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Review Article
Nephrotic syndrome: what's new, what's hot?
Hee Gyung Kang, Hae Il Cheong
Clin Exp Pediatr. 2015;58(8):275-282.   Published online August 21, 2015

While the incidence of nephrotic syndrome (NS) is decreasing in Korea, the morbidity of difficult-to-treat NS is significant. Efforts to minimize treatment toxicity showed that prolonged treatment after an initial treatment for 2-3 months with glucocorticosteroids was not effective in reducing frequent relapses. For steroid-dependent NS, rituximab, a monoclonal antibody against the CD20 antigen on B cells, was proven to...

Case Report
Hypopituitarism and Legg-Calve-Perthes disease related to difficult delivery
Veysel Nijat Baş, Salih Uytun, Ümit Erkan Vurdem, Yasemin Altuner Torun
Clin Exp Pediatr. 2015;58(7):270-273.   Published online July 22, 2015

Legg-Calve-Perthes (LCP) disease is characterized by idiopathic avascular osteonecrosis of the epiphysis of the femur head. The main factor that plays a role in the etiology of the disease is decreased blood flow to the epiphysis. Many predisposing factors have been suggested in the etiology of LCP disease, and most have varying degrees of effects. Here we present the case...

Graves disease following rabbit antithymocyte globulin treatment of severe aplastic anemia in a Korean child
In Su Choi, Han Kyul Kim, Dong Kyun Han, Hee Jo Baek, Hae In Jang, Chan Jong Kim, Hoon Kook
Clin Exp Pediatr. 2015;58(7):267-269.   Published online July 22, 2015

Antithymocyte globulin (ATG) is used as an immunosuppressive treatment (IST) to deplete clonal suppressor T cells in patients with severe aplastic anemia (SAA). The depletion of suppressor T cells by ATG may affect the activation of B cells, which results in an increased risk for autoimmune conditions. A 12-year-old boy was diagnosed with idiopathic SAA. As he did not have...

Original Article
The corrected QT (QTc) prolongation in hyperthyroidism and the association of thyroid hormone with the QTc interval
Ye Seung Lee, Joong Wan Choi, Eun Ju Bae, Won Il Park, Hong Jin Lee, Phil Soo Oh
Clin Exp Pediatr. 2015;58(7):263-266.   Published online July 22, 2015
Purpose

Ventricular repolarization is assessed using the QT interval corrected by the heart rate (QTc) via an electrocardiogram (ECG). Prolonged QTc is associated with an increased risk of arrhythmias and cardiac mortality. As there have been few reports regarding the effects of hyperthyroidism on ventricular repolarization, we studied the association between serum free thyroxine (free T4 [fT4]) and thyroid stimulating hormone...

The strong association of left-side heart anomalies with Kabuki syndrome
Ja Kyoung Yoon, Kyung Jin Ahn, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Chung Il Noh, Jung Min Ko
Clin Exp Pediatr. 2015;58(7):256-262.   Published online July 22, 2015
Purpose

Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center.

Methods

A retrospective analysis was conducted for a total of 13 patients with Kabuki...

Applying the bacterial meningitis score in children with cerebrospinal fluid pleocytosis: a single center's experience
Jungpyo Lee, Hyeeun Kwon, Joon Soo Lee, Heung Dong Kim, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(7):251-255.   Published online July 22, 2015
Purpose

The widespread introduction of bacterial conjugate vaccines has decreased the risk of cerebrospinal fluid (CSF) pleocytosis due to bacterial meningitis (BM) in children. However, most patients with CSF pleocytosis are hospitalized and treated with parenteral antibiotics for several days. The bacterial meningitis score (BMS) is a validated multivariate model derived from a pediatric population in the postconjugate vaccine era and...

Clinical risk factors associated with the development of wheezing in children less than 2 years of age who required hospitalization for viral lower respiratory tract infections
Joon Hwan Kim, Ji-Yeon Choi, Na Yeon Kim, Jin Woo Kim, Ji Hyeon Baek, Hye Sung Baek, Jung Won Yoon, Hye Mi Jee, Sun Hee Choi, Hyeung Yoon Kim, Ki Eun Kim, Youn Ho Shin, Man Yong Han
Clin Exp Pediatr. 2015;58(7):245-250.   Published online July 22, 2015
Purpose

Wheezing following viral lower respiratory tract infections (LRTIs) in children <2 years of age is an important risk factor for the development of asthma later in life; however, not all children with viral LRTIs develop wheezing. This study investigated risk factors for the development of wheezing during viral LRTIs requiring hospitalization.

Methods

The study included 142 children <2 years of age hospitalized...

Review Article
Complement regulation: physiology and disease relevance
Heeyeon Cho
Clin Exp Pediatr. 2015;58(7):239-244.   Published online July 22, 2015

The complement system is part of the innate immune response and as such defends against invading pathogens, removes immune complexes and damaged self-cells, aids organ regeneration, confers neuroprotection, and engages with the adaptive immune response via T and B cells. Complement activation can either benefit or harm the host organism; thus, the complement system must maintain a balance between activation...

Case Report
Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome
In Ah Jung, Won Kyoung Cho, Yeon Jin Jeon, Shin Hee Kim, Kyoung Soon Cho, So Hyun Park, Min Ho Jung, Byung-Kyu Suh
Clin Exp Pediatr. 2015;58(6):234-237.   Published online June 22, 2015

Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia. Mauriac syndrome, which is characterized by hepatomegaly due to hepatic glycogenosis, growth retardation, delayed puberty, and Cushingoid features, is a rare diabetic complication. We report a case of...

A novel association between cerebral sinovenous thrombosis and nonketotic hyperglycinemia in a neonate
Sadık Yurttutan, Mehmet Yekta Oncel, Nursel Yurttutan, Halil Degirmencioglu, Nurdan Uras, Ugur Dilmen
Clin Exp Pediatr. 2015;58(6):230-233.   Published online June 22, 2015

Lethargy in newborns usually indicates central nervous system dysfunction, and many conditions such as cerebrovascular events, infections, and metabolic diseases should be considered in the differential diagnosis. Nonketotic hyperglycinemia is an autosomal recessive error of glycine metabolism, characterized by myoclonic jerks, hypotonia, hiccups, apnea, and progressive lethargy that may progress to encephalopathy or even death. Cerebral sinovenous thrombosis is a...

Original Article
Thyroid dysfunction in very low birth weight preterm infants
Ji Hoon Lee, Sung Woo Kim, Ga Won Jeon, Jong Beom Sin
Clin Exp Pediatr. 2015;58(6):224-229.   Published online June 22, 2015
Purpose

Thyroid dysfunction is common in preterm infants. Congenital hypothyroidism causes neurodevelopmental impairment, which is preventable if properly treated. This study was conducted to describe the characteristics of thyroid dysfunction in very low birth weight infants (VLBWIs), evaluate risk factors of hypothyroidism, and suggest the reassessment of thyroid function with an initially normal thyroid-stimulating hormone (TSH) as part of a newborn...

Cardiopulmonary function and scoliosis severity in idiopathic scoliosis children
Seokwon Huh, Lucy Yougmin Eun, Nam Kyun Kim, Jo Won Jung, Jae Young Choi, Hak Sun Kim
Clin Exp Pediatr. 2015;58(6):218-223.   Published online June 22, 2015
Purpose

Idiopathic scoliosis is a structural lateral curvature of the spine of unknown etiology. The relationship between degree of spine curvature and cardiopulmonary function has not yet been investigated. The purpose of this study was to determine the association between scoliosis and cardiopulmonary characteristics.

Methods

Ninety children who underwent preoperative pulmonary or cardiac evaluation at a single spine institution over 41 months were...

Hepatitis associated with Mycoplasma pneumoniae infection in Korean children: a prospective study
Kyu Won Kim, Jae Jin Sung, Hann Tchah, Eell Ryoo, Hye Kyung Cho, Yong Han Sun, Kang Ho Cho, Dong Woo Son, In Sang Jeon, Yun Mi Kim
Clin Exp Pediatr. 2015;58(6):211-217.   Published online June 22, 2015
Purpose

Mycoplasma pneumoniae (MP) infection is a major cause of respiratory infection in school-aged children. Extrapulmonary manifestations of MP infection are common, but liver involvement has been rarely reported. The aim of this study was to determine the clinical characteristics of MP-associated hepatitis.

Methods

This prospective study included 1,044 pediatric patients with MP infection diagnosed serologically with MP IgM at one medical center...

Initial steroid regimen in idiopathic nephrotic syndrome can be shortened based on duration to first remission
Hee Sun Baek, Ki-Soo Park, Hee Gyung Kang, Cheol Woo Ko, Min Hyun Cho
Clin Exp Pediatr. 2015;58(6):206-210.   Published online June 22, 2015
Purpose

The use of a 12-week steroid regimen (long-term therapy, LT) for the first episode of idiopathic nephrotic syndrome (NS) reportedly induces a more sustained remission and lower relapse rate than previous regimens, including an 8-week steroid regimen (short-term therapy, ST). Here, we assessed the potential for selective application of 2 steroid regimens (LT vs. ST) based on the days to...

Review Article
Haploidentical hematopoietic stem cell transplantation in children and adolescents with acquired severe aplastic anemia
Ho Joon Im, Kyung-Nam Koh, Jong Jin Seo
Clin Exp Pediatr. 2015;58(6):199-205.   Published online June 22, 2015

Severe aplastic anemia (SAA) is a life-threatening disorder for which allogeneic hematopoietic stem cell transplantation (HSCT) is the current available curative treatment. HSCT from matched sibling donors (MSDs) is the preferred therapy for children with acquired SAA. For patients who lack MSDs, immunosuppressive therapy (IST) is widely accepted as a first-line treatment before considering HCT from an unrelated donor (URD)....

Case Report
Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses
Soo Jin Chang, Ji Hyun Lee, Shin Hye Kim, Joon Soo Lee, Heung Dong Kim, Joon Won Kang, Young Mock Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(5):194-198.   Published online May 22, 2015

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at...

Case of seropositive allergic bronchopulmonary aspergillosis in a 10-year-old girl without previously documented asthma
Jeong Eun Shin, Jae Won Shim, Deok Soo Kim, Hae Lim Jung, Moon Soo Park, Jung Yeon Shim
Clin Exp Pediatr. 2015;58(5):190-193.   Published online May 22, 2015

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease due to bronchial colonization of Aspergillus fumigatus that occurs in susceptible patients with asthma or cystic fibrosis. A 10-year-old girl was referred to the Department of Pediatric Pulmonology for persistent consolidations on chest radiography. Pulmonary consolidations were observed in the right upper and left lower lobes and were not resolved with...

Original Article
Validity of bag urine culture for predicting urinary tract infections in febrile infants: a paired comparison of urine collection methods
Geun-A Kim, Ja-Wook Koo
Clin Exp Pediatr. 2015;58(5):183-189.   Published online May 22, 2015
Purpose

Catheter urine (CATH-U) and suprapubic aspiration (SPA) are reliable urine collection methods for confirming urinary tract infections (UTI) in infants. However, noninvasive and easily accessible collecting bag urine (CBU) is widely used, despite its high contamination rate. This study investigated the validity of CBU cultures for diagnosing UTIs, using CATH-U culture results as the gold standard.

Methods

We retrospectively analyzed 210 infants,...

Association of CD4+CD25+FoxP3+ regulatory T cells with natural course of childhood chronic immune thrombocytopenic purpura
Bo Ra Son, Ji Yoon Kim
Clin Exp Pediatr. 2015;58(5):178-182.   Published online May 22, 2015
Purpose

The purpose of this study was to determine the frequency of CD4+CD25+FoxP3+ regulatory T cells (Treg) in the peripheral blood of patients with childhood chronic immune thrombocytopenic purpura (ITP) exhibiting thrombocytopenia and spontaneous remission. The findings of this study indicate the possibility of predicting spontaneous recovery and pathogenesis of childhood chronic ITP.

Methods

Eleven children with chronic ITP (seven thrombocytopenic and four...

Epidemiological comparison of three Mycoplasma pneumoniae pneumonia epidemics in a single hospital over 10 years
Eun-Kyung Kim, You-Sook Youn, Jung-Woo Rhim, Myung-Seok Shin, Jin-Han Kang, Kyung-Yil Lee
Clin Exp Pediatr. 2015;58(5):172-177.   Published online May 22, 2015
Purpose

Mycoplasma pneumoniae (MP) pneumonia epidemics have occurred in 3- to 4-year cycles in Korea. We evaluated the epidemiologic characteristics of MP pneumonia in Daejeon, Korea, from 2003 to 2012.

Methods

We retrospectively analyzed 779 medical records of children (0-15 years of old) with MP pneumonia admitted to our institution and compared the data from 3 recent epidemics.

Results

In 779 patients, the mean age...

Association between serum alanine aminotransferase level and obesity indices in Korean adolescents
Moon Bae Ahn, Woo Ri Bae, Kyung Do Han, Won Kyoung Cho, Kyoung Soon Cho, So Hyun Park, Min Ho Jung, Byung Kyu Suh
Clin Exp Pediatr. 2015;58(5):165-171.   Published online May 22, 2015
Purpose

To analyze the correlation between serum alanine aminotransferase (ALT) and obesity indices including body mass index (BMI), body fat percentage (BFP), total fat mass (FM), truncal fat mass (TFM), waist circumference (WC), and waist-to-height ratio (WHtR) in Korean adolescents.

Methods

This was a cross-sectional study based on data derived from the 2010-2011 Korean National Health and Nutrition Examination Surveys (KNHANES). Subjects were...

Review Article
Approaches to the diagnosis and management of chronic urticaria in children
Sun Hee Choi, Hey Sung Baek
Clin Exp Pediatr. 2015;58(5):159-164.   Published online May 22, 2015

Most guidelines for chronic urticaria (CU) in infants and children are based on limited pediatric evidence. Current evidence used to guide treatment in children is extrapolated from data focusing on older age groups. CU in children is a different and complex condition than that in adults. Furthermore, there is little published information regarding urticaria in Korean children. The aim of...

Case Report
Urosepsis and postrenal acute renal failure in a neonate following circumcision with Plastibell device
Meena Kalyanaraman, Derrick McQueen, Joseph Sykes, Tej Phatak, Farhaan Malik, Preethi S. Raghava
Clin Exp Pediatr. 2015;58(4):154-157.   Published online April 22, 2015

Plastibell is one of the three most common devices used for neonatal circumcision in the United States, with a complication rate as low as 1.8%. The Plastibell circumcision device is commonly used under local anesthesia for religious circumcision in male neonates, because of cosmetic reasons and ease of use. Occasionally, instead of falling off, the device may get buried under...

Autosomal dominant hypocalcemia with Bartter syndrome due to a novel activating mutation of calcium sensing receptor, Y829C
Keun Hee Choi, Choong Ho Shin, Sei Won Yang, Hae Il Cheong
Clin Exp Pediatr. 2015;58(4):148-153.   Published online April 22, 2015

The calcium sensing receptor (CaSR) plays an important role in calcium homeostasis. Activating mutations of CaSR cause autosomal dominant hypocalcemia by affecting parathyroid hormone secretion in parathyroid gland and calcium resorption in kidney. They can also cause a type 5 Bartter syndrome by inhibiting the apical potassium channel in the thick ascending limb of the loop of Henle in the...

Original Article
Neuroprotective effects of mild hypoxia in organotypic hippocampal slice cultures
Seh Hyun Kim, Woo Soon Lee, Na Mi Lee, Soo Ahn Chae, Sin Weon Yun
Clin Exp Pediatr. 2015;58(4):142-147.   Published online April 22, 2015
Purpose

The aim of this study was to investigate the potential effects of mild hypoxia in the mature and immature brain.

Methods

We prepared organotypic slice cultures of the hippocampus and used hippocampal tissue cultures at 7 and 14 days in vitro (DIV) to represent the immature and mature brain, respectively. Tissue cultures were exposed to 10% oxygen for 60 minutes. Twenty-four hours...

Ultrasound-guided internal jugular vein catheterization in critically ill pediatric patients
Eu Jeen Yang, Hyeong Seok Ha, Young Hwa Kong, Sun Jun Kim
Clin Exp Pediatr. 2015;58(4):136-141.   Published online April 22, 2015
Purpose

Continuous intravenous access is imperative in emergency situations. Ultrasound-guided internal jugular vein (IJV) catheterization was investigated in critically ill pediatric patients to assess the feasibility of the procedure.

Methods

Patients admitted to the pediatric intensive care unit between February 2011 and September 2012 were enrolled in this study. All patients received a central venous catheter from attending house staff under ultrasound guidance....

Long-term outcome of patients with p22phox-deficient chronic granulomatous disease on Jeju Island, Korea
Hyun Sik Kang, Geol Hwang, Kyung-Sue Shin
Clin Exp Pediatr. 2015;58(4):129-135.   Published online April 22, 2015
Purpose

This study investigated the long-term clinical outcomes of patients with p22phox-deficient chronic granulomatous disease (CGD) on Jeju Island and retrospectively evaluated the effects of interferon-gamma (IFN-γ) prophylaxis.

Methods

The medical records of 15 patients with CGD were retrospectively reviewed. The efficacy of IFN-γ prophylaxis was evaluated by comparing the frequency of severe infections before and after starting continuous prophylaxis with IFN-γ.

Results

At the...

Review Article
Osteosarcoma in Korean children and adolescents
Jun Ah Lee
Clin Exp Pediatr. 2015;58(4):123-128.   Published online April 22, 2015

Osteosarcoma is the most frequent primary bone tumor. Advances in combination chemotherapy and surgical technique have greatly improved the survival of patients with osteosarcoma. In Korea, improvements in osteosarcoma treatment have been made over the past two decades. The 5-year event-free survival rate of Korean children and adolescents with localized disease is 64.6%, comparable to that of American or European...

Metabolic evaluation of children with global developmental delay
So-Hee Eun, Si Houn Hahn
Clin Exp Pediatr. 2015;58(4):117-122.   Published online April 22, 2015

Global developmental delay (GDD) is a relatively common early-onset chronic neurological condition, which may have prenatal, perinatal, postnatal, or undetermined causes. Family history, physical and neurological examinations, and detailed history of environmental risk factors might suggest a specific disease. However, diagnostic laboratory tests, brain imaging, and other evidence-based evaluations are necessary in most cases to elucidate the causes. Diagnosis of...

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