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Review Article
Pulmonology
Evidence-based management guidelines for noncystic fibrosis bronchiectasis in children and adolescents
Eun Lee, Kyunghoon Kim, You Hoon Jeon, In Suk Sol, Jong Deok Kim, Taek Ki Min, Yoon Ha Hwang, Hyun-Ju Cho, Dong In Suh, Hwan Soo Kim, Yoon Hee Kim, Sung-Il Woo, Yong Ju Lee, Sungsu Jung, Hyeon-Jong Yang, Gwang Cheon Jang
Clin Exp Pediatr. 2024;67(9):418-426.   Published online January 23, 2024
· We suggest offering long-term macrolides to children with noncystic fibrosis bronchiectasis with frequent exacerbations (conditional recommendation, moderate quality of evidence).
· We do not recommend the routine use of mucolytic agents, inhaled corticosteroids, or nonsteroidal anti-inflammatory drugs to prevent exacerbation of bronchiectasis in children (inconclusive, very low quality of evidence).
· We recommend the use of nebulized hypertonic saline to prevent exacerbations and improve the lung function of children with noncystic fibrosis bronchiectasis (weak recommendation, moderate quality of evidence).
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.
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.
Original Article - Clinical trial
WITHDRAWN:Randomized controlled trial of effect of N-acetylcysteine as an antioxidant on iron overload in children with thalassemia major
Yasmen A Mohamed, Mohamed H Meabed, Amany Ashraf, Dalia S Morgan, Mostafa G Abdul Latif, Rehab M Abd-Elkareem, Heba M Ahmed
Background: β-Thalassemias are characterized by the presence of mutations in the globin gene that result in the absence or reduced synthesis of β-globin chains of the hemoglobin tetramer. Several studies have reported increased oxidative stress in β-thalassemia major (β-TM) patients. N-acetylcysteine (NAC), a derivative of L-cysteine amino acid, is commonly used as a mucolytic drug. Numerous studies have reported efficient...
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,...

Neonatology (Perinatology)
Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate
Juneyoug Koh, Euiseok Jung, Se Jin Jang, Dong Kwan Kim, Byong Sop Lee, Ki-Soo Kim, Ellen Ai-Rhan Kim
Clin Exp Pediatr. 2018;61(1):30-34.   Published online January 22, 2018

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS...

Original Article
Infection
Pneumocystis jirovecii pneumonia in pediatric patients: an analysis of 15 confirmed consecutive cases during 14 years
Kyung-Ran Kim, Jong Min Kim, Ji-Man Kang, Yae-Jean Kim
Clin Exp Pediatr. 2016;59(6):252-255.   Published online June 30, 2016
Purpose

Pneumocystis jirovecii pneumonia occurs in various immunocompromised patients. Despite the prophylaxis strategies in clinical practice, certain patients develop P. jirovecii pneumonia. This study was performed to investigate pediatric cases with P. jirovecii pneumonia in a single center.

Methods

We identified pediatric patients younger than 19 years with microbiologically confirmed P. jirovecii pneumonia from January 2000 to February 2014. A retrospective chart review...

Case Report
Gastroenterology
A giant choledochal cyst in infancy: a case report
Nursel Yurttutan, Suleyman Cuneyt Karakus, Naim Koku, Mustafa Demirci, Ramazan Ucak
Clin Exp Pediatr. 2016;59(5):239-241.   Published online May 31, 2016

Choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts. Postnatally, ultrasonography is the initial diagnostic modality of choice, allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge. Symptoms depend on the age at presentation. Common bile duct malformations should be considered as a...

Original Article
Usefulness of serum cystatin C to determine the dose of vancomycin in neonate
Jeong Eun Shin, Soon Min Lee, Ho Seon Eun, Min Soo Park, Kook In Park, Ran Namgung
Clin Exp Pediatr. 2015;58(11):421-426.   Published online November 22, 2015
Purpose

The vancomycin dosage regimen is regularly modified according to the patient's glomerular filtration rate (GFR). In the present study, we aimed to assess the usefulness of serum cystatin C (Cys-C) concentration, compared with serum creatinine (SCr) concentration, for predicting vancomycin clearance (CLvcm) in neonates.

Methods

We retrospectively analyzed the laboratory data of 50 term neonates who were admitted to the neonatal intensive...

Review Article
BK virus-associated hemorrhagic cystitis after pediatric stem cell transplantation
Seung Beom Han, Bin Cho, Jin Han Kang
Clin Exp Pediatr. 2014;57(12):514-519.   Published online December 31, 2014

Hemorrhagic cystitis is a common stem cell transplantation-related complication. The incidence of early-onset hemorrhagic cystitis, which is related to the pretransplant conditioning regimen, has decreased with the concomitant use of mesna and hyperhydration. However, late-onset hemorrhagic cystitis, which is usually caused by the BK virus, continues to develop. Although the BK virus is the most common pathogenic microorganism of poststem...

Case Report
Cystic fibrosis of pancreas and nephrotic syndrome: a rare association
Selvi Kelekçi, Müsemma Karabel, Aydın Ece, Velat Şen, Ali Güneş, İlyas Yolbaş, Cahit Şahin
Clin Exp Pediatr. 2013;56(10):456-458.   Published online October 31, 2013

Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy...

Original Article
Polymorphisms of methylenetetrahydrofolate reductase are not a risk factor for Kawasaki disease in the Korean population
Kyung Lim Yoon, Jin Hee Ko, Kye Shik Shim, Mi Young Han, Sung Ho Cha, Su Kang Kim, Joo Ho Jung
Clin Exp Pediatr. 2011;54(8):335-339.   Published online August 31, 2011
Purpose

Hyperhomocysteinemia is known as a risk factor for atherosclerosis. Preclinical arteriosclerosis is noted and premature atherosclerosis is known to be accelerated in Kawasaki disease (KD) patients. Genetic polymorphisms in the 5,10-methylenetetrahydrofolate reductase (MTHFR) gene result in elevated plasma homocysteine concentrations and are known to be associated with the development of coronary artery disease. Our hypothesis is that single nucleotide polymorphisms...

Risk factor for pituitary dysfunction in children and adolescents with Rathke's cleft cysts
Han Hyuk Lim, Sei Won Yang
Clin Exp Pediatr. 2010;53(7):759-765.   Published online July 31, 2010
Purpose

This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts.

Methods

Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain...

Analysis of reports on orphan lung diseases in Korean children
Sun Jung Jang, Hyun Kyung Seo, Sung Jae Yi, Kyong Min Kim, Hye Mi Jee, Man Yong Han
Clin Exp Pediatr. 2010;53(6):711-717.   Published online June 23, 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...

Spectrum of patients with hypermethioninemia based on neonatal screening tests over 14 years
Se Jung Oh, Yong Hee Hong, Yong Wha Lee, Dong Hwan Lee
Clin Exp Pediatr. 2010;53(3):329-334.   Published online March 15, 2010
Purpose : The neonatal screening test for homocystinuria primarily measures methionine by using a dried blood specimen. We investigated the incidence and clinical manifestations of homocystinuria, isolated hypermethioninemia, and transient hypermethioninemia among patients with hypermethioninemia on a neonatal screening test. Methods : We performed a retrospective study of 58 patients transferred to Shoonchunhyang Hospital because of hypermethioninemia on a neonatal...
Case Report
A case of postoperative nasopharyngeal reflux associated with retropharyngeal lymphangioma in newborn infant
Kyo Yeon Koo, Jun Seok Lee, Soon Min Lee, Min Soo Park, Ran Namgung, Kook In Park, Chul Lee, Choon Sik Yoon, Woo Hee Jung, Hong Shik Choi
Clin Exp Pediatr. 2010;53(2):258-261.   Published online February 15, 2010
Lymphangioma is a rare benign congenital tumor of the lymphatic system, which is commonly diagnosed before 2 years of age. In the natronal report, cystic lymphangioma was usually reported as a huge translucent mass located in the head and neck area. It's occurrence in retropharyngeal space with respiratory obstruction and swallowing difficulty in neonate is extremely rare and postoperative nasopharyngeal...
Original Article
Comparison of various methods of glomerular filtration rate measurements in children
Na Mi Lee, In Seok Lim
Clin Exp Pediatr. 2009;52(9):999-1004.   Published online September 15, 2009

Purpose:Glomerular filtration rate (GFR) is a fundamental parameter in assessing renal function and predicting the progression of chronic renal disease. Because the use of serum creatinine has several disadvantages, many studies have investigated the use of cystatin C for estimating GFR. We compared creatinine clearance and GFR with formulas using serum creatinine and cystatin C. Methods:We retrospectively analyzed 211 patients...
Association study of polymorphism in leukotriene C4 synthase and cysteinyl leukotriene receptor 1 genes with phenotype of asthma and clinical parameters in Korean children
Jung Yeon Shim, Byung-Joo Kim, Young Hwa Song, Mi-Jin Kang, So-Yeon Lee, Jinho Yu, Soo-Jong Hong
Clin Exp Pediatr. 2009;52(6):680-688.   Published online June 15, 2009
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...
Case Report
Intestinal obstruction caused by a duplication cyst of the cecum in a neonate
Seung-woon Keum, Min-Woo Hwang, Jong-In Na, Seung-taek Yu, Dong-Baek Kang, Yeon-Kyun Oh
Clin Exp Pediatr. 2009;52(2):261-264.   Published online February 15, 2009
Duplication cysts are rare congenital malformations, that may be detected anywhere along the alimentary tract, and they may communicate with the intestinal tract. Cystic duplication of the cecum is especially rare. About 80% of these cases are detected in the first 2 years of life as a result of an acute intestinal obstruction, which manifests as vomiting, recurrent abdominal pain,...
A case of megalencephalic leukoencephalopathy with subcortical cysts
Eun Young Park, Young Ok Kim, Ji Youn Kim, Chae Young Yeo, Hee Jo Baek, Chan Jong Kim, Eun Young Kim, Young Jong Woo
Clin Exp Pediatr. 2008;51(12):1342-1345.   Published online December 15, 2008
Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare white matter disorder, first described in the early 1990s. The brain in patients with MLC appears swollen on MRI, with diffuse white matter abnormalities; in addition, there is an invariable presence of subcortical cysts, primarily in the anterior temporal region sparing the deep white matter, basal ganglia, thalami, and cerebellum. Patients...
A case of congenital vallecular cyst associated with gastroesophageal reflux presenting with stridor, feeding cyanosis, and failure to thrive
Mi Ae Yang, Min Jae Kang, Jeana Hong, Seung Han Shin, Sang Duk Kim, Ee-Kyung Kim, Han-Suk Kim, Jung-Hwan Choi, Tack Kyun Kwon, In-One Kim
Clin Exp Pediatr. 2008;51(7):775-779.   Published online July 15, 2008
Vallecular cyst is an uncommon but potentially dangerous condition causing stridor and has been associated with sudden airway obstruction resulting in death due to its anatomical location in neonates. It may also present with various degrees of feeding problems resulting in failure to thrive. When a vallecular cyst is suspected clinically, endoscopic laryngoscopy is necessary to visualize it. Other conditions...
Original Article
A case of cystic fibrosis presented with meconium ileus in a female neonate
In-Ok Hwang, Eun-Sil Lee
Clin Exp Pediatr. 2007;50(12):1252-1256.   Published online December 15, 2007
Meconium ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF) in infants. It arises from the intraluminal accumulation of highly viscid, protein-rich meconium, typically present in the terminal ileum as a neonatal intestinal obstruction. Therefore, the clinical symptoms include abdominal distension, bilious vomiting and delayed passage of meconium. CF is caused by mutations in the transmembrane conductance regulator...
Case Report
Basal cell nevus syndrome (gorlin syndrome) confirmed by PTCH mutations and deletions
Hee Moon Kim, Chul Hee Lee, Sung Koo Kim, Tae Jung Sung
Clin Exp Pediatr. 2007;50(8):789-793.   Published online August 15, 2007
Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder characterized by variable manifestations, including multiple basal cell carcinomas, odontogenic keratocysts of the jaw, skeletal anomalies including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We report a case of a 9-year-old boy with the...
Original Article
Serum homocysteine and tumor necrosis factor-alpha levels after intravenous gammaglobulin treatment in patients with Kawasaki disease
Jung Hwa Cha, Young Mi Hong
Clin Exp Pediatr. 2006;49(10):1093-1099.   Published online October 15, 2006
Purpose : Homocysteine is a strong and independent risk factor for cardiovascular disease. The deleterious effects of homocysteine included endothelial dysfunction, arterial intimal-medial thickening, wall stiffness and procoagulant activity. However, the precise mechanism responsible for homocysteine release in children with coronary artery disease is still unknown. The purpose of this study was to investigate serum homocysteine and tumor necrosis factor(TNF)-α...
Case Report
Intrathyroidal branchial cleft-like cyst in neonate
Joonwon Kang, Sangmin Oh, Jiyoung Sul, Choongsik Lee, Meayoung Chang
Clin Exp Pediatr. 2006;49(9):1005-1009.   Published online September 15, 2006
A rare case is described of an intrathyroidal branchial cleft-like cyst in neonate. The patient was a newborn girl with a mass in the left lateral neck. The ultrasonography and computed tomography revealed a cystic lesion in the left thyroid. The lesion was enucleated surgically from the thyroid. Histologically, the cyst was lined by squamous or columnar epithelium and contained...
A case of multicystic dysplastic kidney and cystic adenomatoid malformation of the lung identified as incidental findings
Sun-Joo Lee, Ji-Hun Lee, Hyun-Hee Kim, So-Young Kim, Seung-Hoon Hahn, Ja-Young Hwang, Wonbae Lee
Clin Exp Pediatr. 2006;49(7):796-799.   Published online July 15, 2006
Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was...
Original Article
Voiding cystourethrography in children with an initial episode of febrile urinary tract infection
Hae Jeong Lee, Won Deok Lee, Hyun Seok Kim, Tae Hong Kim, Joo Seok Lee, Kyung Lae Cho
Clin Exp Pediatr. 2006;49(6):653-658.   Published online June 15, 2006
Purpose : Because voiding cystourethrography(VCUG) is an invasive method, we studied whether VCUG could be postponed through evaluation of alternative non-invasive tests including renal ultrasonography and 99mTc-DMSA renal scan. Methods : We reviewed the medical records of 175 patients initially diagnosed with febrile urinary tract infection during the one year period of 1999, and compared 3-tests : renal ultrasongraphy, 99mTc-DMSA renal...
Homocysteine, insulin, vitamin B12 and folic acid levels in obese children
Jee Yoon Park, Young Mi Hong
Clin Exp Pediatr. 2006;49(5):475-481.   Published online May 15, 2006
Purpose : As the prevalence of childhood obesity is increasing, hypertension, hyperlipidemia, insulin resistance and diabetes mellitus have become problems. High homocysteine levels and low vitamin B12 supplementation are acknowledged to have a role in coronary artery disease, but there are few studies on homocysteine, insulin and vitamin B12 levels in obese children. We aimed to study whether homocysteine, insulin,...
The Value of Serum Concentration of Cystatin C as a Marker for Glomerular Filtration Rate in Children and Adolescents
Young-Su Jung, In-Seok Lim
Clin Exp Pediatr. 2005;48(6):614-618.   Published online June 15, 2005
Purpose : Cystatin C has been proposed for the assessment of glomerular filtration rate, being more accurate than creatinine determination. We measured serum cystatin C concentrations in the pediatric population, and analysed the correlation between cystatin C and glomerular filtration rate. Methods : Cystatin C and creatinine were measured by the particle enhanced nephelometric immunoassay and Jaff method, respectively, in...
Case Report
A Case of Chylous Mesenteric Cyst in a Neonate
Hyun Taek Lim, Jung Hwa Lee, So Hee Lee, Jeong Eun Kim, Seong Jin Hong, Young Chil Choi, Sang Yun Kim
Clin Exp Pediatr. 2005;48(5):569-571.   Published online May 15, 2005
Mesenteric cysts are one of the least common intraabdominal lesions, which are very rare in neonates. Among mesenteric cysts, a chylous cyst is the rarest one of all. This entity can be found anywhere in the gastrointestinal tract from the duodenum to the rectum. Mesenteric cysts may be asymptomatic and found on routine examinations, or can present with symptoms such...