Purpose : We evaluated allele frequencies and distribution of surfactant protein A2(SP-A2) in Korean neonates in order to estimate the prevalence of RDS, to find out new SP-A alleles, and to establish new steroid therapy. Methods : Genomic DNA was extracted from 71 neonates and served as a template in PCR for genotype analysis. SP-A gene-specific amplications and gene-specific allele determinations... |
Purpose : Surfactant protein A(SP-A) is involved in surfactant physiology and structure, and plays a major role in innate host defense and inflammatory processes in the lung. Steroid therapy is widely used for mothers who threaten to deliver prematurely and also used commonly in the management of preterm infants with chronic lung disease. Two SP-A genes(SP-A1, SP-A2) and several alleles... |
Purpose : Recently, clinical trials of steroid add-on therapy were reported with variable results in Kawasaki disease. We analyzed the clinical outcomes of patients at high risk of with Kawasaki disease(≥4 points of Harada score) treated by three commonly used different treatment regimens, with or without corticosteroids. Methods : Medical records of 96 children with Kawasaki disease treated with one of... |
Purpose : We evaluated allele frequencies and distribution of surfactant protein A1(SP-A1) in Korean neonates in order to estimate prevalence of RDS to find out new SP-A alleles, and to establish new steroid therapy. Methods : Genomic DNA was extracted from 100 neonates and served as a template in PCR for genotype analysis. SP-A gene-specific amplications and gene-specific allele determinations were... |
Purpose : Leptin, the product of the ob gene is primarily by adipose tissue but also by the human placenta. Leptin may also have a role as a regulator of fetal growth and development during normal pregnancy as well as in pregnancies associated with anomalous fetal growth. Our study aimed to discover whether or not leptin concentration in neonatal cord... |
Purpose : To asses the short-term effects of the topical corticosteroid, budesonide administered by metered dose inhaler(MDI) and spacer(Aerochamber MV 15) following systemic corticosteroid in premature babies with respiratory distress syndrome at birth. Methods : 19 premature babies <1800gm were randomly assigned to receive steroids(n=9) or not (n=10). The steroid group(n=9, GA[gestational age]=29.6¡¾2.9 weeks, BW[birth weight]=1.29¡¾0.35kg) received systemic dexamethasone for 48... |
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... |
Levamisole has been used for nephrotic syndrome due to its immunostimulating, immunomodulating, and steroid-sparing effects. Agranulocytosis, a serious side effect of levamisole, was rare and mostly associated with autoimmune disease, neoplastic disease and HLA B27 except one case in a nephrotic syndrome who was treated with high-dose(5mg/kg QOD) levamisole. This 15 year-old girl with steroid dependent nephrotic syndrome, who was... |
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 : 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... |
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... |
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 : Percutaneous renal biopsy in children with primary nephrotic syndrome(NS) contributed to establish the renal pathology and clinicopathological correlation. The most common minimal change lesion(MCL) was steroid sensitive and could be predicted by clinical and laboratory findings. It was uniformly agreed that most nephrotic children who were predicted as MCL, should receive an 8 week course of prednisolone before considering renal biopsy. Early indications... |
Purpose : Steroid-resistant nephrotic syndrome in children is difficult to manage and tends to progress to chronic renal failure. We studied clinicopathological correlations in primary nephrotic syndrome in children resistant to 4-week daily steroid therapy. Methods : Among children who had been admitted to Seoul National University Children's Hospital during the period between Oct. 1985 and Jul. 1995 and diagnosed as primary nephrotic syndrome, 87... |
Purpose : Osteoporosis and growth failure have been known one of the serious side effects of corticosteroid therapy especially in children. This study was designed to evaluate the effect of long-term administration of steroids on bone mineral density(BMD) in children with nephrotic syndrome and its relationship to cumulative steroid dose, the duration of the rapy and sex. Methods : The BMD of... |
Focal segmental glomerulosclerosis is the renal histopathologic lesion observed in 10% of children with idopathic nephrotic syndrome. Complications include infection, thrombosis, hypocalcemia and adverse effect of steroid use. The incidnce of thrombosis reported ranges from 10~40%. The pathogenesis are changes in coagulation system, decrease of Antithrombin III, increase platelet aggregability and steroid or diuretics use. These changes take place passively... |
In this study I assesed the efficacy and tolerance of cyclosporin A (CyA) in the treatment of steroid-dependent MCNS (minimal change nephrotic cyndrome) and steroid resistant FSGS (focal segmental glomerulosclerosis). The results showed, that 1) CyA was effective in sustaining a remission in steroid-dependent MCNS without prednisone treatment, and therefore, could be an alternative therapeutic choice. 2) After discontinuation of CyA, relapses reccurred... |
The auther analysed 29 patients who were treated with prednisolone, including nephrotic syndrome(13 cases). Acute leukemia (10 cases), bronchial asthma(2 cases), juvenile rheumatoid arthritis(2 cases) and ulcerative colitis(1 cases) patients in childhood, after remission induction, prolonged alternate or interrupted treatment was used. The auther observed patterns of height velocity of these patients from 1.6 to 10.3 years. The results were as... |
Poststeroid panniculitis occuring after steroid treatment is an extremely rare entity. Multiple subcutaneous, firm, pruritic, tender nodules occur chiefly over the cheeks, arms, and trunk in young children following sudden discontinuation of large dose of steroids. We experiened a case of poststeroid panniculitis in a 2day-old female infant with complaints of multiple subcutaneous nodules on cheeks and neck for 10 days.... |
Poststeroid panniculitis occuring after steroid treatment is an extremely rare entity. Multiple subcutaneous, firm, pruritic, tender nodules occur chiefly over the cheeks, arms, and trunk in young children following sudden discontinuation of large dose of steroids. We experiened a case of poststeroid panniculitis in a 2day-old female infant with complaints of multiple subcutaneous nodules on cheeks and neck... |
Idiopathic pulmonary fibrosis is a heterogeneous group of disorder which predominantly affects the lung parenchyma and spares the airway. We report two cases of idiopathic pulmonary fibrosis in a 2 year 11 month-old female and a 1 year 1 month-old male patients who were treated with steroid. These patients presented with dyspnea and showed fine crepitant rales in both lung... |
A retrospective analysis of clinical findings in 20 patients with Kasabach-Merritt syndrome who visited Department of Pediatrics, Seoul National University Hospital from January, 1979 to June, 1990 was performed. The average age of occurrence was 6.4 month and male to female ratio was 1: 1.2. The mean size of hemangioma was 11.0x8.5 cm and the locations were cutanous in 18 (extrem- ities 5, trunk... |
A clinical study was carried out in 73 children with nephrotic syndrome who had had regular ophthalmologic examination annually at Seoul National University Children’s Hospital from March 1976 to February 1990 with the aim of finding the relationship between the posterior subcapsular cataract (PSC) and the effect of corticosteroids. The results were summarized as follows: 1) The number of patients with PSC were 23 cases... |
A clinical analysis was made on 51 cases with nephrotic syndrome who were admitted to Pediatric Department of Chung Nam National University Hospital from January 1st, 1984 to December 31st, 1988. Clinical manifestation, response to the inital continuous steroid therapy, relapsing patterns, factors affecting response to steroid therapy, pathologic findings and difference in laboratory findings between relapser and nonrelapser were evaluated. Results were as follows; 1) Among... |
Hypereosinophilic syndrome is a state of severe eosinophilia of unknown cause accomanied by multiple organ-system involvement. The criteria of hypereosinophilic syndrome includes. (1) A persistent eosinophil count of more than 1.5 X 10 9 /L for associated with the signs and symptoms of hypereosinohilic disease; (2) a lack of evidence for parasite, allergic, or other known causes of eosinophila; (3) presumptive signs and... |
Clinical observations were made on 55 patients of childhood ITP who were diagnosed at the department of Pediatrics, National Medical Center from January 1977 to April 1986. Boys were 30 cases and girls were 25 cases. The following results were obtained. 1) The highest incidence was seen among the children between three and five years of age and the boys were affected more frequently than girls... |
A clinical study was conducted on children with frequently relapsing nephrotic syndrome to see the therapeutic effectiveness of chlorambucil therapy. Study population consisted of 21 children with biopsy-proven minimal lesion nephrotic syndrome, in whom chlorambucil therapy was given to control their frequent relapses and/or steroid dependency. Eleven patients were frequent relapser and 10 were steroid dependant. Study period extended from December 1980 to August... |
A clinical study was conducted to determined the effectiveness of intravenous gammaglobulin on childhood acute ITP. Study group consisted of 8 children who were treated with IV Globulin(0.4 gm/kg/d, 5 days) only or combined with steroid, and control group of 19 children who were treated with steroid only. Study period was from January 1980 to June 1984, and following results... |
Among total 452 children with percutaneous kidney needle biopsy due to renal disease in the Department of Pediatric, Seoul National University Hospital, from Jan. 75 to Nov.84, 20(4.4%) were confirmed as embranoproliferative glomerulonephritis(MPGN) histopathologically. And 4 out of these 20 were excluded from this report because of association of systemic disease. A review of clinical and pathological findings of the... |
We experienced 2 cases of salt losing form of congenital adrenal hyperplasia. Case I was 3days old female neonate who had dehydration and large clitoris. Case H was 22 days old male neonate who had large pigmented penis. Both cases had hyponatremia and hyperkalemia. Their 24 hours urinary 17-ketosterod was also increased. Fluid therapy, hydrocortisone and florinef were tried with improvement. A brief review related literatures... |