Delta neutrophil index as a predictor of vesicoureteral reflux in children with febrile urinary tract infection

Purpose@#Delta neutrophil index (DNI) indicates immature granulocytes in peripheral blood and has been confirmed to be effective as a prognostic factor for neonatal sepsis. Also, it has been reported to have diagnostic value in acute pyelonephritis and in predicting vesicoureteral reflux (VUR) in the infant. We conducted the study to verify whether DNI is also helpful in the entire pediatric age group with febrile urinary tract infection (UTI). @*Methods@#Medical records of children hospitalized for febrile UTIs were analyzed retrospectively. All subjects underwent kidney ultrasound and voiding cystourethrography. In the group with and without VUR, we compared sex and age, and the following laboratory values: the white blood cell count, neutrophil, polymorphonuclear leucocyte, eosinophil, hemoglobin, platelet count, C-reactive protein, DNI value, and the finding of ultrasound. @*Results@#A total of 315 patients (163 males and 152 females; range, 0–127 months) were eligible, and 41 patients (13%) had VUR. As a result of univariate analysis, the white blood cell count, neutrophil, DNI, and ultrasonic abnormalities were high in the reflux group, and the hemoglobin and lymphocyte fraction values were low. The value of DNI and the abnormal ultrasound were significantly higher in the reflux group on the multivariate analysis. The area under the curve value of the receiver operating curve was higher in DNI (0.640; 95% confidence interval, 0.536–0.744; P=0.004), and the DNI cutoff value for VUR prediction was 1.85%. @*Conclusions@#We identified that ultrasound findings and DNI values were helpful predictors of VUR in pediatric febrile UTIs.

Correlation between Transient Elastography (Fibroscan ® ) and Ultrasonographic and Computed Tomographic Grading in Pediatric Nonalcoholic Steatohepatitis / 대한소아소화기영양학회지

Purpose@#This study aimed to examine the advantages and usefulness of transient elastography (Fibroscan ® ) in diagnosing non-alcoholic steatohepatitis in children and adolescents compared to those of abdominal computed tomography and liver ultrasonography. @*Methods@#Forty-six children and adolescent participants aged between 6 and 16 years who underwent transient elastography (Fibroscan ® ) as well as liver ultrasonography or abdominal computed tomography were included. Thirty-nine participants underwent liver ultrasonography and 11 underwent computed tomography. The physical measurements, blood test results, presence of metabolic syndrome, and the degree of liver steatosis and liver fibrosis were analyzed, and their correlations with transient elastography (Fibroscan ® ), abdominal computed tomography, and liver ultrasonography, as well as the correlations between examinations, were analyzed. @*Results@#Thirty-six participants (78.3%) were boys, and the mean age was 12.29±2.57 years, with a mean body mass index of 27.88±4.28. In the 46 participants, the mean values for aspartate aminotransferase, alanine aminotransferase, and total bilirubin were 89.87±118.69 IU/L, 138.54±141.79 IU/L, and 0.77±0.61 mg/dL, respectively. Although transient elastography (Fibroscan ® ) and abdominal computed tomography grading had a statistically significant positive correlation with aspartate aminotransferase and alanine aminotransferase values, the correlations between the results of grading performed by transient elastography (Fibroscan ® ), abdominal computed tomography, and liver ultrasonography were not statistically. @*Conclusion@#We confirmed that each examination was correlated with the results of some blood tests, suggesting the usefulness and possibility of diagnosis and treatment of steatohepatitis mediated by transient elastography (Fibroscan ® ) in the department of pediatrics.

Saccharomyces boulardii and Lactulose for Childhood Functional Constipation: A Multicenter Randomized Controlled Trial

Background/Aims@#The effects of probiotics in children vary based on diseases and probiotic strains. We aim to investigate the effectiveness of Saccharomyces boulardii and lactulose for treating childhood functional constipation. @*Methods@#This open-label randomized controlled trial was conducted at 10 university hospitals in Korea. Children who were diagnosed with functional constipation were allocated to 3 groups (lactulose monotherapy, combination therapy, and S. boulardii monotherapy). The primary outcome was treatment success rate that was accordingly defined as ≥ 3 bowel movements without incontinence at week 12. The cumulative successful maintenance and drug maintenance rates without drug changes were calculated throughout the study period. We compared stool frequency, incontinence, consistency, and painful defecation at week 2 among the 3 groups. @*Results@#Overall, 187 children were assigned to the lactulose monotherapy (n = 69), combination therapy (n = 68), or S. boulardii monotherapy (n = 50) groups. The primary outcome was significantly higher in the lactulose monotherapy group (26.1%) or combination therapy group (41.2%) than in the S. boulardii monotherapy group (8.0%). The S. boulardii monotherapy group showed a significantly lower cumulative successful maintenance and drug maintenance rate than the other 2 groups. There were no significant intergroup differences in the frequency of defecation, incontinence, painful defecation, or stool consistency during the follow-up at week 2. @*Conclusion@#S. boulardii monotherapy was not superior to lactulose monotherapy or combination therapy and showed a higher drug change rate, supporting the current recommendation of probiotics in the treatment of childhood functional constipation.

Evaluation of methods for fitness of removable partial denture / 北京大学学报(医学版)

OBJECTIVE@#To compare the differences and indications of three evaluation methods for fitness evaluation of removable partial denture (RPD).@*METHODS@#A RPD was fabricated and seated on the stone cast of a partially edentulous mandible, and the spaces between RPD and stone cast were recorded with polyvinyl siloxane (PVS) impression material forming PVS replicas. Using cross sectional measurement, the average thicknesses of PVS replicas were measured under stereomicroscope with different numbers of selected measuring points in the denture base, major connector, occlusal rest of the RPD, and the average thicknesses of the PVS replicas measured with different numbers of measuring points were compared using one-way analysis of variance (ANOVA) and independent sample t test. Three kinds of method, including cross sectional measurement, three-dimensional analysis on the stone cast, and three-dimensional analysis on the polyether cast, were applied to measure the average thicknesses of the PVS replicas, and the average thicknesses of the PVS replicas measured by these three evaluation methods were compared with ANOVA.@*RESULTS@#For cross sectional measurement, statistically significant differences were found in the average thicknesses of the PVS replicas in the denture base and the major connector among the different numbers of measuring points (P < 0.05), but no differences were found in the average thicknesses of the PVS replicas in the occlusal rest (P>0.05). There were significant differences among the average thicknesses of the PVS replicas measured by these three evaluation methods in each component of the RPD (P < 0.01). The average thickness measured by three-dimensional analysis on the stone cast and three-dimensional analysis on polyether cast were smaller than that measured by cross sectional measurement (P < 0.05). And there were no differences between the average thicknesses of PVS replicas measured by three-dimensional analysis on stone cast and three-dimensional analysis on polyether cast (P>0.05).@*CONCLUSION@#For cross sectional measurement, the average thickness of the PVS replicas was influenced by the number of measuring points, and the measurement accuracy of cross sectional measurement was not reliable enough. Three-dimensional analysis on stone cast which is suitable for evaluation in vitro and three-dimensional analysis on polyether cast which is suitable for evaluation in vivo can evaluate the fitness of RPD more comprehensively and effectively than that of cross sectional measurement.

Mortality Rate and Major Causes of Death by Gestational Age in Korean Children under 5 Years of Age

Background@#Preterm birth is associated with increased infant mortality. However, it is not clear whether prematurity is associated with mortality after 1 year of age. There is a lack of research on mortality rate and causes of death after infancy in preterm babies in Korea. We aimed to analyze the mortality rates and causes of deaths up to 5 years of age in Korea. @*Methods@#Using the Microdata Integrated Service of Statistics Korea database, this retrospective cohort study screened infants born between 2010 and 2012. After applying the exclusion criteria, 1,422,913 live births were classified into the following groups by gestational age: those born at < 32 weeks' gestation (n = 10,411), those born between 32 and 36 weeks' gestation (n = 75,657), and those born at ≥ 37 weeks' gestation (n = 1,336,845). The association of gestational age with mortality in infancy (< 1 year of age) and childhood (1–5 years of age) was analyzed, with and without covariates. The major causes of death in infancy and childhood were analyzed by gestational age. @*Results@#Overall, 4,930 (0.3%) children died between birth and 5 years of age, with 19.1% of these deaths occurring after infancy. Adjusted hazard ratios (HRs) for infant death were 78.79 (95% confidence interval [CI], 71.33–87.04) and 4.62 (95% CI, 4.07–5.24) for the < 32 and 32–36 weeks groups, respectively, compared to the full-term group; the adjusted HRs for deaths occurring at ages 1–5 years were 9.25 (95% CI, 6.85–12.50) and 2.42 (95% CI, 1.95–3.01), respectively. In infancy, conditions originating in the perinatal period were the most common cause of deaths in the < 32 and 32–36 weeks groups (88.7% and 41.9%, respectively). Contrarily, in the ≥ 37 weeks group, conditions originating in the perinatal period explained 22.7% of infant deaths, with congenital malformations primarily accounting for 29.6% of these deaths. The most common cause of death in children (after infancy) in the < 32 weeks group was perinatal causes (25.0%); in the 32–36 weeks group, congenital malformation and nervous system disease were the common causes (21.7% and 19.1%, respectively). In the ≥ 37 weeks group, injury, poisoning, and other consequences of external causes explained 26.6% of childhood deaths, followed by neoplasms and nervous system disease (15.7% and 14.7%, respectively). @*Conclusion@#Low gestational age is associated with not only infant mortality but also child mortality. The major causes of death differed by gestational age in infancy and childhood.For the care of preterm infants, especially those born at < 32 weeks' gestation, particular attention and continuous monitoring are needed in consideration of the major causes of deaths until 5 years of age.

14q12q13.3 Deletion Diagnosed Using Chromosomal Microarray Analysis in an Infant Showing Seizures, Hypoplasia of the Corpus Callosum, and Developmental Delay

14q12q13.3 Deletion is a rare microdeletion syndrome associated with neurodevelopmental delay, failure to thrive, seizures, and abnormal brain development. Symptoms vary depending on the sites of gene deletion, and establishing the diagnosis is often difficult, as the condition cannot be detected with routine chromosome analysis. In this report, we present a patient with intrauterine growth retardation, microcephaly, muscle weakness, seizures, and hypoplasia of the corpus callosum who underwent diagnostic tests, including karyotyping in the neonatal period without leading to a specific diagnosis. The patient was confirmed with a serious developmental disorder, and a chromosomal microarray analysis was performed at 8 months of age, revealing a 14q12q13.3 deletion. In this case, the condition was diagnosed in early infancy, in contrast to previously reported cases, and the patient had diverse and severe symptoms. Establishing the diagnosis of 14q12q13.3 deletion syndrome allows better management of patient care and genetic counseling for the parents.

14q12q13.3 Deletion Diagnosed Using Chromosomal Microarray Analysis in an Infant Showing Seizures, Hypoplasia of the Corpus Callosum, and Developmental Delay

14q12q13.3 Deletion is a rare microdeletion syndrome associated with neurodevelopmental delay, failure to thrive, seizures, and abnormal brain development. Symptoms vary depending on the sites of gene deletion, and establishing the diagnosis is often difficult, as the condition cannot be detected with routine chromosome analysis. In this report, we present a patient with intrauterine growth retardation, microcephaly, muscle weakness, seizures, and hypoplasia of the corpus callosum who underwent diagnostic tests, including karyotyping in the neonatal period without leading to a specific diagnosis. The patient was confirmed with a serious developmental disorder, and a chromosomal microarray analysis was performed at 8 months of age, revealing a 14q12q13.3 deletion. In this case, the condition was diagnosed in early infancy, in contrast to previously reported cases, and the patient had diverse and severe symptoms. Establishing the diagnosis of 14q12q13.3 deletion syndrome allows better management of patient care and genetic counseling for the parents.

Juvenile Polyp associated with Hypovolemic Shock Due to Massive Lower Gastrointestinal Bleeding / 대한소아소화기영양학회지

Juvenile polyps are the most common types of polyps in children, and patients usually present with lower gastrointestinal (GI) bleeding as the predominant symptom. These lesions, which are referred to as hamartomas, usually measure approximately 2 cm in size and are benign tumors located mainly in the rectum and sigmoid colon. The most common symptom of a juvenile polyp is mild intermittent rectal bleeding. It is rare for anemic patients because the amount of blood loss is small and often not diagnosed immediately. We present the case of a 6-year-old girl with a juvenile polyp in the distal transverse colon, who developed hypovolemic shock due to massive lower GI bleeding. Pediatricians must perform colonoscopy for thorough evaluation of polyps, because their location and size can vary and they can cause massive bleeding.

Usefulness of Transient Elastography for Non-Invasive Diagnosis of Liver Fibrosis in Pediatric Non-Alcoholic Steatohepatitis

BACKGROUND: Transient elastography (FibroScan®) is a non-invasive and rapid method for assessing liver fibrosis. While the feasibility and usefulness of FibroScan® have been proven in adults, few studies have focused on pediatric populations. We aimed to determine the feasibility and usefulness of FibroScan® in Korean children. METHODS: FibroScan® examinations were performed in 106 children (age, 5–15 years) who visited the Konyang University Hospital between June and September 2018. Liver steatosis was measured in terms of the controlled attenuation parameter (CAP), while hepatic fibrosis was evaluated in terms of the liver stiffness measurement (LSM). Children were stratified into obese and non-obese controls, according to body mass index (≥ or 95% percentile) (P 5.5 kPa), whereas the remaining 29 did not (LSM < 5.5 kPa). Obese children with fibrosis had higher levels of AST (73.57 ± 56.00 vs. 39.86 ± 31.93 IU/L; P = 0.009), ALT (132.47 ± 113.88 vs. 48.66 ± 51.29 IU/L; P = 0.001), and gamma-glutamyl transferase (106.67 ± 69.31 vs. 28.80 ± 24.26 IU/L; P = 0.042) compared to obese children without fibrosis. LSM had high and significant correlation (P < 0.05) with AST, ALT, homeostasis model assessment for insulin resistance, and AST-to-platelet ratio index. CONCLUSION: FibroScan® is clinically feasible and facilitates non-invasive, rapid, reproducible, and reliable detection of hepatic steatosis and liver fibrosis in the Korean pediatric population.

Changes in the Occurrence of Rotavirus Gastroenteritis before and after the Introduction of Rotavirus Vaccine among Hospitalized Pediatric Patients and Estimates of Rotavirus Vaccine Effectiveness

PURPOSE: This study aimed to investigate the changes in the occurrence of rotavirus gastroenteritis (RGE) after the introduction of rotavirus vaccine and estimate rotavirus vaccine effectiveness in hospitalized children. METHODS: We compared the retrospective data of 671 patients with acute gastroenteritis (AGE) admitted to the Department of Pediatrics, Hanyang University Seoul Hospital from January 1, 2014, to December 31, 2015, with retrospective data of 1,243 patients admitted to the same institution with AGE from January 1, 2004, to December 31, 2005. The vaccine effectiveness was estimated using a case-positive control test-negative study. RESULTS: The proportion of RGE in AGE was significantly lower in 2014 to 2015 (9.0%, 48/531) than in 2004 to 2005 (22.7%, 282/1,243) (P <0.001). In particular, there was a significant decrease in the 6- to 11-, 12- to 23-, and 24- to 35-month-old groups (P <0.001), whose rotavirus vaccination rates were higher than the remaining age groups. The monthly distribution of patients with RGE in 2004 to 2005 was higher from November to May, peaked in January, followed by December and February. In 2014 to 2015, the monthly distribution of patients with RGE slightly peaked in January. In 2014 to 2015 study, the complete rotavirus vaccination rate was 66.0% (332/503) and incomplete vaccination rate was 6.2% (31/503). Presumed rotavirus vaccine effectiveness was 83.3% (95% confidence interval [CI], 60.5% to 92.9%) in the complete vaccination group and 27.4% (95% CI, –163.7% to 80.0%) in the incomplete group. CONCLUSIONS: The proportion of RGE in AGE has markedly decreased since the introduction of rotavirus vaccines, and improving vaccination coverage will further reduce the burden of RGE in Korea.

The improvement of right ventricular function after adenotonsillectomy in children with obstructive sleep apnea / 소아과

PURPOSE: Adenotonsillar hypertrophy (ATH) that causes upper airway obstruction might lead to chronic hypoxemic pulmonary vasoconstriction and right ventricular (RV) dysfunction. We aimed to evaluate whether adenotonsillectomy (T&A) in children suffering from obstructive sleep apnea (OSA) due to severe ATH could improve RV function. METHODS: Thirty-seven children (boy:girl=21:16; mean age, 9.52±2.20 years), who underwent T&A forsleep apnea due to ATH, were included. We analyzedthe mean pulmonary artery pressure (mPAP), the presence and the maximal velocity of tricuspid regurgitation (TR), the tricuspid annular plane systolic excursion (TAPSE), and the right ventricular myocardial performance index (RVMPI) with tissue Doppler echocardiography (TDE) by transthoracic echocardiography pre- and post-T&A. The follow-up period was 1.78±0.27 years. RESULTS: Only the RVMPI using TDE improved after T&A (42.18±2.03 vs. 40±1.86, P=0.001). The absolute value of TAPSE increased (21.45±0.90 mm vs. 22.30±1.10 mm, P=0.001) but there was no change in the z score of TAPSE pre- and post-T&A (1.19±0.34 vs. 1.24±0.30, P=0.194). The mPAP was within normal range in children with ATH, and there was no significant difference between pre- and post-T&A (19.6±3.40 vs. 18.7±2.68, P=0.052). There was no difference in the presence and the maximal velocity of TR (P=0.058). CONCLUSION: RVMPI using TDE could be an early parameter of RV function in children with OSA due to ATH.

Clinical manifestation of Campylobacter enteritis in children / 소아과

PURPOSE: Timely antibiotic therapy in selected cases of diarrhea associated with bacterial infections can reduce the duration and severity of illness and prevent complications. The availability of a predictive index before identification of causative bacteria would aid in the choice of a therapeutic agent. METHODS: The study included patients admitted to the pediatrics unit at Konyang University Hospital for acute inflammatory diarrhea from August 1, 2015 to July 31, 2016 who underwent multiplex polymerase chain reaction testing. Of 248 patients, 83 had positive results. The clinical symptoms and blood test results were examined in 61 patients with Campylobacter spp. (25 patients), Salmonella spp. (18 patients), and Clostridium perfringens (18 patients) infections. The mean age of the 61 patients (male:femal=31:30) was 84.0±54.8 months, and the mean hospital stay was 4.6±1.7 days. RESULTS: There were no statistical differences in sex, age, clinical symptoms, or signs. Patients with Campylobacter infection were significantly older (P=0.00). C-reactive protein (CRP) levels in patients with Campylobacter infection were higher than those in the other 2 groups, at 9.6±6.1 mg/dL. The results of receiver-operating characteristic curve analysis showed that the cutoff age was ≥103.5 months (sensitivity, 72%; specificity, 86%) and the CRP cutoff level was ≥4.55 mg/dL (sensitivity, 80%; specificity, 69%). CONCLUSION: Age (≥103.5 months) and higher CRP level (≥4.55 mg/dL) were good predictors of Campylobacter enterocolitis. If neither criterion was met, Campylobacter enterocolitis was unlikely (negative predictive value 97.2%). When both criteria were met, Campylobacter enterocolitis was highly likely.

Sensitization to component antigens in acute idiopathic urticaria in children

PURPOSE: The aim of this study was to evaluate hidden allergens of acute idiopathic urticaria (AIU) in childhood by using the component-resolved diagnostics (CRD). METHODS: We applied CRD using pathogenesis-related protein family number 10 (PR-10) and nonspecific lipid transfer proteins (nsLTP). RESULTS: Twenty-two of the 74 AIU children (29.7%) were found to be positive on CRD. Ten children were positive to nMal d 1 for apple (value range, 1.10–40.59), 6 to rConr a 1 for hazelnut (1.53–11.97), 4 to rPru p 1 for peach (1.32–11.83). 6 to rAra h 8 for peanut (1.20–8.12), 6 to nAct d 8 for kiwi (0.85–3.32), 4 to rBet v 1 for birch (2.49–54.28), and 3 to rAln g 1 for alder (2.32–5.74). Six children were positive to nPru p 3 for peach (1.45–18.77), 4 to rCor a 8 for hazelnut (2.56–9.19), 2 to nArt v 3 for mugwort (3.40–7.42), and 3 to rBet v2 to profilin of birch (2.56–17.46). Ten children with AIU were positive to multiple component proteins. For hazelnut, 5 children were positive to PR-10 (rConr a 1) and nsLTP (rConr a 1). For peach, 3 children were positive to PR-10 (rPru p 1) and nsLTP (nPru p 3). CONCLUSION: IgE sensitization to PR-10 or nsLTP may be allergen components for AIU in childhood.

Trends in Fetal and Perinatal Mortality in Korea (2009–2014): Comparison with Japan and the United States

Fetal death is an important indicator of national health care. In Korea, the fetal mortality rate is likely to increase due to advanced maternal age and multiple births, but there is limited research in this field. The authors investigated the characteristics of fetal deaths, the annual changes in the fetal mortality rate and the perinatal mortality rate in Korea, and compared them with those in Japan and the United States. Fetal deaths were restricted to those that occurred at 20 weeks of gestation or more. From 2009 to 2014, the overall mean fetal mortality rate was 8.5 per 1,000 live births and fetal deaths in Korea, 7.1 in Japan and 6.0 in the United States. While the birth rate in Korea declined by 2.1% between 2009 and 2014, the decrease in the number of fetal deaths was 34.5%. The fetal mortality rate in Korea declined by 32.9%, from 11.0 in 2009 to 7.4 in 2014, the largest decline among the 3 countries. In addition, rates for receiving prenatal care increased from 53.9% in 2009 to 75.0% in 2014. Perinatal mortality rate I and II were the lowest in Japan, followed by Korea and the United States, and Korea showed the greatest decrease in rate of perinatal mortality rate II. In this study, we identified that the indices of fetal deaths in Korea are improving rapidly. In order to maintain this trend, improvement of perinatal care level and stronger national medical support policies should be maintained continuously.

Pathologic Impact of Insulin Resistance and Sensitivity on the Severity of Liver Histopathology in Pediatric Non-Alcoholic Steatohepatitis

PURPOSE: Insulin resistance (IR) has an important role in the development of non-alcoholic steatohepatitis (NASH). We aimed to analyze the association between liver histopathology and IR in pediatric patients with NASH. MATERIALS AND METHODS: In 24 children with non-alcoholic fatty liver disease (NAFLD), we investigated whether the hepatic pathologic characteristics have relations with following three biochemical indices; IR index including homeostasis model assessment of IR (HOMA-IR), quantitative insulin sensitivity check index (QUICKI), and insulin sensitivity indices-free fatty acid (ISI-FFA). RESULTS: Among 24 patients, 16 (66.6%) had a high NAFLD activity score (NAS), which is diagnostic of NASH. Higher serum triglyceride level was significantly correlated with a high NAS. Higher steatosis grades were significantly associated with low insulin sensitivity (p=0.023). In addition, severe lobular inflammation was associated with higher IR: HOMA-IR (p=0.014) and QUICKI (p=0.023). Severe fibrosis correlated with low insulin sensitivity and high IR indexes: ISI-FFA (p=0.049), HOMA-IR (p=0.028), and QUICKI (p=0.007). CONCLUSION: Patients with high IR had more severe lobular inflammation and hepatic fibrosis. Analyses of biochemical and endocrine parameters can be applied to determine the severity of the hepatic pathologic status in patients with NASH, especially in children who cannot undergo a liver biopsy.

Gastrointestinal Mucormycosis in Extremely Low Birth Weight Infants Mimicking Atypical Necrotizing Enterocolitis and Intussusception

Neonatal gastrointestinal mucormycosis, a rare disease with a high mortality rate, shows a rapid progressive course in premature infants with an immature immune system. We report the case of a male neonate weighing 970 g, delivered via cesarean section at 27 weeks, as one of a pair of dizygotic twins. From the 7(th) day after birth, bile was seen to drain through the orogastric tube, and paralytic ileus was noted on performing an abdominal X-ray. Thus, oral feeding was discontinued because necrotizing enterocolitis (NEC) was highly suspected. On the 9(th) day after birth, a firm mass was palpable in left upper abdominal quadrant, but no pneumatosis intestinalis was observed on performing abdominal X-ray. Small bowel intussusception was suspected on performing abdominal ultrasonography. Based on these findings, an exploratory laparotomy was performed, and although no intussusception was found intraoperatively, we performed a partial gastrectomy and hemicolectomy due to the presence of necrotic changes and perforations of the stomach and colon. Postoperatively, he was observed to have hypotension with persistence of hemorrhage at the surgical site. He died on the 11(th) day after birth. Intraoperative histopathological examination of stomach and colon showed fungal aseptate hyphae with broad branching. Gastrointestinal mucormycosis was confirmed based on findings of vascular involvement in the form of fungal hyphae and thrombosis in the transmural blood vessels. We report a case of an extremely low birth weight infant with neonatal gastrointestinal mucormycosis with an initial clinical presentation suggestive of intussusception and atypical NEC.

Interstitial deletion of 5q33.3q35.1 in a boy with severe mental retardation / 소아과

Constitutional interstitial deletions of the long arm of chromosome 5 (5q) are quite rare, and the corresponding phenotype is not yet clearly delineated. Severe mental retardation has been described in most patients who present 5q deletions. Specifically, the interstitial deletion of chromosome 5q33.3q35.1, an extremely rare chromosomal aberration, is characterized by mental retardation, developmental delay, and facial dysmorphism. Although the severity of mental retardation varies across cases, it is the most common feature described in patients who present the 5q33.3q35.1 deletion. Here, we report a case of a de novo deletion of 5q33.3q35.1, 46,XY,del(5)(q33.3q35.1) in an 11-year-old boy with mental retardation; to the best of our knowledge this is the first case in Korea to be reported. He was diagnosed with severe mental retardation, developmental delay, facial dysmorphisms, dental anomalies, and epilepsy. Chromosomal microarray analysis using the comparative genomic hybridization array method revealed a 16-Mb-long deletion of 5q33. 3q35.1(156,409,412-172,584,708)x1. Understanding this deletion may help draw a rough phenotypic map of 5q and correlate the phenotypes with specific chromosomal regions. The 5q33.3q35.1 deletion is a rare condition; however, accurate diagnosis of the associated mental retardation is important to ensure proper genetic counseling and to guide patients as part of long-term management.

P wave dispersion on 12-lead electrocardiography in adolescents with neurocardiogenic syncope / 소아과

PURPOSE: Neurocardiogenic syncope (NCS) is the most frequent cause of fainting during adolescence. Inappropriate cardiovascular autonomic control may be responsible for this clinical event. The head-up tilt test has been considered a diagnostic standard, but it is cumbersome and has a high false-positive rate. We performed a study to evaluate whether P-wave dispersion (PWD) could be a useful electrocardiographic parameter of cardiac autonomic dysfunction in children with NCS. METHODS: Fifty-four patients with NCS (28 boys and 26 girls; mean age, 12.3±1.4 years) and 55 age- and sex-matched healthy controls were enrolled. PWD was obtained as the difference between maximum and minimum durations of the P wave on standard 12-lead electrocardiography in all patients and controls RESULTS: The value of PWD was significantly higher in the syncope group than in the control group (69.7±19.6 msec vs. 45.5±17.1 msec, respectively; P<0.001). The minimum duration of P wave was shorter in the syncope group than in the control group (43.8±16.8 msec vs. 53.5±10.7 msec, respectively; P<0.001). Left atrial volume was not different between the groups on transthoracic echocardiography. CONCLUSION: PWD on echocardiography could be used as a clinical parameter in patients with NCS.

Granular cell tumor of the esophagus in an adolescent / 소아과

Esophageal granular cell tumor (GCT) is a rare neoplasm originating from the Schwann cells of the submucosal neuronal plexus. Histology is the gold standard for its diagnosis. Endoscopic resection or surgical excision should be considered, depending on the potential for malignancy. Here, we report a case of an esophageal GCT in an adolescent. A 12-year-old boy presented with a 1-year history of dysphagia and vomiting. Upper gastrointestinal endoscopic examination and esophagography showed narrowing of the midesophagus, and computed tomography angiography of the thoracic aorta revealed an esophageal or periesophageal mass posterior to the paratracheal segment of the esophagus. The tumor was surgically excised, and based on the pathological findings, esophageal GCT was diagnosed.

Age-adjusted plasma N-terminal pro-brain natriuretic peptide level in Kawasaki disease / 소아과

PURPOSE: Recent reports showed that plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) could be a useful biomarker of intravenous immunoglobulin (IVIG) unresponsiveness and coronary artery lesion (CAL) development in Kawasaki disease (KD). The levels of these peptides are critically influenced by age; hence, the normal range and upper limits for infants and children are different. We performed an age-adjusted analysis of plasma NT-proBNP level to validate its clinical use in the diagnosis of KD. METHODS: The data of 131 patients with KD were retrospectively analyzed. The patients were divided into 2 groups—group I (high NT-proBNP group) and group II (normal NT-proBNP group)—comprising patients with NT-proBNP concentrations higher and lower than the 95th percentile of the reference value, respectively. We compared the laboratory data, responsiveness to IVIG, and the risk of CAL in both groups. RESULTS: Group I showed significantly higher white blood cell count, absolute neutrophil count, C-reactive protein level, aspartate aminotransferase level, and troponin-I level than group II (P<0.05). The risk of CAL was also significantly higher in group I (odds ratio, 5.78; P=0.012). IVIG unresponsiveness in group I was three times that in group II (odds ratio, 3.35; P=0.005). CONCLUSION: Age-adjusted analysis of plasma NT-proBNP level could be helpful in predicting IVIG unresponsiveness and risk of CAL development in patients with KD.