Abstract
Kawasaki disease (KD) is an acute multisystemic vasculitis of unknown etiology that occurs in infants and children. Diagnosis is based on clinical criteria that include fever, exanthema, conjunctivitis, changes in the extremities, erythema of oral mucosa and lips and cervical lymphadenopathy. We investigated the clinical signs and therapy efficacy of patients with KD who admitted to our department. We reviewed 22 patients in Russia, with an age range of 2,5 months to 12 years. Moreover, 426 cases of children with KD admitted to the Children’s Hospital of Shaanxi Provincial People’s Hospital (China) from December 2013 to March 2018 were selected and compared. In case of hospitalization within 10 days of fever we reviewed incomplete KD in 5 patients (25%). In 3 patients KD was finally diagnosed either after antibiotics had been ineffective or when other symptoms of KD emerged, in 70% of cases the diagnosis of KD was undoubted. There was also no doubt of diagnosis in all patients admitted to our department after 10 days of fever. 77% of patients recovered after one dose (2 g/kg) of intravenous immunoglobulin (IVIG) and in 23% of cases it was necessary to insert the second dose. Clinicians should consider the KD diagnosis in young children with persistent inexplicable fever to start IVIG treatment within 10 days to prevent development of coronary aneurysms.
Highlights
The Kawasaki disease (KD) or mucous and skin lymphonodular syndrome – an acute general disease with primary damage of average and small arteries and development of a destructive and proliferative vasculitis
The analysis of thousand of proteinaceous molecules showed that about 190 of them could be found in urine of children with KD. 2 proteins, associated with failure of endothelial and muscle cells, were identified: filamin C and immunoregulator meprin A, which are considerably raised at the patients, who did not respond to starting therapy in comparison with the patients, who ceased to be in a fever after the first dose of intravenous immunoglobulin (IVIG)
Among the 17 children with Kawasaki disease with coronary artery aneurysm, there was no significant difference in the degree of increase of ESR between 1 week and 2 weeks
Summary
The Kawasaki disease (KD) or mucous and skin lymphonodular syndrome – an acute general disease with primary damage of average and small arteries and development of a destructive and proliferative vasculitis. Since 1961, he examined children with persistent fever and such symptoms as cervical lymphadenitis, conjunctivitis, rash, edema of brushes and feet with the subsequent peeling of fingers. This symptom complex did not match any other known disease. T. Kawasaki described about 50 cases, this state had the name “febrile oculo-oro-kutaneo-acrodesquamative syndrome with not purulent cervical lymphadenitis”. Clinical guidelines for the diagnosis and treatment of Kawasaki syndrome have been developed in Russia [3]
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