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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.
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