation and thrombolytic therapy: Patients in the recovery period are treated with aspirin 3-5 mg/kg daily, taken once until the blood sedimentation and platelets return to normal. If there are no coronary artery abnormalities, the drug is usually discontinued 6-8 weeks after the onset of the disease. Thereafter, echocardiography was repeated at 6 months and 1 year. For patients with chronic phase of residual coronary artery, long-term anticoagulant medication and close follow-up are required. Patients with small solitary coronary aneurysms should be given long-term aspirin 3-5 mg/kg?d until the aneurysm subsides. For those who are intolerant to aspirin, use Pansentine 3-6mg/kg/day in 2-3 doses. Review the heart condition annually. If cardiac ultrasound, clinical data or exercise tests suggest myocardial ischemia, coronary angiography should be done. Patients with multiple or large coronary aneurysms should have long-term oral angiography. Patients with multiple or large coronary artery aneurysms should take oral aspirin and pentoxifylline for a long time. Patients with giant aneurysms are prone to thrombosis, coronary artery stenosis or occlusion and may be treated with oral Favarine anticoagulants. These patients should limit their activities and not participate in sports. The heart should be checked every 3 to 6 months. If there are signs of myocardial ischemia or a positive exercise test, coronary angiography should be performed to understand the progression of the stenotic lesion. Patients with occlusion of one or more major coronary arteries should receive long-term anticoagulation therapy, have their hearts checked repeatedly, including myocardial scans, exercise tests, and coronary angiograms, and consider surgical treatment. Intracoronary administration by intravenous or catheter percutaneous puncture of the coronary arteries is used to induce coronary revascularization and myocardial reperfusion in patients with infarcts and thrombosis of the heart. Urokinase is administered within 1 hour of intravenous thrombolysis at 20,000 u/kg, followed by 3000-4000 u/kg per hour. Streptokinase can also be used. 10,000 u/kg of streptokinase can be administered within 1 hour of intravenous thrombolysis, followed by another dose half an hour later. The above drugs dissolve fibrin rapidly, with better effect and no adverse reaction.
Sixth, the prognosis of Kawasaki disease.
The vast majority of children have a good prognosis, self-limiting, and can gradually recover with appropriate treatment. However, coronary artery aneurysms can occur in 15-30% of Kawasaki disease patients. Death due to coronary artery aneurysm, thromboembolism or myocarditis accounts for 1 to 2% of all cases, and sudden death can occur even during the recovery period. The number of sequelae of ischemic heart disease is very small. Recurrence occurs in about 2% of cases.