Kawasaki disease - a generalized vasculitis of unknown etiology, also known as lymph node syndrome mukokutannogo or periarteritis nodosa of childhood. In developed countries, Kawasaki disease has overtaken the prevalence of acute rheumatic fever as a cause of acquired heart disease.
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| Kawasaki disease (Kawasaki syndrome) |
In the event of disease relevant genetic factors, assumed the nature of infectious disease.
It is estimated that the prevalence of the disease in the United States is 10 per 100,000 children younger than 5 years (neaziaty) and 44 children per 100 000 (Asians). The mortality is about 1%. Untreated, aneurysms of the coronary arteries razivayutsya 25% of children. In the treatment of gamma-globulin and with the beginning of the previous 10-day incidence of aneurysms of the coronary arteries is reduced to 5-10%. 80% of patients diagnosed with "Kawasaki Disease" - children under 4 years old. Up to 3 years, the disease is rare.
Pathogenesis
Central role in the genesis of vasculitis attributed to antibodies to endothelial cells and viruses. In diseased vessels develop inflammation with necrosis of the medial smooth muscle cells. Artery aneurysms may be formed by bundles of elastic layer. After 4-8 weeks developed fibrosis of the vessel wall, proliferation and intimal thickening, develop severe general symptoms. In diseased vessels develop stenosis, thrombosis and occlusion of the possible. Thus, when Kawasaki disease may develop a myocardial infarction due to thrombosis or rupture of an aneurysm of coronary artery disease. Affects the average diameter of the arteries: renal, parovarial, splanhnicheskie, axillary, and others, as well as veins, capillaries, arterioles and larger arteries.
Clinic
In young children, after the initial 3-4-week phase at a temperature 38-40 ° C causes the formation of microaneurysms in the coronary arteries. Fever is usually higher than 39,9 ° C for not less than 5 days, is intermittent in nature. No treatment may be delayed for 1-2 weeks, may not respond to antipyretics, but disappears within 1-2 days after starting treatment with intravenous gamma globulin. In addition, the non-specific skin rash appears, the total heavy symptoms, conjunctivitis, cracked lips, crimson tongue and painful swelling of lymph nodes. Characterized by the plantar and palmar erythema, accompanied by swelling and subsequent desquamation.
In the analyzes indicated a leukocytosis due to granulocytosis may be anemia and thrombocytosis in 2-3 weeks (up to 700 * 109 / L). Increased erythrocyte sedimentation rate, C-reactive protein, α1-antitrypsin. Complement is normal. May be slightly elevated transaminases (40% of patients).
In general, urine analysis may show pyuria, proteinutriya.
A heart attack is diagnosed by mikarda kardiospetsifichnym enzymes and ECG. The ECG can also be determined by the signs of myocarditis, myocardial infarction.
WGC on the radiograph may be determined by cardiomegaly. Cardiac status of the requested monitored by echocardiography. Possible to perform CT angiography and MR angiography of the heart.
Kawasaki syndrome is often a diagnosis of exclusion, differential diagnosis is carried out with scarlet fever, staphylococcal skin syndrome, a syndrome of Stephen Jones, a reaction to medication, JRA, measles, mercury poisoning, viral rash.
Treatment
The main objective of therapy for Kawasaki disease - to prevent the development of aneurysms of the coronary arteries, thereby reducing mortality. The basis of therapy - intravenous gamma globulin (IVIG) in the full dose - 2 g / kg for 10-12 hours. In addition, aspirin is usually prescribed, and possibly corticosteroid hormones.
Traditionally, the treatment of Kawasaki disease used aspirin. However, according to some studies, aspirin in high and medium doses (up to 100 mg / kg day 2-4 of admission to the period of fever, after lowering the temperature - 3-5 mg / kg / day for 3 months) even with early therapy did not increase the effectiveness of intravenous gamma globulin, ie, did not affect the duration of fever and incidence of coronary artery aneurysms. Therefore, the advisability of appointing salicylates in Kawasaki disease remains controversial.
Is poorly understood and the use of glucocorticosteroid. According to a randomized study of Japanese, in addition to corticosteroids, intravenous immunoglobulin did not reduce the incidence of aneurysms of the coronary arteries, no significant effect on the duration of fever, C-reactive protein. In another randomized study in the United States, a single dose of methylprednisolone (30 mg / kg) in addition to conventional therapy resulted in a significant reduction in ESR after a week of starting treatment, but had no effect on the frequency of anomalies of the coronary arteries.
During treatment it is important to the dynamic cardiac monitoring (see above). After resolution of fever, development of heart disease is unlikely. It is important to repeat echoscopy heart about a month after the onset of fever. A rare complication is endocarditis with mitral valve lesions requiring valve prosthesis. Patients with large aneurysms of the coronary arteries later appointed antiaggregantnaya therapy (usually aspirin). The frequency of recurrence of the disease - from 1 to 3%.
It is estimated that the prevalence of the disease in the United States is 10 per 100,000 children younger than 5 years (neaziaty) and 44 children per 100 000 (Asians). The mortality is about 1%. Untreated, aneurysms of the coronary arteries razivayutsya 25% of children. In the treatment of gamma-globulin and with the beginning of the previous 10-day incidence of aneurysms of the coronary arteries is reduced to 5-10%. 80% of patients diagnosed with "Kawasaki Disease" - children under 4 years old. Up to 3 years, the disease is rare.
Pathogenesis
Central role in the genesis of vasculitis attributed to antibodies to endothelial cells and viruses. In diseased vessels develop inflammation with necrosis of the medial smooth muscle cells. Artery aneurysms may be formed by bundles of elastic layer. After 4-8 weeks developed fibrosis of the vessel wall, proliferation and intimal thickening, develop severe general symptoms. In diseased vessels develop stenosis, thrombosis and occlusion of the possible. Thus, when Kawasaki disease may develop a myocardial infarction due to thrombosis or rupture of an aneurysm of coronary artery disease. Affects the average diameter of the arteries: renal, parovarial, splanhnicheskie, axillary, and others, as well as veins, capillaries, arterioles and larger arteries.
Clinic
In young children, after the initial 3-4-week phase at a temperature 38-40 ° C causes the formation of microaneurysms in the coronary arteries. Fever is usually higher than 39,9 ° C for not less than 5 days, is intermittent in nature. No treatment may be delayed for 1-2 weeks, may not respond to antipyretics, but disappears within 1-2 days after starting treatment with intravenous gamma globulin. In addition, the non-specific skin rash appears, the total heavy symptoms, conjunctivitis, cracked lips, crimson tongue and painful swelling of lymph nodes. Characterized by the plantar and palmar erythema, accompanied by swelling and subsequent desquamation.
In the analyzes indicated a leukocytosis due to granulocytosis may be anemia and thrombocytosis in 2-3 weeks (up to 700 * 109 / L). Increased erythrocyte sedimentation rate, C-reactive protein, α1-antitrypsin. Complement is normal. May be slightly elevated transaminases (40% of patients).
In general, urine analysis may show pyuria, proteinutriya.
A heart attack is diagnosed by mikarda kardiospetsifichnym enzymes and ECG. The ECG can also be determined by the signs of myocarditis, myocardial infarction.
WGC on the radiograph may be determined by cardiomegaly. Cardiac status of the requested monitored by echocardiography. Possible to perform CT angiography and MR angiography of the heart.
Kawasaki syndrome is often a diagnosis of exclusion, differential diagnosis is carried out with scarlet fever, staphylococcal skin syndrome, a syndrome of Stephen Jones, a reaction to medication, JRA, measles, mercury poisoning, viral rash.
Treatment
The main objective of therapy for Kawasaki disease - to prevent the development of aneurysms of the coronary arteries, thereby reducing mortality. The basis of therapy - intravenous gamma globulin (IVIG) in the full dose - 2 g / kg for 10-12 hours. In addition, aspirin is usually prescribed, and possibly corticosteroid hormones.
Traditionally, the treatment of Kawasaki disease used aspirin. However, according to some studies, aspirin in high and medium doses (up to 100 mg / kg day 2-4 of admission to the period of fever, after lowering the temperature - 3-5 mg / kg / day for 3 months) even with early therapy did not increase the effectiveness of intravenous gamma globulin, ie, did not affect the duration of fever and incidence of coronary artery aneurysms. Therefore, the advisability of appointing salicylates in Kawasaki disease remains controversial.
Is poorly understood and the use of glucocorticosteroid. According to a randomized study of Japanese, in addition to corticosteroids, intravenous immunoglobulin did not reduce the incidence of aneurysms of the coronary arteries, no significant effect on the duration of fever, C-reactive protein. In another randomized study in the United States, a single dose of methylprednisolone (30 mg / kg) in addition to conventional therapy resulted in a significant reduction in ESR after a week of starting treatment, but had no effect on the frequency of anomalies of the coronary arteries.
During treatment it is important to the dynamic cardiac monitoring (see above). After resolution of fever, development of heart disease is unlikely. It is important to repeat echoscopy heart about a month after the onset of fever. A rare complication is endocarditis with mitral valve lesions requiring valve prosthesis. Patients with large aneurysms of the coronary arteries later appointed antiaggregantnaya therapy (usually aspirin). The frequency of recurrence of the disease - from 1 to 3%.
source:heaalthy.blogspot.com
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