Abstract
BackgroundKawasaki disease (KD) is a rare vasculitis seen predominantly in children. In developing countries, it is the leading cause of childhood-acquired heart disease. Besides a case report from 1981 there have been no data published dealing with the epidemiology and clinical aspects of KD in Austria.MethodsThe purpose of the present study was to investigate the clinical spectrum of KD in a geographically determined cohort of infants, children, and adolescents that were diagnosed and treated at the University Hospital of Innsbruck from 2003–2012.ResultsThirty-two patients were included in the study with a median age of 32.96 months (2–192). 59.4% of the patients were aged between six months and four years. The male-to-female ratio was 1:1.13. Clinical examination revealed non-purulent conjunctivitis and exanthema as the most common symptoms (84.4%). 75% showed oropharyngeal changes, 21.9% had gastrointestinal complaints such as diarrhoe, stomachache or vomiting prior to diagnosis. One third of the patients were admitted with a preliminary diagnosis, whereas 78.1% were pre-treated with antibiotics. The median fever duration at the time of presentation was estimated with 4.96 days (1–14), at time of diagnosis 6.76 days (3–15).75% were diagnosed with complete KD, and 25% with an incomplete form of the disease. There was no significant difference in the duration of fever neither between complete and incomplete KD, nor between the different age groups. Typical laboratory findings included increased C-reactive protein (CRP) (80.6%) and erythrocyte sedimentation rate (ESR) (96%),leukocytosis (48.4%) and thrombocytosis (40.6%) without any significant quantitative difference between complete and incomplete KD. Coronary complications could be observed in six patients: one with a coronary aneurysm and five with tubular dilatation of the coronary arteries. Our patient cohort represents the age distribution as described in literature and emphasizes that KD could affect persons of any age. The frequency of occurrence of the clinical symptoms differs from previous reports – in our study, we predominantly observed non-purulent conjunctivitis and exanthema.ConclusionKD should always be considered as a differential diagnosis in a child with fever of unknown origin, as treatment can significantly decrease the frequency of coronary complications.Electronic supplementary materialThe online version of this article (doi:10.1186/1546-0096-12-37) contains supplementary material, which is available to authorized users.
Highlights
Kawasaki disease (KD) is a rare vasculitis seen predominantly in children
Based on the observation that over 80% occur between the age of six months and four years, we categorized our patient cohort into three age groups: younger than six months, six months to four years, and older than four years. 59.4% of our patients were in the group of six months to four years, whereas 18.8% were younger than six months and 21.9% older than four years
Clinical aspects 31.3% of our patients were transferred from a general practitioner, pediatrician, or another hospital with a different primary diagnosis such as sepsis, scarlet fever, exanthema subitum or viral exanthema, superinfection of a dermatitis, urinary tract-infection, tonsillitis, otitis media and aphthous stomatitis
Summary
Kawasaki disease (KD) is a rare vasculitis seen predominantly in children. In developing countries, it is the leading cause of childhood-acquired heart disease. KD has replaced acute rheumatic fever as the leading cause of acquired heart disease in children [2,3]. “Atypical KD” is defined as KD with any unusual presentation [7] and occurs more often in infants [8]. Their young age and the potential for not receiving timely treatment puts them at higher risk of developing coronary artery lesions [8]. It is broadly agreed that KD can be diagnosed in the absence of full criteria when coronary abnormalities are present [9]
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