Familial Mediterranean fever (FMF) is the most common Mendelian autoinflammatory disease, characterized by uncontrolled activation of the innate immune system that manifests as recurrent brief fever and polyserositis (e.g., peritonitis, pleuritic, and arthritis). FMF is caused by autosomal recessive mutations of the Mediterranean fever gene, |
This study aimed to evaluate the correlation, according to postnatal age, between plasma B-type natriuretic peptide (BNP) levels and echocardiographic parameters for the assessment of patent ductus arteriosus (PDA) in preterm infants with respiratory distress. We enrolled 42 preterm infants with respiratory distress who underwent serial echocardiographic evaluation with simultaneous plasma BNP measurements until ductal closure. The correlations between BNP levels... |
Nephrogenesis is normally accompanied by a tightly regulated and efficient vascularization. We investigated the effect of angiotensin II inhibition on angiogenesis in the developing rat kidney. Newborn rat pups were treated with enalapril (30 mg/kg/day) or vehicle (control) for 7 days after birth. Renal histological changes were checked using Hematoxylin & Eosin staining. We also investigated the intrarenal expression of vascular... |
Purpose:To investigate the effects of angiotensin II inhibition on the epithelial to mesenchymal transition (EMT) in the developing kidney, we tested the expression of EMT markers and nestin in angiotensin converting enzyme (ACE) inhibitor-treated kidneys. Methods:Newborn rat pups were treated with enalapril (30 mg/kg/d) or a vehicle for 7 days. Immunohistochemistry for the expression of α-smooth muscle actin (SMA), E-cadherin, vimentin, and nestin were... |
Purpose : We aimed to investigate the efficacy of and functional recovery after intracerebral transplantation of different doses of mouse mesenchymal stem cells (mMSCs) in immature rat brain with hypoxic-ischemic encephalopathy (HIE). Methods : Postnatal 7-days-old Sprague-Dawley rats, which had undergone unilateral HI operation, were given stereotaxic intracerebral injections of either vehicle or mMSCs and then tested for locomotory activity in... |
SmithMagenis syndrome (SMS) is a rare disorder with multiple congenital anomalies caused by a heterozygous interstitial deletion involving chromosome 17p11.2, where the retinoic acid-induced 1 (RAI1) gene is located, or by a mutation of RAI1. Approximately 90% of the patients with SMS have a detectable 17p11.2 microdeletion on fluorescence in-situ hybridization (FISH). SMS is characterized by mental retardation, distinctive behavioral... |
Purpose : Hypertension (HTN) is no longer an exclusively adult disease; the prevalence of pediatric HTN is increasing. To understand the evaluation and treatment of childhood HTN in Korea, we investigated, via a questionnaire, how hypertensive children are currently assessed and managed by pediatric cardiologists (CA) and nephrologists (NE). Methods : We surveyed 82 pediatric CA and 77 NE, regarding... |
Purpose : Herbal medicine is thought to be widely used by children with epilepsy, but there have been few studies. This study aimed to investigate the perceptions and the actual conditions of usage of herbal medicine by children with epilepsy. Methods : From July to August 2007, three hundred seventy eight questionnaires were completed by parents of epileptic children who visited... |
Purpose : Transcatheter closure of atrial septal defects (ASD) is currently established therapy as an alternative to surgery. But rarely, complications are reported in some studies. We report early and intermediate term complications associated with transcatheter closure of atrial septal defects using the Amplatzer septal occluder (ASO). Methods : From June 2003 to May 2006, 64 patients underwent transcatheter closure of... |
Purpose : Oxcabarzepine (OXC), newly recommended antiepileptic drug, has been prescribed for patients with partial seizures and generalized tonic clonic seizures in Korea from 1999. There are limited reports about an efficacy of OXC therapy in epileptic children in Korea. This study evaluated the efficacy and safety of OXC in the light of our experience. Methods : The patients, who had... |
Purpose : This study was designed to clarify the mechanism of proteinuria in nephrotic syndrome patients by using puromycin aminonucleoside (PAN) nephrosis model. Methods : Following administration of various concentrations of PAN and antioxidants we observed the changes of podocyte cytoskeletons in cultured rat glomerular epithelial cells (GEpC) by method of scanning electron microscope, reactive oxyten species (ROS) analysis, permeability assay,... |
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 : Obesity has become a pandemic and especially the prevalence of childhood obesity has increased remarkably over the past years. Childhood obesity is often accompanied by the expected morbidities such as hyperlipidemia, fatty liver and glucose intolerance. We evaluated the pattern of alteration in the body mass index (BMI), serum adiponectin concentration, insulin resistance (HOMA-IR), and complication rates after... |
Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea Purpose : Vascular endothelial cell damage and alteration of a fibrinolytic system was suggested to play a role in the development of coronary artery abnormalities in Kawasaki disease (KD). D-dimer is one of the markers of endothelial damage and fibrinolysis. We evaluated the clinical usefulness of D- dimer to differentiate KD... |
Purpose : This study was conducted to compare the efficacy and safety of ibuprofen and indomethacin in the treatment of patent ductus arteriosus (PDA) in preterm infants and to determine whether ibuprofen can be an alternative drug. Methods : A total of 32 preterm infants with symptomatic PDA were enrolled in the study. Twelve infants received intravenous ibuprofen 10 mg/kg, followed... |
Purpose : This study aimed to evaluate the usefulness of auditory evoked potential (AEP) in clarifying neuronal development in premature small for gestational age (SGA), and appropriate for gestational age (AGA) infants. Methods : A total of 183 premature infants who were born from August 2002 to July 2005, were examined with AEP. They were divided into three groups; AGA, symmetric-SGA... |
Purpose : The comatose mentality can be catastrophic, especially if the condition is severe or the duration is prolonged. Therefore, delayed diagnosis can result in a poor outcome or death. The best radiologic modality to differentiate from cerebral lesions in patients suffering from cerebral diseases is magnetic resonance imaging (MRI) rather than computed tomography (CT). Special apparatuses with metal... |
Necrotizing enterocolitis(NEC) is the most common life-threatening surgical emergency in neonates, and remains a major cause of morbidity and mortality. In addition to conventional laparotomy, intraperitoneal drains have been used for the treatment of perforated NEC, especially in extremely low birth weight(ELBW) infants. We report a case of perforated NEC with bacterial peritonitis in an ELBW infant managed with primary... |
Purpose : This study aimed to evaluate the usefulness of Korean Infant Development Screening Test in predicting delayed development in premature infants. Methods : A total of 74 children with histories of prematurity, who visited the pediatric neurology clinic of Korean University Ansan Hospital from August, 2002 to July, 2004, were examined, using the Korean Infant Development Screening Test. They were... |
The deficiency of enzyme hypoxanthine-guanine phosphoribosyltransferase(HPRT) results in hyperuricemia and subsequently manifests in diverse symptoms. Lesch-Nyhan syndrome is a disorder characterized by hyperuricemia, mental retardation, choreoathetosis, spasticity and self-mutilation, resulting from complete deficiency of the enzyme, whereas partial deficiency of the enzyme shows symptoms of milder forms more often without abnormal neurologic signs. A 7-year-old boy with normal growth and... |
Purpose : Unilateral ureteral obstruction(UUO), a well established experimental model of renal injury, gives rise to tubulointerstitial fibrosis, tubular dilatation and cellular atrophy. Angiotensin(ANG) II may take the prime role in the regulation of this response. The objectives in the current investigation were to determine whether the renal response to UUO involves the dedifferentiation of tubular epithelial cells to mesenchymal... |
Purpose : Long-term prognosis of Henoch-Sch nlein purpura(HSP) is determined by the existence and severity of renal involvement. We evaluated the relationship between various clinical features of HSP and the development of renal involvement using univariate and multivariate analyses for early detection and proper management of HSP nephritis. Methods : We performed a retrospective study of 200 children who were diagnosed... |
Intestinal lymphangiectasia, one of the protein-losing gastroenteropathies, is an uncommon disease characterized by dilated intestinal lymphatics, enteric protein loss, edema, hypoalbuminemia, and lympocytopenia. Small bowel biopsy and CT have been used to confirm the diagnosis of intestinal lymphangiectasia. Small bowel biopsy shows collections of abnormal dilated lacteals in submucosa with distortion of villi and CT findings have been described as... |
Purpose : This study aimed to evaluate risk factors of the first year relapse in children with nephrotic syndrome(NS) without the need for biopsy. Methods : We reviewed, retrospectively, 78 children diagnosed with steroid responsive nephrotic syndrome between July 1997 and June 2002. Median years to follow up were 4.4 years(range : 1-5 years). We divided the patients into two groups(group... |
Purpose : In order to investigate the role of endothelins in the cardiac development, the present study was designed to examine the effects of endothelin A receptor(ETAR) antagonist to the cellular proliferation and apoptosis in the neonatal rat heart. In addition, the expression of various regulatory genes in protein and mRNA levels by ETAR antagonist were examined. Methods : Neonatal... |
Purpose : Urinary tract infection(UTI) is the most common bacterial infectious disease that may induce severe renal injury unless early diagnosis and appropriate treatment are performed. If recurrent UTI is prevented, renal injury can be also reduced. Therefore, we studied the risk factors of recurrent UTI in children. Methods : We performed a retrospective study of 168 children(58 girls and 110... |
Purpose : To determine the postnatal changes in aldosterone action on the renal tubular reabsorption in low birth weight(LBW) infants, we assessed the relation of the aldosterone concentrations to renal parameters during the first 10 days of life. Methods : Twenty LBW infants were evaluated and their gestational ages ranged from 32.4 to 39.3 weeks and their birth weights ranged from... |
Purpose : This study was aimed at finding a diagnostic tool which is useful in predicting the neurologic outcome of the asphyxiated newborns. Methods : 31 newborns with asphyxia were evaluated by brainstem evoked potentials and cranial ultrasonography(CUS). During the neonatal follow-up, we evaluated the development of the babies with Bayley Scale of Infant Development II and INFANIB on corrected age... |
Purpose : This study was undertaken to investigate changes in cardiac performance in early neonatal life by observing the changes of left ventricular(LV) function and volume according to the size of ductus arteriosus. Methods : The study group consisted of 21 full-term Cesarean section neonates who were admitted to the nursery of Korea University Hospital. We serially investigated the... |
Purpose : Diagnosis of a hemodynamically significant patent ductus arteriosus(PDA) that should be treated is difficult to determine by clinical and echocardiographic examinations. The aim of this study was to clarify the interrelationship of plasma atrial natriuretic peptide(ANP) concentrations and clinical signs or echocardiographic signs of PDA in preterm infants and use plasma ANP concentrations as a diagnostic indicator of a hemodynamically significant PDA. Methods... |