• Childhood Kidney Diseases
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• v.5 no.2
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• pp.109-116
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• 2001

Purpose Long- term use of steroid, cyclophosphamide and cyclosporin, which are frequently used in the therapy of SDNS, might cause severe side effects. Recently, the immune-modulator levamisole has been tried as a substitute therapy and it has been reported as a method with less side effects and more effectiveness. We started this research in order to observe the effects of levamisole and compare it to other therapy results. Patients and Methods : We chose 16 steroid dependent nephrotic syndrome children, those who had shown frequent relapse during the immunocompromised therapy period. Mean age was $9.1{\pm}1.4$ years in children and the male to female ratio was 15:1. All of subjects were diagonized with MCNS and had received cyclophosphamide or cyclosporin before receiving levamisole. Levamisole at a dose of 2.5mg/kg was used every other day for 1 year and the relapse rate was observed. Results : On average of 14 days after treatment, complete remission was visible in all of the children, and the relapse percentage was $50\%$, which represents 8 children, while remaining 8 children representing $50\%$ of the cases showed no relapse during treatment. During the levamisole therapy period, tile average relapse rate was reduced significantly from $2.18{\pm}0.9/year\;to\;0.77{\pm}0.9/year$(P=0.027). Also the average relapse rate after the therapy was reduced to $1.34{\pm}1.1/year$, which was a significant level compared to the level before treatment(P=0.003). There was no significant difference in terms of duration of remission maintenance. Duration of remission maintenance showed an average of $12.2{\pm}9.1$ months before the use of levamisole, but it was also $10.1{\pm}6.9$ month after therapy. No other side effects such as leukopenia, skin disease and other clinically significant symptoms appeared at all during therapy. Conclusion : The long-term medication of levamisole for the therapy of SDNS children is thought to be able to maintain stable remission by reducing the relapse frequency without causing severe side effects. Further study with a broader range of subjects is required to eluccidate the long-term effects of this treatment. (J. Korean Soc Pediatr Nephrol 2001;5 : 109-16)

• Proceedings of the KSCN Conference
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• 2000.11a
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• pp.86-86
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• 2000

• 대한임상약리학회:학술대회논문집
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• 1993.12a
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• pp.37-37
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• 1993

• Journal of the Korean Orthopaedic Association
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• v.56 no.2
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• pp.164-167
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• 2021

Down syndrome is the most common chromosomal disorder and is accompanied by hip osteoarthritis in approximately 28% of patients. This paper reports a case of hip arthrodesis as a treatment for severe hip osteoarthritis in adolescent Down syndrome patients. When performing surgical treatment of patients with Down syndrome, it is necessary to take a comprehensive approach that considers not only the medical point of view, but also the psychological and economic requirements of the patient and their social environment.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.91-95
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• 2003

The nephrotic syndrome is characterized by generalized edema, hypoproteinemia(<2 g/dL), proteinuria(>40 $mg/m^2/hr$), and hypercholesterolemia(>200 mg/dL). It is reported that hypoalbuminemia, which is one of the four diagnostic criteria of the nephrotic syndrome, is associated with gallbladder wall thickening. An explanation for the thickened wall in hypoalbuminemic states is the accumulation of fluid in the subserosal layer of the gallbladder wall which contains the most areolar tissue. This report describes a patient who was initially diagnosed with the nephrotic syndrome at the age of 4 and subsequently developed acute acalculous cholecystitis at the age of 5.8 with an albumin level of 1.3 g/dL. The patient responded to fluid therapy, nasogastric suction, and broad spectrum antibiotics.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.437-441
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• 2010

Toxic epidermal necrolysis (TEN) and Stevens Johnson syndrome (SJS) are rare, life-threatening mucocutaneous -diseases, usually attributable to drugs and infections. Corticosteroids have been used in the management of TEN for the last 30 years. This remains controversial and is still much debated. TEN can occur despite administration of high doses of systemic corticosteroids. The psychiatric side effects of corticosteroids can include headache, insomnia, depression, and mood disorders with or without psychotic episodes. Steroid-induced psychosis is dealt with by tapering or discontinuing the steroid; antipsychotics are also sometimes used. We report a case of an 11-year-old boy who was admitted with TEN. He had also been diagnosed as having nephrotic syndrome in the past. Remission was achieved through induction therapy and by maintaining the use of steroids. After a full-dose intravenous dexamethasone for TEN, he showed psychotic symptoms. We diagnosed him as having steroid-induced psychosis. We tapered the steroid use and initiated an atypical antipsychotic medication, olazapine and intravenous immunoglobulin (IV-IG). His symptoms dramatically improved and he was discharged.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.167-174
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• 2005

Purpose : Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. Methods : The study group consisted of 7,3 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. Results : The age($mean{\pm}SD$) of patients was $4.53{\pm}2.53$ years old. The male to female ratio was 52:21. In 95$\%$, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.005). Conclusion : The rapidity of response is expected to be one of the predictive (actors for relapse in children with SRNS. (J Korean Soc Pediatr Nephrol 2005;9:167-174)

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.889-892
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• 2003

Purpose : This study aimed to evaluate risk factors of the first year relapse in children with nephrotic syndrome(NS) without the need for biopsy. Methods : We reviewed, retrospectively, 78 children diagnosed with steroid responsive nephrotic syndrome between July 1997 and June 2002. Median years to follow up were 4.4 years(range : 1-5 years). We divided the patients into two groups(group I : primary responders with no relapse or with only two relapses in the first year after initial response; group II : initial steroid responders with three or more relapses within the first year). We retrospectively reviewed and compared variables - sex, onset age, serum albumin, serum cholesterol, 24 hours urinary protein, creatinine clearance(Ccr), presence of hematuria and hypertension(HTN), and days from initial attack to remission. Results : Of 78 patients(male : 61(78.2%), female : 17(21.8%), age range 1.1 years to 14 years, median $5.1{\pm}3.0years$), 47(60.3%) were in group I and 31(39.7%) were in group II. There were no statistically significant differences in sex, serum albumin, serum cholesterol, 24 hours urinary protein, Ccr, presence of hematuria or HTN. The median age of onset showed no statistical difference between the two groups. However, if the patients are dividing into two groups according to the age of onset of three-years, patients theree yrs old or less fit into group II, as opposed to patients older than three yrs in age(63.2% vs. 32.2%, P<0.05). The days from initial attack to remission was longer in group II($12.9{\pm}0.5$ vs. $16.2{\pm}1.1$, P<0.05). Conclusion : We may conclude that the age of onset of three yrs old or less, and the longer time remission to initial steroid therapy, are risk factors of the first year relapse.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.110-117
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• 1998

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis(HSPN) accompanied by nephrotic syndrome(NS) is known to have a poor prognosis and effective treatment is still controversial, even though both corticosteroids and immunosuppresant have been used for therapy. Cyclosporine A(CsA) is a well known immunosuppresant and widely used in renal transplantation and glomerular diseases especially steroid resistant. The aims of this study was to evaluate the therapeutic effect of CsA and to compare CsA with previously reported our data of rifampin(RFP) and azathioprine(AZA) in children with HSPN accompanied by NS. Methods : 37 HSPN patients with NS confirmed by renal biopsy were selected. Of these, 17 patients were treated with CsA(5 mg/kg/day) fur 6-8 months, 7 children were treated with RFP(10-20 mg/kg/day) for 9-12 months and 13 patients were treated with AZA(2 mg/kg/day) fur 8 months. Along with these regimens, low dose oral prednisolone(0.5-1 mg/kg, qod) was also used. Sequential renal biopsy was done in all patients 1 month after termination of treatment. Results : Complete remission rate of nephrotic syndrome was $58.8\%$ in CsA, $57.1\%$ in RFP and $38.4\%$ in AZA group after 17, 22, 11 months of mean follow-up period. Overall remission rate including partial remission was $88.2\%$ in CsA, $85.7\%$ in RFP and $84.6\%$ in AZA group. Disappearance rate of hematuria was $58.8\%$ in CsA, $57.1\%$ in RFP and $46.2\%$ in AZA group. Improvement of grade of clinical status was observed in 17 out of 17 CsA, 7 out of 7 RFP and 10 out of 13 AZA group. Improvement of pathologic class on sequencial renal biopsy was shown in 5 CsA($29.4\%$), none RFP($0\%$) and 2 AZA group($12.4\%$). Improvement on histologic immune-deposition was seen in 15 CsA($88.2\%$), 6 RFP($85.9\%$) and 4 AZA group($30.8\%$). Conclusion : In conclusion, Both CsA and RFP treated groups showed better result in complete remission rate of nephrotic syndrome and significant improvement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and REP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.170-175
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• 2009

Purpose : FSGS do not respond well to any kind of therapy and gradually progress to end-stage renal disease. This study was conducted to investigate the difference of protein expression between MCNS and FSGS as a preliminary study for understanding the pathophysiology of FSGS. Methods : Renal biopsy samples of MCNS and FSGS were obtained, which was diagnosed by one pathologist. They were solubilized with a conventional extraction buffer for protein extraction. The solution was applied on immobilized linear gradient strip gel (pH 4-7) using IPGphor system. Silver staining was carried out according to standard method. Protein identification was done by searching NCBI database using MASCOT Peptide Mass Fingerprint software. Results : The differences in protein expressions between MCNS and FSGS were shown by increased or decreased protein spots. Most prominently expressed spot among several spots in FSGS was isolated and analyzed, one of which was glutathione S-transferase (GST) P1-1, whereas it was not found in MCNS. So GSTP1-1 was considered as the one of the key biomarkers in pathogenesis of FSGS. Conclusion : This result would be helpful in diagnosing FSGS and researching FSGS. Further studies for glutathione S-transferase P1-1 might be necessary to elucidate the mechanisms regarding FSGS.