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Purpose Ejection fraction, measured as the fraction of blood ejected from the ventricle in each heartbeat using M-mode echocardiography, serves as a primary indicator of left ventricular systolic function. This study explores the correlation between blood pressure and left ventricular systolic function in neonates using M-mode echocardiography. Methods Neonates who underwent echocardiography in the neonatal intensive care unit between January 2011 and December 2020 were retrospectively studied. Results Our analyses showed a significant association between ejection fraction and systolic blood pressure, but not with diastolic or mean blood pressure—both of which are more sensitive to hypotension. Ejection fraction was also not significantly associated with heart rate, urine output, or inotropic support in this study, suggesting that factors influencing urine output may not directly relate to ejection fraction. Additionally, we found that higher systolic blood pressure was correlated with advanced gestational age, the absence of patent ductus arteriosus, and no need for fentanyl administration. Notably, lower gestational age and lack of mechanical ventilation were both associated with increased hourly urine output, suggesting that developmental maturity and respiratory stability may influence renal function. Conclusion Neonatal hypotension occurred secondary to decreased systolic cardiac function and peripheral vascular resistance. Neonatologists should carefully monitor the individual components of blood pressure and prescribe medications accordingly, considering that systolic blood pressure is correlated with ejection function.

Partial trisomy 1q is a rare chromosomal disorder characterized by ventriculomegaly with craniofacial, renal, cardiac, and finger and toe anomalies. Most reported cases of partial trisomy1q have involved stillborn or premature deaths due to cardiac or liver failure. This case report describes an 18-month-old patient with partial duplication of the 1q32-44 segments and consequent developmental delays who exhibited improvement in developmental status with rehabilitation. Prenatal ultrasonography and magnetic resonance imaging of the mother revealed ventriculomegaly and atrophic changes in the left cerebral hemisphere of the fetus. The infant was born with micrognathia, microphthalmia, macrocephaly, low-set ears, polydactyly, and long feet at 37+5 weeks of gestation. A chromosomal study revealed an abnormal male karyotype of 46,XY,rec(1)dup(1)(q32.1q44)inv(1)(p36.3q32.1)pat. In this rare case of a patient with partial trisomy, we observed improvement in developmental delays following treatment using appropriate rehabilitation techniques. Further research is required to help validate the findings of this case study and establish a standardized rehabilitation technique that can be subsequently applied to such cases.

Purpose Previous estimates of the cause- and age-specific frequency of pediatric meningitis in Korea were mainly derived from single- and multi-center studies. Herein, we used data on the number of cases of bacterial and viral meningitis from the Health Insurance Review and Assessment Service database to examine time- and sex-related trends. Methods We extracted data on meningitis diagnoses registered in the Health Insurance Review and Assessment Service from 2009 to 2017, using Korean Standard Classification of Disease and Cause of Death codes. Information on 202,254 children aged 0 to 18 years was extracted. Detailed demographic and disease information was available for 84,543 children who underwent hospitalization. Results Among all hospitalized patients, 2166, 36,155, and 46,192 children were diagnosed with bacterial, viral, or other types of meningitis, respectively. There were 30 cases of fungal meningitis and another 30 cases of meningitis attributable to other pathogens. The number of cases of bacterial and viral meningitis was highest among infants (1,087 [50.2%]) and patients in their early childhood (12,949 [35.9%]), respectively. Meningitis outbreaks were most likely to occur during the summer, and boys were more susceptible to meningitis than girls. The following pathogens most commonly caused infant meningitis: group B Streptococcus, Escherichia coli, and type B Haemophilus influenzae. Conclusion This study reports the number of pediatric meningitis cases, stratified by age, disease type, and month/year. The present findings contribute to a better understanding of pediatric meningitis in Korea and provide a foundation for future research to identify the risk factors for this disease.

Background An increase in the numbers of patients with gastrointestinal symptoms has recently been observed. Purpose To investigate the effects of proton pump inhibitor (PPI) therapy on intestinal inflammation in children and adolescents as confirmed by clinical manifestations and objectively assessed by fecal calprotectin (FC) level measurement. Methods Consecutive children (aged 3–18 years) who presented with gastrointestinal symptoms and were treated with or without PPI for at least 1 month were enrolled. Patients were divided into PPI and non-PPI groups. The PPI group was further subdivided by treatment duration and type of PPI used. Stool samples were collected for FC evaluation at baseline and after treatment and clinical data and FC levels were compared between the groups. Results Fifty-one patients (15 boys, 36 girls) were enrolled in the study. The PPI group included 37 patients, while the non-PPI group included 14 patients. Clinical symptoms were not significantly different. FC levels and laboratory results, including C-reactive protein levels, white blood cell count, and absolute neutrophil count, were not statistically different before versus after PPI treatment. After treatment, FC levels decreased to 8.1 mg/kg (-575.4 to 340.3 mg/kg) in the PPI group and increased to 5.6 mg/kg (-460.0 to 186.9 mg/kg) in the non-PPI group compared to those before treatment (P=0.841). The number of patients with increased FC levels was not significantly different between the 2 groups (48.6% vs. 64.3%, P=0.363), similar to that observed in patients with an FC level > 50 mg/kg (24.3% and 7.1%, P=0.250). PPI therapy type and duration did not affect the FC levels (P=0.811 and P=0.502, respectively). Conclusion Although we aimed to confirm the evidence of intestinal inflammation due to PPI use in children and adolescents through clinical symptoms and FC measurement, no significant changes were observed.

Abstract Background Intussusception is a gastrointestinal condition in which early treatment is critical. Although its epidemiology and comorbidities have been studied, few studies have included the entire pediatric population of a country. Therefore, we aimed to analyze the epidemiologic features of pediatric intussusception patients and identify comorbidities associated with intussusception in South Korea, using the public health database. Methods We analyzed the data of children below 18 years of age, from the national database of South Korea, who were diagnosed with intussusception and managed such as air reduction or surgical methods from 2008 to 2016. Patients were categorized into six groups based on the comorbid diseases. Patients with structural lesion in gastrointestinal tract were divided diagnosis or diagnosis code. Results The number of patients diagnosed with intussusception were 25,023 (16,024 males, 64.0%). Of them, the highest percentage was patients aged between 2 and 36 months (20,703; 82.7%). The incidence per 100,000 individuals aged up to 2 years was 196.7. The number of males were 16,024 (64.0%) and were almost twice the number of 8999 (36.0%) female patients. The maximum number of cases (n = 2517; 10.1%) were seen in September, followed by July (n = 2469; 9.9%). In February, the number of cases was lowest at 1448 (5.8%) patients (P

Bacterial infections (BIs) need to be differentiated from non-BIs (NBIs) to enable prompt administration of antibiotics. Therefore, inflammatory biomarkers are needed as they can accurately identify BIs. This study evaluated the usefulness of procalcitonin (PCT) in the diagnosis of BI in immunocompetent children. We retrospectively reviewed the medical records of patients p = 0.008) and ESR (39.1 ± 32.4 vs. 54.8 ± 28.2 mm/h; p < 0.001) were significantly different. Between the IBI and other groups, WBC (14,797 ± 7148 vs. 12,622 ± 5770 × 106/L; p = 0.007), ESR (35.3 ± 30.3 vs. 51.5 ± 30.3 mm/h; p < 0.001), and PCT (8.1 ± 23.8 vs. 1.0 ± 3.4 ng/mL; p = 0.005) were significantly different. However, none of the biomarkers were useful in differentiating BI from NBI. While WBC (area under curve (AUC) = 0.615, p = 0.003) and PCT (AUC = 0.640, p < 0.001) were useful, they fared poorly in differentiating IBI from other groups. Thus, additional studies are needed to identify more accurate biomarkers capable of differentiating BIs, especially IBIs.

Kawasaki disease (KD) is an acute febrile vasculitis predominately affecting infants and children. The dominant incidence age of KD is from 6 months to 5 years of age, and the incidence is unusual in those younger than 6 months and older than 5 years of age. We tried to identify genetic variants specifically associated with KD in patients younger than 6 months or older than 5 years of age. We performed an age-stratified genome-wide association study using the Illumina HumanOmni1-Quad BeadChip data (296 cases vs. 1,000 controls) and a replication study (1,360 cases vs. 3,553 controls) in the Korean population. Among 26 candidate single nucleotide polymorphisms (SNPs) tested in replication study, only a rare nonsynonymous SNP (rs4365796: c.1106C＞T, p.Thr369Met) in the lymphoid enhancer binding factor 1 (LEF1) gene was very significantly associated with KD in patients younger than 6 months of age (odds ratio [OR], 3.07; pcombined = 1.10 × 10-5), whereas no association of the same SNP was observed in any other age group of KD patients. The same SNP (rs4365796) in the LEF1 gene showed the same direction of risk effect in Japanese KD patients younger than 6 months of age, although the effect was not statistically significant (OR, 1.42; p = 0.397). This result indicates that the LEF1 gene may play an important role as a susceptibility gene specifically affecting KD patients younger than 6 months of age.

Objectives: Computed tomography should be performed after careful consideration due to radiation hazard, which is why interest in low dose CT has increased recently in acute appendicitis. Previous studies have been performed in adult and adolescents populations, but no studies have reported on the efficacy of using low‐dose CT in children younger than 10 years. Methods: Patients (n = 475) younger than 10 years who were examined for acute appendicitis were recruited. Subjects were divided into three groups according to the examinations performed: low‐dose CT, ultrasonography, and standard‐dose CT. Subjects were categorized according to age and body mass index (BMI). Results: Low‐dose CT was a contributive tool in diagnosing appendicitis, and it was an adequate method, when compared with ultrasonography and standard‐dose CT in terms of sensitivity (95.5% vs. 95.0% and 94.5%, p = 0.794), specificity (94.9% vs. 80.0% and 98.8%, p = 0.024), positive‐predictive value (96.4% vs. 92.7% and 97.2%, p = 0.019), and negative‐predictive value (93.7% vs. 85.7% and 91.3%, p = 0.890). Low‐dose CT accurately diagnosed patients with a perforated appendix. Acute appendicitis was effectively diagnosed using low‐dose CT in both early and middle childhood. BMI did not influence the accuracy of detecting acute appendicitis on low‐dose CT. Conclusion: Low‐dose CT is effective and accurate for diagnosing acute appendicitis in childhood, as well as in adolescents and young adults. Additionally, low‐dose CT was relatively accurate, irrespective of age or BMI, for detecting acute appendicitis. Therefore, low‐dose CT is recommended for assessing children with suspected acute appendicitis.

Abstract Background Hepatitis B virus (HBV) infection is the most common cause of liver disease in endemic areas such as South Korea. After HBV vaccination, hepatitis B surface antibody (HBsAb) titers gradually decrease. Trends in HBsAb titers have not been evaluated among children in South Korea over the past decade. Methods We screened 6155 patients (aged 7 months to 17 years) who underwent HBV antigen/antibody testing at Chung-Ang University Hospital from May 2012 to April 2015. Titer criteria were defined as follows: positive, titer ≥100 IU/L; weakly positive, titer 10–99 IU/L; and negative, titer

PurposeThe aim of this study was to investigate the potential effects of mild hypoxia in the mature and immature brain.MethodsWe prepared organotypic slice cultures of the hippocampus and used hippocampal tissue cultures at 7 and 14 days in vitro (DIV) to represent the immature and mature brain, respectively. Tissue cultures were exposed to 10% oxygen for 60 minutes. Twenty-four hours after this hypoxic insult, propidium iodide fluorescence images were obtained, and the damaged areas in the cornu ammonis 1 (CA1), CA3, and dentate gyrus (DG) were measured using image analysis.ResultsIn the 7-DIV group compared to control tissue, hypoxia-exposed tissue showed decreased damage in two regions (CA1: 5.59%±2.99% vs. 4.80%±1.37%, P=0.900; DG: 33.88%±12.53% vs. 15.98%±2.37%, P=0.166), but this decrease was not statistically significant. In the 14-DIV group, hypoxia-exposed tissue showed decreased damage compared to control tissues; this decrease was not significant in the CA3 (24.51%±6.05% vs. 18.31%±3.28%, P=0.373) or DG (15.72%±3.47% vs. 9.91%±2.11%, P=0.134), but was significant in the CA1 (50.91%±5.90% vs. 32.30%±3.34%, P=0.004).ConclusionAlthough only CA1 tissues cultured for 14 DIV showed significantly less damage after exposure to hypoxia, the other tissues examined in this study showed a tendency towards less damage after hypoxic exposure. Therefore, mild hypoxia might play a protective role in the brain.

Kawasaki disease (KD) is an acute systemic vasculitis that can potentially cause coronary artery aneurysms in some children. KD occurs approximately 1.5 times more frequently in males than in females. To identify sex-specific genetic variants that are involved in KD pathogenesis in children, we performed a sex-stratified genome-wide association study (GWAS), using the Illumina HumanOmni1-Quad BeadChip data (249 cases and 1,000 controls) and a replication study for the 34 sex-specific candidate SNPs in an independent sample set (671 cases and 3,553 controls). Male-specific associations were detected in three common variants: rs1801274 in FCGR2A [odds ratio (OR) = 1.40, P = 9.31 × 10-5], rs12516652 in SEMA6A (OR = 1.87, P = 3.12 × 10-4), and rs5771303 near IL17REL (OR = 1.57, P = 2.53 × 10-5). The male-specific association of FCGR2A, but not SEMA6A and IL17REL, was also replicated in a Japanese population (OR = 1.74, P = 1.04 × 10-4 in males vs. OR = 1.22, P = 0.191 in females). In a meta-analysis with 1,461 cases and 5,302 controls, a very strong association of KD with the nonsynonymous SNP rs1801274 (p.His167Arg, previously assigned as p.His131Arg) in FCGR2A was confirmed in males (OR = 1.48, P = 1.43 × 10-7), but not in the females (OR = 1.17, P = 0.055). The present study demonstrates that p.His167Arg, a KD-associated FCGR2A variant, acts as a susceptibility gene in males only. Overall, the gender differences associated with FCGR2A in KD provide a new insight into KD susceptibility.

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

Purpose: As the importance of breastfeeding has been reinforced, human milk is often stored for practical reasons. Therefore, we evaluated optimal storage and processing methods for human milk from a nutritional standpoint. Methods: Human milk samples were collected between June 2017 and February 2018. Also, data about maternal information were collected. Human milk was analyzed for macronutrients and caloric content. The samples were subdivided into groups for nutrient analysis. The control group (fresh milk) was not stored or processed. The other groups (9 groups) consisted of samples analyzed based on different storage temperatures (room temperature, refrigerated, frozen), defrosting methods (bottle warmer, room temperature thawing, microwave oven), and storage period (1 week, 1 month, 2 months) and compared with the control group. Results: There was no statistically significant difference in the nutrient content of human milk among the collected samples. A significant change in the content of macronutrients in milk samples was observed under storage condition at different temperatures for 1 week with subsequent thawing with bottle warmer compared to fresh milk. Under storage at −20°C for 1 week with subsequent thawing with different defrosting methods, a significant change in the content of macronutrients in milk samples was observed compared to fresh milk. After storage at −20°C for different periods and thawing with a bottle warmer, a significant change in macronutrient content in milk samples was observed compared to fresh milk regardless of the storage period. Conclusion: Unlike previous guidelines, changes in macronutrient content in milk samples were observed regardless of the method of storing and thawing. Apparently, it is proposed that mothers should feed fresh human milk to their babies without storing. [ABSTRACT FROM AUTHOR]

Abstract Objectives: Computed tomography should be performed after careful consideration due to radiation hazard, which is why interest in low dose CT has increased recently in acute appendicitis. Previous studies have been performed in adult and adolescents populations, but no studies have reported on the efficacy of using low-dose CT in children younger than 10 years. Methods: Patients (n = 475) younger than 10 years who were examined for acute appendicitis were recruited. Subjects were divided into three groups according to the examinations performed: low-dose CT, ultrasonography, and standard-dose CT. Subjects were categorized according to age and body mass index (BMI). Results: Low-dose CT was a contributive tool in diagnosing appendicitis, and it was an adequate method, when compared with ultrasonography and standard-dose CT in terms of sensitivity (95.5% vs. 95.0% and 94.5%, p = 0.794), specificity (94.9% vs. 80.0% and 98.8%, p = 0.024), positive-predictive value (96.4% vs. 92.7% and 97.2%, p = 0.019), and negative-predictive value (93.7% vs. 85.7% and 91.3%, p = 0.890). Low-dose CT accurately diagnosed patients with a perforated appendix. Acute appendicitis was effectively diagnosed using low-dose CT in both early and middle childhood. BMI did not influence the accuracy of detecting acute appendicitis on low-dose CT. Conclusion: Low-dose CT is effective and accurate for diagnosing acute appendicitis in childhood, as well as in adolescents and young adults. Additionally, low-dose CT was relatively accurate, irrespective of age or BMI, for detecting acute appendicitis. Therefore, low-dose CT is recommended for assessing children with suspected acute appendicitis.

Purpose: This study investigated predictive factors for severe neonatal thrombocytopenia, which greatly increases the need for intensive care and is associated with a high mortality rate in premature infants. Factors adopted for prompt identification of at-risk newborns include blood test results and birth history. This study analyzed the relationship between the presence of severe neonatal thrombocytopenia and the mortality rate. The causes of thrombocytopenia in premature infants were also examined. Methods: This retrospective study evaluated 625 premature infants admitted to the neonatal intensive care unit (NICU) at Chung-Ang University Medical Center. The neonates were classified into 3 groups according to the severity of thrombocytopenia: mild (100×109/L≤platelet<150×109/L), moderate (50×109/L≤platelet<100×109/L), or severe (platelet<50×109/L). Analysis of blood samples obtained at the onset of thrombocytopenia included platelet count, white blood cell (WBC) count, hemoglobin level, hematocrit level, absolute neutrophil count, and high-sensitivity C-reactive protein level. Results: Of the 625 premature infants admitted to our NICU, 214 were detected with thrombocytopenia. The mortality rate in thrombocytopenic neonates was 18.2% (39/ 214), whereas a mortality rate of only 1.0% was observed in non-thrombocytopenic neonates. The major causes of thrombocytopenia were perinatal insufficiency and sepsis in premature infants. Severe thrombocytopenia was noted more frequently in premature infants with higher WBC counts and in those with a younger gestational age. Conclusion: Platelet count, WBC count, and gestational age are reliable predictors for severe neonatal thrombocytopenia. The major causes of thrombocytopenia were perinatal insufficiency and sepsis in premature infants. [ABSTRACT FROM AUTHOR]

A 14-year-old girl diagnosed with Behçet's disease 15 months previously presented at the emergency department with severe headache, fever, and vomiting. Chest radiographic and computed tomography findings were consistent with miliary tuberculosis, and pleocytosis and increased protein levels were found on examination of cerebrospinal fluid (CSF). Tuberculous meningitis associated with miliary pulmonary tuberculosis was suspected, and a four-drug regimen for tuberculosis plus intravenous dexamethasone was initiated. However, negative results of real-time polymerase chain reaction and CSF cultures led us to reconsider our initial diagnosis. On brain magnetic resonance imaging, the results supported neuro-Behçet's syndrome rather than tuberculous meningitis. However, it is unclear how miliary tuberculosis complicated neuro-Behçet's syndrome because the patient had no history of tuberculosis or contact with patients with tuberculosis, and had not used any immunosuppressive agents such as anti-tumor necrosis factor-alpha drugs. This is a very rare case of neuro-Behçet's syndrome associated with miliary tuberculosis. More commonly, cases of neuro-Behçet's syndrome presenting with features mimicking acute tuberculous meningitis have been reported. Neuro-Behçet's syndrome should be considered in patients with a history of Behçet's disease presenting with signs of meningeal irritation. [ABSTRACT FROM AUTHOR]

Congenital chloride diarrhea (CLD) is a rare inherited autosomal recessive disorder. Mutations of the solute carrier family 26 member 3 gene cause profuse, chloride ion rich diarrhea, which results in hypochloremia, hyponatremia and metabolic alkalosis with dehydration. If a fetal ultrasound shows bowel dilatation suggestive of bowel obstruction, or if a neonate shows persistent diarrhea and metabolic alkalosis, CLD should be considered in the differential diagnosis. The severity of CLD varies, but early detection and early therapy can prevent complications including growth failure. We report a case of dizygotic twins affected by CLD who had been born to non-consanguineous parents. Both of them showed growth failure, but one of the twins experienced worse clinical course. He showed developmental delay, along with dehydration and severe electrolyte imbalance. He was diagnosed with CLD first at 6-month age, and then the other one was also diagnosed with CLD. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is an acute self-limited vasculitis of infants and children that manifests as fever and signs of mucocutaneous inflammation. Coronary artery aneurysms develop in approximately 15-25% of untreated children. Although the etiology of KD is largely unknown, epidemiologic data suggest the importance of genetic factors in the susceptibility to KD. In order to identify genetic variants that influence KD susceptibility, we performed a genome-wide association study (GWAS) using Affymetrix SNP array 6.0 in 186 Korean KD patients and 600 healthy controls; 18 and 26 genomic regions with one or more sequence variants were associated with KD and KD with coronary artery lesions (CALs), respectively ( p < 1 × 10). Of these, one locus on chromosome 1p31 (rs527409) was replicated in 266 children with KD and 600 normal controls (odds ratio [OR] = 2.90, 95% confidence interval [CI] = 1.85-4.54, P = 1.46 × 10); and a PELI1 locus on chromosome 2p13.3 (rs7604693) was replicated in 86 KD patients with CALs and 600 controls (OR = 2.70, 95% CI = 1.77-4.12, P = 2.00 × 10). These results implicate a locus in the 1p31 region and the PELI1 gene locus in the 2p13.3 region as susceptibility loci for KD and CALs, respectively. [ABSTRACT FROM AUTHOR]

The article reports an outbreak of Serratia marcescens blood stream infection (BSI) in the pediatric ward at the Chung-Ang University Hospital in Seoul, Korea associated with a contaminated multidose dropper bottle used for nebulization. Hospital investigation revealed poor hand hygiene and possible contamination of the common drug bottles used for nebulization. According to speculation, the absence of mortality was due to the immediate initiation of effective antibiotic therapy, absence of risk factors or history of ventilator care or airway suction.