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... |
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 : To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). Methods : Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated... |
Purpose : It has been shown that a considerable number of acute myeloid leukemia(AML) patients had both myeloid and lymphoid antigens. CD7 is a lymphoid antigen, often expressed in AML cells. We investigated 40 AML patients to clarify the relationship between the cellular characteristics and clinical features. Methods : We reviewed 8 CD7 positive(CD7+) AML patients and 32 CD7 negative(CD7-)... |
Purpose : We evaluated the response to immunomodulation therapy, long-term survival and relapse rate of aplastic anemia during follow-up for more than 1 year. Methods : Twenty-eight children, with moderate to severe aplastic anemia were followed for more than one year and 7 children expired after therapy, were analyzed. Antilymphocyte globulin(ALG) or antithymocyte globulin(ATG) by itself was given to 27 patients, and cyclosporine A(CsA)... |
Purpose : The release of histamine from human basophils is controlled by an intrinsic, as yet unidentified, cellular property termed “releasability.” We carried out this study to ascertain whether there was any difference in the releasability of basophils from asthmatic children compared to those from normal children. We intended also to clarify the correlation between the releasability and the atopic status of asthma. Methods :... |
Autoimmune hepatitis in children is a rare and severe inflammatory disease of unknown etiology, and progress to cirrohosis and liver failure, generally is responsive to immunosuppressive therapy. It is more prevalent in women than men, and characterized by the presence of circulating autoantibodies, a high serum globulin. Extrahepatic manifestations such as thyroiditis, ulcerative colitis, glomerulonephritis and autoimmune hemolytic anemia, are associated. We report, to our... |
Splenic infarction is a rare disease and usually detected as a complication of SBE, atrial fibrillation, hemoglobinopathy, or myeloproliperative disorders. Although thrombotic event, such as deep vein, cerebral or renal thrombosis, is a frequent complication of SLE, there have been only a couple of cases documenting splenic infarction complicated in SLE. This is, to our knowledge, the first case report... |
Purpose : The purpose of this study was to observe the effect of IFN-γ and PGE2 on TNF-α production by human peripheral mononuclear cells(PBMC) that were stimulated with LPS. Methods : PBMC were separated by Ficoll-Hypaque gradient centrifugation from human peripheral venous blood and were incubated for 72 hours with or without LPS, IFN-γ, PGE2, and indomethacin according to various conditions. TNF-α activity of... |
To analyse the age of onset, etiologic microorgnisms, clinical manifestations, managements and genetic variation of chronic granulomatous disease, the authors reviewed four patients who were diagnosed as CGD at Department of Pediatrics, Seoul National Univesity Children's Hospital. They were siblings in relationship-two of them were brothers, the others wete brother-sister. The results were as follows; 1) Initial manifestations developed within 1... |
We experienced 3 cases of Candida esophagitis in infancy which were diagnosed by esophageal endoscopy, First case, 10 month-old boy with combined immune deficiency had suffered from oral thrush and poor feeding for more than 4 months. Esophageal endoscopy revealed multiple whitish creamy patches on the friable erythematous and necrotic mucosa of the esophagus, He was firstly treated with amphotericin-B... |
Rheumatoid factor is a commonly used laboratory test by clinicians to assess the children with possible juvenile rheumatoid arthritis. To assess its diagnositc value and clinical significance we reviewed the case histories of the patients in whom latex agglutinating rheumatoid factor was tested during November 1988 to April 1991 at Seoul National University Children's Hospital. There were 61 patients with... |
The one-hundred thirty five cases with Kawasaki disease who wre diagnosed and treated at the department of pediatrics, Seoul National University Children뭩 Hospital from September 1984 to December 1990, were investigated for clinical pattrns and laboratory correlations. The results were as follows; 1) Most patients (86%) were under 4 years of the age. 2) boys wee more prevalent than girls, as a ratio... |
To determine the safety and efficacy of varicella vaccine, we studied 10 healthy children and 12 children in remission from acute lymphoblastic leukemia. They were immunized with the Oka strain of live attenuated varicella vaccine (Biken®). All the healthy children and 6 children with leukemia received the vaccine of 1,000 plaque-forming units (PFU), and 6 leukemic children received the vaccine of 500 PFU. The... |
A reversible syndrome resembling systemic lupus erythematosus is induced by hydralazine which has been used in treatment of hypertension or congestive heart failure. The incidence of the lupus syndrome induced by hydralazine was estimated at 6.8% in adults (Cameron, 1984) but there was few reports in children. A 9 year old girl had arthralgia, fever, splenomegaly, pleurisy and oral ulcer after 62 months of hydralazine... |
We made a clinical study on 31 cases of histiocytosis X who had been admitted to the Dept, of Ped. of S.N.U.C.H. from Jan. 1981 to Dec. 1987. Among 31 cases, 28 patients received chemotherapy to include prednisolone, vinblastine, methotrex- ate and cyclophosphamide as induction therapy, and 6-mercaptopurine, methotrexate and cyclophos- phamide as maintenance therapy. The results obtained were as follows. 1) The sex incidence revealed male... |
We have experienced a case of congestive cardiomyopathy in a 4-year-old boy who had progressive cardiomegaly, congestive heart failure and weakness of right upper and lower extremities. On necropsy, the heart showed hypertrophy of cardiac muscle with focal granular degeneration. Clinical course of the case was reported with review of literature. |
A 1.5 month-old male with rapid and irregular pulse rate had an uncommon form of atrial tachycardia. The electrocardiogram was compatable with the diagnosis of chaotic atrial tachycardia or multifocal atrial tachycardia. The patient was treated with digoxin. |
A case of Morgagni hernia in 3day old newborn is reported with literature review. The patient developed tachypnea and respiratory difficulty on 3rd day of life and the diagnosis was made by diagnostic pneumoperitoneum. |
Birth weight and gestational age are two important factors in assessing the neonatal mortality and morbidity. We collected the birth weights of 10,242 newborns who were born at Ewha Womans University Hospital from June, 1976 to June, 1981, including birth weight and gestational age. Mean birth weight with standard deviation for each gestational age and fetal growth curve are presented... |
The hyaline membrane disease is not a common disease in Korea. Only a few reports of small scale are avaiable in the literature. We have experienced 5 cases of HMD during approximately 1 year period. The diagnosis was made either on characteristic clinical and roentgenological features or postmortem examination. The birth weights of these cases were in the range of... |
A childhood case of moyamoya disease with subarachnoid hemorrhage is reported. This patient exhibited left hemiparesis, dysarthria, convulsion and mental retardation. He also had characteristic angiographic picture: occlusion of supraclinoid segment of both internal carotid arteries, combined with collateral networks in the base of brain. |
Congenital hepatic fibrosis is a relatively rare liver disease in children and young adults,that is characterized by stony hard hepatomegaly and portal hypertension with relative preservation of liver function and underlying architecture. Since this Condition was first delineated by Kerr et al in 1961, approximately over 150 cases have been reported in the literature. However, congenital hepatic fibrosis was not... |
A hemorrhagic disease due to prothrombin complex deficiency os described in 4 infants from 1 to 11 months of age. This disease is different from the hemorrhagic disease of the newborn. The main differences are that it is found in an older age group and that intracranial hemorrhages are frequent. Rapid clinical and laboratory response after vitamin K therapy are... |
Correlation between electrocardiographic findings and hemodynamics in 108 children with patent ductus arteriosus, confirmed by cardic catheterization at SNUH, was evaluated. The results were as follows: 1. Age at diagnosis: patients(75/108) were before 10 years old, but about one third of patients(33/108) were over 10 years old. 2. Sex distribution: M:F=1:2 3. Correlation between ventricular hypertrophy pattern by ECG and... |
Statistical analysis for mortality of in-patient in pediatric ward in Seoul National University Hospital was carried out during the 3 years from Jan. 1974 to Dec. 1976. Total number of admitted patients was 2,234, of whom 172 expired. The following results were observed. 1. Total number of admission during the 3 years was 2,234, and male to female ratio was... |
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