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.

Trends and Characteristics of Mortality Associated with Congenital Anomalies in Korean Children under 5 Years of Age

Purpose@#Studies have been conducted on the prevalence and infant mortality rate of congenital anomalies; however, studies on child mortality are rare. Therefore, we evaluated the characteristics of deaths associated with congenital anomalies among children born in Korea who died within 5 years of age. @*Methods@#Birth-to-death cohort linked data of children under the age of 5 years from 2010 to 2013, and statistical data on the cause of death by age from 1999 to 2019, both provided by the Korea National Statistical Office's Microdata Integrated Service, were retrospectively investigated. We investigated the trends and characteristics of mortality associated with congenital anomalies. @*Results@#Among 1,858,945 children, 6,510 children who died were under 5 years of age, and among them, 1,229 deaths were associated with congenital anomalies, while 5,281 deaths were due to other causes. Deaths associated with congenital anomalies accounted for 18.9% of all deaths. When comparing congenital anomalies by systems, anomalies of the cardiovascular system (52.6%) were the most common. The mortality rate associated with congenital anomalies and those of other causes showed similar declining trends in 21 years. @*Conclusion@#The mortality rate of congenital anomalies during the first 5 years of life did not increase differently from the prevalence of congenital anomalies but rather decreased. Deaths associated with congenital anomalies accounted for 20.5% of all infant deaths and 12.1% of child deaths, since the major causes of death in infants and children are slightly different, continuous and careful monitoring is required.

Trends and Characteristics of Mortality Associated with Congenital Anomalies in Korean Children under 5 Years of Age

Purpose@#Studies have been conducted on the prevalence and infant mortality rate of congenital anomalies; however, studies on child mortality are rare. Therefore, we evaluated the characteristics of deaths associated with congenital anomalies among children born in Korea who died within 5 years of age. @*Methods@#Birth-to-death cohort linked data of children under the age of 5 years from 2010 to 2013, and statistical data on the cause of death by age from 1999 to 2019, both provided by the Korea National Statistical Office's Microdata Integrated Service, were retrospectively investigated. We investigated the trends and characteristics of mortality associated with congenital anomalies. @*Results@#Among 1,858,945 children, 6,510 children who died were under 5 years of age, and among them, 1,229 deaths were associated with congenital anomalies, while 5,281 deaths were due to other causes. Deaths associated with congenital anomalies accounted for 18.9% of all deaths. When comparing congenital anomalies by systems, anomalies of the cardiovascular system (52.6%) were the most common. The mortality rate associated with congenital anomalies and those of other causes showed similar declining trends in 21 years. @*Conclusion@#The mortality rate of congenital anomalies during the first 5 years of life did not increase differently from the prevalence of congenital anomalies but rather decreased. Deaths associated with congenital anomalies accounted for 20.5% of all infant deaths and 12.1% of child deaths, since the major causes of death in infants and children are slightly different, continuous and careful monitoring is required.

A Case Study on the Customized Nutrition Intervention for a Patient with Primary Gastrointestinal Non-Hodgkin Lymphoma Underlying Chronic Kidney Disease

Non-Hodgkin lymphoma comprises 2.1% of the total number of cancers in South Korea. Among those, diffuse large B cell lymphoma (DLBCL) comprises the largest percentage. Nutrition interventions have been highlighted because nutritional status in non-Hodgkin's lymphoma patients has a significant impact on treatment and prognosis, but relevant studies are inadequate. Therefore, the aim of this study was to share the case of a nutrition intervention for a patient with primary gastrointestinal non-Hodgkin lymphoma underlying chronic kidney disease who was comorbid with tumor lysis syndrome, which was a complication of a specific chemotherapy. The subject is a 76-year-old patient who was diagnosed with DLBCL. He had abdominal pain, constipation, and anorexia. After chemotherapy, he experienced the tumor lysis syndrome. The patient's condition was continuously monitored, and various nutrition interventions, such as nutrition counseling and education, provision of therapeutic diet, oral nutritional supplement, change of meal plans, and parenteral nutrition support were attempted. As a result of the nutrition intervention, oral intake was increased from 27% of the energy requirement to 70% and from 23% of the protein requirement to 77%. Despite the various nutrition interventions during the hospitalization, there were no improvements in weight and nutrition-related biochemical parameters or malnutrition. However, it was meaningful in that the patient was managed to prevent worsening and the planned third chemotherapy could be performed. These results can be used as the basis for establishing guidelines for nutritional interventions customized to patients under the same conditions.

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.

A Case Study on the Customized Nutrition Intervention for a Patient with Primary Gastrointestinal Non-Hodgkin Lymphoma Underlying Chronic Kidney Disease

Non-Hodgkin lymphoma comprises 2.1% of the total number of cancers in South Korea. Among those, diffuse large B cell lymphoma (DLBCL) comprises the largest percentage. Nutrition interventions have been highlighted because nutritional status in non-Hodgkin's lymphoma patients has a significant impact on treatment and prognosis, but relevant studies are inadequate. Therefore, the aim of this study was to share the case of a nutrition intervention for a patient with primary gastrointestinal non-Hodgkin lymphoma underlying chronic kidney disease who was comorbid with tumor lysis syndrome, which was a complication of a specific chemotherapy. The subject is a 76-year-old patient who was diagnosed with DLBCL. He had abdominal pain, constipation, and anorexia. After chemotherapy, he experienced the tumor lysis syndrome. The patient's condition was continuously monitored, and various nutrition interventions, such as nutrition counseling and education, provision of therapeutic diet, oral nutritional supplement, change of meal plans, and parenteral nutrition support were attempted. As a result of the nutrition intervention, oral intake was increased from 27% of the energy requirement to 70% and from 23% of the protein requirement to 77%. Despite the various nutrition interventions during the hospitalization, there were no improvements in weight and nutrition-related biochemical parameters or malnutrition. However, it was meaningful in that the patient was managed to prevent worsening and the planned third chemotherapy could be performed. These results can be used as the basis for establishing guidelines for nutritional interventions customized to patients under the same conditions.

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.

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.

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.

Thyroid Hormone Resistance in a Preterm Infant with a Novel THRB Mutation

Resistance to thyroid hormone (RTH) is a condition caused by a mutation in the thyroid hormone receptor gene. It is rarely reported in individuals with no family history of RTH or in premature infants, and its clinical presentation varies. In our case, a premature infant with no family history of thyroid diseases had a thyroid stimulating hormone level of 85.0 µIU/mL and free thyroxine level of 1.64 ng/dL on a thyroid function test. The patient also presented with clinical signs of hypothyroidism, including difficulties in feeding and weight gain. The patient was treated with levothyroxine; however, only free thyroxine and triiodothyronine levels increased without a decrease in thyroid-stimulating hormone levels. Taken together with thyroid gland hypertrophy observed on a previous ultrasound examination, RTH was suspected and the diagnosis was eventually made based on a genetic test. A de novo mutation in the thyroid hormone receptor β gene in the infant was found that has not been previously reported. Other symptoms included tachycardia and pulmonary hypertension, but gradual improvement in the symptoms was observed after liothyronine administration. This report describes a case involving a premature infant with RTH and a de novo mutation, with no family history of thyroid disease.

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.

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.

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.

Comparison of Birth Outcomes Based on Maternal Ethnicity in Korea: Korean, Vietnamese, and Chinese, 2010–2015

PURPOSE: The purpose of this study is to compare birth outcomes based on maternal ethnicity in Korea. METHODS: Using the birth data of Statistics Korea from 2010 to 2015, this study selected data from infants with a Vietnamese mother and Korean father (Vietnamese/Korean group), and a Chinese mother and Korean father (Chinese/Korean group), to compare them with that of a Korean mother and Korean father (Korean/Korean group). The newborn infants' birth outcomes and parental characteristics were investigated, and trends in annual changes were compared. In addition, this study investigated whether the mother's ethnicity affected the mean birth weight. RESULTS: Gestational age and birth weight were highest in the Chinese/Korean group, and were slightly lower in the Korean/Korean and Vietnamese/Korean group, in that order. The highest rate of preterm birth before 37 weeks and low birth weight rate were observed in the Vietnamese/Korean group; 4.62% and 4.26%, respectively. From 2010 to 2015, the mean gestational age decreased in all the three groups, and the preterm birth rate increased at gestational ages less than 37 weeks. However, the birth weight decreased only in the Korean/Korean group, but increased in the Chinese/Korean and Vietnamese/Korean groups. In addition, we found that factors such as parents' educational levels, percentage of hospital births, and appropriate maternal age improved in the Vietnamese/Korean group. CONCLUSION: This study confirms that the mean birth weight and low birth weight rate are affected by maternal ethnicity in Korea. Therefore, careful research and active national support policies are needed.

Bronchial Hyperresponsiveness to Methacholine and AMP in Children With Atopic Asthma

PURPOSE: Bronchial hyperresponsiveness (BHR) is typically measured by bronchial challenge tests that employ direct stimulation by methacholine or indirect stimulation by adenosine 5'-monophosphate (AMP). Some studies have shown that the AMP challenge test provides a better reflection of airway inflammation, but few studies have examined the relationship between the AMP and methacholine challenge tests in children with asthma. We investigated the relationship between AMP and methacholine testing in children and adolescents with atopic asthma. METHODS: The medical records of 130 children with atopic asthma (mean age, 10.63 years) were reviewed retrospectively. Methacholine and AMP test results, spirometry, skin prick test results, and blood tests for inflammatory markers (total IgE, eosinophils [total count, percent of white blood cells]) were analyzed. RESULTS: The concentration of AMP that induces a 20% decline in forced expiratory volume in 1 second [FEV1] (PC20) of methacholine correlated with the PC20 of AMP (r2=0.189, P<0.001). No significant differences were observed in the levels of inflammatory markers (total eosinophil count, eosinophil percentage, and total IgE) between groups that were positive and negative for BHR to methacholine. However, significant differences in inflammatory markers were observed in groups that were positive and negative for BHR to AMP (log total eosinophil count, P=0.023; log total IgE, P=0.020, eosinophil percentage, P<0.001). In contrast, body mass index (BMI) was significantly different in the methacholine positive and negative groups (P=0.027), but not in the AMP positive and negative groups (P=0.62). The PC20 of methacholine correlated with FEV1, FEV1/forced vital capacity (FVC), and maximum mid-expiratory flow (MMEF) (P=0.001, 0.011, 0.001, respectively), and the PC20 of AMP correlated with FEV1, FEV1/FVC, and MMEF (P=0.008, 0.046, 0.001, respectively). CONCLUSIONS: Our results suggest that the AMP and methacholine challenge test results correlated well with respect to determining BHR. The BHR to AMP more likely implicated airway inflammation in children with atopic asthma. In contrast, the BHR to methacholine was related to BMI.

Bronchiectasis in Children: 10-Year Experience at a Single Institution

PURPOSE: Bronchiectasis in children is still one of the most common causes of childhood mortality in developing countries. The aim of this study was to investigate the epidemiological characteristics, clinical features, underlying etiologic factors, and distinct change in the management of patients with bronchiectasis at Asan Medical Center Children's Hospital of Seoul. METHODS: A retrospective study of children diagnosed with bronchiectasis was conducted between January 1999 and December 2008. All patients underwent a comprehensive examination to identify etiologic factors. Data analysis in terms of age at onset, initial presenting symptoms, underlying etiology, distinct change in treatment, distribution of pulmonary involvement on computed tomography (CT), and causative microbiological flora triggering secondary infections was performed. RESULTS: The median age at the time of the diagnosis of bronchiectasis was 7.6 years (range, 2 months to 18 years). Persistent coughing was the most common symptom. The underlying etiologies identified in 79 patients (85.8%) included bronchiolitis obliterans (32.6%), childhood respiratory infection (20.6%), interstitial lung disease (17.3%), immunodeficiency (8.6%), and primary ciliary dyskinesia (4.3%). In 53 children (67%), the identified cause led to a distinct and individualized change in management. The distribution of CT abnormalities had no correlation with the underlying cause of bronchiectasis. CONCLUSIONS: Selected Korean children with bronchiectasis were reviewed to identify diverse underlying etiologies. All children with bronchiectasis should be comprehensively investigated because identifying underlying causes may have a major impact on their management and prognosis.

The Effects of Lactobacillus rhamnosus on the Prevention of Asthma in a Murine Model

PURPOSE: Lactobacilli are probiotic bacteria that are effective in the management of allergic diseases or gastroenteritis. It is hypothesized that such probiotics have immunoregulatory properties and promote mucosal tolerance. Our goal was to investigate whether Lactobacillus casei rhamnosus Lcr35 could inhibit airway inflammation in an ovalbumin (OVA)-induced murine model of asthma. METHODS: BALB/c mice aged 6 weeks were used in the present study. Lactobacillus casei rhamnosus Lcr35 was administered daily, starting 1 week prior to the first OVA sensitization (group 1) and 2 days before the first 1% OVA airway challenge (group 2). Mice that received only saline at both sensitization and airway challenge time points were used as negative controls (group 3), and those that had OVA-induced asthma were used as positive controls (group 4). Airway responsiveness to methacholine was assessed, and bronchoalveolar lavage (BAL) was performed. At the endpoint of the study, total IgE as well as OVA-specific IgE, IgG1 and IgG2a in serum was measured by enzyme-linked immunosorbent assay. Lung pathology was also evaluated. RESULTS: Airway hyperresponsiveness, total cell counts and the proportion of eosinophils in BAL fluid were significantly decreased in group 1 compared with group 4 (P<0.05). Total serum IgE levels were also significantly decreased in group 1 compared with group 4. Serum levels of OVA-specific IgE, IgG1 and IgG(2a) were not significantly influenced by treatment with Lcr35. There was significantly less peribronchial and perivascular infiltration of inflammatory cells in group 1 compared with group 4; however, there were no significant differences in methacholine challenge, BAL, serology or histology between groups 2 and 4. CONCLUSIONS: Oral treatment with Lcr35 prior to sensitization can attenuate airway inflammation and hyperreactivity in a mouse model of allergic airway inflammation. These results suggest that Lcr35 may have potential for preventing asthma.

Association study of polymorphism in leukotriene C4 synthase and cysteinyl leukotriene receptor 1 genes with phenotype of asthma and clinical parameters in Korean children / 소아과

PURPOSE: Cysteinyl leukotrienes are important proinflammatory mediators in asthma. Recently, it was suggested that a promoter polymorphism in the genes encoding for leukotriene C4 synthase (LTC4S), a key enzyme in the leukotriene synthetic pathway, and cysteinyl leukotriene receptor 1 (CysLTR1) might be associated with aspirin-intolerant asthma. We investigated whether polymorphisms in LTC4S and CysLTR1 genes or their interactions were associated with the asthma phenotype, lung function, or bronchial hyperreactivity (BHR) in Korean children. METHODS: A total of 856 asthmatic children and 254 non-asthmatic controls were enrolled; a skin prick test, lung function test and bronchial provocation test were performed. Of those enrolled, 395 children underwent exercise challenge tests. The LTC4S A(-444)C and CysLTR1 T(+927)C were genotyped by polymerase chain reaction-restriction fragment length polymorphism analysis. RESULTS: Of those enrolled, 699 children were classified as having atopic asthma and 277 children, as having exercise-induced asthma (EIA). LTC4S and CysLTR1 polymorphisms were not associated with atopic asthma, EIA, or asthma per se. Lung function and BHR were not significantly different between the wild type (AA or TT) and the variant (AC+CC or TC+CC) genotypes in asthmatics, atopic asthmatics, and EIA (+) asthmatics, while total eosinophil counts were higher in the variant type of LTC4S than in the wild type in atopic asthmatics. There were no associations between the gene-gene interactions of LTC4S and CysLTR1 genotypes and the asthma phenotypes. CONCLUSION: LTC4S A(-444)C and CysLTR1 T(+927)C polymorphisms and their gene-gene interactions are not associated with asthma phenotype, lung function, or BHR in Korean children.