• Journal of Korean Neurosurgical Society
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• v.30 no.11
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• pp.1291-1299
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• 2001

Objectives : The authors present a retrospective analysis of 14 patients treated for spinal cord hemangioblastoma (SCH) between Dec. 1986 and Mar. 2000. This study was conducted to evaluate and compare the difference of the functional outcomes associated with the extent of surgical removal of SCH. Methods : Eleven patients were male and three patients were female. Their mean age was 37.2 years that ranged from 19 to 62 years. Preoperative magnetic resonance(MR) imaging of the spine was performed in all cases, and preoperative angiography in eleven cases. They were followed from 15 months to 161 months(median follow-up period, 47 months), and we investigated the change of neurological symptoms and functional outcomes with radiological features, especially on MR imaging. Results : Six patients were accompanied by von Hippel-Lindau disease, and three of them had multiple CNS tumors. Thirteen patients had intramedullary tumor, and the remaining one had extradural. Syringomyelia around the tumor was observed in ten cases. All patients underwent surgical removal, and gross total removal(GTR) was achieved in ten cases. Preoperative embolization was performed in four cases. In four patients who were treated with preoperative embolization, intraoperative loss of blood was minimal and GTR was possible. One patient developed a transient swallowing difficulty postoperatively without permanent postoperative neurological deficits. In three of four patients in whom GTR was not possible, their functional outcomes were worsened postoperatively. The functional status at discharge was improved in seven patients, stationary in four patients, and worse in three. At the last follow-up(15-161 months), one of four patients who had been stationary at discharge showed improvement but, the rest did not show any change. All patients who showed neurological improvement were patients with GTR, and the patients with GTR had significant better outcome than those without GTR(p=0.015). Conclusion : Surgical treatment, and especially, GTR is considered as treatment of choice for spinal cord hemangioblastoma. Preoperative embolization may prevent intraoperative bleeding and improve surgical outcome.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.149-158
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• 2005

Purpose : To determine the histological findings and treatment outcome in cases of child hood nephrotic syndrome which required renal biopsy. Methods : We retrospectively reviewed the clinical, laboratory, pathologic findings and therapeutic outcomes of 159 nephrotic children who received a renal biopsy at the Department of Pediatrics, Kyunghee Medical University Hospital, Seoul from 1984 to 2004 over a period of 21 years. The renal biopsy was performed in nephrotic children who showed atypical features at presentation, or needed cytotoxic therapy because of frequent-relapsing, steroid-dependent, or steroid-resistant nephrotic syndrome(SRNS). Results : Minimal change disease(MCD) was found in 52.1$\%$ of the patients, followed by diffuse mesangial proliferation(33.1$\%$), focal segmental gomerulosclerosis(5.3$\%$), membranoproliferative glomerulonephritis(2.4$\%$), membranous nephropathy(2.4$\%$), and IgA nephropathy(1.8$\%$). In MCD children, 14.8$\%$ had hematuria, 22.7$\%$ had hypertension, 5.7$\%$ showed decreased renal function, and no patient was found to have an abnormal complement level. Among patients diagnosed with diseases other than MCD, 43.2$\%$ had hematuria, 21.0$\%$ was found to be hypertensive, 7.4$\%$ of children showed decreased renal function and only 3(3.7$\%$) had decreased complement level; the rates of hematuria and SRNS were found to be significantly higher than MCD patients. Among 37 SRNS patients, 30(81.0$\%$) showed a final remission state with long-term steroid therapy, including methylprednisolone pulse therapy, over 4 months, with or without cytotoxic therapy. Conclusion : Almost half of the cases of childhood nephrotic syndrome requiring renal biopsy were not diagnosed with MCD. Among atypical features, hematuria and steroid-resistance would be the most probable indicators for a diagnosis other than MCD. Even in patients with SRNS, long-term methylprednisolone pulse therapy may result in a good remission rate. (J Korean Soc Pediatr Nephrol 2005;9:149-158)

• Biomedical Science Letters
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• v.5 no.1
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• pp.75-84
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• 1999

In order to study the effects of $\alpha$-tocopherol acetate in glomerular injury, the minimal change nephrosis disease was induced by puromycin aminonucleoside (PAN) in spontaneously hypertensive rats, and we examined biochemical analysis in serum and morphological changes. The experimental animals were divided to control, PAN-treated (30 mg/kg, I.p.), vitamin E-treated (200 mg/kg, P.O.), and PAN+vitamin E-treated groups. After PAN injection, the rate of increase of body weight was lower than the other treatments. In addition, at 8 days after PAN injection, total protein content in serum was the lowest, whereas both blood urea nitrogen and serum creatinine contents were the highest in all experimental groups, which their changes of serum parameters were statistically significant. In morphological changes, the glomerular tissue at 8 days after PAN injection clearly showed obstruction of urinary space and proliferation of mesangial cells, and that loss and fusion of pedicles, vacuolization and edema of endothelial cells, and thickness of basal lamina were ultrastructurally showed in the glomerulus. Glomerular injury was significantly prevented by administration of vitamin E having an antioxidant effect. It suggested that the glomerular injury induced by PAN was accelerated by hypertension, and renal dysfunction might be induced by oxidative injury.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.42-47
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• 1999

Angiotensin-converting enzyme inhibitors lower urinary protein excretion in hypertensive and normotensive patients with renal disease. Most children with nephrotic syndrome have minimal change histology and the great majority of these patients respond to the treatment with oral prednisone. Here we have studied the effects of combination of Inhibace and oral prednisone in pediatric patients with nephrotic syndrome. 45 patients with nephrotic syndrome were selected. Of these, 29 patients were treated with prednisone(2mg/kg/day) and 16 children were treated with prednisone and Inhibace(2.5mg/day). Urinary protein, blood pressure, creatinine clearance, serum creatinine, serum albumin and serum cholesterol were measured in both control and Inhibace group. Also the duration to remission after treatment was compared in both groups. The amounts of proteinuria before and after treatment were not significantly different in both group. The duration to remission of proteinuria was significantly shorter in Inhibace group compared to that in control group. The changes of blood pressure and creatinine clearance were not significant in Inhibace group. In conclusion, the combination therapy of oral prednisone and ACE inhibitor have shortened the duration to remission of proteinuria in nephrotic syndrome of children.

• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.4
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• pp.581-585
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• 2002

Focal epithelial hyperplasia(FEH) is Human papilloma virus - induced, localized proliferation of oral squamous epithelium. FEH usually occurs in the childhood, but occasionally affects the young and middle-aged adults. Sites of the greatest involvement include the labial, buccal and lingual mucosa, but lesions of gingiva or tongue have also been reported. This disease is typically characterized by multiple soft, non-tender flattened papules and plaques. Occasional lesions show a slight papillary surface change. Individual lesions are small, discrete and well demarcated. The histopathologic hallmark of FEH is acanthosis of the oral epithelium. Cells demonstrating viral cytopathic changes including koilocytes or mitosoid cells may be present. The 5-year-old female of this case visited Department of Pediatric Dentistry, College of Dentistry, Yonsei University with a chief complaint of exophytic lesions on gingiva. Sessile papillary papules were detected by clinical examination on buccal gingiva at the maxillary left and right second deciduous molars. The patient did not complain of pain by palpation. An excisional biopsy was carried out for a histological examination and acanthosis was observed. The lesions were diagnosed as FEH. FEH would regress spontaneously after several months or years. Conservative excision may be performed for diagnostic or esthetic purpose. The risk of recurrence after this therapy is minimal, and there is no malignant transformation.

• Journal of Preventive Medicine and Public Health
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• v.27 no.2 s.46
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• pp.175-185
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• 1994

In order to anticipate disease pattern and health problems of Koreans in the 1st part of 21st century (by the year 2020), transition of population characteristics, mortality and morbidity data during the last 30 years Koreans have experienced were reviewed. On the actual basis of epidemiologic transition process that has undergone during last 30 years since 1960 along with socioeconomic development and successful implementation of selective national health policies (family planning, medical insurance and etc.), following changes can be expected in the 21st century in Korea, under the assumption that the current rate of progress is maintained. The population of South Korea alone will be doubled the population of 1960 by the year 2013 : aged Population older than 65 years will be increased from 3.3% in 1960 to 11.4% in 2020 with increased average age of the population from 23.6 year in 1970 to 39.2 year in 2020; urban population from 28% in 1960 to 83% in 2005. GNP/capita has increased tremendously from U.S. $120 in 1970 to $6,749 in 1992, and the government estimated it would be 519,350 in 2010 and $29,460 in 2020. Growth and developmental indices of children, educational achievement and social status of women also showed a remarkable improvement and anticipated to make futher progress. Leading causes of mortality and morbidity have shown a striking change during the last 30 years, from infectious diseases to chronic degenerative diseases and man-made injuries. Occurrence of communicable diseases may become minimal although viral hepatitis, venereal diseases Including AIDS, and well adapted herpes virus infections will maintain their endemic level. Newly evolving infectious agents, however, should be carefully monitored because of rapidly changing environments and human behaviours. Tuberculosis may increase up to the epidemic level when AIDS prevails. Ischemic heart diseases may increase steadily with increasing occurrence of hypertension and diabetes mellitus whereas cerebrovascular diseases may be decreased slowly. Musculaskeletal diseases which contribute a lot to the disability of aged people may be a major health problems due to increased aged population. Mental diseases, particularly that caused by alcohol and drug abuse, and senile dementia may become a prominent health problem. On the other hand injuries caused by traffic and industrial accidents that have shown most striking increase till now may be decreased considerably by intensive intervention. The health policies in the 21st century will be oriented to the health promotion for good quality life rather than life-savings.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.161-169
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• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.4-12
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• 1997

Purpose: To evaluate the prevalence and clinical manifestations of various glomerulonephritis (GN) in children, a clinicopathological anlysis of 310 biopsied cases were performed. Method: We conducted retrospective study with review of histopathologic findings and clinical manifestations of the 310 cases diagnosed as glomerulonephritis by percutaneous renal biopsy which were done between January 1986 and December 1996 at department of pediatrics, Pusan Paik hospital. Results: 1) Male to female ratio was 1.54:1 and the range of age was from 13 months to 15 years 10 months. 2) Among these, 217 (70.0%) patients were belong to primary GN and 93 (30.0%) patients were belong to secondary GN. As a whole, the most common pathologic diagnosis was minimal change lesion (MC, 32.6%), which was followed by IgA nephropathy (IgAN, 15.8%), $Henoch-Sch\"{o}nlein$ purpura nephritis (HSPN, 13.5%), Poststreptococcal glomerulonephritis (PSAGN, 8.1%). 3) Clinical manifestations of patients were asymptomatic urinary abnormality (43.2%), nephrotic syndrome (41.0%), acute glomerulonephritis (14.2%), chronic glomerulonephritis (1.0%), rapidly progressive glomerulonephritis (0.6%). 4) In primary GN, the most common pathologic diagnosis was MC (46.5%), IgAN (22.6%), thin glomerular basement membrane (GBM) disease (7.8%), membranoproliferative glomerulonephritis (MPGN, 5.5%), mesangial proliferative glomerulonephritis (MesPGN,4.6%), focal segmental glomerulosclerosis (FSGS, 4.6%), membranous nephropathy (MN, 0.9%), sclerosing glomerulonephritis (SCGN, 0.9%), crescentic glomerulonephritis (CreGN, 0.5%) and non-specific glomerulonephritis (NonspGN, 6.0%). 5) Major causes of secondary GN were HSPN (45.2%), PSAGN (26.9%), hepatitis B associated glomerulonephritis (HBGN, 17.2%), lupus nephritis (LN, 6.5%), Alport syndrome (2.2%), hemolytic uremic syndrome (1.0%), fibrillary glomerulonephritis (1.0%) in descending order. Conclusions: There are some differences of the results of clinicopathological stuidies of glomerulonephritis in children because of its different indications of renal biopsy, pathologic classification of renal disease and methods of analysis among investigators. In order to establish more reliable data of incidence and classification of childhood glomerulonephritis in Korea, multicenter cooperative study were necessary.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.465-473
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• 1995

Background: The prevalence of pulmonary tuberculosis has decreased progressively after the control of the tuberculosis began as national control. But as diabetes, malignancy, immunodeficiency disease recently tend to be increased, the tuberculosis become to the important national health problem. So, this study was designed to observe the state and the change of the prevalence and the clinical status of pulmonary tuberculosis for recent 10 years at one women's university. Method: We retrospectively investigated the epidemiology and the clinical status of 612 patients who were registered at the Ewha Womans University Health Center by analyzing records from 1983 to 1992. Results: 1) The prevalence rate had been steadily decreased from 0.63% in 1983 to 0.11 % in 1992. The prevalence of freshman and the incidence rate according to the entrance year while in the university significantly decreased since 1989. 2) In classifying by registered source, 45.6% of students were detected by annual periodical health examination, 34.5% by entrance physical examination, 12.0% by hospital, 5.4% by health center clinic, 2.5% by reentrance physical examination, sequentially. 3) The students with past history of tuberculosis were 70(11.4%) and 61(10%) suffered from pulmonary tuberculosis. The patients with family history of tuberculosis were 142(23.3%). 4) There were 530(86.6%) with minimal disease, 79(12.9%) with moderate and only 3(0.5%) with far advanced, when classified by the severity of disease. 5) The initial symptoms were mild breathing difficulty in 30.1%, sweating in 14.9%, fatigue in 14.3%, febrile sense in 11.7%, hemoptysis in 8.2%, sequentially. 6) The duration of treatment was $10.6{\pm}3.6$ months in mild group, $14.9{\pm}5.2$ months in the moderate group(P<0.05). 7) The side reactions of the drug were GI trouble in 7.2%, hepatitis in 1.8%, skin rash in 0.8% and streptomycin side in used patients in 9.1%. Conclusion: The prevalence of pulmonary tuberculosis among the students in one women's university was significantly lower than that of university students and 20-24 year-old age group announced in tuberculosis survey on a national scale, and significantly decreased since 1989. The treatment effect was desirable in student's group managed by university health center.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.117-124
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• 2001

Purpose : Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulosclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid- dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. Methods : We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy Period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methylprednisolone, often in combination with oral cyclophosphamide for 82 weeks. Results : In all the 10 children with SD-MCNS, complete remission was visible on average of $18{\pm}9$ days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was $2.1{\pm}1.0$ relpases/year, which was reduced to $1.4{\pm}0.9$ relapses/year during MP therapy(P>0.05) and rose to $2.7{\pm}1.0$ relapse/year after MP therapy. Of the 6 children with FSGS, 4 children($67\%$) showed complete remission, of whom 3 children($50\%$) remained in the remission status during the follow up period, $1.2{\pm}0.7$ years, after the end of MP therapy. 2 children($33\%$) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, $1.7{\pm}0.6$ years The only side effect of MP therapy was transient hypertension in 10 children of ail subjects during the intravenous infusion of methylprednisolone. Conclusion : We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 117-24)