| Typical hypoplastic left heart syndrome(HLHS) is a distinct pathologic entity with aortic atresia, mitral atresia, very hypoplastic or absent left ventricle and thread like ascending aorta. Occasionally, the lesser degree of hypoplasia is found and is called hypoplastic left heart complex(HLHC) by some authors. This HLHC is often associated with critical aortic stenosis. Fetal echocardiography has enabled us to observe... |
| Purpose : This study was undertaken to obtain basic data about the megakaryocyte colony formation of fetal liver cells by using immunocytochemical staining and ex vivo culture with growth factors. Methods : The mononuclear cells were isolated from fetal liver and bone marrow with idiopathic thrombocytopenic purpura(ITP) and pancytopenia. These mononuclear cells were cultured in MegaCultTM-C(Stem Cell Tech, Canada) media in... |
| Purpose : It is not clear that the development of glomerular injury and aggravation by tumor necrosis factor alpha(TNF-α) is related to intrarenal or serum concentration of TNF-α. So, we studied the relationship between the concentration of TNF-α and aggravation of glomerular damage in the Henoch-Schönlein nephritis(HSN) and idiopathic nephrotic syndrome(INS). Methods : We collected the sera and urines of... |
| Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with... |
| Purpose : Weight changes, especially weight gain, is a side effect of antiepileptics(especially valproate and carbamazepine). This may be sufficiently severe to cause noncompliance or to require the withdrawal of effective treatment. Unfortunately, the exact mechanism of weight change is not illustrated. Several reports and our experiment suggested that weight gain highly correlated with a familial tendency of obesity. The genetic makeup is a... |
| Purpose : A nationwide survey was conducted to investigate the most common causative organisms in neonatal sepsis in Korea. Methods : By reviewing medical records of newborn infants who were confirmed as neonatal sepsis by isolating organisms from blood culture during a one year study period from January to December in 1997, data for causative organisms, risk factors, accompanying focal infections and combinations of antibiotics... |
| Purpose : The acute scrotum is a clinical syndrome that is defined as an acute, painful swelling of the scrotum or its contents. The symptoms and signs of acute scrotum are similar and its pathognomonic features are rarely detected. This study was performed to find a proper way to diagnose and treat acute scrotum promptly and correctly. Methods : From January 1990 through December... |
| Purpose : We'd like to evaluate the relationship between the degree of cardiac damage and that of cardiac function according to the total injected dose of doxorubicin. Methods : 12 rabbits(body weight : 2.0-3.2 kg) were used and 30 mg/m2/week of doxorubicin hydrochloride was injected intravenously. The cardiac function was checked under anesthesia, after which the thorax was opened and cardiac samples were evaluated... |
| Wernicke,s encephalopathy(WE), a neurological disorder caused by thiamine deficiency, is characterized by the triad of ocular symptoms, ataxia, and mental confusion. More than 90% of the cases are observed in chronic alcoholics. Other conditions less frequently associated with WE are anorexia nervosa, prolonged parenteral nutrition, hemodialysis, uremia, hyperemesis gravidarum, gastroplasty for morbid obesity, and acquired immunodeficiency syndrome(AIDS). One of the... |
| Purpose : To describe clinical features of cyclic vomiting syndrome(CVS), managements during episodes, prophylactic drugs as long term therapy and to investigate the disease course and prognostic factors of CVS. Methods : Thirty two children who fulfilled the diagnostic criteria of CVS and who presented to Seoul National University Children's Hospital from March 1989 to December 2000, were included. Data were... |
| We report on two Korean siblings with multiple congenital anomalies : microcephaly, gyral abnormality, minor facial anomalies, and congenital nephrotic syndrome. The first infant developed proteinuria at age 3 days. This condition appeared similar to that described by Galloway and Mowat and reviewed by Cooperstone, et al, especially the presence of abnormal gyral patterns. She died at 19 months. The second infant; the brother... |
| Status epilepticus has been occasionally reported as a cause of acute disseminated intravascular coagulation(DIC), but little is known regarding the pathogenesis of this uncommon association. The occurrence of DIC in status epilepticus may be related to widespread endothelial damage secondary to seizure-induced hyperpyrexia or status epilepticus due to high fever. We experienced two cases of status epilepticus accompanying fever in which laboratory data demonstrated... |
| Malignant ectomesenchymoma is a rare tumor originating from remnants of migratory neural crest(ectomesenchyme) and composed of neuroectodermal as well as mesenchymal components. Neuroblasts and ganglion cells constitute the neuroectodermal components and rhabdomyosarcoma is the most frequently encountered mesenchymal components. We report a case of malignant ectomesenchymoma in a two-month-old boy who was presented with abdominal pain and urinary difficulty. The... |
| Purpose : To evaluate the usefulness of magnetic resonance cholangiography(MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods : Fifty consecutive infants with cholestatic jaundice underwent single-shot MRC for 3 years. The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. The diagnosis of biliary atresia was... |
| Purpose : The purpose of this study was to evaluate M-mode echocardiographic abnormalities in infants of relatively well controlled diabetic mothers and to determine the timing of spontaneous regression. Methods : Echocardiographic study was prospectively carried out in 9 neonates born to diabetic mothers(infants of diabetic mothers, IDM) and 6 newborn infants(control) matched for weight and age. And the study were... |
| Intravenous immunoglobulin(IVIG) has been widely used to treat idiopathic thrombocytopenic purpura in childhood. Aseptic meningitis has been reported as a rare complication of IVIG therapy. This report is on an 11 year-old boy with ITP who suffered from aseptic meningitis following the administration of IVIG. He was given 1 g/kg of IVIG for 2 days, and on the fourth day... |
| Purpose : Long-term administration of anticonvulsants for pediatric epileptic patients may cause changes of bone metabolism. This study was performed to evaluate the influence of antiepileptics on the bone metabolism and growth in pediatric patients who received antiepileptics. Methods for early detection of adverse bone metabolism were also evaluated. Methods : Forty-eight epileptic patients were enrolled in this study.... |
| The 49, XXXXY Klinefelter variant is distinctive; classic findings include mental retardation, hypogonadism and radioulnar synostosis. Its incidence is estimated to be 1/80,000 to 1/100,000 male births. We report a case of 49, XXXXY syndrome confirmed by chromosomal study in neonatal period, whose clinical features included a coarse face, short neck, micropenis, and other multiple minor anomalies including simian creases.... |
| Purpose : It is very important to study the individual risk factors which cause periventricular leukomalacia(PVL) and those which affect the process of cystic formation from liquefied periventricular white matter, in order to prevent and permit early diagnosis of cystic PVL that contributes to mortality and neurologic sequelaes in preterm infants. Therefore, the present study was designed to assess the... |
| Five to fifteen percent of patients affected by human immunodeficiency virus(HIV) are children in developing countries. In Korea, most of HIV infections in children have been transfusion-related, and cases of vertical transmission have been very rare so far and are usually suspected due to a maternal positive history. We experienced a case of vertical transmission of HIV in a 19... |
| Purpose : Widespread use of MRI now gives us increased insights into the different expressions of cortical malformations. We carried out this study to characterize the clinical and EEG features of focal cortical dysplasia(FCD) which also caused intractable epilepsy requiring surgical treatment. Methods : A retrospective analysis was conducted in 8 children. The history of seizures, imaging studies, electroencephalographic findings,... |
| Purpose : The purpose of this study was to evaluate the hemodynamic effect of transposition of the great arteries(TGA) on neuro-development by measuring the cerebral metabolites before and 1 year after open heart surgery(OHS) by localized in vivo 1H-magnetic resonance spectroscopy(1H- MRS) along with Bayley scales of infants development II(BSID II). Methods : Full-term newborns(N=13) with TGA and intact ventricular septum... |
| Purpose : Williams syndrome(WS) is an autosomal dominant disorder resulting from a submicroscopic deletion of contiguous genes on the long arm of chromosome 7. We evaluated the physical, neurodevelopmental, and behavioral characteristics of children and adults with WS confirmed by fluorescence in situ hybridization. Methods : The clinically suspicious twelve cases with cardiac anomaly were confirmed as WS with fluorescence in... |
| Purpose : We'd like to know the relationship between the changes of cardiac function and systemic O2 consumption according to the increasing dose of dopamine. Methods : Ten rabbits(from 2kg to 2.8kg) were used in this experiment. Anesthesia was induced with intraperitoneal pentobarbital sodium(35mg/kg) and tracheostomy was done. It was maintained by ventilation with a mixture of 1-3% halothane and 67-69%... |
| Purpose : The aims of this study are to investigate the results of balloon aortic valvuloplasty (BAV) in congenital aortic stenosis(CAS) and, especially, to compare the results between BAV performed before two months of age(Group A) and BAV after two month of age(Group B). Methods : From January 1993 to June 2000, 14 patients who were diagnosed as CAS were treated... |
| Purpose : Hemolytic uremic syndrome(HUS) is the most common cause of renal failure in childhood in western countries, but it has been a relatively rare disease in Korea. To analyze the clinical and epidemiologic characteristics of HUS in Korea, we reporte our experience. Methods : We reviewed retrospectively the medical records of 9 patients who were diagnosed the hemolytic uremic syndrome... |
| Purpose : To asses the short-term effects of the topical corticosteroid, budesonide administered by metered dose inhaler(MDI) and spacer(Aerochamber MV 15) following systemic corticosteroid in premature babies with respiratory distress syndrome at birth. Methods : 19 premature babies <1800gm were randomly assigned to receive steroids(n=9) or not (n=10). The steroid group(n=9, GA[gestational age]=29.6¡¾2.9 weeks, BW[birth weight]=1.29¡¾0.35kg) received systemic dexamethasone for 48... |
| Purpose : The identification of antigenic specificity of Streptococcus pyogenes using T serotyping is important to understand biologic characteristics of microorganisms. We would like to disover the association of the occurrence of predominant T type, with possible outbreak of erythromycin resistant Streptococcus pyogenes in this country, which has been documented since the late 1990s. Methods : Throat swab cultures were taken... |
| Caffey`s disease(infantile cortical hyperostosis) is an uncommon benign and self limited disorder of unknown etiology, affecting the skeleton and its contiguous fasciae and muscles. Its occurrence in isolated cases or in multiple members in families suggests the existence of two different forms, namely a sporadic form and a familial form. The tibia is the predominant bone known to be affected... |
| Purpose : To determine the incidence, etiology, sex, age, seizure type and prognosis of acute symptomatic seizures in children and evaluate the hypothesis that acute symptomatic status epilepticus( SE) is associated with an increased risk of subsequent unprovoked seizure compared with the risk of acute symptomatic seizure without SE. Methods : Five hundred and sixty-eight convulsive children visited the Pediatric Department of Chungbuk National University... |
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