Background: Multisystem inflammatory syndrome in children (MIS-C) is a new hyperinflammatory variant that evolved during the coronavirus disease 2019 pandemic. Although the precise pathophysiology of MIS-C is uncertain, it is thought to be due to immune dysregulation occurring after recovery from acute infection. Purpose: Our study aimed to analyze the clinical spectrum, laboratory parameters, imaging characteristics, treatment strategies, and short-term outcomes of children with a diagnosis of MIS-C. Methods: This retrospective and prospective observational study included children less than 16 years of age who were admitted to the pediatric unit of a tertiary care teaching hospital in south India between August 2020 to January 2021 with a diagnosis of MIS-C according to World Health Organization criteria. Results: Twenty-one children were included in the analysis; all had fever with variable combinations of other symptoms. The mean age was 6.9 years; 71.4% were male. Gastrointestinal (80.9%) and cardiovascular (80.9%) systems were the most commonly affected. The majority of children had elevated inflammatory markers, and 16 (76.2%) had echocardiographic abnormalities mimicking Kawasaki disease. Eleven children (52.4%) required intensive care admission, 3 (14.3%) required supplemental oxygen, and 4 (19%) required inotropes. Nine (42.9%) were treated with intravenous immunoglobulin alone, 6 (28.6%) with steroids alone, and 3 (14.3%) with steroids and immunoglobulin. The median hospital stay was 6 days; there were no fatalities. Overweight/obesity, elevated ferritin, and mucocutaneous involvement were significantly associated with a prolonged hospital stay (≥7 days). Sixteen children (76.2%) were followed up till now and all of them had no clinical concerns. Conclusion: MIS-C is an emerging disease with variable presentation. A high index of suspicion is necessary for its early identification and appropriate management. Further research is essential for developing optimal treatment strategies.
Choi, Chang Hwan;Byun, Sung Hwan;Jeon, Je Duk;Choi, Jong-Woon
Clinical and Experimental Pediatrics
/
v.50
no.1
/
pp.47-51
/
2007
Purpose : The early diagnosis of Kawasaki disease (KD) is difficult sometimes, especially in atypical or incomplete cases presenting 4 or less principal clinical features without coronary arterial lesions (coronary arterial ectasia or aneurysm). The authors investigated the incidence of echocardiographic abnormalities in patients with KD to discover whether abnormal echocardiographic findings might be helpful in the early diagnosis of KD. Methods : Echocardiography was done in the acute stage of 103 patients with KD (Kawasaki group) and 40 patients with other acute febrile illnesses (control group). Abnormal echocardiographic findings were classified into 4 categories and defined as follows; 1) significant pericardial effusion, 2) significant valvular dysfunctions, 3) left ventricular systolic dysfunction, 4) coronary arterial ectasia or aneurysm. Results : In the Kawasaki group, significant pericardial effusion was present in 24 patients (23.3 percent), significant valvular dysfunctions in 30 patients (29.1 percent), left ventricular systolic dysfunction in 10 patients (9.7 percent), and coronary arterial lesions in 27 patients (26.2 percent). In the control group, significant pericardial effusion was present in only one patient (2.5 percent). The number of patients with any one of echocardiographic abnormalities was 57 (55.3 percent) in Kawasaki group and one (2.5 percent) in control group. The sensitivity of echocardiography in the diagnosis of KD was 55.3 percent, the specificity 97.5 percent, the positive predictive value 98.3 percent, and the negative predictive value 45.9 percent. Conclusion : If abnormal echocardiographic findings, even if other than coronary arterial lesions, are confirmed in patients in whom KD is suspected, it seems desirable to inifiate specific treatment for KD.
Bae, Hyun Kyung;Lee, Do Kyung;Kwon, Jung Hyun;Kim, Hae Soon;Sohn, Sejung;Hong, Young Mi
Clinical and Experimental Pediatrics
/
v.57
no.8
/
pp.357-362
/
2014
Purpose: The incidence of Kawasaki disease (KD) is rare in young infants (less than 3 months of age), who present with only a few symptoms that fulfill the clinical diagnostic criteria. The diagnosis for KD can therefore be delayed, leading to a high risk of cardiac complications. We examined the clinical characteristics and measured the serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) levels of these patients for assessing its value in the early detection of KD. Methods: We retrospectively reviewed the data of young infants diagnosed with KD from 2004 to 2012. The control group included 20 hospitalized febrile patients. Laboratory data, including NT-proBNP were obtained for each patient in both groups. Results: Incomplete KD was observed in 21/24 patients (87.5%). The mean fever duration on admission was $1.36{\pm}1.0$ days in the KD group. Common symptoms included erythema at the site of Bacille Calmette-Guerin inoculation (70.8%), skin rash (50.0%), changes of oropharyngeal mucosa (29.1%), and cervical lymphadenopathy (20.8%). The mean number of major diagnostic criteria fulfilled was $2.8{\pm}1.4$. Five KD patients (20.8%) had only one symptom matching these criteria. The incidence of coronary artery complications was 12.5%. The mean serum NT-proBNP level in the acute phase, in the KD and control groups, were $4,159{\pm}3,714pg/mL$ and $957{\pm}902pg/mL$, respectively, which decreased significantly in the convalescent phase. Conclusion: Incomplete KD was observed in 87.5% patients. Serum NT- proBNP might be a valuable biomarker for the early detection of KD in febrile infants aged <3 months.
Purpose: Activation of Toll-like receptor 2 (TLR2) present on circulating monocytes in patients with Kawasaki disease (KD) can lead to the production of proinflammatory cytokines and interleukin-10 (IL-10). We aimed to determine the association of the frequency of circulating TLR2+/ CD14+ monocytes (FTLR2%) with the outcomes of KD, as well as to compare FTLR2% to the usefulness of sIL-10. Methods: The FTLR2% in patients with KD was measured by flow cytometry. Serum levels of IL-10 (sIL-10) were determined in 31 patients with KD before the initial treatment with intravenous immunoglobulin (IVIG) and in 21 febrile controls by using enzyme-linked immunosorbent assay. Patients were classified as having coronary artery lesions (CALs) based on the maximal internal diameters of the proximal right coronary artery and proximal left anterior descending coronary artery one month after the initial diagnosis. Results: We found that FTLR2% greater than 92.62% predicted CALs with 80% sensitivity and 68.4% specificity, whereas FTLR2% more than 94.61% predicted IVIG resistance with 66.7% sensitivity and 71.4% specificity. Moreover, sIL-10 more than 15.52 pg/mL predicted CALs and IVIG resistance with 40% and 66.7% sensitivity, respectively, and 73.7% and 76.2% specificity, respectively. Conclusion: We showed that measuring FTLR2% before the initial treatment could be useful in predicting CAL development with better sensitivity than sIL-10 and with results comparable to sIL-10 results for the prediction of IVIG resistance in patients with KD. However, further studies are necessary to validate FTLR2% as a marker of prognosis and severity of KD.
Background: We analyzed the changes in QT dispersion (QTd) in children with Kawasaki disease (KD), and determined the presence of repolarization abnormality in these children even in the absence of coronary artery abnormalities. Methods: Ninety-one children with KD and 20 healthy controls were enrolled in this retrospective study. Serial echocardiographic and electrocardiographic (ECG) measurements in the beginning of treatment, 2nd month and 6th month after the diagnosis were compared. Fifty-one of 91 children had at least 2 serial ECG data. The number of patients who had 3 consecutive ECG data was 23. Results: Among the 67 KD patients with no coronary artery changes, the consecutive mean QTd values were 41.86 ms, 37.84 ms, and 25.47 ms, respectively (26 ms for controls). In the analysis of changes among KD patients without coronary artery abnormalities, QTd showed a significant decrease with time (p=0.01). Especially, the 1st month and the 6th month QTd values were significantly different (p=0.028). The mean QTd values in KD patients with coronary artery changes were significantly higher than those in KD patients with no coronary artery changes at each time (1st, 2nd, and 6th month exam). Conclusion: QTd is significantly increased in children during the early stage of KD. Repolarization abnormality may exist during the acute stage of KD, regardless of the echocardiographic changes.
Lee, Hoon Sang;Kim, Ji Yong;Song, Bo Kyung;Kim, Yong-Woo;Park, Su Eun
Pediatric Infection and Vaccine
/
v.23
no.3
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pp.217-222
/
2016
Purpose: This study aimed to identify the differential clinical, laboratory, and imaging characteristics between patients with cervical lymphadenitis as first presentation of Kawasaki disease (CLKD) and those with acute unilateral cervical lymphadenitis (AUCL). Methods: We surveyed 372 patients who visited Pusan National University Children's Hospital because of fever and cervical lymph node enlargement, and underwent neck computed tomography (CT) from January 2010 to December 2014. We compared 28 confirmed cases of Kawasaki disease and 28 cases of AUCL based on a retrospective review of the medical records of the patients. Results: Patients with CLKD and AUCL showed no differential clinical characteristics in terms of the duration of fever, antibiotic use, or the size of lymph nodes. Patients with CLKD had higher white blood cell count, absolute neutrophil count, erythrocyte sedimentation rate, and C-reactive protein levels (P<0.05) than those of patients with AUCL. The presence of retropharyngeal edema on neck CT was similar between the groups (64% vs. 33%, P=0.686). Conclusions: CLKD and AUCL showed no differentiating clinical and radiological characteristics; hence, Kawasaki disease should be the presumptive diagnosis in patients with fever and cervical lymph node enlargements who fail to respond to antibiotic treatment.
Lee, Ho Seok;Yun, Sin Weon;Jung, Young Soo;Chae, Soo Ahn;Lim, In Seok;Lee, Dong Keun;Choi, Eung Sang;Yoo, Byung Hoon;Lee, Mi Kyung
Clinical and Experimental Pediatrics
/
v.50
no.10
/
pp.995-1004
/
2007
Purpose : Kawasaki disease is a systemic vasculitis, leading cause of pediatric acquired heart disease. Vascular endothelial growth factor (VEGF) has functions as vascular permeability factor, plays an important role in coronary artery lesion (CAL). We studied the clinical significance of serum VEGF in Kawasaki disease. Methods : Kawasaki group was 49 patients, and control group was 15 patients. Diagnosis followed AHA (American Heart Association) diagnostic criteria, with blood sampling in acute, subacute, and convalescent phase. Echocardiographic abnormalities were defined and the definition of intravenous gamma globulin (IVGG)-responsive and IVGG-resistant was determined. Results : Serum VEGF of Kawasaki group was significantly higher than of control group. Comparison of serum VEGF between CAL and non-CAL group, between carditis group and non-carditis group showed no significant differences. Subacute serum VEGF was statistically higher in IVGG-resistant group than in IVGG-responsive group, and serum VEGF of IVGG-resistant group in subacute phase was statistically higher than in the other phases. Serum VEGF of convalescent CAL and non-CAL group in acute and subacute phase had meaningful differences. Total fever duration and subacute serum VEGF had positive correlation. Acute serum VEGF had positive correlation with ESR and CRP, all phases serum VEGF had also positive correlation with WBC. Acute and subacute serum VEGF had negative correlations with hemoglobin and albumin. Conclusion : Serum VEGF can help to determine the severity of Kawasaki disease, especially subacute serum VEGF seems to be used as a prognostic factor of coronary complication. Afterward, further studies needed with more strict diagnostic criteria and more study groups.
Lee, Soo Jeong;Kim, So Jung;Kim, Hae Soon;Sohn, Sejung
Clinical and Experimental Pediatrics
/
v.46
no.6
/
pp.591-596
/
2003
Purpose : Kawasaki disease(KD) is rare in infants <3 months of age. In this younger group, the diagnosis may be delayed due to lack of most of the clinical criteria, resulting in a high risk of cardiac complications. We examined clinical characteristics in these patients for early recognition and treatment. Methods : We conducted a retrospective study on the infants with KD aged three months or younger treated at our hospital from January 1998 to July 2002. Results : Of a total of 291 patients treated during the study period, 11(3.8%) were three months old or younger. Of the 11 patients, 10 had atypical presentations. Infants had fewer of the accepted criteria, and the most common findings were fever(100%) and oral mucosal changes(72.7%). Erythema at the site of BCG inoculation was observed in six of the 11 patients(unknown in the remaining five). This feature proved a definite diagnostic clue in two patients in whom cardiac complications developed in the subacute phase. Cardiac complications were found in six patients(54.5%) : three had coronary dilatation, two had coronary wall irregularity, and one had mitral valve prolapse with regurgitation. Defervescence occurred within $1.1{\pm}0.3day$ in 10 of the 11 patients treated with intravenous immunoglobulin(IVIG) and one was given a second course of IVIG. Echocardiographic abnormality persisted in only one patient with mitral regurgitation at the 6-month follow-up. Conclusion : Most patients with KD younger than three months of age have atypical presentations and a high complication rate. For early diagnosis, erythema at the BCG inoculation site, if present, could be used as a valid diagnostic clue to atypical KD in this age group.
Heo, Mi Young;Choi, Su Jung;Kim, Hae Soon;Sohn, Sejung
Clinical and Experimental Pediatrics
/
v.45
no.3
/
pp.376-382
/
2002
Purpose : To identify clinical and laboratory features of atypical Kawasaki disease(KD), and to develop criteria for early diagnosis of atypical KD patients. Methods : All patients with KD treated at our hospital from January 1998 to June 2000 were reviewed retrospectively. Results : Among a total of 167 patients, 28(16.8%) were atypical KD of which seven(25%) were infants. Among the five cardinal symptoms, oral mucosal change(96.4%) occurred most frequently, followed by conjuntivitis(57.1%) and rash(46.4%). Most notable laboratory findings were anemia, and increased erythrocyte sedementation rate(ESR) or C-reactive protein(CRP). Coronary artery abnormalities developed in seven(25.8%) atypical KD patients, compared with 14.4% in typical KD patients. We considered oral mucosal change as major criterion, and conjunctivitis, rash, hematocrit <35% and ESR >30 mm/hr or CRP >3.1 mg/dL as minor criteria. Proposed modification in diagnostic criteria for atypical KD include fever of ${\geq}5$ days+major criterion+${\geq}2$ minor criteria, or fever of ${\geq}5$ days+4 minor criteria. Conclusion : The modified diagnostic criteria has yielded a sensitivity 89.3%. Our diagnostic criteria may be used for early diagnosis of atypical KD.
Kang, Ha Young;Joo, Eun Young;Kim, Dong Hyun;Hong, Young Jin
Pediatric Infection and Vaccine
/
v.24
no.1
/
pp.60-64
/
2017
Kawasaki disease (KD) is a systemic vasculitis that occurs predominantly in infants and young children. The etiology of KD is unknown and coronary heart disease is a major complication of KD. Acute scrotum is a rare complication of acute KD, and not as well recognized as other manifestations of the disease. We report a 2-month-old boy with acute scrotum in the acute phase of KD. He was treated with intravenous immunoglobulin (total 2 g/kg) and aspirin (50 mg/kg/day). The treatment was effective in resolving his fever and other clinical symptoms, but 2 days after starting treatment he experienced scrotal swelling. Scrotal ultrasound and transillumination were used in the diagnosis of acute scrotum. After 2 months, a follow-up testicular ultrasound revealed a remission of the acute scrotum. Subsequently, he has been followed up for KD.
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