• Clinical and Experimental Pediatrics
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• v.53 no.6
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• pp.711-717
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• 2010

Purpose: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. Methods: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. Results: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. Conclusion: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.

• Pediatric Infection and Vaccine
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• v.27 no.1
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• pp.24-34
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• 2020

The Korean Society of Pediatric Infectious Diseases and the Korea Centers for Disease Control and Prevention issued the guidelines about coronavirus disease 2019 (COVID-19) for children and adolescents. Case definitions and management of COVID-19 in neonates, infants, children and adolescents are presented in this guideline. In addition, guidelines for caregiver management are also provided. In this review, we introduce the contents of the current guidelines for COVID-19 in children and adolescents in Korea.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.75-82
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• 2020

Objectives: We aimed to investigate the incidence, manifestations, and outcomes of malignancy after pediatric kidney transplantation (KT) at our center over 30 years. Methods: We retrospectively reviewed the medical records of 155 patients under 18 years of age who underwent KT between January 1990 and February 2020 at Asan Medical Center. Results: Twelve patients (7.7%) were diagnosed with a malignancy after KT. Malignancy was diagnosed after a mean period of 6.4±5.9 years (median 4.6, range 0.5-20.6 years) after KT. Nine (75.0%) of the 12 cancer patients were diagnosed with post-transplant lymphoproliferative disease (PTLD), and the other three had papillary thyroid cancer, mucoepidermoid cancer of the hard palate, and T-cell acute lymphoblastic leukemia, respectively. PTLD was diagnosed within a mean of 3.7±3.4 years (median 3.7, range 0.5-9.8 years) after KT. Five patients diagnosed with PTLD were cured without recurrence. Three patients with PTLD died from the disease, and one patient with mucoepidermoid cancer from a non-PTLD malignancy died after progression, despite surgical resection and chemotherapy. Three (33.3%) of the nine survivors progressed to end-stage renal disease (ESRD) after completing cancer treatment. No patient with post-transplant malignancy (PTM) experienced critical renal deterioration during cancer treatment. Conclusion: PTLD was the most common PTM, occurring at 5.8% of the pediatric KT patients after KT in our center. Careful follow up is needed particularly considering the risk of PTLD after KT in children.

• Clinical and Experimental Pediatrics
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• v.62 no.1
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• pp.30-35
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• 2019

Purpose: Obesity is risk factor for nonalcoholic fatty liver disease (NAFLD). However, nonobese patients are also increasingly susceptible to NAFLD. The aim of this study was to compare the clinical characteristics of obese and nonobese pediatric patients with NAFLD. Methods: We retrospectively studied 68 patients who were diagnosed with NAFLD between January 2010 and October 2016 at 10-18 years of age. Body mass index ${\geq}95th$ percentile for age and sex was defined as obesity. Abdominal ultrasonography and laboratory, anthropometrics measurements were evaluated. Results: Among the 68, 26 (38.2%) were nonobese patients. The ratio of male to female was 5.8:1, and the median age at diagnosis was 13 years (range, 10-17 years). Significant higher triglyceride (223.0 mg/dL vs. 145.9 mg/dL, P=0.047) and total cholesterol levels (211.6 mg/dL vs. 173.2 mg/dL, P=0.011) were shown in nonobese than obese patients. High-density lipoprotein cholesterol level <40 mg/dL (hazard ratio [HR], 6.5; 95% confidence interval [CI], 2.13-7.10; P=0.048), total cholesterol level >200 mg/dL (HR, 5.6; 95% CI, 1.23-15.31; P=0.038) and abdominal obesity (HR, 2.53; 95% CI, 1.22-4.68; P=0.013) were significant risk factors for NAFLD in nonobese patients. Conclusion: Nonobese patients present a substantial proportion of pediatric NAFLD cases. Significant abnormal lipid concentrations were found in nonobese and abdominal obesity was important risk factor for nonobese NAFLD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.2
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• pp.109-120
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• 2022

Purpose: While regurgitation is a common and often benign phenomenon in infants and younger children, it can also be a presenting symptom of gastroesophageal reflux disease (GERD). If untreated, GERD can lead to dangerous or lifelong complications. Clinical practice guidelines (CPGs) have been published to inform clinical diagnosis and management of pediatric GERD, but to date there has been no comprehensive review of guideline quality or methodological rigor. Methods: A systematic literature search was performed, and a total of eight CPGs pertaining to pediatric GERD were identified. These CPGs were evaluated using the Appraisal of Guidelines for Research and Evaluation instrument. Results: Three CPGs were found to be "high" quality, with 5 of 6 domains scoring >60%, one "average" quality, with 4 of 6 domains meeting that threshold, and the remaining four "low" quality. Conclusion: Areas of strength among the CPGs included "Scope and Purpose" and "Clarity and Presentation," as they tended to be well-written and easily understood. Areas in need of improvement were "Stakeholder Involvement," "Rigor of Development," and "Applicability," suggesting these CPGs may not be appropriate for all patients or providers. This analysis found that while strong CPGs pertaining to the diagnosis and treatment of pediatric GERD exist, many published guidelines lack methodological rigor and broad applicability.

• Korean Journal of Radiology
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• v.22 no.11
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• pp.1886-1893
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• 2021

Objective: To assess the feasibility of quantitatively assessing pancreatic steatosis using magnetic resonance imaging (MRI) and its correlation with obesity and metabolic risk factors in pediatric patients. Materials and Methods: Pediatric patients (≤ 18 years) who underwent liver fat quantification MRI between January 2016 and June 2019 were retrospectively included and divided into the obesity and control groups. Pancreatic proton density fat fraction (P-PDFF) was measured as the average value for three circular regions of interest (ROIs) drawn in the pancreatic head, body, and tail. Age, weight, laboratory results, and mean liver MRI values including liver PDFF (L-PDFF), stiffness on MR elastography, and T2^* values were assessed for their correlation with P-PDFF using linear regression analysis. The associations between P-PDFF and metabolic risk factors, including obesity, hypertension, diabetes mellitus (DM), and dyslipidemia, were assessed using logistic regression analysis. Results: A total of 172 patients (male:female = 125:47; mean ± standard deviation [SD], 13.2 ± 3.1 years) were included. The mean P-PDFF was significantly higher in the obesity group than in the control group (mean ± SD, 4.2 ± 2.5% vs. 3.4 ± 2.4%; p = 0.037). L-PDFF and liver stiffness values showed no significant correlation with P-PDFF (p = 0.235 and p = 0.567, respectively). P-PDFF was significantly associated with obesity (odds ratio 1.146, 95% confidence interval 1.006-1.307, p = 0.041), but there was no significant association with hypertension, DM, and dyslipidemia. Conclusion: MRI can be used to quantitatively measure pancreatic steatosis in children. P-PDFF is significantly associated with obesity in pediatric patients.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.197-200
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• 2011

Respiratory syncytial virus (RSV) is a major cause of respiratory infection in children. Most of the pediatric population have RSV infection before the age of 2, and recurrent infections are common even within one season. Chronic lung disease, prematurity, along with congenital heart disease (CHD) are major risk factors in severe lower respiratory infection. In hemo-dynamically significant CHD patients with RSV infection, hospitalization is usually needed and the possibility of treatment in intensive care unit and the use of mechanical ventilator support are known to increase. Therefore the prevention of RSV infection in CHD patients is mandatory. The current standard for RSV prevention is immunoprophylaxis by palivizumab. Immunoprophylaxis is recommended monthly in hemodynamically significant CHD patients, up to 5 months. Motabizumab, a second generation drug and newly developing RSV vaccines are also expected to play a key role in RSV prevention in the future. The prophylaxis of RSV infection in CHD patients is cost-effective in both the medical aspect of the patients as well as the socio-economic aspect. Therefore an effort to promote prevention should be made by not only the family of the patients but also by the government.

• Childhood Kidney Diseases
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• v.25 no.2
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• pp.92-111
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• 2021

Purpose: Nephrotic syndrome (NS) is the most common form of glomerulopathy in children. Most pediatric patients respond to glucocorticosteroid treatment (steroid-sensitive NS, SSNS), while approximately 10-15% will remain unresponsive or later become steroid-resistant. There has been a long-standing effort to find biomarkers that may predict steroid responsiveness. Methods: We systematically reviewed current studies which investigated clinically relevant biomarkers for predicting steroid responsiveness in pediatric NS. We performed a PubMed and EMBASE search to identify eligible articles. We collected data on urinary markers, blood/serum markers (including cellular phenotypes and mRNA expression), genotypes and HLA allele frequency. Results: A total of 659 articles were identified following electronic and manual searches. After reviewing the titles, abstracts, and full texts, 72 eligible articles were finally included. Vitamin D-binding protein (VDBP) seemed to be significantly elevated in SRNS than in SSNS, in both serum and urine specimen, although further validation is required. Conclusions: The present paper narratively illustrates current understandings of potential biomarkers that may help predict steroid responsiveness. Further investigation and collaboration involving a larger number of patients are necessary.

• The Journal of Pediatrics of Korean Medicine
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• v.37 no.3
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• pp.17-34
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• 2023

Objectives Few studies to date reviewed herbal medicines prescribed to pediatric patients with coronavirus disease 2019 (COVID-19). We aimed to summarize and analyze these herbal medicines. Methods Nine databases were searched for studies published up until November 24, 2020. Nine case reports and one case series involving 17 pediatric patients were included in the study. The clinical symptoms, interventions, outcomes, pattern identification, composition, and frequency of herbal medicine administration were analyzed. Results The symptoms of pediatric patients with COVID-19 treated with herbal medicines were mild or moderate. Herbal medicines have been used to alleviate symptoms, such as fever and cough. No adverse events were reported. All of the patients who received herbal medicines were discharged from the hospital after symptomatic relief. Pattern identification revealed that the lungs were the most affected organs. Epidemic toxins, wind, dampness, and heat were the primary etiological factors. More pediatric patients with COVID-19 were prescribed a combination of herbal and Western medicines than herbal medicines alone. The frequently used herbs include Glycyrrhizae Radix et Rhizoma, Lonicerae Flos, Scutellariae Radix, Armeniacae Semen, Citri Reticulatae Pericarpium, and Astragali Radix. Conclusions Our review can serve as a reference for the treatment of COVID-19 in children.

• Clinical and Experimental Pediatrics
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• v.64 no.5
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• pp.225-226
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• 2021