Kawasaki disease - symptoms and signs, treatment
Symptoms and signs, treatment of Kawasaki disease
Kawasaki disease- It is vasculitis, sometimes with a lesion of the coronary arteries, which tends to occur in infants and children aged 1 to 8 years. This disease is characterized by fever, exanthema, conjunctivitis, inflammation of the mucous membranes and lymphadenopathy. Coronary artery aneurysm may develop and rupture or cause myocardial infarction. Diagnosis is based on clinical criteria; at statement of the diagnosis carry out an echocardiography. Treatments include aspirin and intravenous immunoglobulin. Thrombosis of the coronary arteries may require fibrinolytic therapy or percutaneous intervention.
Kawasaki disease - It is a vasculitis of arteries of medium caliber, the most important is the defeat of the coronary arteries, which are involved in approximately 20% of patients with no treatment.Early manifestations include acute myocarditis with heart failure, arrhythmias, endocarditis, and pericarditis. Subsequently, coronary artery aneurysms can form. Giant coronary artery aneurysms, despite the fact that they are rare, have the highest risk of cardiac tamponade, thrombosis or infarction. BK is the leading cause of acquired heart disease in children. Inflammation can also develop in extravascular tissues, including the upper respiratory tract, pancreas, bile ducts and kidneys.
Etiology is unknown, but epidemiology and clinical manifestations suggest the role of an infection or an abnormal immune response to infection in genetically predisposed children. Especially high incidence among children of Japanese origin, but BC is everywhere. In the United States, there are 3,000–5,000 cases annually. The ratio of boys to girls is approximately 1.5: 1. 80% of patients under 5 years old. Rarely, this disease occurs in adolescents, adults and infants less than 4 months old. Cases of the disease are recorded all year round, however most often in the spring and winter.Group cases of Kawasaki disease have been reported, with no obvious signs of transmission from person to person. Approximately 2% of patients have repeated episodes, usually months and years later.
Symptoms and signs of Kawasaki disease
The disease tends to progress in stages, starting with fever for at least 5 days, usually intermittent and above 39 ° C, combined with anxiety, sometimes drowsiness or intermittent colitis-like abdominal pain. Usually, within 1 or 2 days from the onset of fever, a bilateral injection of the vessels of the conjunctiva of the eye appears without signs of exudation. Within 5 days a polymorphic erythematous spotted rash appears, first on the body, often with thickening in the crotch area. The rash can be urtikarnoy, core-like, scarlet-like or erythema multiforme. It is accompanied by injection of the vessels of the pharynx; red, dry, cracked lips and red "crimson" language. During the 1st week, there may be pallor of the proximal areas of the nail bed on the hands and feet. At about the 3-5th day of Kawasaki disease, an erythema or violet-red hue and variable swelling of the palms and feet appear.Although the edema can be expressed weakly, the edematous area is often tense, dense, there is no depression on it when pressed. Approximately on the 10th day, desquamation begins near the nails, on the palms, on the feet, and in the crotch area. The surface layer of the skin sometimes exfoliates large fragments, exposing the new unaltered skin. Painful purulent lymphadenopathy in the neck is noted during the disease in 50% of patients. Kawasaki disease can last from 2 to 12 weeks or even longer. There may be incomplete or atypical cases of the disease, especially in very young children with a high risk of developing coronary artery disease. These manifestations are observed in approximately 90% of patients.
Other less specific signs indicate the involvement of many systems. Arthritis and arthralgia develop at about1/3patients. Other clinical manifestations include urethritis, aseptic meningitis, hepatitis, otitis media, vomiting, diarrhea, and anterior uveitis.
Cardiac manifestations usually appear in the subacute phase of the syndrome approximately 1-4 weeks after the debut, when the rash, fever and other early acute symptoms begin to disappear.
The diagnosis is based on clinical criteria. Similar symptoms can occur with scarlet fever, staphylococcal exfoliative syndromes, measles, drug reactions and juvenile RA; less common masks are leptospirosis and Rocky Mountain spotted fever.
Laboratory tests are not diagnostic, but may be performed to rule out other diseases. Typically, a complete blood count, determination of antinuclear antibody titer, rheumatoid factor, ESR, and blood cultures and pharyngeal swab are usually shown. In the acute period, leukocytosis is often noted, often with a significant shift towards immature cells. Other hematologic changes include mild normocytic anemia, thrombocytosis at the 2nd or 3rd week of the disease, as well as increased ESR and C-reactive protein. AHA, RF and crops are negative. Other disorders, depending on the damage to a particular organ, include sterile pyuria, elevated liver enzymes, proteinuria and pleocytosis in the cerebrospinal fluid.
It is important to consult a child with a pediatric cardiologist. In the diagnosis of Kawasaki disease, ECG and echocardiography are performed; since violations may not appear until the late stages, these studies are repeated after 2–3 weeks,6–8 weeks and 6–12 months after the onset of the disease. On the ECG, you can see arrhythmias, reduced voltage, left ventricular hypertrophy. Echocardiography can reveal coronary artery aneurysms, valve regurgitation, pericarditis, or myocarditis. Sometimes it is useful to conduct coronary arteriography, mainly in patients with aneurysms and abnormal stress test results.
Without treatment of Kawasaki disease, mortality can reach 1%, usually death occurs within 6 weeks of the onset of the disease. Mortality is less than 0.01% in the United States with adequate treatment. Prolonged fever increases the risk of heart damage. Death is most often the result of cardiac complications and can be sudden and unpredictable: more than 50% of deaths occur within 1 month from the onset of the disease, 75% - within 2 months and 95% - within 6 months, but death can even occur after 10 years. Effective therapy reduces the acute manifestations and, more importantly, the incidence of coronary artery aneurysms from 20% to less than 5%. In the absence of coronary artery disease, the prognosis for complete recovery is favorable. About2/3coronary aneurysms regress within 1 year, although it is not known whether residual coronary stenosis remains. Giant coronary aneurysms are less likely to regress and require more intensive dynamic observation and treatment.
Kawasaki disease treatment
The patient should be treated by an experienced pediatric cardiologist and / or a child infectious disease specialist or with their direct involvement. Treatment of Kawasaki disease begins as quickly as possible, optimally during the first 10 days of the disease, a combination of high doses of intravenous immunoglobulin is given, a dose of 2 g / kg is administered once for 10-12 hours, and high doses of aspirin are given orally, 20-25 mg / kg orally 4 times a day. The dose of aspirin is reduced to 3-5 mg / kg once a day after the child has a normal temperature for 4-5 days; some experts prefer to continue therapy with high doses of aspirin before the 14th day of the disease. The metabolism of aspirin is unstable during the acute period of the BC, which partly explains the requirements for the administration of high doses. Some specialists monitor serum levels of aspirin during high-dose therapy, especially if performed for 14 days.
Most children have a sharp response during the first 24 hours of therapy. A small proportion of children continue to fever for several days; in such cases, repeated IVIG administration is required. An alternative regimen that can lead to a slower disappearance of symptoms of Kawasaki's disease can be shown to children with cardiac dysfunction who can not tolerate infusion volume for the administration of 2 g / kg of IVIG, includes IVIG 400 mg / kg once daily for 4 days . The effectiveness of IVIG / aspirin therapy at its onset after the 10th day of illness is unknown, but you should still think about it.
After the child has an improvement in the course of 4-5 days, the treatment of the disease is continued with aspirin at a dose of 3-5 mg / for at least 8 weeks after the start of the re-echocardiographic examination. Aspirin can be canceled if coronary artery aneurysms and signs of inflammation are absent. In connection with the anti-rombotic effect of aspirin, they are continued to be treated for a long time in the presence of a lesion of the coronary arteries. Children with giant coronary aneurysms may need additional administration of anticoagulants.
Children who receive IVIG therapy may experience a decreased response to live viral vaccines. Therefore, vaccination against measles, mumps and rubella should be postponed for 11 months after treatment with IVIG, and vaccination against varicella for 11 months or more. If the risk of measles is high, vaccination should be carried out, but revaccination should be carried out after 11 months.
In children receiving long-term therapy with aspirin, during a flu or chickenpox disease there is a small risk of developing Ray's syndrome, therefore annual vaccination against influenza is indicated for children receiving aspirin for a long time. In the future, the parents of the child receiving aspirin should be instructed about the need to consult a doctor who has their child, if he was in contact or was ill with influenza or chicken pox. Aspirin can be temporarily discontinued.
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