Purpose : We reviewed 100 cases of HLA-matched sibling allogeneic bone marrow transplantation( allo-BMT) in children and wish to share these results. Methods : One hundred children had undergone allo-BMT from HLA-identical siblings between Nov. 1983 and May 1998. There were 50 males and 50 females with a median age of 10 years and a median follow-up of 38 months. Out of 100 cases,... |
Purpose : Antineutrophil cytoplasmic antibody(ANCA) has been identified in various disorders including Wegener' s granulomatosis, microscopic polyarteritis and Kawasaki disease. Measuring this antibody has a diagnostic role. It facilitates monitoring disease activity and may also help understand the pathogenesis of the diseases in which it is found. We investigated the correlation between the hematologic findings and ANCA in acute Kawasaki disease and the diagnostic potential... |
Purpose : It is increasingly important to diagnosis asymptomatic infections which make up a majority(90%) of congenital cytomegalovirus(CMV) infections and that they may have sequeles such as sensorineural hearing loss and mental retardation. Recently antigenemia assay has been developed by using monoclonal antibodies against early structural protein pp65 of CMV. This CMV antigenemia assay seems to be more quicker to... |
von Gierke disease(type Ia glycogen storage disease) is an inherited disease associated with accumulation of glycogen in the liver, kidney, intestine and erythrocytes due to the defect of glucose-6-phosphatase activity. Hepatomegaly, doll face, anemia, bleeding tendency and increased susceptability to infection are common features observed during infancy. Hypoglycemia especially fasting hypoglycemia is typical metabolic derangement in this disease, followed by metabolic acidosis, lactic acidemia, hyperlipidemia, hyperuricemia,... |
Pena-Shokeir syndrome is a rare, often lethal disease, characterized by intrauterine growth retardation, craniofacial anomalies, limb ankylosis, polyhydramnios and pulmonary hypoplasia. This autosomal recessive disease should be differentiated from trisomy 18, which the second most common multiple congenital malformation syndrome. It is therefore clear that the two syndromes have certain features in common, the most consistent being craniofacial and limb abnormalities and intrathoracic pathology. Therefore,... |
Purpose : The pertussis vaccines are divided into whole-cell vaccine and acellular vaccine. Purified component acellular pertussis vaccine, originally developed in Japan, has been used for primary immunization series in Korea since 1984. We performed this study to evaluate the prevalence of pertussis antibodies following vaccination with acellular pertussis vaccine. Methods : We randomized 80 children who were immunized according to vaccine schedules... |
Purpose : The diagnosis for chromosomal anomality has been made by obtaining cells in metaphase from peripheral blood and fresh tissue culture. But this method is difficult to perform because somewhat invasive and necessary time for cell culture and staining(48-72hr). Recently, the method to find numerical abnormalities in, autosome and sex chromosome of adult buccal mucosal smear by FISH was... |
Between 1986 and 1990, four children with recurrent CNS leukemia who had previous NS prophylaxis therapy were treated with intermittent central nervous system irradiation and intrathecal chemotherapy (IIC). There was no isolated CNS recurrence. One patient died from bone marrow relapse. Three patients are alive without evidence of disease for 33/12 year to 36/12 years after the diagnosis of recurrence... |
We performed this study to evaluate the therapeutic responses of bone marrow transplantation and immunosuppressive therapy for aplastic anemia in 103 cases(68 severe and 35 moderate). Among them, 15 cases with severe aplastic anemia having HLA-identical sibling donor underwent bone marrow transplantation, and cases (53 severe and 35 moderate cases) without available HLA-identical sibling donor had received immunosuppressive therapy using... |
Bile peritonitis due to spontaneous perforation of choledochal cyst is a rare disease and the etiology of spontaneous perforation is unknown in most of infant cases. Recently, we experienced a case of bile peritonitis caused by spontaneous perforation of choledochal cyst in a 6 month-old female infant. She had progressive abdominal distention with ascites, mild jaundice and intermittent passage of acholic... |
In Kawasaki disease, coronary arterial involvement is an important prognostic factor. Many studies have reported about risk factors of coronary artery disease in Kawasaki disease. This study was designed to evaluate factors influencing coronary arterial involvement and to evaluate the effect of intravenous gammaglobulin (IVG) when admitted on the widely used criteria in Korea. Fiftyfour patients meeting the CDC criteria... |
A Unilateral Absence of Pulmonary Artery(UAPA), which develops occasionally as one of the associated anomaly in congenital cardiovascular defect like as tetralogy of Fallot, is rare anomaly as an isolated congenital defect. We have recently experienced a case of UAPA in a 9 years old boy, which was diagnosed by perfusion scan, digital subtraction angiography. So we report this case... |
Twenty, six children younger than 15 years with severe aplastic anemia underwent either bone marrow transplantation or immunomodulation therapy (antilymphocyte globulin with cyclosporin A therapy). Six patients who had an HLA-identical sibling donor underwent bone marrow transplantation after conditioning with cyclophosphamide, procarbazine and antilymphocyte globulin. Twenty children who did not have an HLA-identical donor recieved antilymphocyte globulin and cyclosporin A. The results were as follows; 1) Hematologic responses:... |
Immunoglobulin (Ig) and T cell receptor 0 chain (TcR分) gene rearrangements can be used as ”genetic marker” of lineage and clonality in the study of lymphoproliferative disease. We analyzed genomic DNA of the bone marrow mononuclear cells from twenty six patients with acute leukemia in children. The patients were seventeen cases of acute lymphocytic leukmia (ALL), seven cases of acute nonlymphocytic leukemia (ANL) and... |
The hybrid acute leukemia is defined as acute leukemia with both myeloid and lymphoid features. We experienced and report two cases of hybrid leukemia which were identified by morpholgy, cytochemical stain, reactivity with monoclonal antibody, analysis of immunoglobulin and T cell receptor genes rearrangement in 14 years old boy and 10 years old girl. |
A retrospective study of 10 years* experience with surgical treatment of congenital heart disease in the St. Mary’s Hospital from 1978 to 1987 was made. Data were analyzed on 188 children with congenital heart disease who had received corrective heart surgery under the age of 18 years. The results were as follows: 1) Out of 188 patients, ventricular septal defect(54.2% of all) was the most... |
Acute rheumatic fever and rheumatic carditis were still important disease of the acquired heart disease in children and adults, in spite of improving socioeconomic status in Korea. Recently, many outbreaks of acute rheumatic fever have been reported in the papers from developed countries. We should be aware of such outbreaks of acute rheumatic fever and should pay attention to their treatment of the patients... |
The Korean pediatric association is conducting the analysis of disease patterns of children by the body organ system. For the purpose of assessing the disease patterns in patients of the pediatric age in Korea, we performed a statistical analysis of diseases of patients admitted to the department of pediatrics in 36 hospitals having pediatric residency programs in 1987. The results obtained were as follows, 1) Total... |
We recently experienced a case of systemic-onset juvenile rheumatoid arthritis with protracted and variable clinical course with multiple complications and sequeles including pericarditis, disseminated intravascular coagulation, severe ankylosis and bony fusion of neural arches of C2 through C6 vertebrae, and multiple chronic arthritis causing joint deformities at relatively young age of nine. A brief review of related literatures is also presented. |
Pulsed Doppler echocardiography, a sensitive and specific non-invasive method enabled the various congenital and acquired heart diseases to be detected sine 1970,s For determining the time of functional closure of ductus arteriosus, 30 normal newborn infants were examined by pulsed wave Doppler (PWD) from March 1 to April 30, 1987, in Holy Family Hospital, Catholic Medical Center. A PDA was diagnosed when abnormal diastolic ductal... |
Statistical analysis of pediatric diseases in patients admitted to the department of pediatrics in 34 hospitals having pediatric residency program for 2 years from Jan. 1985 to Dec. 1986 was underwent for the purpose of assessing the disease pattern in the pediatric aged people in Korea. The results obtained were as follows 1) Total number of inpatients was 112,309, of which 69,266 were male and... |
We observed the patients admitted to the department of pediatrics of St. Mary*s Hospital, Catholic Medical College during the past 20 years from Jan. 1966 to Dec. 1985 and classified them by year and disease according to the International Classification of Disease by W.H.O. (1965 Revision). The results were as follows: 1) The total number of pediatric inpatients during 20 years was 18,686, of which... |
Pulmonary arteriovenous fistula is a congenital vascular malformation in lung resulting from abnormal capillary development and thus unoxygenated, desaturated arterial blood enters into the pulmonary venous system and results in various symptoms such as exertional dyspnea, cyanosis, clubbing fingers and secondary polycythemia. The exact etiology of pulmonary arteriovenous fistula is not well known but it may occur with or without hereditary... |
A 8-year old boy with acute lymphocytic leukemia who had shown resistance to repeated combination chemotherapies, was successfully grafted with bone marrow from his HLA- matched sister. He was in the third partial remission at the time of bone marrow transplantation. He was conditioned with cyclophosphamide 60 mg/kg/day on two consecutive days, followed by 800 rads total body irradiation. Marrow... |
Mucocutaneous Lymphnode Syndrome (MCLS) becomes familiar to pediatrician recently as a new disease entity. However, the pathogenesis of this syndrome is not know yet. Since Dr. Kawasaki’s first cases had reported in 1963, over 30,000 cases of this disease has "been reported in Japan. This study was undertaken to know the epidemiologic status of this dis- ease in this country. The materials were collected from... |
Left ventricular-right atrial shunt is a relatively uncommon defect, which permits as communication between the left ventricle and right atrium. This paper is fifth case report in our country. This patient was noted to have congenital heart disease in two years old. The pathology of this lesion was classified into four types by Perry. The type C is compatible with... |
This study was attempted to know the recent changes of the patterns of diseases among Korean children living inurban and rural areas. 21,498 sick-children, who admitted to the thirteen general hospitals which located in urban and rural areas during past five years from Jan. 1994 to Dec. 1978, were analysed for this purpose. 13,759 sick-children(64.0%) were male, others were female... |
Congentally corrected transposition of the great vessels can best be defined as a malformation in which the aorta and pulmonary artery are transposed in relation to each other but in which the flow of blood is in the physiologic direction. And in addition to the malposition of great vessels, there is an inversion of the ventrice,. Unfortunately this basic malformation... |
Morgagni hernia is an uncommon finding in adult and rarer still in infants and children, and they say that Morgagni hernia occur about one in every 300 diaphragmatic hernias diagnosed. This paper presents a 16 months oId Korean male infant with symptomatic Morgagni’ s hernia in whom the diagnosis was established during the course of treatment of upper respiratory tract infection and who were... |
This paper presents a case of Di Guglielmo Syndrome which is veryrrare in childhood. We experienced this syndrome in 6 year old male Korean. He visited with the chief complaints of abdominal pain and pale face appearance. We confirmed this patient by the bone marrow aspiration and have been performed a serial follow up checks. And also we have observed a... |
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