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Review Article
Genetics and Metabolism
Neurofibromatosis type I: points to be considered by general pediatricians
Eungu Kang, Hee Mang Yoon, Beom Hee Lee
Clin Exp Pediatr. 2021;64(4):149-156.   Published online July 15, 2020
Neurofibromatosis type 1 (NF1), a prevalent genetic disease that is transmitted in an autosomal dominant manner, is characterized by multiple cutaneous café-au-lait spots and neurofibromas as well as various degrees of neurological, skeletal, and neoplastic manifestations. The clinical features of NF1 increase in frequency with age, while the clinical diagnosis can remain undetermined in some pediatric patients. Importantly, affected patients...
Review article
Immunology
Systematic review of literature and analysis of big data from the National Health Insurance System on primary immunodeficiencies in Korea
Sohee Son, Ji-Man Kang, Younsoo Hahn, Kangmo Ahn, Yae-Jean Kim
Clin Exp Pediatr. 2021;64(4):141-148.   Published online July 13, 2020
In this article, a systematic review of reported primary immunodeficiency disease (PID) cases in Korea was performed and we attempted to estimate the number of PID patients and healthcare costs for the first time in Korea. Our review revealed that Korean PID cases are greatly underreported in the literature based on health insurance. Physicians in the field and health care policymakers should be aware of the disease burden of PID.
Perspective
Other
Role of the Korea National Institute of Health in the children’s health effect study of particulate matter
Young Youl Kim
Clin Exp Pediatr. 2021;64(4):139-140.   Published online November 17, 2020
Particulate matter (PM) adversely affects children’s lungs and cognitive development, and an early exposure to it can lead to chronic metabolic diseases. The Korea National Institute of Health convened a Task Force on the Health Impact of PM to produce scientific evidence and promote technological developments to prevent PM-induced health effects.
Clinical Note
Neonatology (Perinatology)
Secondary renal tubular dysgenesis in a newborn exposed to angiotensin Ⅱ receptor antagonist during gestation
Mi Hyeon Gang, Yong Wook Lee, Mea-young Chang
Clin Exp Pediatr. 2021;64(3):136-138.   Published online July 21, 2020
Question: The use of renin-angiotensin system (RAS) blockers during pregnancy causes lethal neonatal complications.
Finding: A neonate, whose mother took a RAS blocker during pregnancy, was diagnosed with renal tubular dysgenesis. He presented with respiratory failure, severe refractory hypotension, and anuria requiring continuous dialysis.
Meaning: Physicians should consider any degree of RAS fetopathy in a newborn born to a hypertensive mother who had use of RAS blockers during her pregnancy.
Original Article
General Pediatrics
Significance of the tethered maxillary frenulum: a questionnaire-based observational cohort study
Sody A. Naimer, Ariel Israel, Aviezer Gabbay
Clin Exp Pediatr. 2021;64(3):130-135.   Published online September 7, 2020
Question: Does a tethered maxillary frenulum in the newborn result in breastfeeding difficulty or other oral symptomatology?
Finding: The analysis of subjects with a tethered maxillary frenulum surveyed beyond a mean 5-year follow-up did not reveal an increase in oral issues versus those of a random agematched control group.
Meaning: These data demonstrate no need to intervene upon the diagnosis of a tethered maxillary frenulum.
Critical Care Medicine
Can central venous access device care bundles and regular feedback reduce central line-associated complications in pediatric patients?
Chanapai Chaiyakulsil, Onsuthi Pharadornuwat
Clin Exp Pediatr. 2021;64(3):123-129.   Published online July 14, 2020
Question: Can central line bundles and feedback reduce central line-associated complications in pediatric patients?
Finding: The central line-related bloodstream infection rate decreased from 10.0 catheter-days to 1.4/1,000 catheter-days at 6-month postintervention. The central line occlusion rate was also decreased.
Meaning: Reinforcing central line care bundles with direct feedback can significantly decrease central line-associated complications in pediatric patients.
Editorial
Nephrology (Genitourinary)
Inhibition of the renin-angiotensin system during fetal kidney development
Eujin Park
Clin Exp Pediatr. 2021;64(3):121-122.   Published online September 22, 2020
· Fetal exposure to renin-angiotensin system (RAS) inhibitors leads to short- and long-term kidney complications.
· Women of reproductive age who are absolutely indicated for RAS inhibitors should be adequately informed of the risks for the duration of treatment.
Infection
Catheter care bundle and feedback to prevent central line-associated bloodstream infections in pediatric patients
Hye-Kyung Cho
Clin Exp Pediatr. 2021;64(3):119-120.   Published online December 8, 2020
• Intravascular catheter-related infection is an important cause of morbidity and mortality in children, and care bundles are effective and cost-saving in pediatric and neonatal patients.
• Providing regular feedbacks to critical care practitioners is helpful to maintain compliance to care bundle.
• Establishing a bundle policy (insertion and maintenance), monitoring compliance, and providing regular feedbacks are necessary for prevention of central line-associated bloodstream infections in pediatric patients.
Other
Is correcting exposure misclassification bias an additional option in meta-analyses?
Ki Eun Kim
Clin Exp Pediatr. 2021;64(3):117-118.   Published online January 14, 2021
Systematic reviews and meta-analyses examine various existing research results. Such studies are conducted according to a technically determined algorithm to minimize errors. It is particularly important to understand basic analytical methods such as the fixed-effect and random-effects models and apply appropriate statistical techniques to verify interstudy heterogeneity. A design that eliminates possible bias from the early stages of the research in a step-by-step manner is required whenever possible.
Review Article
Other
Behavioral insomnia in infants and young children
Eun Kyeong Kang, Seung Soo Kim
Clin Exp Pediatr. 2021;64(3):111-116.   Published online July 15, 2020
• Behavioral insomnia is common and affects a child’s daytime functioning and emotions.
• Diagnosing insomnia using the child’s sleep history, a sleep diary/log, and sleep questionnaires is important.
• Behavioral intervention, the main treatment, involves creating positive associations with sleep, establishing a consistent sleep schedule and bedtime routines, and the development of selfsoothing skills.
Neurology
Myelin oligodendrocyte glycoprotein antibody-associated disorders: clinical spectrum, diagnostic evaluation, and treatment options
Yun-Jin Lee, Sang Ook Nam, Ara Ko, JuHyun Kong, Shin Yun Byun
Clin Exp Pediatr. 2021;64(3):103-110.   Published online May 14, 2020
MOG antibody-associated disorder exhibits different pathophysiological and phenotypic findings than both aquaporin-4 antibody-associated neuromyelitis optica spectrum disorder and typical MS. MOG-antibody is of particular interest in pediatric patients with clinical or radiological non-MS typical findings. MOG-antibody was included in a diagnostic algorithm for children recommending for the first time a standardized use in clinical practice except in cases of typical MS.
General Pediatrics
Association between maternal smoking during pregnancy and risk of bone fractures in offspring: a systematic review and meta-analysis
Erfan Ayubi, Saeid Safiri, Kamyar Mansori
Clin Exp Pediatr. 2021;64(3):96-102.   Published online February 5, 2020
Question: What is the effect of maternal smoking during pregnancy (MSDP) on the risk of bone fractures in the offspring?
Finding: After the adjustment for misclassification, MSDP may be associated with a 27% increased risk of bone fracture in the offspring (pooled odds ratio, 1.27; 95% confidence interval, 1.00–1.62; I2=0%; P=0.537)
Meaning: Preventive measures and health education programs should be designed and implemented to encourage women to stop smoking, especially during.
Clinical note
Immunology
Salivary and lacrimal gland enlargement with serum immunoglobulin G4 elevation
Jinseok Lee, Da-Eun Kim, Jeong-Hwan Yang, Misun Lim, Hye Yung Yum, JoongGon Kim
Clin Exp Pediatr. 2021;64(2):93-95.   Published online December 8, 2020
Question: What diseases should be considered in a 15-year-old girl presenting enlarged lacrimal and salivary glands?
Finding: Laboratory tests revealed elevated IgG4 levels. A submandibular gland biopsy showed lymphoplasmacytic infiltration and more than 40 immunoglobulin G4 (IgG4)-positive cells per high-power field. The patient was diagnosed with IgG4-related disease.
Meaning: Although rare in children, IgG4-related disease should be considered a differential diagnosis in children with enlarged salivary and lacrimal glands.
Original Article
Hematology
Changes in the prevalence of anemia in Korean adolescents, 1998–2018
Jun Young An, Yoo Rha Hong, Seom Gim Kong
Clin Exp Pediatr. 2021;64(2):86-92.   Published online November 16, 2020
Question: Over the past 21 years, has the prevalence of anemia decreased among Korean adolescents?
Finding: The prevalence of anemia in boys aged 10–18 years decreased from 3.0% to 0.5% over the study period, whereas that in girls did not change significantly over time (increased from 7.9% to 8.5%).
Meaning: The prevalence of anemia in female adolescents remains high, requiring attention and efforts to improve it.
Gastroenterology
Value of the International Classification of Diseases code for identifying children with biliary atresia
Pornthep Tanpowpong, Chatmanee Lertudomphonwanit, Pornpimon Phuapradit, Suporn Treepongkaruna
Clin Exp Pediatr. 2021;64(2):80-85.   Published online August 24, 2020
Question: What is the value of the diagnostic code in identifying cases of biliary atresia in a large administrative database?
Finding: The diagnostic code’s accuracy and sensitivity are acceptable for identifying algorithm-defined cases. A history of pale stool and a presumed diagnosis of biliary atresia prior to referral added value.
Meaning: The addition of clinical data to the diagnostic code significantly increased the diagnostic yield.
Editorial
Hematology
Time to pay attention to anemia in female adolescents
Jae Min Lee
Clin Exp Pediatr. 2021;64(2):78-79.   Published online February 1, 2021
Cardiology
Where should we start to improve pediatric heart transplantation outcomes?
Jinyoung Song
Clin Exp Pediatr. 2021;64(2):76-77.   Published online November 25, 2020
· Pediatric heart transplantation is achieving better outcomes.
· The waitlist mortality is still high, especially in Korea, and should be reduced.
· The use of ventricular assist device is promising and might offer improved waitlist mortality rates.
Review Article
General Pediatrics
Clinical features, diagnosis, and outcomes of multisystem inflammatory syndrome in children associated with coronavirus disease 2019
Ji Hee Kwak, Soo-Young Lee, Jong-Woon Choi; the Korean Society of Kawasaki Disease
Clin Exp Pediatr. 2021;64(2):68-75.   Published online December 30, 2020
Hundreds of cases of children and adolescents with hyperinflammatory responses such as Kawasaki disease have been reported amid the coronavirus disease 2019 (COVID-19) pandemic, leading to coining of the new term COVID-19–associated multisystem inflammatory syndrome in children. In this review article, we introduce the illness and describe its case definitions, epidemiology, pathogenesis, clinical features, treatments, and outcomes.
Allergy
Eosinophils and childhood asthma
Bong Seok Choi
Clin Exp Pediatr. 2021;64(2):60-67.   Published online January 6, 2021
•In allergic eosinophilic asthma, eosinophils act as important effector cells and antigen-presenting cells, while in nonallergic eosinophilic asthma, type 2 innate lymphoid cells play an important role in eosinophil activation.
•Sputum eosinophil counts can be helpful for evaluating allergic airway inflammation in asthma.
• Anti-interleukin-5 has broadened the scope of asthma treatment.
Cardiology
Pediatric heart transplantation: how to manage problems affecting long-term outcomes?
Young Hwue Kim
Clin Exp Pediatr. 2021;64(2):49-59.   Published online April 8, 2020
Since the initial International Society of Heart Lung Transplantation registry was published in 1982, the number of pediatric heart transplantations has increased markedly, reaching a steady state of 500–550 transplantation annually and occupying up to 10% of total heart transplantations. Heart transplantation is considered an established therapeutic option for patients with end-stage heart disease. The long-term outcomes of pediatric heart...
Perspective
Infection
Addressing children’s health amid the coronavirus disease 2019 pandemic
Young June Choe, Bilal Aurang Zeb Durrani, Stefan Swartling Peterson
Clin Exp Pediatr. 2021;64(2):46-48.   Published online December 15, 2020
In the coronavirus disease 2019 (COVID-19) pandemic, children experience heightened barriers to health and protective services. Children may also be disproportionately affected due to their reliance on the education system for daily tasks and lack of access to remote learning options. Republic of Korea findings on how vaccination coverage could be sustained in children and schools could be reopened without aggravating COVID-19 underlie the need for coordinated efforts across sectors.
Letter to the Editor
Neonatology (Perinatology)
Role of neutrophil CD11b expression in diagnosis of earlyonset neonatal sepsis in full-term infant
Safaa ELMeneza, Walaa Mohamed, Iman Elbagoury, Karima Bahagat
Clin Exp Pediatr. 2021;64(1):44-45.   Published online April 14, 2020
Question: Can CD11b detect sepsis in full-term infants with suspected sepsis?
Finding: The percentage of neutrophils expressing CD11b was significantly upregulated in the sepsis and suspected sepsis groups versus the control group.
Meaning: CD11b is a sensitive marker for sepsis and suspected sepsis in full-term neonates and it may be added to sepsis markers. This information would allow the neonatologist to confidently discontinue antibiotic use as long as the neonate is clinically stable.
Original Article
Neonatology (Perinatology)
The protective effect of CXC chemokine receptor 2 antagonist on experimental bronchopulmonary dysplasia induced by postnatal systemic inflammation
Seung Hyun Lee, Chang Won Choi
Clin Exp Pediatr. 2021;64(1):37-43.   Published online July 15, 2020
Question: Can CXC chemokine receptor 2 (CXCR2) antagonist preserve alveolarization by attenuating the inflammation induced by systemic lipopolysaccharide (LPS) administration in a rat model of bronchopulmonary dysplasia (BPD)?
Finding: CXCR2 antagonist significantly decreased neutrophil counts in bronchoalveolar lavage fluid and peripheral blood induced by systemic LPS administration and restored alveolarization in newborn rats.
Meaning: CXCR2 antagonist protected the lungs from the inflammation in a rat model of BPD.
Gastroenterology
Noninvasive markers for esophageal varices in children with cirrhosis
Parisa Rahmani, Fatemeh Farahmand, Ghobad Heidari, Azadeh Sayarifard
Clin Exp Pediatr. 2021;64(1):31-36.   Published online July 21, 2020
Question: Can noninvasive biomarkers identify esophageal varices among children with esophageal cirrhosis?
Finding: The spleen size, platelet count, international normalized ratio, aspartate aminotransferase to platelet ratio index, platelet count to spleen size ratio, and risk score differed significantly between the patients with and those without esophageal varices.
Meaning: These biological parameters can predict esophageal varices among pediatric patients and indicate the need for esophagogastroduodenoscopy.
Editorial
Neonatology (Perinatology)
Neutrophil CD11b as a promising marker for early detection of neonatal sepsis
Ju Sun Heo
Clin Exp Pediatr. 2021;64(1):28-30.   Published online September 1, 2020
· Neonatal sepsis is a global problem and significant cause of neonatal mortality and adverse short- and long-term outcomes.
· Due to severe limitations diagnosing neonatal sepsis, there is a critical need to identify reliable specific biomarkers for early detection.
· nCD11b might be an accurate and rapid biomarker for the early detection of neonatal sepsis.
General Pediatrics
Why should we be concerned about early menarche?
Hae Sang Lee
Clin Exp Pediatr. 2021;64(1):26-27.   Published online July 13, 2020
· Early menarche is associated with several health problems in later life.
· Early menarche can be caused by environmental factors such as increased subcutaneous fat, a high body mass index, and sugar-sweetened beverages as well as genetic factors.
· Health education can prevent early menarche by aiming to reduce the consumption of fructose, high concentrations of which are present in sugar-sweetened beverages.
Review Article
Gastroenterology
Changing prevalence of Helicobacter pylori infection in children and adolescents
Ji Sook Park, Jin Su Jun, Ji-Hyun Seo, Hee-Shang Youn, Kwang-Ho Rhee
Clin Exp Pediatr. 2021;64(1):21-25.   Published online July 15, 2020
Although Helicobacter pylori infection rate in children is unclear due to diversity and limitation of diagnostic tests unlike in adults, investigation the childhood prevalence is important for predicting H. pylori-related diseases in the future.
H. pylori infection occurred in early childhood, and declined during 30 years in our study.
Change in risk factors of H. pylori transmission and consensus for eradication therapy in children might further reduce the infection rate.
Endocrinology
Early menarche and its consequence in Korean female: reducing fructose intake could be one solution
Ji Hyun Kim, Jung Sub Lim
Clin Exp Pediatr. 2021;64(1):12-20.   Published online May 14, 2020
In Korea, the average age of menarche has declined sharply. Early menarche is associated with psychosocial and behavioral problems and cardiometabolic disease. Excess fructose intake has been suggested as one cause of early menarche in recent studies, so reducing fructose intake may be one solution.
Other
Review of epidemiological studies on air pollution and health effects in children
Jong-Tae Lee
Clin Exp Pediatr. 2021;64(1):3-11.   Published online June 10, 2020
This review summarized the accumulated epidemiologic evidence with emphasis on studies conducted in Korea and heterogeneity in the literature. Based on systematic reviews and meta-analyses, there is consistent evidence on the association between exposure to ambient air pollution and children’s health, especially respiratory health and adverse birth outcomes, and growing evidence on neurodevelopmental outcomes.
Perspective
Neurology
How can pediatricians treat neurodevelopmental disorders
Young-Hoon Kim
Clin Exp Pediatr. 2021;64(1):1-2.   Published online July 27, 2020
∙ Recently neurodevelopmental therapy for preschool-aged children with neurodevelopmental disorders is paid for by health insurance in Korea.
∙ There are good evidences that parenting programs and neurodevelopmental therapy can work in attention deficit hyperactivity disorder and autism spectrum disorder.
∙ Pediatricians must be able to pass away important information to parents.