Purpose : The safety of MMR(Measles, Mumps, and Rubella) immunization to children with egg allergies has been debated for decades because the live attenuated virus used in the vaccine is grown in cultured chick-embryo fibroblasts. Many studies that investigated adverse reactions to MMR vaccine have been reported abroad, but there has been no report in Korean children. So, this study is aimed at determining... |
Purpose : This study was carried out to obtain the immunization rate of MMR, and to know the related factors. Methods : Subjects of the study were 3,364 students of 8 elementary schools of Kyonggi province. Surveys accompanied with questionnaires were conducted in 1996 and 1999, respectively. Results : The salient features of the findings were as follows : The immunization rate... |
On Xp21 region several genes such as adrenal hypoplasia congenita(AHC) gene, glycerol kinase (GK) gene and Duchenne muscular dystrophy(DMD) gene are located contiguously. If there is a long deletion in that region, various combination of genetic defect can be occurred from one kind of genetic defect to all three kinds of genetic defect simultaneously. In case of more than two... |
Chloral hydrate is widely used as a sedative or hypnotic, especially in pediatric patients, but cardiac arrhythmia following chloral hydrate administration has rarely been reported in literature up to date. The most common cardiac arrhythmia is ventricular extrasystole. We describe a 17- year-old Down syndrome patient who developed a life-threatening cardiac arrhythmia developed after chloral hydrate administration of 100mg/kg body... |
Purpose : In this study we tried to look at the spreading, duration of colonization, and acquisition of new streptococci which were obtained in one geographical area, as well as the bacteriologic and molecular epidemiology of normal school children carrying group A streptococci and their clonal relationship through the combined application of the serotype of T antigen and Pulsed Field... |
Acute promyelocytic leukemia(APL) is a unique entity in the spectrum of acute myelogenous leukemia. It has several characteristic features, including distinctive morphology, chromosomal translocation, t(15:17), disseminated intravascular coagulation and effect on retinoic acid. Retinoic acid which is a derivative of vitamin A induces differentation of APL cells in vitro and in vivo, but its cessation induces relapse of APL. Arsenic... |
Purpose : To evaluate the effectiveness of transcatheter arterial chemoembolization(TACE) as a preoperative treatment for initially unresectable hepatoblastomas. Methods : From January 1995 to July 1999, we identified two boys and three girls(age range : 5- 27 months; mean age : 12 months) with pathologically-confirmed hepatoblastoma. To assess response to treatment, we utilized an identical TACE procedure for all the patients.... |
Purpose : To produce a new generation of artificial pulmonary surfactant(PS), surfactant protein (SP)-B from human PSwas isolated, and the amino acid sequences of these proteins were studied. Artificial peptides of human SP-B were synthesized. New artificial PS preparations which were composed of phospholopids and two artificial synthetic SP-B peptides were made, and the surface physical properties of these new PS preparations were tested. Methods... |
Apert syndrome is an uncommon congenital disorder characterized by malformation of the skull in association with symmetrical syndactyly of both hands and feet. This syndrome is autosomal dominant. The original description was presented by Apert in 1906. Since then more than 200 cases have been reported in the world. Recently, we experienced a case of newhorn male infant with congenital... |
Nonketotic hyperglycinemia is an extremely rare congenital metabolic disorder, which is caused by the lack of a glycine cleavage system. The onset of hyperglycinemic symptom is during the neonatal or early infant period. Progressing grave neuromotor dysfunction is one of the main symptoms. They include myoclonic seizure, hiccup, apnea, decreased deep tendon reflex, lethargy and coma. The prognosis is mostly very poor. Furthermore, there... |
Purpose : Insulin-like growth factor(IGF-I) and IGF binding protein(IGFBP)-3 is thought to play an important role in fetal erythropoiesis. The objective of this study was to establish a relation between IGF-I, free IGF-I, IGFBP-1, and -3 with hemoglobin level in healthy term, 3-month, and 12-month old infants. Methods : Healthy term infants(n=20) were enrolled at birth, as well as 3 months(n=30) and 12 months(n=30) of... |
Neonatal alloimmune thrombocytopenia(NAIT) is a rare disease caused by maternal alloimmunization against fetal platelet surface antigen, which is mainly platelet specific alloantigen or human leukocyte antigen(HLA). During routine hemotology, we accidentally discovered thrombocytopenia in a female fullterm newborn admitted due to jaundice. We excluded NAIT due to human platelet alloantigen(HPA), because the HPA of the mother and baby were the... |
Purpose : To evaluate the clinical characteristics, treatments and outcome of patients with hemorrhagic shock and encephalopathy(HSE) syndrome. Methods : We performed a clinical study on 14 patients who were diagnosed as hemorrhagic shock and having encephalopathy syndrome in the Department of Pediatrics, from 1984 to 1998. Age, sex, clinical symptoms and physical findings at admission, the most deranged laboratory findings,... |
Purpose : Cytokines found at a inflammatory site may be a good indicator of clinical severity of an infectious inflammatory disease. We assayed interleukin 6(IL-6) concentrations in cerebrospinal fluid(CSF) from patients affected with viral meningitis, and verified the relationship between IL-6 and inflammatory parameters and whether IL-6 can be used as a diagnostic marker in the diagnosis of viral meningitis. Methods... |
Purpose : To survive in an ecological environment, an individual must develop immunity to various antigens. Therefore, populations of peripheral blood mononuclear cells(PBMC) in humans change continuously with growth. The object of this study is to evaluate the apoptosis of peripheral blood mononuclear cells(PBMC) in normal children of different ages. Methods : PBMC were isolated from the study groups. Ten... |
Purpose : Acute promyelocytic leukemia(APL or AML, M3) represents an unique model for cancer research in terms of biological and clinical features. Since 1988, it has been widely confirmed that all-trans retinoic acid(ATRA) can induce complete clinical remission in over 85% of APL patients by a differentiation process, with PML-RARα protein possibly being the direct target of ATRA. However, ATRA... |
Purpose : Persistent hyperinsulinemic hypoglycemia of infancy(PHHI), which is characterised by inappropriate insulin secretion in spite of hypoglycemia, needs urgent treatment to prevent cerebral hypoglycemic damage. Although pancreatectomy is the treatment of choice for PHHI, there are several complications which follow treatment. We suggest that aggressive medical therapy, when effective, is preferable to partial pancreatectomy. Methods : We evaluated 8 patients... |
Greig cephalopolysyndactyly syndrome(GCPS) is a disorder characterized by postaxial polydactyly of the hand, broad or occasionally bifid thumbs, preaxial polydactyly of the feet, broad halluces, syndactyly of the fingers or toes, macrocephaly, frontal bossing, hypertelorism and a broad nasal bridge. Intelligence is usually normal, although borderline IQ has been reported. Advanced bone age, mild hydrocephalus, craniosynostosis and agenesis of the corpus callosum are occasionally associated... |
Sometimes physicians and clinical pathologists find very high levels of alkaline phosphatase(ALP) activity in their patients. This condition is associated with transient, marked increase in serum ALP in healthy infants and children. It has also been described in adults. Clinical and biochemical features of transient hyperphosphatasemia in infancy and early childhood are reviewed in six patients that we have studied. The diagnosis is suggested... |
Purpose : Apoptosis is active cell death which plays an important role in developing normal tissues. Various conditions such as genetic defects, drugs, ischemia or infections are known to induce apoptosis. We studied the effect of maternal infection on fetal brain development during pregnancy. Methods : We treated 46 C3H pregnant mice with lipopolysaccharide(LPS) or phosphat-buffered saline and observed the changes in apoptosis and... |
Purpose : Apoptosis is a process of active cell death which has been suggested to be part of hippocampal cell loss caused by kainic acid(KA). Immature rats showed higher susceptibility and mortality to KA but did not develop recurrent seizure, long term behavioral or neuropathologic changes. We investigated whether this was due to age-dependent resistance, and elucidated the molecular mechanics which mediate P53-induced apoptosis,... |
Purpose : Antenatal dexamethasone administration is associated with a significant lowering respiratory distress syndrome(RDS) incidence, but can increase neonatal infection. Ambroxol has been accepted as an alternative treatment to dexamethasone and is of at least equal efficacy but without adverse reaction. The aim of this study was to evaluate the effectiveness of ambroxol versus dexamethasone in RDS incidence and neonatal... |
Purpose : The purpose of this study is to estimate the side effects of antiepileptic drug(AED) in children. Methods : Subjects were 267 children who had received AED for at least 1 month during January 1995 to July 1998. We reviewed their sex, age at start of seizure onset, age at medication, class and number of AED, developmental delay, type and... |
Purpose : This study was aimed to investigate the relationship between changes in intrapulmonary right to left shunt(QSP/QT) and improvements in oxygenation during the 2-hr study period after Surfactant-TA(S-TA) administration. Methods : Umbilical arterial blood gases and corresponding respiratory indexes of 27 preterm neonates with respiratory distress syndrome were studied. The neonates were separated into group A(n=20) and group B(n=7) according... |
We experienced a case of split hand split foot(SHSF) anomaly in a male neonate who had a deficiency of the middle finger, syndactyly of the 1st and 2nd finger and a deep median cleft in both hands. He also had a deep median cleft between 2nd and 3rd toe, syndactyly of the 1st and 2nd, 3rd and 4th toe without... |
Congenital acute leukemia is a rare disorder with approximately 200 cases reported. It is defined as a childhood leukemia occurring at birth or before 1 month of age at a rate of 1%. Acute leukemias are generally classified according to morphology, cytochemistry and cell surface marker expression. Most leukemias conform to an ordered lineage-specific pattern of gene expression, but a... |
Purpose : This study was undertaken to characterize molecular defects in Korean families with ornithine transcarbamylase(OTC) deficiency, correlate it with phenotype using in vitro expression study, and utilize it for making prenatal molecular diagnosis. Methods : To investigate molecular lesions resulting in OTC deficiency in 15 unrelated Korean families, the OTC genes of probands were amplified exon by exon and analyzed... |
Percutaneous transluminal coronary angioplasty(PTCA) is rarely performed on patients with coronary arterial stenosis that resulted from Kawasaki disease. We experienced a 3 year 10-month- old male with a history of Kawasaki disease who developed a few numbers of fusiform aneurysm on the right and left coronary artery. We examined and followed up the patient for 21 months using eletrocardiography,... |
Purpose : Infection by Haemophilus influenzae Type B(Hib) occurs most often in infants and children who are 5 years old and less. The incidence is highest around 6 months of age, and then decreases thereafter as infants gain natural immunity. Hi PRP-D conjugate vaccine is poorly immunogenic in infants. However, immunogenicity PRP-D vaccine in Korean infants is unknown. This... |
MELAS(mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome is a major subgroup of mitochondrial myopathy. Recent advances in molecular genetics revealed specific mutations in mitochondrial DNA which cause MELAS. We described here clinical and molecular genetic findings of sister and brother with MELAS syndrome. For molecular genetic studies, DNAs from peripheral blood nucleated cells were used. And the substitution... |