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Case Report
A case of Hyper-IgE syndrome with a mutation of the STAT3 gene
Ji-man Kang, Jungmun Suh, Jihyun Kim, Hee-Jin Kim, Yae-jean Kim, Hun Seok Lee, Young Kee Shin, Kangmo Ahn, Sang-Il Lee
Clin Exp Pediatr. 2010;53(4):592-597.   Published online April 15, 2010
Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene (stat3) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent...
Original Article
The natural history and prognostic factors of Graves' disease in Korean children and adolescents
Seung Min Song, Ji-Seok Youn, Jung Min Ko, Chong Kun Cheon, Jin-Ho Choi, Han Wook Yoo
Clin Exp Pediatr. 2010;53(4):585-591.   Published online April 15, 2010
Purpose : Graves' disease is the most common cause of hyperthyroidism in children and adolescents. In this study, we investigated the natural course and the prognostic factors of Graves' disease in Korean children and adolescents. Methods : One-hundred thirteen (88 girls and 25 boys) patients were included in this study. A retrospective analysis was made of all patients who were diagnosed...
Phonetic analysis of Korean elementary students who had overseas study at early ages
Mee Heun Ryu, Chang Woo Lee
Clin Exp Pediatr. 2010;53(4):579-584.   Published online April 15, 2010
Purpose : The number of the repatriated Korean students who had overseas study at early ages is increasing. They received foreign education, they can speak international languages, but they have many difficulties in articulation and intonation of the Korean language. This study aims to measure closure and aspiration duration, length of consonants, length of subsequent vowels, and ratio of consonants...
Efficacy of levetiracetam in refractory childhood epilepsy
Keon Su Lee, Joon Won Kang
Clin Exp Pediatr. 2010;53(4):571-578.   Published online April 15, 2010
Purpose : To evaluate the efficacy and safety of levetiracetam adjunctive therapy for reducing the rate of seizure frequency in children with intractable pediatric epilepsy. Methods : We reviewed the medical records of 86 patients with intractable pediatric epilepsy who visited our hospital between March 1989 and February 2009. Levetiracetam was included in the previous anticonvulsant regimen for at least 6...
Clinical efficacy and safety of lamotrigine monotherapy in newly diagnosed pediatric patients with epilepsy
Ji Hye Han, Jung Eun Oh, Sun Jun Kim
Clin Exp Pediatr. 2010;53(4):565-570.   Published online April 15, 2010
Purpose : To verify the efficacy and safety of lamotrigine (LTG) monotherapy in newly diagnosed children with epilepsy. Methods : We prospectively enrolled 148 children who had undergone LTG monotherapy at our institution between September 2002 and June 2009. Twenty-nine patients were excluded: 19 due to incomplete data and 10 were lost to follow up. The data of the remaining...
Change of interictal epileptiform discharges after antiepiletic drug treatment in childhood epilepsy
Mun Ju Kim, Sang Ook Nam
Clin Exp Pediatr. 2010;53(4):560-564.   Published online April 15, 2010
Purpose : Electroencephalography (EEG) findings can play a critical role in a variety of decisions, including initiation and withdrawal of antiepileptic drugs (AEDs) therapy. Interictal epileptiform discharges (IEDs) are predictor of recurrent seizures. We investigated IEDs in EEG after AED therapy and related factors in epileptic children. Methods : The subjects were 257 children [151 males and 106 females; age,...
A study on the therapeutic effects of Topiramate according to the types of migraine
Jun Hwa Lee, Kyung Lae Cho
Clin Exp Pediatr. 2010;53(4):554-559.   Published online April 15, 2010
Purpose : The purpose of this study was to evaluate whether the therapeutic effects of topiramate differ according to the types of migraine. Methods : We recruited 38 children and adolescents with migraine who had been treated with topiramate. The effect of topiramate was evaluated on the basis of the change in the frequency of migraine attacks after treatment. Results : Among...
Association of HLA-G gene promoter haplotype with childhood IgA nephropathy in the Korean population
Hwan Hee Jung, Won Ho Hahn, Byoung Soo Cho, Sung Do Kim
Clin Exp Pediatr. 2010;53(4):548-553.   Published online April 15, 2010
Purpose : IgA nephropathy (IgAN) is the most commonly occurring form of chronic glomerulonephritis in pediatric cases.Human leukocyte antigen (HLA) genes have been implicated in various inflammatory and autoimmune diseases. The present study was conducted to investigate the association between 2 single nucleotide polymorphisms (SNPs) of the HLA-G gene and childhood IgAN. Methods : The authors analyzed and compared HLA-G gene...
Comparison of total body irradiation-based or non-total body irradiation-based conditioning regimens for allogeneic stem cell transplantation in pediatric leukemia patients
Sang Jeong Kim, Dong Kyun Han, Hee Jo Baek, Dong Yeon Kim, Taek Keun Nam, Tai Ju Hwang, Hoon Kook
Clin Exp Pediatr. 2010;53(4):538-547.   Published online April 15, 2010
Purpose : This study aims to compare the outcome of total body irradiation (TBI)- or non-TBI-containing conditioning regimens for leukemia in children. Methods : We retrospectively evaluated 77 children conditioned with TBI (n=40) or non-TBI (n=37) regimens, transplanted at Chonnam National University Hospital between January 1996 and December 2007. The type of transplantation, disease status at the time of transplant, conditioning regimen,...
Early postoperative arrhythmias after open heart surgery of pediatric congenital heart diseas
Hee Joung Choi, Yeo Hyang Kim, Joon Yong Cho, Myung Chul Hyun, Sang Bum Lee, Kyu Tae Kim
Clin Exp Pediatr. 2010;53(4):532-537.   Published online April 15, 2010
Purpose : Early postoperative arrhythmias are a major cause of mortality and morbidity after open heart surgery in the pediatric population. We evaluated the incidence and risk factors of early postoperative arrhythmias after surgery of congenital heart disease. Methods : From January 2002 to December 2008, we retrospectively reviewed the medical records of the 561 patients who underwent cardiac surgery in Kyungpook National University...
Apoptosis and upregulation of TNF-α and TRAIL receptor 1 (DR4) in the pathogenesis of food protein-induced enterocolitis syndrome
Jin-Bok Hwang, Sang Pyo Kim, Yu Na Kang, Seong-Ryong Lee, Seong-Il Suh, Taeg Kyu Kwon
Clin Exp Pediatr. 2010;53(4):525-531.   Published online April 15, 2010
Purpose : Expression levels of tumor necrosis factor (TNF)-α expression on the mucosa of the small intestine is increased in patients with villous atrophy in food protein-induced enterocolitis syndrome (FPIES). TNF-α has been reported to induce apoptotic cell death in the epithelial cells. We studied the TNF family and TNF-receptor family apoptosis on the duodenal mucosa to investigate their roles in the pathogenesis...
NT-proBNP as a useful tool in diagnosing incomplete Kawasaki disease
Dong Won Lee, Yeo Hyang Kim, Myung Chul Hyun, Tae Chan Kwon, Sang Bum Lee
Clin Exp Pediatr. 2010;53(4):519-524.   Published online April 15, 2010
Purpose : To determine the efficacy of the N-terminal fragment of B-type natriuretic peptide (NT-proBNP) as a useful diagnostic method in children with incomplete Kawasaki disease (KD). Methods : Ninety-six patients who were diagnosed as having KD between January 2008 and June 2009 were enrolled in the study. American Heart Association recommendations for diagnosis were used, and patients were divided into...
Clinical significance of matrix metalloproteinase 9 and tissue inhibitor of metalloproteinase 1 and 2 in Kawasaki disease
Ki Wook Yun, Sin Weon Sin, Jung Ju Lee, Soo Ahn Chae, In Seok Lim, Eung Sang Choi, Byoung Hoon Yoo, Mi-Kyung Lee
Clin Exp Pediatr. 2010;53(4):510-518.   Published online April 15, 2010
Purpose : Kawasaki disease (KD) is a systemic vasculitis, a leading cause of pediatric acquired heart disease. Histopathological findings of coronary artery lesion (CAL) in KD indicate destruction of the coronary artery wall with diffuse vasculitis. Matrix metalloproteinases (MMPs) and their endogenous tissue inhibitors (TIMPs) might play central roles in this process. Special attention to MMP-9 has recently been emerging....
The risk factors and prognosis associated with neonatal pulmonary hemorrhage
Su Jin Park, Ki Tae Yun, Won Duck Kim, Sang Geel Lee
Clin Exp Pediatr. 2010;53(4):503-509.   Published online April 15, 2010
Purpose : Although neonatal pulmonary hemorrhage is rare, it is associated with high mortality. We aimed to evaluate the risk factors associated with pulmonary hemorrhage in preterm infants and to describe the clinical course, including neonatal morbidity, of infants who developed pulmonary hemorrhage. Methods : We performed a retrospective case-control study of 117 newborn infants aged less than 37 gestational weeks admitted...
Analysis of neonatal sepsis in one neonatal intensive care unit for 6 years
Peter Chun, Seom Gim Kong, Shin Yun Byun, Su Eun Park, Hyung Du Lee
Clin Exp Pediatr. 2010;53(4):495-502.   Published online April 15, 2010
Purpose : Sepsis is a significant cause of morbidity and mortality in the newborn, particularly in preterm. The objective of this study was to analyze the incidence rate, causative pathogens and clinical features of neonatal sepsis in one neonatal intensive care unit (NICU) for 6 years. Methods : This study was retrospectively performed to review the clinical and laboratory characteristics including sex, gestational age,...
A report on operating a nationwide human milk bank in Korea
Kang Hoon Song, Yoo Min Lee, Ji Young Chang, Eun Young Park, Sung Ae Park, Nam Kyu Cho, Chong-Woo Bae
Clin Exp Pediatr. 2010;53(4):488-494.   Published online April 15, 2010
Purpose : A human milk bank collects, processes, eliminates, and stores breast milk from donors and provides breast milk to those in need. The authors hereby present the experiences and the objective lessons obtained through operating a nationwide human milk bank over a period of 2 years. Methods : The characteristics of the donors and the recipients and the amounts of breast milk...
The effects of early allergen/endotoxin exposure on subsequent allergic airway inflammation to allergen in mouse model of asthma
Yeong-Ho Rha, Sun-Hee Choi
Clin Exp Pediatr. 2010;53(4):481-487.   Published online April 15, 2010
Purpose : Recently many studies show early exposure during childhood growth to endotoxin (lipopolysaccharides, LPS) and/or early exposure to allergens exhibit important role in development of allergy including bronchial asthma. The aim of this study was to evaluate the role of endotoxin and allergen exposure in early life via the airways in the pathogenesis of allergic airways inflammation and airway hyperresposiveness...
Review Article
Late physical effects of childhood cancer survivors
Young-Ho Lee
Clin Exp Pediatr. 2010;53(4):477-480.   Published online April 15, 2010
Advances in research and medical and supportive care have contributed to a growing population of adults formerly treated for childhood cancer. History of cancer and its therapy can have significant life-long health implications. Late effects of cancer therapy can be insidious on onset, occur outside the pediatric age, and contribute to premature morbidity and mortality. In this review, I have focused on the...
Psychosocial aspects of childhood cancer survivors
Jong Jin Seo
Clin Exp Pediatr. 2010;53(4):471-476.   Published online April 15, 2010
The majority of childhood cancer survivors and their families will be psychologically healthy, but may desire and benefit from preventive care. A significant portion of the survivor population will be psychosocially distressed in various aspects by their harsh experience of long cancer treatment, and may warrant professional intervention and treatment. Pediatricians should be aware of the late psychological effects that can occur a...
Long-term follow-up study and long-term care of childhood cancer survivors
Hyeon Jin Park
Clin Exp Pediatr. 2010;53(4):465-470.   Published online April 15, 2010
The number of long-term survivors is increasing in the western countries due to remarkable improvements in the treatment of childhood cancer. The long-term complications of childhood cancer survivors in these countries were brought to light by the childhood cancer survivor studies. In Korea, the 5-year survival rate of childhood cancer patients is approaching 70%; therefore, it is extremely important to undertake...
Case Report
A case of steroid-induced psychosis in a child having nephrotic syndrome with toxic epidermal necrolysis
Sae Yoon Kim, Jae Min Lee, Yong Hoom Park
Clin Exp Pediatr. 2010;53(3):437-441.   Published online March 15, 2010
Toxic epidermal necrolysis (TEN) and Stevens–Johnson syndrome (SJS) are rare, life-threatening mucocutaneous diseases, usually attributable to drugs and infections. Corticosteroids have been used in the management of TEN for the last 30 years. This remains controversial and is still much debated. TEN can occur despite administration of high doses of systemic corticosteroids. The psychiatric side effects of corticosteroids can include...
Transient neonatal diabetes mellitus with macroglossia diagnosed by methylation specific PCR (MS-PCR)
Hye Young Jin, Jin-Ho Choi, Gu-Hwan Kim, Han-Wook Yoo
Clin Exp Pediatr. 2010;53(3):432-436.   Published online March 15, 2010
Transient neonatal diabetes mellitus (TNDM) has been associated with paternal uniparental isodisomy of chromosome 6, paternally inherited duplication of 6q24, or a methylation defect at a CpG island of the ZAC or HYMAI gene. We experienced a case of TNDM in which the patient presented with hyperglycemia, macroglossia, and intrauterine growth retardation, caused by a paternally derived HYMAI. An 18-day-old...
Original Article
Thrombotic thrombocytopenic purpura with decreased level of ADAMTS-13 activity and increased level of ADAMTS-13 inhibitor in an adolescent
Eun Mi Yang, Dong Kyun Han, Hee Jo Baek, Young Ok Kim, Myung Geun Shin, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2010;53(3):428-431.   Published online March 15, 2010
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other...
Endocrine dysfunction after bone marrow transplantation during childhood and adolescence
Hye Young Jin, Jin-Ho Choi, Ho-Joon Im, Jong-Jin Seo, Hyung-Nam Moon, Han-Wook Yoo
Clin Exp Pediatr. 2010;53(3):420-427.   Published online March 15, 2010
Purpose : Several complications can occur in patients who received bone marrow transplantation (BMT) during childhood and adolescence. This study aims to investigate endocrine dysfunctions after BMT so that better care can be provided to care for long-term survivors of BMT. Methods : One hundred patients (61 males, 39 females) were included in this study. Clinical parameters such as initial diagnosis,...
Comparison of clinical and laboratory characteristics in children with type 1 diabetes according to pancreatic autoantibodies
Ji Hae Choi, Min Sun Kim, Chan Jong Kim, Jong Duk Kim, Dae-Yeol Lee
Clin Exp Pediatr. 2010;53(3):414-419.   Published online March 15, 2010

Purpose:The purpose of this study was to determine whether there is any difference in the clinical and laboratory characteristics of patients with autoantibody-positive and patients with autoantibody-negative type 1 diabetes at initial presentation. Methods:We analyzed 96 patients under 18 years of age with newly diagnosed type 1 diabetes. One or both of the pancreatic autoantibodies-glutamic acid decarboxylase autoantibodies (GADA) and...
Limitations of 99mTc-DMSA scan in diagnosing acute pyelonephritis in children
Byung Gee Kim, Jae Ryoung Kwak, Ji Min Park, Ki Soo Pai
Clin Exp Pediatr. 2010;53(3):408-413.   Published online March 15, 2010
Purpose : We aimed to prove the relative limitation of 99mTc-DMSA scintigraphy (DMSA) compared to computed tomography (CT) in diagnosing acute pyelonephritis (APN) in children. Methods : Since September 2006, after a 64-channel CT was imported, 10 DMSA false-negative patients have been identified: these patients underwent a CT scan for acute abdomen or acute febrile symptoms and were diagnosed as...
Direct detection of hemophilia B F9 gene mutation using multiplex PCR and conformation sensitive gel electrophoresis
Ki Young Yoo, Hee Jin Kim, Kwang Chul Lee
Clin Exp Pediatr. 2010;53(3):397-407.   Published online March 15, 2010
Purpose : The F9 gene is known to be the causative gene for hemophilia B, but unfortunately the detection rate for restriction fragment length polymorphism-based linkage analysis is only 55.6%. Direct DNA sequencing can detect 98% of mutations, but this alternative procedure is very costly. Here, we conducted multiplex polymerase chain reactions (PCRs) and conformation sensitive gel electrophoresis (CSGE) to...
Relationship between iron deficiency anemia and febrile convulsion in infants
Youn Soo Jun, Ho Il Bang, Seung Taek Yu, Sae Ron Shin, Du Young Choi
Clin Exp Pediatr. 2010;53(3):392-396.   Published online March 15, 2010
Purpose : The association between iron deficiency anemia and febrile convulsion in infants has been examined in several studies with conflicting results. Therefore, the authors aimed to evaluate the precise relationship involved. Methods : In this case-control study, the authors assessed 100 children with a diagnosis of febrile convulsion, aged between 9 months and 2 years, during January 2007 to...
Respiratory syncytial virus infection cases in congenital heart disease patients
Woo Sup Shim, Jae Yeong Lee, Jin Yong Song, Soo Jin Kim, Sung Hye Kim, So Ick Jang, Eun Yong Choi
Clin Exp Pediatr. 2010;53(3):380-391.   Published online March 15, 2010
Purpose : Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV...
Clinical characteristics of acute lower respiratory tract infections due to 13 respiratory viruses detected by multiplex PCR in children
Jeong-Sook Lim, Sung-Il Woo, Hyuk-Il Kwon, Hyuk-Il Kwon, Young-Ki Choi, Youn-Soo Hahn
Clin Exp Pediatr. 2010;53(3):373-379.   Published online March 15, 2010
Purpose : This study was performed to investigate the epidemiologic and clinical features of 13 respiratory viruses in children with acute lower respiratory tract infections (ALRIs). Methods : Nasopharyngeal aspirates were prospectively obtained from 325 children aged 15 years or less from May 2008 to April 2009 and were tested for the presence of 13 respiratory viruses by multiplex real-time-polymerase chain...
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