Change in Severity and Clinical Manifestation of MIS-C Over SARSCoV-2 Variant Outbreaks in Korea

Background@#There is difference in the incidence of multi-system inflammatory syndrome in children (MIS-C) in patients with different variants of severe acute respiratory syndrome coronavirus 2, however, little is known about the epidemiology in Asian countries. We investigated and compared the epidemiology of the MIS-C during omicron-dominant period with that of previous periods in South Korea. @*Methods@#We obtained clinical, epidemiological and laboratory data on MIS-C cases from national MIS-C surveillance in South Korea. We defined pre-delta period as January 2020–May 2021; delta period as June 2021–December 2021; and omicron period as January 2022–April 2022. We describe the clinical characteristics and outcomes of MIS-C patients by period. @*Results@#A total of 91 cases were assessed to be MIS-C cases. Number of MIS-C cases have increased from six cases during pre-delta period to 66 cases during omicron period, while the incidence rate (the number of MIS-C cases per 100,000 cases of reported coronavirus disease 2019) has decreased from 38.5 cases per 100,000 (95% confidence interval [CI], 14.1–83.9) during pre-delta period to 1.6 cases per 100,000 (95% CI, 1.2–2.0) during omicron periods. During pre-delta period, 66.7% and 100% had hypotension and gastrointestinal involvement, respectively; while during omicron period, 12.1% and 6.1% had such clinical manifestations. Fifty percent of pre-delta MIS-C patients were taken intensive care unit (ICU) cares, while 10.6% of patients during omicron periods were in ICUs. @*Conclusion@#Omicron period were associated with less severe clinical manifestation compared to pre-delta and delta periods. Although incidence rate of MIS-C was lower for the omicron period than pre-delta and delta periods, number of patients reported with MIS-C may pose a substantial clinical burden.

Human Parechovirus: an Emerging Cause of Sepsis-Like Syndrome in Infants Aged under 3 Months

Purpose@#This study aimed to investigate the clinical characteristics of human parechovirus (HPeV) infection in sepsis-like syndrome in infants aged under 3 months. @*Methods@#Medical records of infants aged under 3 months with sepsis-like symptoms who were admitted between July 1, 2018 and August 31, 2018 were reviewed. A multiplex reverse transcription-polymerase chain reaction panel test was performed on the cerebrospinal fluid (CSF). Thirty-nine enrolled infants were categorized into three groups: 11 in group 1 (HPeV detected in the CSF), 13 in group 2 (enterovirus detected in the CSF), and 15 in group 3 (no virus detected in the CSF). @*Results@#Compared with groups 2 and 3, a higher proportion of group 1 had tachycardia, tachypnea, apnea, and hypotension (P<0.05). A significantly lower white blood cell (WBC) count was noted in group 1 than in groups 2 and 3 (5,622±2,355/μL, 9,397±2,282/μL, and 12,312±7,452/μL, respectively; P=0.005). The CSF WBC count was lower in group 1 than in groups 2 and 3 (0.9±1.7/μL, 85.1±163.6/μL, and 3.7±6.9/μL, respectively; P=0.068). The proportion of patients requiring inotrope support (36.6% vs. 0% and 6.6%), mechanical ventilation (18.1% vs. 0% and 0%), and high flow nasal cannula (45.4% vs. 15.3% and 6.6%) was higher in group 1 than in groups 2 and 3. All patients recovered completely without complications. @*Conclusions@#HPeV infection shows a severe clinical course and can cause a severe sepsis-like syndrome in infants aged under 3 months. Early diagnosis and proper treatment of HPeV infection are required.

Does Early Drain Removal Affect Postoperative Pericardial Effusion after Congenital Cardiac Surgery?

Background@#Patients undergoing cardiac surgery require postoperative chest drainage. However, the drain is difficult to keep in place in children with congenital heart disease. Since 2015, at Kyungpook National University Hospital, the chest tube is removed on postoperative day 1 in patients who have undergone simple congenital cardiac surgery (i.e., closure of an atrial or ventricular septal defect). In this study, we evaluated the relationship between the duration of drain placement and the likelihood of pericardial effusion after congenital cardiac surgery. @*Methods@#The medical records of patients who underwent closure of an atrial or ventricular septal defect at our hospital between January 2014 and December 2016 were reviewed. In total, 162 patients who received follow-up echocardiography and had information available on postoperative pericardial effusion after the repair procedure were enrolled. @*Results@#Echocardiography was performed at a median of 5 days (range, 4 to 6 days) postoperatively before discharge from the hospital. Pericardial effusion occurred in 21 patients (13.0%), of whom only 3 (1.9%) had moderate or greater pericardial effusion, regardless of the drain duration. All patients improved during outpatient follow-up without invasive management. No patient had severe complications because of pericardial effusion. The duration of drain placement did not affect the incidence of postoperative pericardial effusion (p=0.069). Operative survival was 100%. @*Conclusion@#Based on our study, we recommend removing the drain as soon as its role is complete, generally on postoperative day 1, because early removal does not increase the incidence of pericardial effusion in patients undergoing simple congenital cardiac surgery.

Diagnosis and management of Kawasaki disease

Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a common form of vasculitis in childhood. Among the recent trends in Kawasaki disease, it is notable that the prevalence rate is increasing, and the proportion of “incomplete” Kawasaki disease is also increasing. A delayed diagnosis with prolonged fever can cause complications, such as coronary artery aneurysm and decreased myocardial contractility. These complications might lead to significant morbidity and mortality. Kawasaki disease is diagnosed when the patient meets the principal criteria for clinical symptoms. Following the diagnostic criteria can prevent overdiagnosis but lead to missing of the incomplete form of the disease. Therefore, it is important to accurately understand Kawasaki disease. As the coronavirus disease 2019 (COVID-19) pandemic continues, concerns about a novel severe Kawasaki-like disease in children related to COVID-19 emerges. Indeed, there have been several reports of Kawasaki-like disease related to COVID-19 in children. Kawasaki disease is no longer rare and is often encountered at hospitals. This paper will provide useful and accurate information on Kawasaki disease.

Correlation between Cytokine and Chemokine levels and Clinical Severity in Children with Mycoplasma pneumoniae Pneumonia

PURPOSE: The aim of this study was to evaluate the relationships between cytokine and chemokine levels and the clinical severity of Mycoplasma pneumoniae pneumonia. METHODS: A retrospective analysis of clinical and laboratory parameters were performed. Serum levels of interleukin (IL)-6, IL-8, IL-10, IL-18, interferon-γ-inducible protein-10 (IP-10), macrophage inflammatory protein-1β, and tumor necrosis factor-α were measured. The severity of patients' clinical course and radiologic findings were also assessed. RESULTS: Seventy-two patients (35 males and 37 females) with a median age of 3.9 years (range, 1–16 years) were enrolled. Patients with lobar pneumonia (n=29) had significantly higher C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and IL-18 values than those with broncho-interstitial pneumonia (n=43). However, the cytokine and chemokine values did not differ between the group that was treated with corticosteroids (n=31) and the one that was not (n=41). The CRP, ESR, lactate dehydrogenase (LDH), IL-18, and IP-10 values showed positive correlations with fever duration prior to admission. The CRP and ESR values were positively correlated with IL-18, and LDH, with IP-10 levels. CONCLUSIONS: CRP, ESR, LDH, IL-18, and IP-10 values were associated with the severity of the disease, manifesting lobar pneumonia or prolonged fever duration prior to admission.

Array Comparative Genomic Hybridization as the First-line Investigation for Neonates with Congenital Heart Disease: Experience in a Single Tertiary Center

BACKGROUND AND OBJECTIVES: The purpose of the present study was to investigate the advantages and disadvantages of verifying genetic abnormalities using array comparative genomic hybridization (a-CGH) immediately after diagnosis of congenital heart disease (CHD). METHODS: Among neonates under the age of 28 days who underwent echocardiography from January 1, 2014 to April 30, 2016, neonates whose chromosomal and genomic abnormalities were tested using a-CGH in cases of an abnormal finding on echocardiography were enrolled. RESULTS: Of the 166 patients diagnosed with CHD, 81 underwent a-CGH and 11 patients (11/81, 13.5%) had abnormal findings on a-CGH. 22q11.2 deletion syndrome was the most common (4/11, 36.4%). On the first a-CGH, 4 patients were negative (4/81, 5%). Three of them were finally diagnosed with Williams syndrome using fluorescent in situ hybridization (FISH), 1 patient was diagnosed with Noonan syndrome through exome sequencing. All of them exhibited diffuse pulmonary artery branch hypoplasia, as well as increased velocity of blood flow, on repeated echocardiography. Five patients started rehabilitation therapy at mean 6 months old age in outpatient clinics and epilepsy was diagnosed in 2 patients. Parents of 2 patients (22q11.2 deletion syndrome and Patau syndrome) refused treatment due to the anticipated prognosis. CONCLUSIONS: Screening tests for genetic abnormalities using a-CGH in neonates with CHD has the advantage of early diagnosis of genetic abnormality during the neonatal period in which there is no obvious symptom of genetic abnormality. However, there are disadvantages that some genetic abnormalities cannot be identified on a-CGH.

The change of QRS duration after pulmonary valve replacement in patients with repaired tetralogy of Fallot and pulmonary regurgitation / 소아과

PURPOSE: This study aimed to analyze changes in QRS duration and cardiothoracic ratio (CTR) following pulmonary valve replacement (PVR) in patients with tetralogy of Fallot (TOF). METHODS: Children and adolescents who had previously undergone total repair for TOF (n=67; median age, 16 years) who required elective PVR for pulmonary regurgitation and/or right ventricular out tract obstruction were included in this study. The QRS duration and CTR were measured pre- and postoperatively and postoperative changes were evaluated. RESULTS: Following PVR, the CTR significantly decreased (pre-PVR 57.2%±6.2%, post-PVR 53.8%±5.5%, P=0.002). The postoperative QRS duration showed a tendency to decrease (pre-PVR 162.7±26.4 msec, post-PVR 156.4±24.4 msec, P=0.124). QRS duration was greater than 180 msec in 6 patients prior to PVR. Of these, 5 patients showed a decrease in QRS duration following PVR; QRS duration was less than 180 msec in 2 patients, and QRS duration remained greater than 180 msec in 3 patients, including 2 patients with diffuse postoperative right ventricular outflow tract hypokinesis. Six patients had coexisting arrhythmias before PVR; 2 patients, atrial tachycardia; 3 patients, premature ventricular contraction; and 1 patient, premature atrial contraction. None of the patients presented with arrhythmia following PVR. CONCLUSION: The CTR and QRS duration reduced following PVR. However, QRS duration may not decrease below 180 msec after PVR, particularly in patients with right ventricular outflow tract hypokinesis. The CTR and ECG may provide additional clinical information on changes in right ventricular volume and/or pressure in these patients.

Outcome of neonatal palliative procedure for pulmonary atresia with ventricular septal defect or tetralogy of Fallot with severe pulmonary stenosis: experience in a single tertiary center / 소아과

PURPOSE: The present study aimed to evaluate progression and prognosis according to the palliation method used in neonates and early infants aged 3 months or younger who were diagnosed with pulmonary atresia with ventricular septal defect (PA VSD) or tetralogy of Fallot (TOF) with severe pulmonary stenosis (PS) in a single tertiary hospital over a period of 12 years. METHODS: Twenty with PA VSD and 9 with TOF and severe PS needed initial palliation. Reintervention after initial palliation, complete repair, and progress were reviewed retrospectively. RESULTS: Among 29 patients, 14 patients underwent right ventricle to pulmonary artery (RV-PA) connection, 11 palliative BT shunt, 2 central shunt, and 2 ductal stent insertion. Median age at the initial palliation was 13 days (1–98 days). Additional procedure for pulmonary blood flow was required in 5 patients; 4 additional BT shunt operations and 1 RV-PA connection. There were 2 early deaths among patients with RV-PA connection, one from RV failure and the other from severe infection. Finally, 25 patients (86%) had a complete repair. Median age of total correction was 12 months (range, 2–31 months). At last follow-up, 2 patients had required reintervention after total correction; 1 conduit replacement and 1 right ventricular outflow tract (RVOT) patch enlargements. CONCLUSION: For initial palliation of patients with PA VSD or TOF with severe PS, not only shunt operation but also RV-PA connection approach can provide an acceptable outcome. To select the most proper surgical strategy, we recommend thorough evaluation of cardiac anomalies such as RVOT and PA morphologies and consideration of the patient's condition.

A novel GLA mutation in a Korean boy with an early cardiac manifestation of Fabry disease

Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by the deficiency of α-galactosidase A. Patients with classical FD present acroparesthesia, hypohidrosis, cornea verticillata, disseminated angiokeratoma, and microalbuminuria in childhood, and develop life-threatening renal, cardiac, and cerebrovascular complications typically after the fourth decade of life. To date, more than 700 mutations responsible for FD have been identified in the human GLA gene. Herein, we report a novel GLA mutation, c.1117_1141del25 (p.Gly373Profs*10), identified in an 11-year-old Korean boy with FD presenting early cardiac and neurologic manifestation and in other affected family members. The boy had acroparesthesia, hypohidrosis, cornea verticillata, and left ventricular hypertrophy. His mother and sister also had acroparesthesia. Two males on the mother's side had similar pain and died of unknown causes. The plasma α-galactosidase A activity (4.1 nmol/hr/mg protein) of the patient was markedly lower than the mean value of the controls. The plasma level of globotriaosylsphingosine was elevated in the patient and all the carriers. We concluded the novel GLA mutation c.1117_1141del25 is a pathogenic mutation for FD, probably related to the early cardiac manifestation of FD.

Array Comparative Genomic Hybridization as the First-line Investigation for Neonates with Congenital Heart Disease: Experience in a Single Tertiary Center

BACKGROUND AND OBJECTIVES@#The purpose of the present study was to investigate the advantages and disadvantages of verifying genetic abnormalities using array comparative genomic hybridization (a-CGH) immediately after diagnosis of congenital heart disease (CHD).@*METHODS@#Among neonates under the age of 28 days who underwent echocardiography from January 1, 2014 to April 30, 2016, neonates whose chromosomal and genomic abnormalities were tested using a-CGH in cases of an abnormal finding on echocardiography were enrolled.@*RESULTS@#Of the 166 patients diagnosed with CHD, 81 underwent a-CGH and 11 patients (11/81, 13.5%) had abnormal findings on a-CGH. 22q11.2 deletion syndrome was the most common (4/11, 36.4%). On the first a-CGH, 4 patients were negative (4/81, 5%). Three of them were finally diagnosed with Williams syndrome using fluorescent in situ hybridization (FISH), 1 patient was diagnosed with Noonan syndrome through exome sequencing. All of them exhibited diffuse pulmonary artery branch hypoplasia, as well as increased velocity of blood flow, on repeated echocardiography. Five patients started rehabilitation therapy at mean 6 months old age in outpatient clinics and epilepsy was diagnosed in 2 patients. Parents of 2 patients (22q11.2 deletion syndrome and Patau syndrome) refused treatment due to the anticipated prognosis.@*CONCLUSIONS@#Screening tests for genetic abnormalities using a-CGH in neonates with CHD has the advantage of early diagnosis of genetic abnormality during the neonatal period in which there is no obvious symptom of genetic abnormality. However, there are disadvantages that some genetic abnormalities cannot be identified on a-CGH.

Comparison of cytokine expression profiles in infants with a rhinovirus induced lower respiratory tract infection with or without wheezing: a comparison with respiratory syncytial virus / 소아과

PURPOSE: The aim of this study was to evaluate whether infants with rhinovirus (RV) infection-induced wheezing and those with respiratory syncytial virus (RSV) infection-induced wheezing have different cytokine profiles in the acute stage. METHODS: Of the infants with lower respiratory tract infection (LRTI) between September 2011 and May 2012, 88 were confirmed using reverse transcription polymerase chain reaction and hospitalized. Systemic interferon-gamma (IFN-γ), interleukin (IL)-2, IL-12, IL-4, IL-5, IL-13, and Treg-type cytokine (IL-10) responses were examined with multiplex assay using acute phase serum samples. RESULTS: Of the 88 patients, 38 had an RV infection (RV group) and 50 had an RSV infection (RSV group). In the RV group, the IFN-γ and IL-10 concentrations were higher in the patients with than in the patients without wheezing (P=0.022 and P=0.007, respectively). In the RSV group, the differences in IFN-γ and IL-10 concentrations did not reach statistical significance between the patients with and the patients without wheezing (P=0.105 and P=0.965, respectively). The IFN-γ and IL-10 concentrations were not significantly different between the RV group with wheezing and the RSV group with wheezing (P=0.155 and P=0.801, respectively), in contrast to the significant difference between the RV group without wheezing and the RSV group without wheezing (P=0.019 and P=0.035, respectively). CONCLUSION: In comparison with RSV-induced LRTI, RV-induced LRTI combined with wheezing showed similar IFN-γ and IL-10 levels, which may have an important regulatory function.

Clinical usefulness of serum procalcitonin level in distinguishing between Kawasaki disease and other infections in febrile children / 소아과

PURPOSE: The aims of this study were to compare serum procalcitonin (PCT) levels between febrile children with Kawasaki disease (KD) and those with bacterial or viral infections, and assess the clinical usefulness of PCT level in predicting KD. METHODS: Serum PCT levels were examined in febrile pediatric patients admitted between August 2013 and August 2014. The patients were divided into 3 groups as follows: 49 with KD, 111 with viral infections, and 24 with bacterial infections. RESULTS: The mean PCT level in the KD group was significantly lower than that in the bacterial infection group (0.82±1.73 ng/mL vs. 3.11±6.10 ng/mL, P=0.002) and insignificantly different from that in the viral infection group (0.23±0.34 ng/mL,P=0.457). The mean erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level in the KD group were significantly higher than those in the viral and bacterial infection groups (P1.0 ng/mL was significantly higher in the nonresponders to the initial intravenous immunoglobulin treatment than in the responders (36% vs. 8%, P=0.01). CONCLUSION: PCT levels may help to differentiate KD from bacterial infections. A combination of disease markers, including ESR, CRP, and PCT, may be useful for differentiating between KD and viral/bacterial infections.

Apparent life-threatening event in infancy / 소아과

An apparent life-threatening event (ALTE) is defined as the combination of clinical presentations such as apnea, marked change in skin and muscle tone, gagging, or choking. It is a frightening event, and it predominantly occurs during infancy at a mean age of 1–3 months. The causes of ALTE are categorized into problems that are: gastrointestinal (50%), neurological (30%), respiratory (20%), cardiovascular (5%), metabolic and endocrine (2%–5%), or others such as child abuse. Up to 50% of ALTEs are idiopathic, where the cause cannot be diagnosed. Infants with an ALTE are often asymptomatic at hospital and there is no standard workup protocol for ALTE. Therefore, a detailed initial history and physical examination are important to determine the extent of the medical evaluation and treatment. Regardless of the cause of an ALTE, all infants with an ALTE should require hospitalization and continuous cardiorespiratory monitoring and evaluation for at least 24 hours. The natural course of ALTEs has seemed benign, and the outcome is generally associated with the affected infants' underlying disease. In conclusion, systemic diagnostic evaluation and adequate treatment increases the survival and quality of life for most affected infants.

Comparison between Kawasaki disease with lymph-node-first presentation and Kawasaki disease without cervical lymphadenopathy / 소아과

PURPOSE: We evaluated the characteristics of patients with Kawasaki disease (KD) who presented with only fever and cervical lymphadenopathy on admission, and compared them with the characteristics of those who presented with typical features but no cervical lymphadenopathy. METHODS: We enrolled 98 patients diagnosed with KD. Thirteen patients had only fever and cervical lymphadenopathy on the day of admission (group 1), 31 had typical features with cervical lymphadenopathy (group 2), and 54 had typical features without cervical lymphadenopathy (group 3). RESULTS: The mean age (4.3+/-2.1 years) and duration of fever (7.5+/-3.6 days) before the first intravenous immunoglobulin (IVIG) administration were highest in group 1 (P=0.001). Moreover, this group showed higher white blood cell and neutrophil counts, and lower lymphocyte counts after the first IVIG administration as compared to the other groups (P=0.001, P=0.001, and P=0.003, respectively). Group 1 also had a longer duration of hospitalization and higher frequency of second-line treatment as compared to groups 2 and 3 (group 1 vs. group 2, P=0.000 and P=0.024; group 1 vs. group 3, P=0.000 and P=0.007). A coronary artery z score of >2.5 was frequently observed in group 1 than in group 3 (P=0.008). CONCLUSION: KD should be suspected in children who are unresponsive to antibiotics and have prolonged fever and cervical lymphadenopathy, which indicates that KD is associated with the likelihood of requiring second-line treatment and risk of developing coronary artery dilatation.

Comparison of Infective Endocarditis between Children and Adults with Congenital Heart Disease: A 16-Year, Single Tertiary Care Center Review

PURPOSE: This study aimed to evaluate and compare the characteristics of infective endocarditis (IE) between children and adults with congenital heart disease (CHD) at a single tertiary care center. METHODS: In this retrospective medical record review, we extracted the demographic characteristics, diagnostic variables, and outcomes of patients diagnosed with IE and CHD between 2000 and 2016. RESULTS: We identified a total of 14 pediatric patients (nine male; median age at diagnosis, 3 years). Of the 14 patients, six had a history of previous open heart surgery, while four had undergone tetralogy of Fallot repair, with transannular patch or Rastelli procedure. Among the 10 children with positive blood cultures, the most common isolated organism was Staphylococcus spp. (8/10, 80%). Eleven adult patients had IE and CHD. Among the adult patients, only four were diagnosed with CHD before IE, and ventricular septal defect was the most common CHD. The most common isolated organism was Streptococcus spp. (6/11, 55%). Compared with adult patients, pediatric patients had a higher incidence of previously diagnosed CHD (P=0.001), with Staphylococcus spp. as the causative organism (P =0.027). The median duration between the onset of symptoms and diagnosis of IE was 9 days in children and 42 days in adults (P=0.012). CONCLUSIONS: Significant differences with regard to the diagnosis and progress of IE were observed between children and adults. Ageadjusted and systematic reassessment may be necessary for the diagnosis and management of IE.

Persistent Truncus Arteriosus with Aortic Dominance in Female Adult Patient

Persistent truncus arteriosus categories associated with different natural histories and various surgical approaches were reported. Although pulmonary overflow and severe heart failure are common, some patients who have hypoplastic pulmonary artery systems may show lesser symptoms of heart failure and remain in relatively stable condition. We experienced a 33-year-old woman with uncorrected type II persistent truncus arteriosus who presented with cyanosis rather than congestive heart failure, and are presenting her images.

Relationship between the Clinical Characteristics and Intervention Scores of Infants with Apparent Life-threatening Events

We investigated the clinical presentations, diagnostic and therapeutic modalities, and prognosis from follow-up of infants with apparent life-threatening events (ALTE). In addition, the relationship between the clinical characteristics of patients and significant intervention scores was analyzed. We enrolled patients younger than 12 months who were diagnosed with ALTE from January 2005 to December 2012. There were 29 ALTE infants with a peak incidence of age younger than 1 month (48.3%). The most common symptoms for ALTE diagnosis were apnea (69.0%) and color change (58.6%). Eleven patients appeared normal upon arrival at hospital but 2 patients required cardiopulmonary resuscitation during the initial ALTE. The most common ALTE cause was respiratory disease, including respiratory infection and upper airway anomalies (44.8%). There were 20 cases of repeat ALTE and 2 cases of death during hospitalization. Four patients (15.4%) experienced recurrence of ALTE after discharge and 4 patients (15.4%) showed developmental abnormalities during the follow-up period. The patients with ALTE during sleep had lower significant intervention scores (P=0.015) compared to patients with ALTE during wakefulness and patients with previous respiratory symptoms had higher significant intervention scores (P=0.013) than those without previous respiratory symptoms. Although not statistically significant, there was a weak positive correlation between the patient's total ALTE criteria and total significant intervention score (Fig. 2, r=0.330, P=0.080). We recommend that all ALTE infants undergo inpatient observation and evaluations with at least 24 hr of cardiorespiratory monitoring, and should follow up at least within a month after discharge.

Intravenous Immunoglobulin Nonresponsive Symptomatic Myocarditis during the Acute Stage of Incomplete Kawasaki Disease

We report the case of a 7-year-old boy who showed treatment-nonresponsive hypotension (59/29 mmHg) and decreased left ventricular systolic function (fractional shortening 22%) in the acute stage of Kawasaki disease (KD). The present case serves to highlight that methylprednisolone pulse therapy should be considered in patients with intravenous immunoglobulin nonresponsive symptomatic myocarditis during the acute stage of KD.

T-Helper Cytokine Profiles in Patients with Kawasaki Disease

BACKGROUND AND OBJECTIVES: Kawasaki disease is an acute systemic vasculitis of which pathogenesis suspected is caused by immune dysregulation. The goal of this study is to evaluate the activation pattern of T helper cell type 1 (Th1) and T helper cell type 2 (Th2) in patients with Kawasaki disease. SUBJECTS AND METHODS: Prospective study of 60 patients (male 36, female 24) with diagnosis of Kawasaki disease were enrolled. One hundred and eighty blood samples from these patients were collected according to the different clinical stages {before initial intravenous immunoglobulin (IVIG), 5 days after initial IVIG, 2 months after initial IVIG}. The plasma level of Th1 cytokines; interferon-gamma (IFN-gamma) & interleukin (IL)-2 and Th2 cytokines; IL-4 & IL-10 were measured by enzyme-liked immunosorbent assay. RESULTS: In all patients, the plasma level of Th1 cytokines (IFN-gamma, IL-2) and Th2 cytokines (IL-4 and IL-10) were markedly elevated during the acute stage of Kawasaki disease. Since then, the plasma level of all these cytokines decreased significantly along with the process of clinical stages. Regardless of the existence of coronary artery lesion or no response to initial IVIG treatment, there were no significant differences between them. CONCLUSION: These data suggest that both Th1 and Th2 cells may be activated simultaneously during the acute stage of Kawasaki disease. Further studies are therefore required to establish the difference of activation pattern of T helper cells between Kawasaki disease and other inflammatory diseases.

Sensorineural hearing loss in patients with Kawasaki disease / 소아과

PURPOSE: Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. METHODS: Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. RESULTS: Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. CONCLUSION: The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.