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Original Article
Polysomnographic features of children with obesity: body mass index predict severe obstructive sleep apnea in obese children?
Rungrat Sukharom, Prakarn Tovichien, Kanokporn Udomittipong, Pinyapach Tiamduangtawan, Wattanachai Chotinaiwattarakul
Background: Few studies have explored the polysomnographic features of children with obesity.
Purpose: This study aimed to explore the demographic and polysomnographic features of obese children and determine whether body mass index (BMI) could predict severe obstructive sleep apnea (OSA). Methods: This cross-sectional study recruited obese children who underwent diagnostic polysomnography between January 2019 and March 2022. We explored demographic and...
Review Article
Endocrinology
Lifelong medical challenges and immunogenetics of Turner syndrome
Won Kyoung Cho
Clin Exp Pediatr. 2024;67(11):560-568.   Published online July 31, 2024
· This summary emphasizes the importance of the early diagnosis of Turner syndrome (TS) and presents a multidisciplinary approach to its prevention and management, high-lighting the need for customized care.
· Advancements in immunogenetic research may improve our understanding of TS and improve its outcomes.
· TS encompasses a wide array of medical challenges, including cardiovascular, endocrine, autoimmune, and mental health issues, as well as a heightened cancer risk.
General Pediatrics
Metabolic complications of obesity in children and adolescents
Hyunjin Park, Jung Eun Choi, Seunghee Jun, Hyelim Lee, Hae Soon Kim, Hye Ah Lee, Hyesook Park
Clin Exp Pediatr. 2024;67(7):347-355.   Published online November 16, 2023
· Pediatric obesity increases the risk of metabolic complications (insulin resistance, dyslipidemia, nonalcoholic fatty liver disease) and long-term cardiovascular diseases.
· A new obesity definition and various indicators (continuous metabolic syndrome score, pediatric simple metabolic syndrome score, fatty liver index) have been proposed to evaluate children’s susceptibility to metabolic disorders.
· Laboratory and body composition tests in pediatric screenings can identify groups at high risk of metabolic complications of obesity.
Nephrology (Genitourinary)
Kidney complications associated with COVID-19 infection and vaccination in children and adolescents: a brief review
Hee Sun Baek, Min Hyun Cho
Clin Exp Pediatr. 2023;66(10):424-431.   Published online June 28, 2023
Several observational studies have shown that acute kidney injury affects up to 46% of children and adolescents who develop severe postinflammatory responses, such as multisystem inflammatory syndrome in childhood, due to coronavirus disease 2019 (COVID-19). Although causality has not been established, some cases of glomerulopathy or nephrotic syndrome occurring after COVID-19 infection or vaccination have been reported. Therefore, kidney complications associated with these conditions in children and adolescents warrant attention.
Pulmonology
Long COVID in children and adolescents: prevalence, clinical manifestations, and management strategies
Eun Kyo Ha, Ju Hee Kim, Man Yong Han
Clin Exp Pediatr. 2023;66(11):465-474.   Published online June 19, 2023
· Current definitions of long coronavirus disease (COVID) in children and adolescents vary in duration, ranging from 4 to 12 weeks or more.
· The clinical spectrum of long COVID in children and adolescents comprises a wide range of symptoms and might be a multisystem disorder.
· Persistent health problems with a prevalence of 1%–66.5% were reported in children and adolescents after COVID-19, with a higher incidence of persistent single or multiple symptoms.
Original Article
Gastroenterology
Relationship between nonalcoholic fatty liver disease and hyperandrogenemia in adolescents with polycystic ovary syndrome
Ozlem Kara, Hanife Aysegul Arsoy, Murat Keskin
Clin Exp Pediatr. 2023;66(9):395-402.   Published online June 14, 2023
Question: Is polycystic ovary syndrome (PCOS) a risk factor for nonalcoholic fatty liver disease (NAFLD) in adolescents?
Finding: The frequency of NAFLD did not increase in adolescents with PCOS. However, hyperandrogenemia was a risk factor for NAFLD.
Meaning: Adolescents with PCOS and hyperandrogenemia should be closely monitored for hepatic steatosis.
Review Article
Infection
Etiological and pathophysiological enigmas of severe coronavirus disease 2019, multisystem inflammatory syndrome in children, and Kawasaki disease
Jung-Woo Rhim, Jin-Han Kang, Kyung-Yil Lee
Clin Exp Pediatr. 2022;65(4):153-166.   Published online November 23, 2021
· Severe cases of coronavirus disease, Kawasaki disease (KD), and multisystem inflammatory syndrome in children (MIS-C) share similar findings: a protracted clinical course, multiorgan involvement, and similar activated biomarkers.
· Here we propose etiological agents in KD and MIS-C as species in the microbiota and introduce a common pathogenesis through the protein-homeostasis-system hypothesis.
· Early proper dose of corticosteroids and/or intravenous immunoglobulin may help to reduce morbidity and mortality in these diseases.
Original Article
Endocrinology
Effects of probiotics combined with dietary and lifestyle modification on clinical, biochemical, and radiological parameters in obese children with nonalcoholic fatty liver disease/nonalcoholic steatohepatitis: a randomized clinical trial
Thushara Rodrigo, Samaranayake Dulani, Sumudu Nimali Seneviratne, Arjuna P. De Silva, Jerad Fernando, H. Janaka De Silva, Jayasekera , V. Pujitha Wickramasinghe
Clin Exp Pediatr. 2022;65(6):304-311.   Published online November 11, 2021
Question: Could probiotics be used as a therapeutic modality in nonalcoholic fatty liver disease/nonalcoholic steatohepatitis?
Finding: There seem no added advantages over lifestyle modifications compared to Probiotics.
Meaning: There does not seem to be an advantage of probiotics over lifestyle modifications in improving obesity-associated metabolic derangement in children.
Other
Clinical spectrum and short-term outcomes of multisystem inflammatory syndrome in children in a south Indian hospital
Muruganantham Balagurunathan, Thrilok Natarajan, Jothilakshmi Karthikeyan, Venkateshwaran Palanisamy
Clin Exp Pediatr. 2021;64(10):531-537.   Published online August 4, 2021
Question: What are the clinical spectrum, course, and short-term outcomes of multisystem inflammatory syndrome in children (MIS-C)?
Finding: MIS-C can have variable clinical manifestations. Fever is most common, followed by gastrointestinal and cardiovascular symptoms. Early identification and appropriate management lead to favorable outcomes.
Meaning: MIS-C can present in a myriad of ways and severities. High suspicion is necessary to ensure its early identification and appropriate management and favorable patient outcomes.
Neonatology (Perinatology)
Comparison of minimally invasive surfactant therapy with intubation surfactant administration and extubation for treating preterm infants with respiratory distress syndrome: a randomized clinical trial
Mohammad Kazem Sabzehei, Behnaz Basiri, Maryam Shokouhi, Sajad Ghahremani, Ali Moradi
Clin Exp Pediatr. 2022;65(4):188-193.   Published online July 28, 2021
Question: Are the short-term outcomes of minimally invasive surfactant therapy (MIST) relatively superior to those of INtubation, SURfactant administration, and Extubation (INSURE) in preterm infants with respiratory distress syndrome (RDS)?
Finding: MIST could be an appropriate substitution for INSURE in preterm infants with RDS since it reduced hospitalization time and number of side effects.
Meaning: MIST is recommended for surfactant administration for its proven advantages over the INSURE technique.
Review Article
Hematology
Thrombosis and severe acute respiratory syndrome coronavirus 2 vaccines: vaccine-induced immune thrombotic thrombocytopenia
Young Shil Park
Clin Exp Pediatr. 2021;64(8):400-405.   Published online June 30, 2021
· Thrombosis and thrombocytopenia occurring within 4–28 days after severe acute respiratory syndrome coronavirus 2 vaccination require attention.
· The terms vaccine-induced immune thrombotic thrombocytopenia (VITT) and thrombosis with thrombocytopenia syndrome (TTS) are used.
· VITT is pathogenetically similar to heparin-induced thrombocytopenia.
· VITT/TTS could be associated with the development of platelet-activating anti-platelet factor 4 antibodies.
· For suspected VITT/TTS, early treatment decisions (intravenous immunoglobulin, non-heparin anticoagulant, and avoidance of platelet transfusions) are important.
Original Article
General Pediatrics
Gross motor dysfunction and balance impairments in children and adolescents with Down syndrome: a systematic review
Preyal D. Jain, Akshatha Nayak, Shreekanth D. Karnad, Kaiorisa N. Doctor
Clin Exp Pediatr. 2022;65(3):142-149.   Published online June 11, 2021
Question: What are the primary motor and balance dysfunctions in children with Down syndrome?
Finding: These individuals have gross delays, altered balance, and inefficient compensatory mechanisms.
Meaning: Neuromuscular and musculoskeletal impairments due to the chromosomal abnormality lead to developmental delay. These children also exhibit poor balance with greater instability and inefficient compensatory mechanisms including altered center of pressure displacement and trunk stiffening that predisposes them to falls.
Endocrinology
Association between polycystic ovary syndrome and risk of attention-deficit/hyperactivity disorder in offspring: a meta-analysis
Azam Maleki, Saeid Bashirian, Ali Reza Soltanian, Ensiyeh Jenabi, Abdollah Farhadinasab
Clin Exp Pediatr. 2022;65(2):85-89.   Published online April 15, 2021
Question: Have polycystic ovary syndrome (PCOS) increased risk of having an offspring with attention-deficit/hyperactivity disorder (ADHD)?
Finding: Six articles (3 cohort and 3 case-control studies; 401,413 total ADHD cases) met the study criteria. Maternal PCOS was associated with an increased risk of ADHD in the offspring based on odds ratio (OR) and relative ratio (RR) (OR, 1.42; 95% confidence interval [CI], 1.27–1.57) and (RR, 1.43; 95% CI, 1.35–1.51), respectively.
Meaning: Our study showed that maternal PCOS is a risk factor for ADHD.
Review Article
Immunology
Modern diagnostic capabilities of neonatal screening for primary immunodeficiencies in newborns
Evgenia Olegovna Khalturina, Natalia Dmitrievna Degtyareva, Anastasiia Vasi’evna Bairashevskaia, Alena Valerievna Mulenkova, Anna Vladimirovna Degtyareva
Clin Exp Pediatr. 2021;64(10):504-510.   Published online March 25, 2021
· Neonatal screening for primary immunodeficiency diseases (PIDs) enables early diagnosis and subsequent initiation of therapy.
· Excision of T-cell receptors and kappa-recombination excision circles are cheaper alternative PID screening methods.
· Sanger DNA sequencing remains the reference method for detecting PID; however, next-generation sequencing technology is increasingly used to diagnose it.
· Here we developed a graphical algorithm for diagnosing primary immunodeficiency syndrome based on modern methods of screening for primary immunodeficiencies in newborns.
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.
Original Article
Nephrology (Genitourinary)
Expression profiling of cultured podocytes exposed to nephrotic plasma reveals intrinsic molecular signatures of nephrotic syndrome
Stuti Panigrahi, Varsha Chhotusing Pardeshi, Karthikeyan Chandrasekaran, Karthik Neelakandan, Hari PS, Anil Vasudevan
Clin Exp Pediatr. 2021;64(7):355-363.   Published online November 1, 2020
Question: Is it possible to classify nephrotic syndrome (NS) through gene expression profiling of podocytes exposed to NS plasma?
Finding: Our data showed different expression profiles in podocytes exposed to nephrotic plasma from different clinical groups, suggesting the molecular stratification of patients into intrinsic subtypes.
Meaning: Transcriptome profiling of podocytes treated with NS plasma can stratify patients into intrinsic subtypes and provide insight into the molecular mechanisms of podocyte injury.
Review Article
Hematology
Genetic classification and confirmation of inherited platelet disorders: current status in Korea
Ye Jee Shim
Clin Exp Pediatr. 2020;63(3):79-87.   Published online February 6, 2020
Inherited platelet disorders (IPDs), which manifest as primary hemostasis defects, often underlie abnormal bleeding and a family history of thrombocytopenia, bone marrow failure, hematologic malignancies, undefined mucocutaneous bleeding disorder, or congenital bony defects. Wide heterogeneity in IPD types with regard to the presence or absence of thrombocytopenia, platelet dysfunction, bone marrow failure, and dysmegakaryopoiesis is observed in patients. The individual...
Original Article
General Pediatrics
Prevalence of hyperuricemia and its association with metabolic syndrome and cardiometabolic risk factors in Korean children and adolescents: analysis based on the 2016–2017 Korea National Health and Nutrition Examination Survey
Jung Hyun Lee
Clin Exp Pediatr. 2019;62(8):317-323.   Published online June 24, 2019

Purpose: Investigating the prevalence of hyperuricemia and its association with metabolic syndrome (MetS) and cardiometabolic risk factors (CMRFs) in Korean children and adolescents. Methods: This cross-sectional survey used data from the 7th Korea National Health and Nutrition Examination Survey (2016–2017); 1,256 males and females aged 10–18 years were included. Hyperuricemia was defined as serum uric acid levels were >6.6 mg/dL at...
Review Article
Neonatology (Perinatology)
Surfactant preparations for preterm infants with respiratory distress syndrome: past, present, and future
Ga Won Jeon
Clin Exp Pediatr. 2019;62(5):155-161.   Published online February 8, 2019
Following the first successful trial of surfactant replacement therapy for preterm infants with respiratory distress syndrome (RDS) by Fujiwara in 1980, several animal-derived natural surfactants and synthetic surfactants have been developed. Synthetic surfactants were designed to overcome limitations of natural surfactants such as cost, immune reactions, and infections elicited by animal proteins contained in natural surfactants. However, first-generation synthetic surfactants...
Original Article
Endocrinology
Comparison of effectiveness of growth hormone therapy according to disease-causing genes in children with Noonan syndrome
Kyo Jin Jo, Yoo Mi Kim, Ju Young Yoon, Yeoun Joo Lee, Young Mi Han, Han-Wook Yoo, Hyang-Sook Kim, Chong Kun Cheon
Clin Exp Pediatr. 2019;62(7):274-280.   Published online December 3, 2018

Purpose: To analyze the growth response to growth hormone (GH) therapy in prepubertal patients with Noonan syndrome (NS) harboring different genetic mutations. Methods: Twenty-three patients with prepubertal NS treated at Pusan National University Children’s Hospital between March 2009 and July 2017 were enrolled. According to the disease-causing genes identified, the patients with NS were divided into 4 groups. Three groups were...
Case Report
Nephrology (Genitourinary)
De novo mutations in COL4A5 identified by whole exome sequencing in 2 girls with Alport syndrome in Korea
Kyoung Hee Han, Jong Eun Park, Chang-Seok Ki
Clin Exp Pediatr. 2019;62(5):193-197.   Published online November 26, 2018
Alport syndrome (ATS) is an inherited glomerular disease caused by mutations in one of the type IV collagen novel chains (α3, α4, and α5). ATS is characterized by persistent microscopic hematuria that starts during infancy, eventually leading to either progressive nephritis or end-stage renal disease. There are 3 known genetic forms of ATS, namely X-linked ATS, autosomal recessive ATS, and...
Original Article
Neonatology (Perinatology)
Association between vitamin D level at birth and respiratory morbidities in very-low-birth-weight infants
Ian Kim, Sung Shin Kim, Jee In Song, Seock Hwa Yoon, Ga Young Park, Yong-Wha Lee
Clin Exp Pediatr. 2019;62(5):166-172.   Published online October 24, 2018

Purpose: This study aimed to evaluate vitamin D status at birth in very-low-birth-weight infants (VLBWIs: <1,500 g) and to determine the association between vitamin D level and respiratory morbidity. Methods: A retrospective study was conducted at Soonchunhyang University Bucheon Hospital between November 2013 and November 2017. We collected blood samples and data on respiratory morbidity from 230 VLBWIs on the first...
Nephrology (Genitourinary)
Changes in the thyroid hormone profiles in children with nephrotic syndrome
Sun Hee Jung, Jeong Eun Lee, Woo Yeong Chung
Clin Exp Pediatr. 2019;62(3):85-89.   Published online October 4, 2018

Purpose: We compared thyroid hormone profiles in children with nephrotic syndrome (NS) during the nephrotic phase and after remission. Methods: This study included 31 pediatric NS patients. The thyroid hormone profiles included serum levels of triiodothyronine (T3), thyroxine (T4), thyroid-stimulating hormone (TSH), and free T4. Results: Of the 31 patients, 16 (51.6%) showed abnormal thyroid hormone profiles: 6 had overt hypothyroidism, 8...
Case Report
Genetics and Metabolism
The first Korean case with Floating-Harbor syndrome with a novel SRCAP mutation diagnosed by targeted exome sequencing
Eun Mi Choi, Dong Hyun Lee, Seok Jin Kang, Ye Jee Shim, Heung Sik Kim, Jun Sik Kim, Jong In Jeong, Jung-Sook Ha, Ja-Hyun Jang
Clin Exp Pediatr. 2018;61(12):403-406.   Published online September 16, 2018
Floating-Harbor syndrome is a rare autosomal dominant genetic disorder associated with SRCAP mutation. To date, approximately 50 cases of Floating-Harbor syndrome have been reported, but none have been reported in Korea yet. Floating-Harbor syndrome is characterized by delayed bony maturation, unique facial features, and language impairment. Here, we present a 6-year-old boy with a triangular face, deep-set protruding eyes, low-set...
Original Article
Neonatology (Perinatology)
Transient intubation for surfactant administration in the treatment of respiratory distress syndrome in extremely premature infants
Ji Won Koh, Jong-Wan Kim, Young Pyo Chang
Clin Exp Pediatr. 2018;61(10):315-321.   Published online September 16, 2018

Purpose: To investigate the effectiveness of transient intubation for surfactant administration and extubated to nasal continuous positive pressure (INSURE) for treatment of respiratory distress syndrome (RDS) and to identify the factors associated with INSURE failure in extremely premature infants. Methods: Eighty-four infants with gestational age less than 28 weeks treated with surfactant administration for RDS for 8 years were included. Perinatal...
Cardiology
Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide
Jung Eun Choi, Yujin Kwak, Jung Won Huh, Eun-Sun Yoo, Kyung-Ha Ryu, Sejung Sohn, Young Mi Hong
Clin Exp Pediatr. 2018;61(5):167-173.   Published online May 28, 2018
Purpose

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone.

Methods

We performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared...

Case Report
Gastroenterology
Intestinal duplication revealed by posterior reversible encephalopathy syndrome
Yosra Kerkeni, Hela Louati, Mourad Hamzaoui
Clin Exp Pediatr. 2018;61(4):132-134.   Published online April 23, 2018

We report a unique case of intestinal duplication detected on posterior reversible encephalopathy syndrome (PRES) in a 13-year-old girl. She was admitted to the pediatric Emergency Department because of generalized seizures. Radiological assessment revealed a large, well-defined, thick-walled cystic lesion in the mid abdomen, suggestive of duplication cyst associated to a PRES. Exploration confirmed the diagnosis of ileal duplication cyst,...

Review Article
Nephrology (Genitourinary)
Atypical hemolytic uremic syndrome and eculizumab therapy in children
Seong Heon Kim, Hye Young Kim, Su Young Kim
Clin Exp Pediatr. 2018;61(2):37-42.   Published online February 28, 2018

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is...

Original Article
Cardiology
Subtle inflammation: a possible mechanism of future cardiovascular risk in obese children
Watchareewan Sontichai, Prapai Dejkhamron, Peraphan Pothacharoen, Prachya Kongtaweelert, Kevalee Unachak, Nuthapong Ukarapol
Clin Exp Pediatr. 2017;60(11):359-364.   Published online November 27, 2017
Purpose

The risk of cardiovascular disease (CVD) has been shown to be associated with systemic inflammation in obese adults with metabolic syndrome (MetS). The aims of this study were to evaluate the prevalence of MetS and its relation to inflammatory markers in obese Thai children.

Methods

A cross-sectional study was conducted. Children with history of endogenous obesity, chronic diseases, drug ingestion, and any...

Neurology
Serum neuron specific enolase is increased in pediatric acute encephalitis syndrome
Dian Pratamastuti, Prastiya Indra Gunawan, Darto Saharso
Clin Exp Pediatr. 2017;60(9):302-306.   Published online September 21, 2017
Purpose

This study aimed to investigate whether serum neuron-specific enolase (NSE) was expressed in acute encephalitis syndrome (AES) that causes neuronal damage in children.

Methods

This prospective observational study was conducted in the pediatric neurology ward of Soetomo Hospital. Cases of AES with ages ranging from 1 month to 12 years were included. Cases that were categorized as simple and complex febrile seizures...