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Joubert syndrome is frequently associated with developmental delay and mental retardation, neonatal tachypnea and apnea, abnormal eye movements, and ataxia. It is inherited by an autosomal recessive trait. The most significant and constant neuropathological finding is partial or total agenesis of cerebellar vermis. Absence of the vermis results in a triangular-shaped mid-fourth ventricle and a "bat-wing" shaped fourth ventricle superiorly. The superior cerebellar peduncles... |
Purpose : Neonatal respiratory distress syndrome is caused by the deficiency of lung surfactant in premature babies. For the treatment of RDS at present surfactants such as Surfacten․ (Tokyo-Tanabe Co., Japan) and Exosurf․ (Wellcome Co., USA) are used. As awarded the grant from the Ministry of Science and Technology for a model research project of Medium-Technology program, we have modified (supplemented) the bovine lung... |
Purpose : In order to elucidate the neurotoxic mechanism of oxygen radicals which are pathological factor of ischemia, we evaluated the oxidant-induced neurotoxicity and the neuroprotective effect of antioxidant on cultured cerebral neurons derived from neonatal mouse. Methods : Neurotoxic effect was investigated after cultured mouse neuronal cells were exposed to oxygen radicals which were generated enzymatically by reaction of xanthine oxidase(XO) and hypoxanthine(HX). And also... |
Purpose : The immature neonatal immune system is thought to result in increased risk of infection. Receptors for the Fc moiety of IgG(FcγR) are important in antibody-mediated clearance of microbes by granulocytes and monocytes/macrophages. As an approach to understand their role in neonatal life, we compared the constitutive expression of the three Fc receptors-FcγRI(CD64), Fcγ RII(CD32) and FcγRIII(CD16)-of neonatal granulocytes with those of adult... |
Purpose : The purpose of this study was to standardize the somatosensory evoked potential(SEP) latency and amplitude of healthy preterm infants according to the postconceptional age. Methods : The median nerve SEPs were recorded in 30 healthy preterm infants who were followed up until 6 months and were neurodevelopmentally normal. Results : The mean peak latency and interpeak amplitude were following : P0=20.89±6.09(msec), N1=26.32±5.18, P1=31.16±6.59, N2=36.52±7.22... |
Purpose : This study was performed to examine the impact of gestational diabetes mellitus (GDM) on perinatal outcomes, especially macrosomia and metabolic complications in Korean women. Methods : We studied perinatal outcomes of 65 women with GDM who delivered a singleton infant and were not complicated by medical disease that might effect the fetal growth, 65 women with normal glucose tolerance were selected to match subject... |
Purpose : Initial drug resistance of children with tuberculosis is a useful performance indicator of tuberculosis control programme in the concerned country because it represented infection transmitted from adult patients with either primary or acquired drug resistance. But there have been a few studies available. The present study was made to know the incidence of drug-resistant tuberculosis in hopes of... |
Purpose : Most of the gastroenteritis due to viruses and some bacteria can be successfully managed by oral and/or intravenous fluid-electrolyte replacement and antibiotic therapy is unnecessary and even harmful. It is, however, not uncommon practice to prescribe antibiotics when acute gastroentritis is suspected. Therefore authors analysed the clinical courses of the patients treated for acute non-bacterial gastroenteritis in a university hospital to assess... |
Purpose : The value of serum lipid in children after recovery of Kawasaki disease may be important because of the predilection of this disease for the coronary artery. Methods : We measured serum high density lipoprotein(HDL)-cholesterol, total cholesterol, triglycerides in 22 patients (mean age 38months, range 6 to 93 months) with Kawasaki disease during 10 days or less after onset and 2 months later... |
Purpose : There is an urgent need for rapid and accurate diagnosis of childhood tuberculosis. Recently, developments in molecular biology have raised hopes about the possibilities of new strategies for tuberculosis diagnosis. Most of these methods have focused on the application of PCR to sputum samples from patients with suspected mycobacterial disease. We used a nested PCR to detect circuclating Mycobacterial tuberculosis DNA in... |
Purpose : We performed this study to determine the diagnostic significance and useful cut-off value of reticulated platelet, which can be used for discriminating the destructive thrombocytopenia from underproductive thrombocytopenia. Methods : We evaluated 37 patients with thrombocytopenia who were admitted to the Wonju christian hospital from March to July, 1995. All patients were evaluated with bone marrow megakaryocyte count. We divided them into two... |
Two families of dilated cardiomyopathy are reported. In the first family, two sons were affected. The elder child presented with congestive symptoms at the age of 5 months and was diagnosed to have dilated cardiomyopathy. The etiology of cardiomyopathy was thought to be idiopathic until his younger brother presented with near collapse at the age of 5 days and was diagnosed to have the... |
Familial Hypercholesterolemia is the most common hyperlipoproteinemia during the childhood, which occurs from the mutation of genes that regulates low-density lipoproteins(LDL), and is classified into two types, the homozygote presented abnormal genes from both parents and the heterozygote presented an abnormal gene from each parent. Type IIa familial hypercholesterolemia is characterized by, especially, increased level of LDL which is common type and normal level... |
Ischemic colitis is a common gastrointestinal disease not in childhood but in sixties decade. It' s developed due to ischemic injury of colon and classified to transient reversible form, chronic form and acute fulminant form. Uniquely Authors had experienced a case of ischemic colitis diagnosed as barium enema in childhood. This patient was brought to our hospital due to abdominal pain, abdominal distension and... |
Congenital pulmonary lymphangiectasia is a rare and perphaps underestimated cause of fatal respiratory distress in the neonate or infant. Pathologically, this condition is characterized by wide lymphatic channels in the subpleural and peribronchovascular spaces and the interlobular septa. Noonan syndrome is characterized by a phenotype similar to Turner syndrome but with a normal karyotype. Both pulmonary and intestinal lymphangiectasia have been reported in patients... |
Congenital megacalyces is a congenital renal disease characterized by hypoplasia of the medulla and a non-obstructive dilatation of the calyces. More than 100 cases have been reported in the literature after first described by Puigvert in 1963, but it has been reported only three cases(two cases of adults and one case of child) in the literatures in Korea. The etiology of the congenital megacalyces... |
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