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Purpose : The product of the ob gene, leptin is an adipocyte specific hormone that might be involved in the regulation of food intake by the central neuroendocrine system. Recent studies reported that serum leptin concentrations were correlated with Body mass index(BMI) and other obesity measurements. The aims of this study were to evaluate leptin concentrations according to degree of... |
Purpose : This study was performed to compare the growth and neurodevelopmental outcome at 15 months of corrected age in very-low-birth weight infants between chronic lung disease(CLD) group and the control group. Methods : Very-low-birth-weight infants who were admitted and survived in the NICU(neonatal intensive care units) of Chonnam Univeristy Hospital from Jan. 1997 to Jan. 2000 were divided into... |
Purpose : Leptin, the product of the ob gene is primarily by adipose tissue but also by the human placenta. Leptin may also have a role as a regulator of fetal growth and development during normal pregnancy as well as in pregnancies associated with anomalous fetal growth. Our study aimed to discover whether or not leptin concentration in neonatal cord... |
Purpose : The aims of this study was to assess the indication, diagnostic, accuracy and prognostic influence of fetal echocardiography. Methods : From 1995 to 1999, 283 pregnancies were evaluated with fetal echocardiography at Dong-A University Hospital. We reviewed medical records included indication, fetal echocardiographic findings, postnatal echocardiographic findings, methods of treatment and outcome of prenataly diagnosed patients. Results : Family... |
Purpose : Pediatric cardiac arrhythmias are increasing but there is no normal values of cardiac electrophysiologic studies(EPS) in Korean children. So we performed cardiac EPS in children undergoing cardiac catheterization for mild congenital heart disease without cyanosis and ECG abnormality. Methods : We studied 24 children(male : 9, female : 15) with mild congenital heart disease(PDA : 10, VSD : 8,... |
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... |
Purpose : It has been reported that the Nitroblue Tetrazolium(NBT) test is more accurate than the urine pH, leukocyte esterase and nitrite test as a screening test of urinary tract infection (UTI). The purpose of this study is to compare the NBT test with other screening tests and evaluate the clinical usefulness of the NBT test as a screening test. Methods... |
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 : Serum levels of G-CSF and GM-CSF were measured and CFU-GM assay using G- CSF, GM-CSF and SCF was conducted to evaluate the influence of hematopoietic growth factor on the precursor cells of cyclic neutropenia. Methods : A 7-year-old male with cyclic neutropenia was studied. Marrow mononuclear cells were isolated at neutrophil nadir and recovery and cultured in methylcellulose media... |
Cutis laxa is a rare disorder of the elastic tissue characterized by loosely hanging and folded skin giving a premature senile appearance, often with internal organ involvement. Recently, we experienced a case of cutis laxa in a neonate. The patient who presented with dyspnea and loose skin at birth was delivered by Cesarean section in our hospital. He was the... |
Childhood onset nemaline myopathy, first described in 1963 by Shy, et al and Conen, et al, is rare congenital myopathy, characterized by nonprogressive or slowly progressive muscle weakness associated with rod-like structures in muscle fibers, often with dysmorphic features. This myopathy was confirmed by muscle biopsy. The light microscopic features noted generally small round fibers that showed variation in size... |
Chondrodysplasia punctata is a heterogenous skeletal dysplasia characterized by small focal calcifications in articular and other cartilages in infancy, with subsequent epiphysial dysplasia and associated anomalies of the face, eyes, and skin. Chondrodysplasia punctata is classified with autosomal recessive rhizomelic type, autosomal dominant nonrhizomelic type(Conradi-Hunermann type), and X-linked dominant type. These types have different clinical manifestations respectively. We report cases... |
Annular pancreas is a rarely found gastrointestinal tract malformation, and is frequently associated with Down syndrome. Endocardial cushion defect is a type of congenital heart disease, commonly also related to Down syndrome. However, a combination of endocardial cushion defect with annular pancreas has not been reported previously. We recently experienced such a case in 2 month old boy. Moreover Down... |
We report the case of a 4 year-old boy with Menetrier's disease, who was presented with edema and hypoalbuminemia. Gastroduodenal endoscopy showed the characteristic features of giant hypertrophy of the gastric rugae and large quantities of adherent gelatinous material. Histologic findings revealed foveolar hyperplasia. The presence of CMV infection was identified by serology. His symptoms resolved spontaneously, and he has... |
Fanconi-Bickel syndrome is a rare autosomal recessive disorder of the carbohydrate metabolism recently demonstrated to be caused by mutations in GLUT2, the gene for the glucose transporter protein 2 expressed in the liver, pancreatic β islet-cells, intestine and kidney. Typical clinical and laboratory findings of Fanconi-Bickel syndrome are hepatomegaly secondary to glycogen accumulation, glucose and galactose intolerance, fasting hypoglycemia, a... |
Hyperornithinemia-hyperammonemia-homocitrullinuria(HHH) syndrome is a rare autosomal recessive disorder caused by a defect in the urea cycle. Protein intolerance, mental retardation, seizure, ataxia, and stupor are characteristic symptoms. Patients showing these symptoms may also present symptoms of acute hepatic disease at the same time. When fed with a high protein diet, they may refuse to eat, vomit, become lethargic, or go... |