P086 Kawasaki syndrome and Epstein Barr virus: a case report (again!)

Background Kawasaki disease (kDa), also known as mucocutaneous lymph node syndrome, is always updating. We present the case of a toddler boy with concomitant positive EBV serology. Material The infant is referred to the pediatric ward for stomatitis with cheilitis, fever> 40 ° C, asthenia, irritability evolving for 4–5 days. In addition, clinical examination revealed multiple, small cervical lymph nodes, < 1 cm. There is no conjunctivitis, no skin rash, nor reactivation of the BCG scar. Biology reveals hyperleukocytosis with very high ESR and CRP. ECG and Echo-heart, requested for suspicion of Kawasaki syndrome, are normal. Results Empirical treatment with Aciclovir—Cefalexin with topical care was started. Viral serologies return HSV (-), CMV (-), but EBV (+). Clinical improvement was noted within 36 h. However, inflammatory markers improve within 2–3 days. At 15 days, the child presents with typical convalescent peeling of the fingers and toes; associated with marked thrombocytosis. The diagnosis of Kawasaki Syndrome is definitively confirmed. Discussion The exact aetiology of Kawasaki syndrome remains unclear. Among the factors frequently encountered, the Epstein Barr Virus is reported for almost 30 years. The syndrome frequency is highest before the age of 5 years; similar to Infectious Mono-Nucleosis (due to EBV…). Our case depicts a concomitant Kawaski syndrome and positive EBV serology; without any detectable coronary artery abnormality. Conclusion Kawasaki syndrome is a real “chameleon” condition with possible cardiovascular complications. The viral aetiology springs up through “an old friend”: the Epstein Barr Virus ...Even without any aneurysm (also associated with EBV), the American Heart Association recommends long-term monitoring for all Kawasaki syndromes.

Predictive value of brachial artery flow-mediated dilation on coronary artery abnormality in acute stage of Kawasaki disease

Coronary artery abnormalities (CAAs) are a severe complication of Kawasaki disease (KD) that may lead to cardiovascular events. Given the evidence that brachial artery flow-mediated dilation (FMD) decreases in children after the onset of KD, we hypothesized that it could be an early marker of CAA development in the acute stage and investigated its relationship with variation in the coronary artery diameter. A total of 326 sex- and age-matched children were enrolled, including 120 with KD, 109 febrile children and 97 healthy controls. In this study, FMD was significantly decreased in the KD group compared with the febrile and healthy groups. FMD was lower in the CAA group than in the no coronary artery abnormality group. The comparison of FMD showed an obvious difference among the CAA subgroups. The FMD in the coronary aneurysm (CA) group showed a strong negative correlation with the pretreatment maximum coronary artery Z-score (preZmax). While preZmax was 2.5, the receiver operating characteristic curve indicated an optimal cutoff point of 3.44% for FMD. FMD ≤ 3.44% could be considered as a signal of coronary lesions in acute stage of KD.

Surgical Management of a Giant Left Sinus of Valsalva Aneurysm with Coronary Artery Abnormality

Coronary insufficiency caused by unruptured left sinus of Valsalva aneurysm (SVA) is exceedingly rare in the literature. Herein, we present a successful surgically treated case of giant left SVA with severe aortic regurgitation and coronary insufficiency, thus introducing a tailored valve-sparing aortic root repair technique.

Changes in and significance of platelet function and parameters in Kawasaki disease

Kawasaki disease (KD) is a systemic febrile, inflammatory vascular disease of unknown etiology. The coronary artery abnormality (CAA) caused by KD has become the most commonly acquired heart disease in children. Initial treatment of intravenous immunoglobulin (IVIG) can reduce the incidence of CAA. Thrombocytosis is common during the course of KD, but changes in and significances of platelet function and parameters are unclear. In this study, we enrolled 120 patients, including 40 patients with KD, 40 febrile controls, and 40 afebrile controls. The platelet function was assessed using the platelet function analyzer (PFA)-200. Platelet parameters, including platelet count (PLT), mean platelet volume (MPV), platelet distribution width (PDW), and platelet hematocrit (PCT) were measured. In the febrile period, the PDW and MPV were lower in KD patients (P < 0.05). The platelet function did not change significantly during the febrile period of KD but weakened in the defervescence phase. No significant differences between the CAA and normal groups, and between IVIG resistance and response groups. The diagnostic cutoff value of the PDW level for predicting KD was 10.85 fL with a sensitivity of 55% and a specificity of 77.5% (area under curve (AUC) = 0.690, 95% confidence interval (CI): 0.574–0.806, P < 0.01). Besides, the MPV level was 9.55 fL with sensitivity of 75% and specificity of 70% (AUC = 0.733, 95%CI: 0.620–0.846, P < 0.001). This is the first longitudinal study of platelet function changes in KD patients using PFA-200. Besides, lower PDW and MPV may be available markers for early diagnosis of KD.

Clinical Manifestations Vary with Different Age Spectrums in Infants with Kawasaki Disease

Background. Kawasaki disease (KD) is an acute systemic vasculitis with unknown etiology. The diagnosis of KD depends on clinical manifestations. The prevalence of coronary artery abnormality (CAA) is 11.0% and results in cardiac sequelae, such as myocardial infarction or coronary aneurysm, which are the most serious complications in KD.Methods. We divided KD's children into different age groups: ≤6 months old, 7 months to 1 year old, and >1 year old, respectively. Different parameters were compared in each group.Results. Infants ≤6 months old are less likely to fulfill KD's major diagnostic criteria within 10 days, are prone to develop incomplete KD with the lowest cholesterol level, and have the greatest chance to have CAA and the laboratory features associated with CAA, such as the longest time needed to confirm CA diagnosis, lower hemoglobin level, lower albumin level, and higher platelet count. Infants <1 year old develop higher percentage of leukocytosis and sterile pyuria. But this group has fewer patients with neck lymphadenopathy.