scholarly journals KAWASAKI DISEASE IN INFANTS LESS THAN ONE YEAR OF AGE: AN ITALIAN COHORT FROM A SINGLE CENTER

2019 ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovan Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  

Abstract Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms’ diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months.

2019 ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovan Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  

Abstract Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms’ diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months.


2019 ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovan Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  

Abstract Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms’ diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months.


2019 ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovan Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  

Abstract Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms’ diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months.


2021 ◽  
Vol 10 (11) ◽  
pp. 2347
Author(s):  
Ling-Sai Chang ◽  
Hong-Ren Yu ◽  
Chiao-Lun Chu ◽  
Kuang-Den Chen ◽  
Ying-Hsien Huang ◽  
...  

The Fc gamma receptor family contains several activating receptors and the only inhibitory receptor, FcγR2B. In this study, we investigated the dynamic methylation change of FcγR2B in different stages of Kawasaki disease (KD). We enrolled a total of 116 participants, which included patients with febrile diseases as controls and KD patients. Whole blood cells of KD patients were collected prior to intravenous immunoglobulin (IVIG) treatment (KD1), three to seven days after IVIG (KD2), three weeks after IVIG treatment (KD3), six months after IVIG (KD4), and one year after IVIG treatment (KD5). In total, 76 KD patients provided samples in every stage. Leukocytes of controls were also recruited. We performed DNA extraction and pyrosequencing. FcγR2B methylation levels were higher in KD3 compared to both the controls and KD1. A significantly higher methylation of FcγR2B was found in KD5 when compared with KD1. FcγR2B methylation levels in the IVIG-resistant group were lower than those in the IVIG-responsive group at KD1-3 (p = 0.004, 0.004, 0.005 respectively). This study is the first to report the dynamic change of FcγR2B methylation and to demonstrate long-term hypermethylation one year after disease onset. Hypomethylation of FcγR2B is associated with IVIG resistance.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovani Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Hideyuki Nakaoka

Backgroud: Kawasaki disease (KD) is a typically acute inflammatory syndrome that takes the form of systemic vasculitis. The acute inflammation and subsequent reparative process may lead to lasting changes in arterial structure even in the convalescence of KD including increased endothelial dysfunction. Endothelial microparticles (EMPs) are vesicles formed by the cell membrane after endothelial activation, and their composition can be used to characterize the status of the parent endothelial cell. EMPs were reported in cardiovascular diseases with endothelial dysfunction, such as acute coronary syndrome, pulmonary hypertension, diabetes, and vasculitis. Our aim of this study is to elucidate whether EMPs are involved in vasculitis during acute stage of Kawasaki disease. Method: We enrolled 9 patients (aged 3 months to 14 years, 6 male, 3 female), 7 common febrile children and 5 healthy children. KD patients in the convalescent phase were divided into two subgroups; coronary artery lesion (CAL, n=2) and no coronary lesion (NCAL, n=7). Blood samples were collected at the time of diagnosis before the initiation of IVIG treatment, then immediately after the first IVIG infusion and at 2-4 weeks after disease onset. Samples were measured using flow cytometry. Result: The percentage counts of EMPs were 2.90±1.26% in KD children before initial treatment, which were significantly higher (P<0.005) than those of disease controls (0.12±0.13%) and healthy controls (0.09±0.08%) before initial treatment, and reached normal levels within 4 weeks (0.05±0.05%). The highest percentage count of EMPs (5.47%) was observed in the patient with CAL before initial treatment. Further, prolonged high percentage count of EMPs (3.34%) was recognized in the patient with multiple gaint aneurysms at 2 weeks after onset. Conclusion: The relation between the increased levels of EMPs and the involvement of CAL may suggest that EMPs could serve as a sensitive marker of the severity of endothelial dysfunction and vasculitis in patients with KD. Although the function of EMPs has not been fully elucidated, there is evidence that it plays an important role for distinct inflammatory reactions in endothelium.


2020 ◽  
Vol 79 (8) ◽  
pp. 999-1006 ◽  
Author(s):  
Marie Pouletty ◽  
Charlotte Borocco ◽  
Naim Ouldali ◽  
Marion Caseris ◽  
Romain Basmaci ◽  
...  

BackgroundCurrent data suggest that COVID-19 is less frequent in children, with a milder course. However, over the past weeks, an increase in the number of children presenting to hospitals in the greater Paris region with a phenotype resembling Kawasaki disease (KD) has led to an alert by the French national health authorities.MethodsMulticentre compilation of patients with KD in Paris region since April 2020, associated with the detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (‘Kawa-COVID-19’). A historical cohort of ‘classical’ KD served as a comparator.ResultsSixteen patients were included (sex ratio=1, median age 10 years IQR (4·7 to 12.5)). SARS-CoV-2 was detected in 12 cases (69%), while a further three cases had documented recent contact with a quantitative PCR-positive individual (19%). Cardiac involvement included myocarditis in 44% (n=7). Factors prognostic for the development of severe disease (ie, requiring intensive care, n=7) were age over 5 years and ferritinaemia >1400 µg/L. Only five patients (31%) were successfully treated with a single intravenous immunoglobulin (IVIg) infusion, while 10 patients (62%) required a second line of treatment. The Kawa-COVID-19 cohort differed from a comparator group of ‘classical’ KD by older age at onset 10 vs 2 years (p<0.0001), lower platelet count (188 vs 383 G/L (p<0.0001)), a higher rate of myocarditis 7/16 vs 3/220 (p=0.0001) and resistance to first IVIg treatment 10/16 vs 45/220 (p=0.004).ConclusionKawa-COVID-19 likely represents a new systemic inflammatory syndrome temporally associated with SARS-CoV-2 infection in children. Further prospective international studies are necessary to confirm these findings and better understand the pathophysiology of Kawa-COVID-19.Trial registration numberNCT02377245


2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S248-S248
Author(s):  
Aileen Martinez ◽  
Lyssette Cardona ◽  
Nina Ricci

Abstract Background The 2019 coronavirus SARS-CoV-2 continues to affect global population health. Patients with severe disease that require hospitalization due to COVID-19 pneumonia remain at further risk of bacterial co-infections. There is limited evidence suggesting up to 3.5% bacterial co-infection upon admission and up to 13.5% of secondary infections after hospitalization for pneumonia yet antibacterial therapy usage remain as high, or even higher, than data seen for viral pneumonia, such as influenza. Unnecessary use of antimicrobial therapy may lead to further resistance and requires stewardship attention. Methods A single-center retrospective chart review was conducted in a community health system on all inpatient influenza admissions between October 1st 2019 to March 31st 2020 and all COVID-19 admissions during the same 6-month period one year later. Patients were excluded if age &lt; 18, observation or emergency visit. The study aims to determine the percentage of patients that were prescribed antibacterial therapy during influenza season compared to during the COVID-19 pandemic. Results A total of 175 patients were included in the influenza group while 1411 patients were included in the COVID-19 group (Table 1). The percent of inpatients with positive bacterial respiratory cultures were 12% in both influenza and COVID-19 groups. Positive bacterial respiratory cultures collected within 48 hours of admission were 3.4% in the influenza group compared to 1.2% in the COVID -19 group. Seventy-three percent of patients in the influenza group received antibiotics during admission compared to 78% in the COVID -19 group. Azithromycin and/or ceftriaxone was most commonly prescribed (58% vs. 60%) (Figure 1). The median length of stay was 3 days in the influenza group compared to 5 days in the COVID-19 group. In hospital mortality was higher in the COVID-19 group (1.7% vs. 9%). Conclusion Despite the viral origin of influenza and COVID-19 and low incidence of bacterial infection, antibacterials were frequently prescribed in both indications but it appears to trend more so in the COVID-19 group. There is an opportunity to enhance antimicrobial stewardship for the treatment of COVID-19 in acute care settings. Disclosures All Authors: No reported disclosures


2014 ◽  
Vol 9 (4) ◽  
pp. 30-35
Author(s):  
S Datta ◽  
S Maiti ◽  
G Das ◽  
A Chatterjee ◽  
P Ghosh

Background The diagnosis of classical Kawasaki Disease was based on clinical criteria. The conventional criteria is particularly useful in preventing over diagnosis, but at the same time it may result in failure to recognize the incomplete form of Kawasaki Disease. Objective To suspect incomplete Kawasaki Disease, because early diagnosis and proper treatment may reduce substantial risk of developing coronary artery abnormality which is one of the leading causes of acquired heart disease in children. Method Nine cases of incomplete Kawasaki Disease were diagnosed over a period of one year. The diagnosis of incomplete Kawasaki Disease was based on fever for five days with less than four classical clinical features and cardiac abnormality detected by 2D- echocardiography. A repeat echocardiography was done after 6 weeks of onset of illness. The patients were treated with Intravenous Immunoglobulin and/or aspirin. Result The mean age of the patients was 3.83 years and the mean duration of symptoms before diagnosis was 12.1 days. Apart from other criteria all of our patients had edema and extreme irritability. All the patients had abnormal echocardiographic finding. Five patients received only aspirin due to nonaffordability of Intravenous Immunoglobulin and four patients received both aspirin and Intravenous Immunoglobulin, but the outcome was excellent in all the cases. Conclusion Incomplete Kawasaki Disease can be diagnosed with more awareness and aspirin alone may be used as a second line therapy in case of non affordability of Intravenous Immunoglobulin. Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 30-35 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10234


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