| 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... |
| The most common obstruction of left ventricular outflow in childhood is produced either by a fibrous ring below the aortic valve, or by localized or diffuse muscular hypertrophy of the interventricular septum. Cardiac tumors causing subaortic stenosis in the newborn infant are extremely rare. This report describes two cases of subaortic stenosis caused by a tumor of the left ventricle... |
| 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... |
| Thin basement membrane nephropathy(TBMN) is defined histologically as follows : 1) By light microscopy only minor abnormalities are detected in the glomeruli at most minor mesangial widening. 2) By electron microscopy, diffuse thinning of glomerular basement membrane is demonstrated. 3) By immunofluorescence, absence of immunoglobulins and complement components is demonstrated. 4) Alport`s syndrome and systemic diseases that may affect the... |
| Purpose : Tumor lysis syndrome is characterized by hyperuricemia, hyperphosphatemia, hyperkalemia and hypocalcemia due to destruction of tumor cells. The purpose of this study is to estimate in children the incidence, onset time, differences in outcomes between hemodialysis and conservative therapy, and predictive factor of tumor lysis syndrome before treatment with chemotherapy. Methods : Subjects were 108 children who had received... |
| Purpose : Multicystic dysplastic kidney(MCDK) is characterized by the presence of multiple cysts of varying sizes. It is associated with ureteral or pelviureteral atresia, and a non-functioning kidney. The purpose of this study was to examine the clinical feature and the natural history of MCDK. Methods : A study was performed in 36 children with prenatal diagnosis of MCDK from 1989 to 1999. All... |
| Purpose : Diagnosis of a hemodynamically significant patent ductus arteriosus(PDA) that should be treated is difficult to determine by clinical and echocardiographic examinations. The aim of this study was to clarify the interrelationship of plasma atrial natriuretic peptide(ANP) concentrations and clinical signs or echocardiographic signs of PDA in preterm infants and use plasma ANP concentrations as a diagnostic indicator of a hemodynamically significant PDA. Methods... |
| Critical valvular aortic stenosis(AS) is a life threatening congenital heart anomaly in neonates or infants. The main cause of death is rapidly progressing left ventricular failure. The treatment goal of critical AS is to save left ventricular function early. Before the 1980s, the preferred treatment was surgical valvotomy, however, that operative method had a high mortality rate and risk of... |
| Purpose : The aims of this study were to investigate the frequencies and role of hepatitis B virus(HBV) precore and core promotor mutations in children with chronic hepatitis B infection. Methods : Sera from 46 children with chronic hepatitis B infection were analyzed by direct sequencing of polymerase chain reaction product of HBV DNA. In this study, the patients were divided... |
| Purpose : This prospective pilot study is a part of the Korean NIH’s effort to characterize congenital anomalies and genetic disorders in Korea and to establish a National Genetic Database. Methods : This population-based study was conducted at all hospitals that deliver in the province of Suwon; a total of 39 hospitals from May 1, 1997 to April 30, 1999 for... |
| Neuromyelitis optica(Devic disease) is a rare demyelinating disorder of unknown etiology in which unilateral or bilateral optic neuritis and transverse myelitis occur within 8 weeks. The disease has no clinical involvement beyond the spinal cord or optic nerves. This illness occurs more commonly in adults than in children. The prognosis is better in children than in adults. The authors experienced... |
| Purpose : Antimicrobial resistance has become a major medical and public health problem. This study was performed to evaluate the changes in antimicrobial susceptibility patterns. Methods : The susceptibility of 8,224 recent clinical isolates during a 5-year period from 1993 to 1997 were tested against 8 antimicrobial agents. Antimicrobial susceptibility test was performed by disc diffusion method. Results : E. coli... |
| 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... |
| Intrahepatic portosystemic venous shunt(IPVS) is an uncommon pathologic condition which occurs mostly in adult patients with portal hypertension and cirrhosis of the liver with only scattered reports are on children. However, asymptomatic IPVS have been detected in an increasing number of patients with recent advances in imaging techniques, such as sonography, CT, MR imaging, and color Doppler sonography. The cause... |
| Common variable immunodeficiency(CVID) is a heterogeneous collection of disorders with hypogammaglobulinemia with recurrent bacterial infections and high incidence of autoimmune disorders as its hallmark. We report a 7-year-old girl suffering from CVID with Coombs' test positive hemolytic anemia. She had been relatively well until 23-months old when she was admitted to Taejon St. Mary' s Hospital with pneumonia 5 years ago. Afterwards, she had... |
| Cystic lymphangiomas are comparatively rare, benign tumors of lymphatic system and their histogenesis are uncertain. About 75% of theses lesions are in the neck, 20% are in the axillary region, and 5% are in the mediastinum, retroperitoneal region or groin. But retroperitoneal cysticlymphangiomas are very rare. Retroperitoneal cystic lymphangiomas are usually found incidentally during diagnostic procedures performed for unrelated clinical... |
| Purpose : Extrahepatic biliary atresia is the most common indication for liver transplantation in children, but the etiology of this disorders remains unknown. It would be very signficant to identify genes that are specifically expressed in pathologic liver tissue of biliary atresia and analyze the pattern of expression in those genes. Methods : We made dot blot panels consisting of 1,730 different EST(expressed sequence... |
| Purpose : Interleukin(IL)-10 is an antiinflammatory cytokine produced by monocytes/macrophages. Granulocyte colony-stimulating factor(G-CSF) plays an important role, not only as a hemopoietic factor but also as a regulating factor for a biologic defense system by neutrophils, in the foci of infection. We studied G-CSF and IL-10 levels in the serum and cerebrospinal fluid(CSF) of patients with aseptic meningitis and investigated... |
| Purpose : A retrospective study was undertaken to test the hypothesis that febrile neonates, who have bacterial infections, can be accurately predicted early by diagnostic criteria. Methods : We conducted a retrospective study of 152 infants, 28 days or less of age, whose rectal temperature was at least 38℃. Past history, family history, finding on physical examinations and results of CBC,... |
| Purpose : To assess the effect of gestational age and illness severity, and the effect of antenatal exposure to magnesium sulfate, glucocorticoids, and antibiotics, on the timing of the first stool in preterm infants. Methods : Medical records of all preterm infants admitted to the neonatal ward at Kangnam Sacred Heart Hospital between March 1998 and August 1998 were reviewed. We... |
| The most clearly delineated condition with prominent cutaneous leukocytoclastic vasculitis which presents to a pediatrician is Henoch-Schönlein purpura. There is general agreement that in Henoch-Schönlein syndrome the skin manifestations have a very benign long-term course and that corticosteroids are of little value. Dapsone appears to be of special value in diseases characterized by an accumulation of polymorphonuclear neutrophils notably leukocytoclastic... |
| 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... |
| We report a case of septic arthritis due to H. influenzae associated with atypical Kawasaki disease. A 2-year-old boy was admitted to our orthopedic department due to limping gait of the right leg. CT finding of right hip joint revealed fluid collection. He was diagnosed as transient synovitis and septic arthritis. He was treated with antibiotics, but no incision nor... |
| Purpose : The purpose of this study was to assess the frequency of acute rheumatic fever(ARF) among children in Korea and to describe trends in its clinical characteristics compared to data from a previous study(1978-1987). Methods : A mailed questionnaire survey sent to the pediatric departments of 13 general hospitals in Korea requested the total number of patients with ARF and... |
| It is well known that both acute glomerulonephritis and rheumatic fever are a common sequelae of group A streptococcal infection. However, their simultaneous occurrence is uncommon. Only a few cases have been reported because they are different in epidemiologic, clinical, serologic and immunopathogenic characteristics. We experienced a 10-year-old boy who presented the manifestation of acute rheumatic fever and acute poststreptococcal... |
| Purpose : To evaluate neurotoxic effects induced by oxygen-radicals, which were generated by adding xanthine oxidase(XO) and hypoxanthine(HX), and protective effects of glutamate receptor antagonist such as MK-801 and 6-cyano-7-nitroquinoxaline(CNQX). Methods : Dissociated cell cultures were prepared from cerebrum of neonatal mouse. Tissues were dissected and diced into small pieces in phosphate buffered saline and were incubated at 37℃. Isolated cells... |
| Purpose : Long-term administration of anticonvulsants for children with convulsive disorder may cause osteomalacia, depending on dosage, duration, types, and combination of anticonvulsants administered. This study was performed to examine the effect of anticonvulsant drugs on bone mineral density in epileptic children. Methods : Forty-eight epileptic children aged 3 to 15 years were enrolled in this study. They were treated with... |
| We report a 10-year-old male with steroid-responsive nephrotic syndrome which was complicated by cerebral venous sinus thrombosis including superior sagittal sinus and left popliteal artery thrombosis following 5 months later. At each thrombotic episode, he suffered from relapse of massive proteinuria with hypovolemic crisis, and from hemoconcentration with dehydration due to vomiting or diarrhea. Brain MR angiography confirmed thromboses of... |
| 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 : There was an explosive prevalence of aseptic meningitis for three years from 1996 to 1998. In this study, we reported the time of outbreak, clinical manifestation, CSF examination of patient, and CSF virus culture. Methods : We reviewed clinical records of 794 aseptic meningitis patient who had been admitted to Chounju Presbyterian Medical Center. The total number of patients... |
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