The First Korean Case Report of Siblings with 12q24.22q24.33 Duplication / 계명의대학술지

Live-born cases of partial trisomy 12q are rare, and only a few fetuses with this unbalanced translocation have survived to term. To our knowledge, only about 40 patients have been reported as having 12q duplication, and among them are no Korean reports. Here, we report the first Korean case of siblings with a 12q24.22q24.33 duplication. An 11-year-old boy visited our clinic for short stature. He was born small for his gestational age and had distinctive facial features, a history of surgery for anorectal malformation, psychomotor delay, intellectual disabilities, and attention-deficit/hyperactivity disorder (ADHD). He had an older sister with similar clinical features. The chromosomal microarray of the patient and his sister showed identical results: a 16.2 Mb duplication of 12q24.22q24.33. They had an identical cutoff point, but their symptoms were not. Symptoms common to both included growth retardation, psychomotor delay, intellectual disability, ADHD, and small for their gestational age.

Newly Developed Sex-Specific Z Score Model for Coronary Artery Diameter in a Pediatric Population

Background@#This study aimed to generate a Z score calculation model for coronary artery diameter of normal children and adolescents to be adopted as the standard calculation method with consensus in clinical practice. @*Methods@#This study was a retrospective, multicenter study that collected data from multiple institutions across South Korea. Data were analyzed to determine the model that best fit the relationship between the diameter of coronary arteries and independent demographic parameters. Linear, power, logarithmic, exponential, and square root polynomial models were tested for best fit. @*Results@#Data of 2,030 subjects were collected from 16 institutions. Separate calculation models for each sex were developed because the impact of demographic variables on the diameter of coronary arteries differs according to sex. The final model was the polynomial formula with an exponential relationship between the diameter of coronary arteries and body surface area using the DuBois formula. @*Conclusion@#A new coronary artery diameter Z score model was developed and is anticipated to be applicable in clinical practice. The new model will help establish a consensus-based Z score model.

Pediatric Extracorporeal Membrane Oxygenation in Korea: A Multicenter Retrospective Study on Utilization and Outcomes Spanning Over a Decade

Background@#Over the last decade, extracorporeal membrane oxygenation (ECMO) use in critically ill children has increased and is associated with favorable outcomes. Our study aims to evaluate the current status of pediatric ECMO in Korea, with a specific focus on its volume and changes in survival rates based on diagnostic indications. @*Methods@#This multicenter study retrospectively analyzed the indications and outcomes of pediatric ECMO over 10 years in patients at 14 hospitals in Korea from January 2012 to December 2021. Four diagnostic categories (neonatal respiratory, pediatric respiratory, postcardiotomy, and cardiac-medical) and trends were compared between periods 1 (2012–2016) and 2 (2017–2021). @*Results@#Overall, 1065 ECMO runs were performed on 1032 patients, with the annual number of cases remaining unchanged over the 10 years. ECMO was most frequently used for post-cardiotomy (42.4%), cardiac-medical (31.8%), pediatric respiratory (17.5%), and neonatal respiratory (8.2%) cases. A 3.7% increase and 6.1% decrease in pediatric respiratory and post-cardiotomy cases, respectively, were noted between periods 1 and 2.Among the four groups, the cardiac-medical group had the highest survival rate (51.2%), followed by the pediatric respiratory (46.4%), post-cardiotomy (36.5%), and neonatal respiratory (29.4%) groups. A consistent improvement was noted in patient survival over the 10 years, with a significant increase between the two periods from 38.2% to 47.1% (P = 0.004). Improvement in survival was evident in post-cardiotomy cases (30–45%, P = 0.002).Significant associations with mortality were observed in neonates, patients requiring dialysis, and those treated with extracorporeal cardiopulmonary resuscitation (P < 0.001). In pediatric respiratory ECMO, immunocompromised patients also showed a significant correlation with mortality (P < 0.001). @*Conclusion@#Pediatric ECMO demonstrated a steady increase in overall survival in Korea;however, further efforts are needed since the outcomes remain suboptimal compared with global outcomes.

Necrotizing fasciitis due to late-onset group B streptococcal bacteremia in a 2-month-old girl

Necrotizing fasciitis (NF) is a severe soft tissue infection, characterized by rapid and fulminant progression. Thus, early suspicion, and prompt medical and aggressive surgical management are important for the clinical outcomes of NF. Despite the rarity of NF in infants and group B streptococcus as its cause, a 2-month-old preterm girl presented with NF involving the suprapubic, inguinal, and lower lateral abdominal regions caused by group B streptococcus. The girl recovered after early medical and surgical management.

Pediatric Emergency Department Utilization and Coronavirus Disease in Daegu, Korea

Background@#Limited data exist on children's utilization of the emergency department (ED) in the ongoing coronavirus disease 2019 (COVID-19) pandemic. Thus, we aimed to examine ED utilization among pediatric patients and the impact of COVID-19 in one large city affected by the outbreak. @*Methods@#This retrospective study included data from six EDs in Daegu, Korea. We compared the demographic and clinical data of patients presenting to the ED during the COVID-19 pandemic (February 1st–June 30th 2020) with those of patients who visited the ED in this period during 2018 and 2019. @*Results@#Fewer patients, particularly children visited the EDs during the study period in 2020 than those in the previous (2018/2019) year period: the number of adult patient decreased by 46.4% and children by 76.9%. Although the number of patients increased from the lowest point of the decrease in March 2020, the number of pediatric patients visiting the ED remained less than half (45.2%) in June 2020 compared with that of previous years. The proportion of patients with severe conditions increased in adults, infants, and school-aged children, and consequently resulted in increased ambulance use and higher hospitalization rates. Fewer infants and young children but more school-aged children visited the ED with febrile illnesses in 2020 than in 2018/2019. @*Conclusion@#The COVID-19 pandemic has led to a substantial decrease in pediatric ED utilization. These findings can help reallocate human and material resources in the EDs during infectious disease outbreaks.

Erratum: Correction of Text in the Article “Pediatric Emergency Department Utilization and Coronavirus Disease in Daegu, Korea”

To the Editor:The authors regret that there were errors in the text. This notice corrects the numbers in the result of the Abstract. We changed the percentages indicating the decrease in the number of patients (adults and children) who visited the emergency department (EDs) during the study period.Content of correction:Before:“ Fewer patients, particularly children visited the EDs during the study period in 2020 than those in the previous (2018/2019) year period: the number of adult patient decreased by 46.4% and children by 76.9%.”After:“ Fewer patients, particularly children visited the EDs during the study period in 2020 than those in the previous (2018/2019) year period: the number of adult patient decreased by 26.0% and children by 62.0%.”

Evaluation and diagnostic approach for heart murmurs in children

Heart murmurs are common in children. Most of them are innocent murmurs with normal heart anatomy and function, and only a few are pathologic murmurs with congenital heart anomaly or abnormal heart function. However, a heart murmur may be the sole symptom of serious heart disease. Therefore, careful evaluation of heart murmurs for distinguishing pathologic murmurs from innocent murmurs is important. Heart murmurs are described by their intensity, timing in the cardiac cycle, location, transmission, and quality. Murmurs, such as a holosystolic or diastolic murmur, of grade 3 or higher intensity, harsh quality, an abnormal S2, a systolic click, or increased intensity when the patient stands are more likely to be pathologic murmurs. Innocent murmurs are more likely to be systolic murmurs, with soft sounds, short duration, low pitch, and varying intensity with phases of respiration and posture (disappears with standing). Not only auscultation but also physical examination findings are important to evaluate heart murmurs. The gold standard test for the evaluation of any potentially pathologic murmur is echocardiography. For the appropriate use of echocardiography, close physical examination, including auscultation, is essential and requires considerable practice.

Toxic Epidermal Necrolysis complicating Infliximab therapy in Refractory Kawasaki disease / 계명의대학술지

Severe skin reactions complicating tumor necrosis factor-alpha (TNF-α) antagonist therapy were somewhat reported in adults. We report a case of 24-month-old boy with refractory Kawasaki disease who developed toxic epidermal necrolysis (TEN) after one dose of 5mg/kg infliximab. The patient responded to intravenous hydrocortisone and was able to discharge without any other serious complications. The clinicians should be aware of the potential for severe skin reactions from the use of TNF-α antagonists in children.

Toxic Epidermal Necrolysis complicating Infliximab therapy in Refractory Kawasaki disease / 계명의대학술지

Severe skin reactions complicating tumor necrosis factor-alpha (TNF-α) antagonist therapy were somewhat reported in adults. We report a case of 24-month-old boy with refractory Kawasaki disease who developed toxic epidermal necrolysis (TEN) after one dose of 5mg/kg infliximab. The patient responded to intravenous hydrocortisone and was able to discharge without any other serious complications. The clinicians should be aware of the potential for severe skin reactions from the use of TNF-α antagonists in children.

Importance of coronary artery dominance in children to determine coronary artery dilatation / 소아과

No abstract available.

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.

Colchicine for steroid-resistant recurrent pericarditis in a child / 영남의대학술지

Recurrent pericarditis is rare in children and is considered idiopathic in most cases. Its course is chronic, and preventing recurrences is important for the patient's quality of life. Although a treatment strategy in pediatric recurrent pericarditis has not yet been established, non-steroidal anti-inflammatory drugs (NSAIDs) are the most common treatment for management of this condition, followed by corticosteroids, colchicine, immunosuppressive agents, immunoglobulins, and interleukin-1β receptor antagonists (e.g. anakinra). Herein, we report a case of recurrent pericarditis with pericardial effusion in a 5-year-old child who presented with fever and epigastric pain. He responded poorly to NSAIDs and corticosteroid therapy, but was successfully treated with colchicine.

Colchicine for steroid-resistant recurrent pericarditis in a child / 영남의대학술지

Recurrent pericarditis is rare in children and is considered idiopathic in most cases. Its course is chronic, and preventing recurrences is important for the patient's quality of life. Although a treatment strategy in pediatric recurrent pericarditis has not yet been established, non-steroidal anti-inflammatory drugs (NSAIDs) are the most common treatment for management of this condition, followed by corticosteroids, colchicine, immunosuppressive agents, immunoglobulins, and interleukin-1β receptor antagonists (e.g. anakinra). Herein, we report a case of recurrent pericarditis with pericardial effusion in a 5-year-old child who presented with fever and epigastric pain. He responded poorly to NSAIDs and corticosteroid therapy, but was successfully treated with colchicine.

Clinical presentation of croup in children according to causative viruses

PURPOSE: We evaluated the clinical features of croup in children according to viral etiology. METHODS: This study enrolled pediatric patients with croup, who showed positive results on respiratory virus reverse transcriptase polymerase chain reaction performed between January 2012 and December 2017. We retrospectively reviewed the medical records. RESULTS: A total of 179 patients (119 boys and 60 girls) were enrolled with the mean age of 18.9±14.7 months. The viruses commonly identified were parainfluenza, respiratory syncytial virus, rhinovirus, and influenza. Among these 4 viruses, patients with rhinovirus infection showed significantly shorter fever and admission durations. Patients with parainfluenza infection showed significantly lower incidences of epinephrine nebulization and patients with influenza infections showed significantly higher incidences of steroid treatment. CONCLUSION: Clinical manifestations of croup differ according to causative viruses. Further studies should be conducted to evaluate the severity and prognosis of croup according to viral etiology.

A cross-sectional retrospective study to analyze the underlying causes and clinical characteristics of children with reactive thrombocytosis at a Korean tertiary medical center

BACKGROUND: Reactive thrombocytosis (RT) is a common condition among children, although no studies have examined the etiology or clinical characteristics of RT among Korean children. METHODS: This retrospective study evaluated children with RT at a single Korean tertiary center during a 10-year period. RESULTS: RT accounted for 13.5% of children who were admitted to the pediatric ward (4,113/30,355): mild RT, 82.7%; moderate RT, 14.1%; severe RT, 1.1%; and extreme RT, 2.1%. There was a negative correlation between platelet count and Hb level (P=0.008). There were positive correlations between platelet count and WBC (P=0.001), erythrocyte sedimentation rate (ESR) (P=0.007), and admission duration (P=0.006). The most common cause of RT was infection and the second most common was Kawasaki disease (KD). The highest proportion of lower respiratory tract infection was observed in extreme RT (P < 0.001). The proportion of KD was highest in extreme RT (P < 0.001) and in children aged 1–7.9 years (P < 0.001). The proportion of refractory KD was highest in extreme RT (P=0.005). In cases of KD, there was a positive correlation between platelet count and fever duration (P=0.006). Non-KD autoimmune inflammation was only observed in mild/moderate RT, and its proportion was highest in children aged 8–18 years (P < 0.001). CONCLUSION: In children, more severe RT was associated with lower Hb, increased WBC, ESR, and prolonged admission. With respiratory infection or KD, extreme RT was associated with more severe disease course.

Clinical Utility of Echocardiography for Early and Late Pulmonary Hypertension in Preterm Infants: Relation with Bronchopulmonary Dysplasia

BACKGROUND: We evaluated early and late pulmonary hypertension (PH) in preterm infants and its relation with bronchopulmonary dysplasia (BPD). METHODS: Sixty-seven preterm infants 0.38 and TAPSE 0.38 than patients with mild BPD, and a significantly lower systolic EI and a significantly higher incidence of systolic EI < 0.81 than patients without BPD. CONCLUSION: Systolic EI, RV MPI, and TAPSE were well represented symptomatic early PH, while systolic EI and RV MPI could be useful parameters for identifying late PH in preterm infants with BPD, even if they did not present PH symptoms.

Clinical characteristics of lower respiratory infections in preterm children with bronchopulmonary dysplasia

PURPOSE: We evaluated the clinical characteristics of lower respiratory infections of preterm children with bronchopulmonary dysplasia (BPD) and compared them between those with and without lower respiratory infections that of preterm patients without BPD. METHODS: This study enrolled preterm patients under 2 years old, who admitted with acute lower respiratory infection from March 2014 to May 2016. The patients were divided into 2 groups according to BPD, and we retrospectively reviewed their medical records. RESULTS: A total of 71 patients (106 cases) were enrolled; the BPD group consisited of 29 patients (54 cases) and the control group 42 patients (52 cases). Compared to the patients in the control group, those in the BPD group were older (P=0.001), had lower gestational age and birth weight (P<0.001), and showed more frequent readmission in hospital (P=0.017). The most common causative virus was human rhinovirus (hRV) in the BPD group, whereas respiratory syncytial virus (RSV) in the control group. The patients in the BPD group showed a higher incidence of tachypnea, decreased aeration, and chest retraction (P<0.001, P=0.009, and P=0.026, respectively), a higher respiratory symptom score (P=0.011), a longer duration of cough and wheezy sounds (P=0.004 and P=0.009, respectively), and higher incidence and longer duration of treatment with oxygen, and mechanical ventilator support (P=0.016 and P=0.017, respectively) than those in the control group. In the BPD group, the patients with RSV showed a higher incidence of tachypnea and rales (P=0.033 and P=0.033, respectively) than those with hRV. CONCLUSION: The preterm children with BPD may have more severe clinical manifestations than those without.

Respiratory Syncytial Virus Infection Complicated by Extrapulmonary Manifestations

Respiratory syncytial virus (RSV) typically causes lower respiratory tract infections in children, and most patients recover successfully. However, some infants and young children can have a severe course of disease with respiratory failure, and extrapulmonary manifestations can occur in severe RSV disease. We report one case of severe RSV bronchiolitis complicated with acute myocarditis, fulminant hepatic failure, and disseminated intravascular coagulation.

Respiratory Syncytial Virus Infection Complicated by Extrapulmonary Manifestations

Respiratory syncytial virus (RSV) typically causes lower respiratory tract infections in children, and most patients recover successfully. However, some infants and young children can have a severe course of disease with respiratory failure, and extrapulmonary manifestations can occur in severe RSV disease. We report one case of severe RSV bronchiolitis complicated with acute myocarditis, fulminant hepatic failure, and disseminated intravascular coagulation.

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.