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Italian Journal of Pediatrics

, 42:92

Allergology and Immunology

Abstract

BackgroundKawasaki disease KD is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin IVIG develop coronary artery lesions CAL. Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL.

Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity.

MethodsWe studied the clinical data of 70 KD Sicilian children 36 males: 51 %; 34 females: 49 %, analysed retrospectively, including: demographic and laboratory parameters; echocardiographic findings at diagnosis, at 2, 6 and 8 weeks, and at 1 year after the onset of the illness.

ResultsForty-seven had Typical KD, three Atypical KD and twenty Incomplete KD. Age at the disease onset ranged from 0.1 to 8.9 years. IVIG were administered 5 ± 2 days after the fever started. Defervescence occurred 39 ± 26 hours after the first IVIG infusion. Fifty-six patients 80 % received 1 dose of IVIG responders; 14 patients 20 % had a resistant KD, with persistent fever after the first IVIG dose non responders. Ten 14 % non responders responded to the second dose, 4 5 % responded to three doses; one needed treatment with high doses of steroids and Infliximab.

Cardiac involvement was documented in twenty-two cases eighteen with transient dilatation-ectasia, fifteen with aneurysms. Pericardial effusion, documented in eleven, was associated with coronaritis and aneurysms, and was present earlier than coronary involvement in seven.

Hypoalbuminemia, D-dimer pre-IVIG, gamma-GT pre-IVIG showed a statistically significant direct correlation with IVIG doses, highlighting the role of these parameters as predictor markers of refractory disease. The persistence of elevated CRP, AST, ALT levels, a persistent hyponatremia and hypoalbuminemia after IVIG therapy, also had a statistical significant correlation with IVIG doses.

Non responders showed higher levels of D-dimer and gamma-GT pre-IVIG, persistent high levels of D-dimer, CRP, AST, ALT, hypoalbuminemia and hyponatremia after IVIG.

ConclusionsThis is the first study on KD in Sicily. We suggest some laboratory parameters as predictive criteria for resistant KD. Patients who show early pericarditis need careful surveillance for coronary lesions.

KeywordsKawasaki Disease Vasculitis Cardiovascular Disease Small Vessel Vasculitis  Download fulltext PDF



Author: Maria Cristina Maggio - Giovanni Corsello - Eugenia Prinzi - Rolando Cimaz

Source: https://link.springer.com/







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