Purpose : Convulsive status epilepticus(SE) is a serious, life-threatening neurological condition that requires immediate treatment to avoid significant morbidity and mortality. Despite improvements in the diagnosis and treatment of SE in the last two decades, SE in young infancy is still associated with high morbidity and mortality. Thus, understanding the varied etiology and clinical presentation and prognosis of SE is... |
Purpose : As morbidity and mortality resulting from physical trauma have significantly increased, the importance of trauma concerning medical, legal, and socioeconomic issues has been widely documented. Studies of post-traumatic seizure after head trauma have been reported, but mostly in adults. So this study was performed to analyse clinical findings on head trauma patients under 20 years of age. Methods :... |
Individuals with Down syndrome have a high incidence of hematologic diseases such as transient myeloproliferative disorder(TMD) & acute leukemia. Because it is difficult to distinguish TMD from acute myeloblastic leukemia, the diagnosis in neonate, who have Down syndrome, should be made with extreme caution. TMD usually undergoes spontaneous remission within a few months, but acute leukemia can develope after remission... |
Purpose : The aim of this study is to assess the cardiac effect of long-term steroid therapy in nephrotic syndrome and the validity of LV functional parameters as an early predictor of subclinical cardiac dysfunction. Methods : The study group was composed of 21 patients diagnosed as minimal change nephrotic syndrome(NS), being managed with prednisone over 6 months or within 6... |
Purpose : Williams syndrome is characterized by supravalvular aortic stenosis, mental retardation and peculiar facial appearance. Its genetic etiology is considered to be a hemizygotic deletion in Chromosome 7q11.23, which includes the elastin gene. We examined the hemizygotic deletion of Chromosome 7q11.23 in 12 Korean Williams syndrome patients and 8 patients with isolated supravalvular aortic stenosis and performed deletion mapping... |
Purpose : Acute respiratory distress syndrome(ARDS) is the final course of acute lung injury. It results from various etiological origins and pathophysiologic mechanisms, and has a mortality rate of approximately 60-70%. Although the confirmative incidence of ARDS in children is yet unknown, the increasing incidence of ARDS has been reported in Korea. In the present study, we report ARDS diagnosed... |
Purpose : This study was aimed to assess the clinical manifestations and courses of autosomal recessive polycystic kidney disease(ARPKD). Methods : The medical records of 10 children diagnosed as infantile or juvenile ARPKD at Seoul National University Children's Hospital between January, 1984 and December, 1996, were reviewed, retrospectively. Results : The average age at diagnosis was 3 8/12 years(4months-7 3/12 years)... |
Purpose : Most of childhood MCNS has a long disease course with frequent relapses. This study was designed to analyze the long-term clinical course of childhood MCNS, focusing at relapsing pattern, treatment response and complications. Mothods : The medical records of 137 children with biopsy-proven MCNS observed during 1976 ti 1996 were analyzed retrospectively. They were classified as initial responders(111 patients,... |
Purpose : Good compliance with an anticonvulsant medication regimen is essential for the successful management of epilepsy. However, pediatric compliance with anticonvulsant therapy did not well be studied. The aim of this study was to identify the following key in noncompliance in pediatric out patients receiving anticonvulsant therapy determining factors. Methods : Eighty-one epileptic outpatients under fifteen years of age with... |
Purpose : The aim of this study was to elucidate the pathogenesis of vertical transmission of hepatitis B virus(HBV), and to study the protective efficacy of the vaccine and hepatitis B immune globulin(HBIG). Methods : Eighty-two infants born from HBV carrier mothers were studied. HBV plasma-derived vaccine and HBIG were administered to their neonates. Then, changes of viral markers, anti-preS2... |
Congenital self-healing reticulohistiocytosis(CSHR) is a rare variant of cutaneous reticulohistiocytosis. We report a newborn who had about 25 reddish brown papulonodular lesions on whole body at birth. No extracutaneous involvement was found. All skin lesions involuted within 4 months spontaneously. Biopsy of a skin lesion showed marked aggregated histiocytic infiltration with some eosinophils. S-100 protein and lysozyme were positive. Electron... |
Purpose : Long-term steroid therapy for various glomerular disorders in children has been known to decrease serum Vit D3 level and develop osteomalacia. The aim of this study was to observe the effect of long-term steroid therapy on growth and bone density in children with nephrotic syndrome. Methods : Bone density of 17 steroid-treated nephrotic syndrome was compared with that of... |
Purpose : The purposes of this study were to determine the normal values of spectral and chaotic indexes and to assess the maturational process of autonomic and chaotic control of fetal heart rate according to gestational age. Methods : Three hundred and thirty-seven mothers with normal singleton pregnancies, who were 23 weeks or more in gestational age, was randomly chosen. For... |
Purpose : Neonatal hepatitis and congenital extrahepatic biliary atresia are two major causes of neonatal cholestasis. But the method of therapeutic trials used for each disease is essentially different. Nonetheless, it is very difficult to differentiate these diseases clinically and histologically. This study is aimed to find out major differences between the two by clinical characteristics and scoring of various... |
Septic pulmonary embolization is an uncommon condition in which the clot or fibrin matrix, contaminated with micro-organisms, lodge in the pulmonary arterial tree, leading to infarction, suppuration and other complications. The septic emboli reach the lung from a variety of sources, including acute suppurative pelvic thrombophlebitis, infective endocarditis involving tricuspid and pulmonary valve, osteomyelitis, suppurative lesions of the neck, infected... |
Purpose : This study was designed to evaluate the safety and efficacy of early cessation of antibiotic treatment regardless of absolute neutrophil count in children with febrile neutropenia and no identifiable infectious source. Methods : A prospective randomized clinical trial was performed in 93 episodes of fever in 37 neutropenic children with cancer, who were admitted to the Department of... |
Purpose : Clinical and pathological prognostic factors of idiopathic IgA nephropathy have been reported, but mostly in adults and a few in children. Especially studies about correlation between those factors are very rare. Methods : We studied 58 children patients who were hospitalized to our clinics and diagnosed as IgA nephropathy by renal biopsy from Jan. 1989 to Jun 1996. They... |
Abnomalities in the production and transport of airway secretions play an important role in the pathophysiology of asthma. Segmental atelectasis as a complication of asthma, is relatively common in children. On the other hand, massive atelectasis such as the collapse of one lung is a very rare occurence. We report a seven-year-old male asthmatic patient with right lung collapse caused by mucoid impaction. Fourteen... |
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 : Despite the use of improved therapeutic modalities, long-term survival remains poor in patients in stage Ⅲ or Ⅳ of neurobalstoma and for over 1 year olds at diagnosis. We evaluated the long-term survival rate and related prognostic factors in advanced neuroblastoma. Methods : Twenty-nine children diagnosed with stage Ⅲ or Ⅳ neuroblastoma after 1 year of age, between May... |
Purpose : In acyanotic congenital heart disease of children with left to right shunt, 99mTc- Macroaggregate albumin(MAA) was employed to determine the scintigraphic severity of pulmonary parenchymal damage. Methods : These data of lung perfusion scan using 99mTc-MAA were compared with hemodynamic values obtained from cardiac catheterization. Results : The mean Pulmonary arterial pressure(≥30mmHg), total pulmonary resistance(≥2 Wood unit)... |
Purpose : The accuracy of bacteriologic diagnosis of beta-hemolytic streptococcal pharyngotonsillitis depends on the degree of carrier rate in that area. It is the purpose of this study to obtain the carrier rate from seven different areas and to find results and epidemiologic factors. Methods : Throat swab was obtained from the tonsillar fossa of normal school children during March through... |
The diaphragm is especially important in sustaining minute ventilation in the neonate. Consequently, diaphragmatic paralysis is not tolerated well by the neonate and often results in prolonged respiratory failure. We experienced a case of unilateral diaphragmatic paralysis with the brachial plexus palsy in a male newborn infant who presented with apnea and cyanosis due to birth asphyxia. After endotracheal intubation, mechanical ventilaton was started.... |
It is generally agreed that theophylline preparations and steroids should be given intravenously for status asthmaticus. Theophylline can potentially have adverse gastrointestinal effects including abdominal pain, nausea, vomiting, and hematemesis. Upper gastrointestinal bleeding in patients treated with corticosteroids without a past history of upper gastrointestinal bleeding has been reported rarely. But the etiologic significance of the stress of status asthmaticus, administration of theophylline or corticosteroids... |
In hydranencephaly, the cerebral hemispheres are absent or represented by membranous sacs with remnants of frontal, temporal or occipital cortex dispersed over the membrane. The brain stem is relatively intact. The cause of hydranencephaly is unknown, but bilateral occlusion of the internal carotid arteries during early fetal development can explain most of the pathologic abnormalities. We evaluated a case of hydranencephaly by magnetic resonance(MR)... |
Purpose : Acute disseminated encephalomyelitis(ADEM) is an inflammatory demyelinating disease of the central nervous system. The pathogenesis is not well known, but it is thought to be an immune-mediated disease and may follow a viral infection or vaccinations. MRI demonstrates decreased signal on T1 weighting and increased signal on T2-weighted image. We report clinical features and characteristic MRI findings in seven patients with ADEM... |
Purpose : To evaluate the diagnostic reliability of the single serum titers of the specific serum antibody determiantion method, we compared antimycoplasma antibody titers of 177 healthy children with 353 children who had respiratory symptoms indicative of Mycoplasma pneumoniae infection. Methods : We used Serodia-Myco II particle agglutination test and the titers of ≥1:40 were regarded as positive. Results : Age distribution of 177 healthy... |
Purpose : In this study, natural pulmonary surfactant was extracted from bovine lung lavage and its surface activity was determined. To investigate the usefulness of synthetic peptides reconstituted with phospholipid as artificial surfactant, truncated peptides from surfactant protein (SP)-B were synthesized and restored the surface tension lowering activities when appropriately recombined with phospholipid. Methods : Crude natural surfactant(CNS) was isolated from lung lavage by centrifugation... |
Chronic iron overload is associated with life-threatening complications, such as cardiomyopathy, liver cirrhosis, diabetes, hypothyroidism, hypoparathyroidism and hypogonadism. We experienced a case of secondary hemosiderosis with hepatic fibrosis in a 8-year-old boy who had been transfused 56 pints of packed red blood cells for a supportive therapy of acute myelogenous leukemia for a 27-month period. Intramuscular injection of Desferroxamine(40mg/kg/day) was... |