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, post-cardiotomy, 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.

Hypertrophic cardiomyopathy as the initial presentation of mitochondrial disease in an infant born to a diabetic mother.

In contrast to hypertrophic cardiomyopathy caused by maternal diabetes, neonatal mitochondrial cardiomyopathy is rare and has a poor prognosis. We report an infant born to a mother with maternal diabetes with persistent ventricular hypertrophy, who was diagnosed with mitochondrial disease associated with m.3243A>G mutation in a mitochondrial tRNA leucine 1 gene. The hypertrophic cardiomyopathy was his initial and only clinical presentation.

Clinical indications for Kawasaki disease in febrile infants aged less than three months.

The purpose of this study was to determine the clinical and laboratory indicators of KD in febrile infants aged less than three months. Febrile infants aged less than three months were enrolled and divided into three groups: KD, bacterial infection (BI), and non-bacterial infection (NBI). Laboratory data were analyzed at two time points: initially upon admission and at follow-up after 48-72 h of admission. A total of 491 patients (280 males and 211 females) were enrolled. According to the final diagnosis, 29 patients had KD, 120 had BI, and 342 had NBI. The infants in the KD group were older (p < 0.001), had prolonged pre-admission and total fever duration (p < 0.001), and had a lower rate of decreased activity (p < 0.05) compared to the infants in the BI and NBI groups. The KD group had a significantly higher level of initial c-reactive protein (CRP) compared to the BI and NBI groups, with a cutoff value of 4.85 mg/dL in comparison with the BI group and 3.32 mg/dL in comparison with the NBI group. Moreover, the KD group had a significantly higher follow-up platelet count compared to the BI and NBI groups, with a cutoff value of 533 × 109/L in comparison with the BI group and 541 × 109/L in comparison with the NBI group. When febrile infants aged less than three months have prolonged fever, less decreased activity, and a higher initial CRP level and follow-up platelet count, we must consider the possibility of KD.

Importance of Serum Ferritin Level for Early Diagnosis and Differentiation in Patients with Kawasaki Disease with Macrophage Activation Syndrome.

We aimed to evaluate the utility of the serum ferritin level as an early screening test of Kawasaki disease with macrophage activation syndrome (KD-MAS). We analyzed the serum ferritin levels on the first day of admission and the clinical progress of patients diagnosed with complete or incomplete KD. Of the 158 patients, 5 were diagnosed with KD-MAS. Conjunctival injection was significantly more frequent in KD group (p = 0.035), although there were no significant differences in other clinical features. On the first day of admission, the serum ferritin level in the KD-MAS group was >500 ng/mL, which was higher than that in the KD group (p = 0.001). In the KD-MAS group, total bilirubin, triglyceride, and lactate dehydrogenase (LDH) were significantly higher, and erythrocyte sedimentation rate (ESR), total protein, albumin, and fibrinogen were significantly lower than the KD group (p < 0.05). Four patients were diagnosed with MAS within 7 days after admission, and 4 (80%) patients with KD-MAS survived. In conclusion, carrying out an early ferritin screening test is important in patients with principal clinical features that may suspect KD. We propose to include ferritin level in the primary laboratory test to differentiate between KD with and without MAS early.

Clinical implications of thrombocytosis in acute phase Kawasaki disease.

In Kawasaki disease (KD), thrombocytosis is commonly found in the subacute phase. However, the exact significance of thrombocytosis in the acute phase of KD is unclear. To evaluate serum platelet counts in patients during the acute phase of KD and assess the clinical outcomes according to the degree of thrombocytosis, we collected data of KD patients between 2009 and 2017. A total of 505 patients with KD were enrolled, and 249 (49.3%) patients had thrombocytosis, including mild (69.5%), moderate (21.7%), severe (4.8%), and extreme (4.0%) thrombocytosis. Correlation analysis revealed a positive correlation between the maximum platelet count and admission duration (r = 0.359, p < 0.001) and fever duration (r = 0.204, p < 0.001). The maximum platelet count was significantly higher in IVIG non-responders than that in IVIG responders (629 ± 201 × 109/L vs. 499 ± 154 × 109/L, p < 0.001), and in patients with coronary artery dilatation (CAD) than in those without CAD (602 ± 201 × 109/L vs. 512 ± 164 × 109/L, p < 0.001).Conclusion: Thrombocytosis in acute phase KD was associated with poor clinical outcomes such as IVIG non-responsiveness, CAD, and prolonged admission and fever durations. What is Known: • Thrombocytopenia in the acute phase of KD is related to non-responsiveness to IVIG and the risk of coronary artery dilatation. • The exact significance of thrombocytosis in the acute phase of KD as a benign phenomenon or a signal of poor outcome of KD is unclear. What is New: • Thrombocytosis in acute phase KD was associated with poor clinical outcomes such as IVIG non-responsiveness, CAD, and prolonged admission and fever durations.

Accompanied anomalies in anal atresia or tracheo-esophageal fistula: Comparison with or without VACTERL association.

PURPOSE: We evaluated the various accompanied malformations in patients with anal atresia or tracheoesophageal fistula (TEF). Furthermore, we determined the prevalence of VACTERL association and compared the clinical findings with those of patients without VACTERL association. METHODS: We enrolled the patients with anal atresia or TEF with/without esophageal atresia. We collected the patient data pertaining to accompanied vertebral, cardiovascular, renal or limb anomalies, single umbilical artery, maternal diabetes mellitus or drug history, and gene research. RESULTS: A total 155 patients (65 boys and 90 girls) were enrolled with 147 cases of anal atresia, 3 cases of TEF, and 5 cases of anal atresia with TEF. The prevalence of accompanied anomalies was 67.1% in cardiovascular, 27.1% in renal, 9.7% in vertebral, 2.6% in limb anomalies, and 3.9% in single umbilical artery. Thirty-six (23.2%) patients were diagnosed with VACTERL association. The patients with VACTERL association had a significantly higher number of male patients (58.3 vs. 37.0%, p = .033) and single umbilical artery (11.1 vs. 1.7%, p = .026), and had a significantly lower birth weight (2.8 vs. 3.1 kg, p = .033) than the patients without VACTERL association. Genetic studies were performed in 111 patients, and 8 (7.2%) had chromosomal abnormalities-3 in VACTERL and 5 in no VACTERL group. CONCLUSION: We recommend a careful evaluation for VACTERL association in patients with anal atresia or TEF. It is particularly important to screen for a single umbilical artery for features of VACTERL association as well as for other congenital anomalies.

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".

This corrects the article on p. e11 in vol. 36, PMID: 33398945.

Anomalous Connection of Umbilical Vessels to the Left Ventricle: Case Report.

The anomalous connection of umbilical vessels to the heart is rare and has not yet been reported in the international scientific literature. Herein, we report the case of a newborn who was diagnosed with an anomalous connection of the umbilical vessels to the left ventricle. These anomalous vessels were functionally open for 2 weeks, and cellulitis was present in the area of the blood vessels connected to the skin. We performed division of these abnormal vessels and removal of the skin lesion.

N-terminal pro-B-type natriuretic peptide levels in children: comparison in cardiac and non-cardiac diseases.

BACKGROUND: N-terminal pro-B-type natriuretic peptide (NT-proBNP), a well-known marker of cardiac disease, may be elevated in acute infections and other non-cardiac diseases. The aim of this study was to evaluate NT-proBNP levels in cardiac and non-cardiac diseases and found out the patient's clinical status that affects the NT-proBNP levels. METHODS: NT-proBNP levels were measured in three groups of children: a cardiac disease group, an infectious disease group, and a non-cardiac, non-infectious disease group. RESULT: In total, 348 children were enrolled, including 134 patients (38.5%) with cardiac disease, 170 patients (48.9%) with infectious disease, and 44 patients (12.6%) with non-cardiac, non-infectious disease. The NT-proBNP level of the cardiac disease group (median: 548 pg/mL; range: 5-35,000 pg/mL) was significantly higher than that of the infectious disease group (median: 193 pg/mL; range: 10-35,000 pg/mL) and the non-cardiac, non-infectious disease group (median: 280 pg/mL; range: 6-35,000 pg/mL). Regarding clinical status, the NT-proBNP levels were significantly higher in patients who needed mechanical ventilation support, oxygen therapy, or inotropic medication or had a change in mental status than in other patients. However, the systemic inflammatory response syndrome and mortality were not related to the NT-proBNP level. CONCLUSION: Among heterogeneous group of children, NT-proBNP level can be a useful marker of cardiac disease. Furthermore, the NT-proBNP levels were related to patients' clinical deteriorations, such as shock rather than the inflammatory status of patients.

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.

Colchicine for steroid-resistant recurrent pericarditis in a child.

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-1ß 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.

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.

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.

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 (P<0.001 and P<0.001 for ESR, P<0.001 and P=0.005 for CRP, respectively). The proportion of patients in the KD group with PCT levels of >1.0 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.

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 < 30 weeks' gestation underwent echocardiography within 14 days after birth for early PH and over 28 days after birth for late PH. We measured tricuspid regurgitation (TR) peak velocity, pulse Doppler-derived myocardial performance index (MPI) of right ventricle (RV) (RV MPI), eccentricity index (EI), and tricuspid annular plane systolic excursion (TAPSE). RESULTS: The median gestation age of patients was 27 weeks (range, 23-30 weeks) and median birth weight was 1030 g (range, 450-1780 g). TR peak velocity was measured only in 19 patients (28.4%). Patients with symptomatic early PH (n = 11) showed a significantly lower systolic EI and a significantly higher incidence of RV MPI > 0.38 and TAPSE < 0.5 cm than patients without PH. The incidence of symptomatic early PH was highest in severe BPD, although this was not statistically significant. Early echocardiographic parameters are not associated with BPD development. Patients with severe BPD showed a significantly higher RV MPI and a significantly higher incidence of RV MPI > 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.

Apparent life-threatening event in infancy.

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.