Purpose : Bartter syndrome is a renal tubular defect in electrolyte transport characterized by hypokalemia, metabolic alkalosis and other clinical signs and symptoms. The aims of this study were to analyze the clinical manifestations and the short- and long-term outcomes of Bartter syndrome. Methods : We retrospectively reviewed clinical history, laboratory finding of blood and urine, renal ultrasonography, and hearing... |
Purpose : Multiple transfusions in patients with chronic anemia can result in excessive iron deposition in tissues and organs. Effective iron chelation therapy in chronically transfused patients can only be achieved when iron chelators remove sufficient amounts of iron equivalent to those accumulated in the body from transfusions, thus leading to maintain body iron load at a non-toxic level. This... |
Purpose : Due to its high potency against leukemic blasts, our institution has opted for the use of dexamethasone during acute lymphoblastic leukemia (ALL) remission induction, but in our most recent treatment protocol, CMCPL-2005, we shortened the length of steroid treatment from 4 to 3 weeks. We compared both the rates of remission induction and significant complications observed during induction... |
Purpose : To determine optimal maintenance dose and to evaluation the efficacy and safety of polyethylene glycol 4000 (PEG4000) in children with chronic functional constipation. Methods : This study enrolled 41 children with chronic functional constipation at the Konkuk university hospital August 2005, then June, 2007. Effective maintenance dose was designed as initial amount of PEG4000 that improved frequency, bowel movement,... |
Purpose : The aim of this study was to investigate the risk of low birth weight associated with delayed first childbearing in Korea. Methods : The national birth certificate data of the singletons and first babies in Korea from January 2001 to December 2003 (n=736,167) was used. Outcome measures were rates of low birth weight infant, very low birth weight... |
Purpose : We performed this study to investigate the outcome of surfactant replacement therapy (SRT) in above nearterm neonates who were required mechanical ventilatory care due to meconium aspiration pneumonia (MAP), respiratory distress syndrome (RDS) or other severe pneumonia (PN). Methods : 48 patients, gestational period ≥36 weeks, who were admitted in NICU of Dongsan Medical Center, Keimyung University between July... |
Purpose : It was generally accepted now a days that the pathogenesis of preeclampsia, small for gestational age (SGA), intrauterine growth retardation and fetal origin of adult diseases were related with a endothelial cell dysfunction. The purpose of this study was to know the relation of such diseases by assessing the level of endothelin-1. Methods : SGA babies, newborns of... |
Purpose : The prevalence of rickets in the world is on the rise not only in developing but also in developed countries. In Korea, breastfeeding has increased. There have been few studies on the possible association of rickets with breastfeeding. The purpose of this study was to identify the development and the clinical presentation of subclinical rickets in breastfed infants. Methods... |
Systemic lupus erythematosus (SLE) is an episodic, multi-system, autoimmune disease characterized by widespread inflammation of blood vessels and connective tissues and by the presence of antinuclear antibodies (ANAs), especially antibodies to native (double-stranded) DNA (dsDNA). Its clinical manifestations are extremely variable, and its natural history is unpredictable. Untreated, SLE is often progressive and has a significant fatality rate. The most... |
The diagnosis of juvenile rheumatoid arthritis (JRA) is based on patient's age at disease onset, symptom duration, gender, and clinical manifestations. JRA is of unknown origin, begins under the age of 16, and persists for a minimum of 6 weeks. JRA is categorized into three principal types, systemic, oligoarticular and polyarticular. Infection, other connective tissue diseases, malignancy, trauma, and immunodeficiency... |
Self/non-self discrimination and unresponsiveness to self is the fundamental properties of the immune system. Self-tolerance is a state in which the individual is incapable of developing an immune response to an individual's own antigens and it underlies the ability to remain tolerant of individual's own tissue components. Several mechanisms have been postulated to explain the tolerant state. They can be... |
Since the tuberculosis (TB) in adolescents has unique clinical characteristics, special attention should be paid to this age group. Adolescents are more susceptible to developing TB disease and more likely to have cavitary pulmonary disease. Also, adolescent patients with TB more frequently present with extrapulmonary disease. We report three adolescents with active pulmonary and/or intestinal TB: one had pulmonary and... |
Osteopetrosis, a rare osteosclerotic bone disease characterized by a defect in osteoclast function and the reduced generation of superoxide by leukocytes, can be classified into several types based on their mode of inheritance, age of onset, severity, and associated clinical symptoms. Stem cell transplantation is the only curative therapy for the infantile malignant type, although alternative treatments, such as corticosteroids,... |
Epidural hematoma is relatively rare in newborn infants and frequently associated with instrumental deliveries or other complications during labor and delivery. Although surgical evacuation has been the most common therapy, many other procedures have been described. Although many epidural hematomas require surgical evacuation rather than nonsurgical management, conservative or aspiration of hematoma have been attempted. In the case of EDH... |
Purpose : Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM. Methods : Forty seven patients identified at Seoul National University Children's Hospital from January 1986... |
Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and... |
Purpose : Since 1998, school urinary screening tests have been performed on Korean school children. We could detect and treat so many asymptomatic chronic renal disease in early stage. We investigated the efficacy of school urinary screening tests from children with membranoproliferative glomerulonephritis (MPGN) type I. Methods : We analyzed the characteristics and prognosis of 18 patients with MPGN type I... |
Purpose : Acute symptomatic seizure is defined as a temporary seizure together with acute systemic, metabolic, or toxic insult in association with an acute central nervous system insult. And unprovoked seizure is defined as seizure without provocating factors. We studied the risk factors of unprovoked seizures after acute symptomatic seizure in children. Methods : We retrospectively reviewed the records of... |
Purpose : The of common carotid artery intima media thickness (IMT) is an acknowledged noninvasive marker for early atherosclerotic changes. We investigate whether common carotid IMT is different between obese and normal-weight children and also evaluate the relationships IMT with cardiovascular risk factors. Methods : We collected the clinical data (age, sex, pubertal stage, body mass index) and measured blood... |
Purpose : Radiofrequency catheter ablation (RFCA) has become an effective therapeutic modality for treating pediatric tachyarrhythmias. Using conventional RFCA catheters, ablation of parahisian accessory pathways may be difficult and have high risk for heart block. We reviewed the efficacy and complications of the RFCA in children and adolescent with arrhythmias including parahisian accessory pathways. Methods : We studied 48 patients... |
Purpose : The prevalence of childhood obesity has doubled over the last 30 years. Obesity-associated sequelae in the vasculature begins in the early stages of life. The purpose of this study was to investigate how pulse wave velocity (PWV) and ankle brachial index (ABI) change with height, weight and body mass index (BMI) in obese adolescents. Methods : Seventy-nine obese... |
Purpose : It has been described that the incidence of breastfeeding jaundice is 13% and that of breast milk jaundice is 2%. The incidence in Korea was believed to be higher, but there were no studies to prove this assumption. The purpose of this study was to investigate the incidence of jaundice of healthy breastfed full-term infants in Korea. Methods :... |
Purpose : The aim of this study was to determine whether improved survival of extremely low birth weight infants (ELBWI) was associated with decreased neurodevelopmental disability later in life, and also to identify the factors influencing this disability. Methods : ELBWI admitted to the neonatal intensive care unit of Samsung Medical Center, survived, and followed up until the corrected age... |
Purpose : Early identification of developmental disabilities allows intervention at the earliest possible point to improve the developmental potential. The Ages and Stages Questionnaire (ASQ), a parent- completed questionnaire, can be used as a substitute for formal screening tests. The purpose of this study was to evaluate the validity of the Korean version of the ASQ (K-ASQ) as a screening... |
Right-sided heart failure is a major problem among patients with congenital heart diseases, due to the prevalence of congenital heart defects and the association of pulmonary hypertension. More attention is focused on the structure of the right heart particularly in association with congenital heart defects and chronic lung disease. The right ventricle (RV) may support the pulmonary circulation, and sometimes... |
Myocarditis represent an important condition encountered by general pediatricians & general practitioners. Its presentation is varied, and therefore a high index of suspicion must be maintained when the possibility of myocarditis is raised. A progression from viral myocarditis to dilated cardiomyopathy has long been hypothesized. Treatment is initially aimed at achieving hemodynamic stability and is largely supportive. There is currently... |
Not a few patients in children and adolescents are suffering from right ventricular (RV) dysfunction resulting from various conditions such as chronic lung disease, left ventricular dysfunction, pulmonary hypertension, or congenital heart defect. The RV is different from the left ventricle in terms of ventricular morphology, myocardial contractile pattern and special vulnerability to the pressure overload. Right ventricular failure (RVF)... |
A pulmonary thromboembolism (PTE), which is a sudden blockage in a pulmonary artery, usually due to a blood clot, is rare in children. The clinical presentation is often subtle or masked by the underlying clinical condition and the condition must be suspected during clinical testing. Although the choice of treatment depends on the clinical presentation, anticoagulation is the mainstay of... |
Costello syndrome (CS) is a rare multiple congenital abnormality syndrome characterized by a typical coarse face, developmental delay, psychomotor and growth retardation, neurologic abnormalities, cardiac and cutaneous anomalies, severe feeding difficulties with postnatal growth failure, and increased risk of tumors. Since Costello first described it in 1971 and again in 1977, over 100 cases have been reported worldwide. It was... |
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