• 대한가정학회:학술대회논문집
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• 대한가정학회 1997년도 Program & Abstracts * The 7th Korea-Japan Home Economics Joint Syposium
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• pp.226-226
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• 1997

• Childhood Kidney Diseases
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• 제9권2호
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• pp.149-158
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• 2005

목 적 : 소아 신증후군 환아 중 신생검을 시행한 경우 그 임상적, 검사적 특징과 조직학적 소견, 그리고 치료 길과를 보기 위해 연구를 시행하였다. 방 법 : 1984년부터 2004년까지 21년 동안 경희의료원 소아과에서 신생검을 시행 받은 169명(남아 130명, 여아 39명)에 대해 그 임상적 특징, 검사 소견, 병리 소견들과 치료 결과를 후향적으로 조사하였다. 신생검의 적응증으로는 입원 당시 비특이적 소견을 보이거나 빈발재발형, 스테로이드 의존성, 스테로이드 저항성 등의 소견으로 세포 독성 약제 치료가 필요했던 경우였다. 결 과 : 약 반수의 환아(52.1$\%$)에서만 미세변화형을 보였고, 그 다음으로는 미만성 메산지움증식형 33.1$\%$, 초점 분절사구체신염 5.3$\%$, 막증식사구체신염 2.4$\%$, 막성신병증 2.4$\%$, Ig A 신염 1.8$\%$ 등이었다. 미세변화형의 경우보다 미세변화형이 아닌 진단명인 경우 혈뇨와 스테로이드에 저항성을 보일 확률이 높았다. 총 37명의 스테로이드 저항성 환아 중 장기간에 걸쳐 스테로이드 치료를 시행한 결과 30명(81.0$\%$)에서 마침내 완해를 보였다. 결 론 : 신생검이 필요했었던 환아의 약 절반에서 미세변화형이 아닌 결과를 보였다. 비정형적 특징들 중 혈뇨와 스테로이드 저항성이 미세변화형이 아닌 진단과 가장 연관되었다. 스테로이드 저항성 환아에서도 장기간의 메틸스테로이드 충격요법으로 좋은 완해율을 보였다.

• Childhood Kidney Diseases
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• 제4권2호
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• pp.102-110
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• 2000

목 적 : 고농도 인알부민은 신증후군성 부종의 치료에 효과적으로 사용되어 왔으나 고혈압, 심부전등의 위험이 있고 알부민 투여에 따른 고단백 부하가 병적인 사구체 상피세포의 손상을 가중시켜 신증후군의 임상적 경과에 나쁜 영향을 끼칠 수 있는 것으로 보고되었다. 이에 저자들은 신증후군 환아에서 인알부민 투여가 신증후군의 임상 결과에 미치는 영향을 관찰하기 위하여 전향적인 연구를 시행하였다. 방 법 : 1997년 3월부터 1999년 9월까지 이화의대 부속 목동병원 소아과에 신증후군으로 입원한 환아중 신조직 검사상 미세변화형이었으나 재발하였거나 임상적으로 미세변화형에 합당하였던 환아에게 무작위로 20$\%$ 알부민 1 g/kg과 furosemide 2 mg/kg를 1-3일간 정맥 투여하였다. 이중 표준 스테로이드 치료(prednisolone 2 mg/kg 또는 calcort-한독약품 2.4 mg/kg)에 8주 내에 반응을 보이고 1년 이상 추적 관찰이 가능했던 스테로이드 반응성 신증후군 환아 26명 (알부민 투여군 13명, 대조군 13명)을 대상으로 임상경과를 비교하였다. 결 과 : 1) 완전 관해까지의 스테로이드 치료기간은 알부민 투여군에서 $13.7{\pm}6.4$일의 대조군의 $7.5{\pm}2.8$일보다 유의하게 길었다 (P<0.05). 2) 첫 재발까지의 기간은 알부민 투여군에서 $94{\pm}63.5$일로 대조군의 $190{\pm}106.4$일에 비하여 유의하게 짧았다 (P<0.05). 완전 관해후 1년내 재발율은 알부민 투여군에서 77$\%$ (10/13)로 대조군의 46$\%$(6/13)보다 높았으나 두군간에 유의한 차이는 없었다 (P>0.05). 완전 관해후 1년간 재발 횟수는 알부민 투여군이 $1.9{\pm}0.8$회로 대조군의 $1.5{\pm}0.5$회와 유의한 차이는 없었다 (P>0.05). 3) 일회뇨단백/크레아티닌 비는 알부민 투여군에서 투여 후 2, 4일에 유의하게 증가하였다 (P<0.05). 4) 일일체중감소, 혈압, 혈청 Na, K 농도의 변화에는 두 군간에 유의한 차이가 없었다 (P>0.05). 결 론 : 스테로이드 반응성 신증후군에서 인알부민 투여는 스테로이드에 대한 반응을 지연시키고 첫 재발까지의 기간을 단축시키는등 임상결과를 악화시킬 수도 있으므로 알부민 투여에 신중을 기하여야 할 것으로 생각된다.

• Childhood Kidney Diseases
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• 제19권2호
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• pp.105-111
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• 2015

Purpose: Steroid dependent nephrotic syndrome (SDNS) is a chronic illness in childhood hard to treat. Steroid sparing drugs are often used, because long-term steroid therapy can cause severe side effects. We studied to compare efficacy between MMF and other drugs including cyclosporine and levamisole. Methods: This study was performed retrospectively on patients with SDNS, who were treated at Pusan National University Children's hospital. MMF group included 11 patients who were treated with MMF for at least six months between June 2012 and July 2014. As control groups, cyclosporine group (n=15) and levamisole group (n=18) included patients treated between January 2008 and July 2014. Number of relapse was analyzed in patients treated more than six months, and relapse free for one year was analyzed in patients treated more than one year. Results: In MMF group, ten were boys and mean age at onset was 5.8 years. Mean age at starting of MMF was 8.6 years. Number of relapse in MMF group was reduced significantly after treatment from 3.4 /year to 0.2 /year (P=0.003). There was no significant difference in number of relapse among groups (MMF: 0.2 /year, cyclosporine: 0.5 /year, levamisole: 0.5 /year). Comparing the early relapse within six months after treatment levamisole group was significantly higher than the other two groups (P=0.04). Conclusions: MMF which is used in SDNS significantly reduced the relapse and side effects were rare. In addition, MMF did not show any significant difference in comparison with the other two groups in number of relapse and relapse free for one year.

• Childhood Kidney Diseases
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• 제22권1호
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• pp.1-6
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• 2018

Rituximab (RTX) is a chimeric monoclonal antibody that inhibits CD20-mediated B-cell proliferation and differentiation. Several studies have examined its use in intractable nephrotic syndrome (NS) with some positive results. However, those studies examined such effects for a short-term period of 1 year, and some patients continued to relapse after a lapse in RTX treatment. Our use of RTX as a maintenance therapy (RTX injection when the CD19 cell count exceeded $100-200/{\mu}L$ before relapse) showed some noticeable efficacy. We used RTX in 19 patients with steroid-dependent NS (SDNS). In 12 patients treated with RTX maintenance therapy, only one relapse occurred. The mean treatment period was $23.4{\pm}12.7months$, and the mean number of RTX administrations was $3.9{\pm}1.6$. The relapse rates were decreased (from 2.68/year to 0.04/year), and the drug-free period also increased (from 22.5 days/year to 357.1 days/year) during maintenance therapy. The other seven patients were treated with one cycle of RTX or additional cycles in case of relapse (non-maintenance therapy). Relapse rates were significantly decreased after RTX treatment (from 1.76/year to 0.96/year, P=0.017). The relapse-free period was $15.55{\pm}7.38$ (range, 5.3-30.7) months. No severe side effects of RTX were found except for a hypersensitivity reaction such as fever and chills during its infusion. In conclusion, RTX is considered an effective and safe option to reduce the relapse rate by a single- or maintenance-interval therapy in SDNS.

• Childhood Kidney Diseases
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• 제20권2호
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• pp.45-49
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• 2016

Purpose: This study was performed to evaluate the effects of cyclosporine-A (CsA) on linear growth in pediatric patients with steroid-dependent (SDNS) or resistant nephrotic syndrome (SRNS). Methods: Thirty-five pediatric patients with SDNS or SRNS undergoing glucocorticoid (GC) and/or CsA treatment were retrospectively reviewed. Seventeen patients were treated with GC alone and 18 were treated with GC and CsA. The cumulative doses of GC and CsA were quantified (mg/kg/day). Linear growth during the follow-up period was defined as the difference in Z-score between the initial and final height according to the follow-up period (${\Delta}$ height Z score/year). The associations between linear growth and clinical parameters were analyzed. Results: The linear growth of patients in the two groups was not significantly different (P=0.262). The ${\Delta}$ height Z score/year did not show a significant correlation with the cumulative doses of CsA, but was negatively correlated with the cumulative dose of GC and positively correlated with the Z score for height at the time of diagnosis. Conclusion: In children with SDNS or SRNS undergoing GC therapy, added CsA treatment may not have harmful effects on linear growth.

• Childhood Kidney Diseases
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• 제21권2호
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• pp.61-68
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• 2017

Purpose: To investigate differences in clinical features, blood/urinary findings, and prognosis in different age groups of patients with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods: A total of 469 patients with HSP were analyzed retrospectively from June 2003 to February 2016. We classified patients into child or adult groups based on their age. Results: The adult group had more patients with anemia (child vs. adult; 7.5% vs. 16.4%), and higher immunoglobulin A (IgA) (30.0% vs. 50.0%) levels, C-reactive protein (34.2% vs. 54.0%) and uric acid (3.1% vs. 12.1%) levels than the child group. The child group was highly positive for Mycoplasma pneumoniae immunoglobulin M (IgM) (34.4%). More patients in the child group presented with high levels of antistreptolysin O (24.7% vs. 2.9%) and high C4 (11.5% vs. 4.2%). Low C3 (1.1% vs. 10.2%) levels, and renal involvement with gross hematuria (8.6% vs. 21.5 %), nonnephrotic proteinuria (1.1% vs. 11.2%), and nephrotic syndrome (1.1% vs. 6.0%) were common in the adult group. Adults also had poorer renal outcomes [persistent hematuria/proteinuria (10.5% vs. 32.8%), and chronic kidney disease (0% vs. 11.2%)] than the child group. Risk factors for renal involvement such as older age and higher level of uric acid were only found in the child group. The risk factors for poor renal outcome were nephrotic syndrome in the child group and gross hematuria in the adult group. Conclusion: In this study, child and adult groups presented with different clinical manifestations of HSP. We found that risk factors for renal involvement included age and high uric acid level in the child group. Moreover, nephrotic syndrome in the child group and gross hematuria in the adult group increased the risk of poor renal outcome.

• Clinical and Experimental Pediatrics
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• 제58권6호
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• pp.206-210
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• 2015

Purpose: The use of a 12-week steroid regimen (long-term therapy, LT) for the first episode of idiopathic nephrotic syndrome (NS) reportedly induces a more sustained remission and lower relapse rate than previous regimens, including an 8-week steroid regimen (short-term therapy, ST). Here, we assessed the potential for selective application of 2 steroid regimens (LT vs. ST) based on the days to remission (early responders [ER] vs. late responders [LR]) for the first idiopathic NS episode in children. Methods: Patients were divided into 4 subgroups (ST+ER, ST+LR, LT+ER, and LT+LR) according to the initial steroid regimen used and rapidity of response; the baseline characteristics, relapse rates, and cumulative percentage of children with sustained remission were then compared among the 4 subgroups. Results: Fifty-four children received ST, and the remaining 45 children received LT. As observed in previous studies, children receiving LT showed significantly lower relapse rates during the first year after the first NS episode than those receiving ST. The ST+ER group showed significantly lower relapse rates during the first one year and two years after the first NS episode than the the ST+LR group, whereas there were no significant differences of the relapse rates and duration to the first relapse between the ST+ER and LT+ER groups. Conclusion: We suggest that the initial steroid regimen in idiopathic NS patients can be shortened according to the duration to remission i.e., LT in patients achieving remission after the first week of steroid therapy, and ST in those achieving remission within the first week of steroid therapy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제19권3호
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• pp.175-185
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• 2016

Purpose: To investigate the epidemiology, clinical manifestations, investigations and management, and prognosis of patients with Henoch-Schonlein purpura (HSP). Methods: We performed a retrospective review of 212 HSP patients under the age of 18 years who were admitted to Inje University Sanggye Paik Hospital between 2004 and 2015. Results: The mean age of the HSP patients was 6.93 years, and the ratio of boys to girls was 1.23:1. HSP occurred most frequently in the winter (33.0%) and least frequently in the summer (11.3%). Palpable purpura spots were found in 208 patients (98.1%), and gastrointestinal (GI) and joint symptoms were observed in 159 (75.0%) and 148 (69.8%) patients, respectively. There were 57 patients (26.9%) with renal involvement and 10 patients (4.7%) with nephrotic syndrome. The incidence of renal involvement and nephrotic syndrome was significantly higher in patients with severe GI symptoms and in those over 7 years old. The majority of patients (88.7%) were treated with steroids. There was no significant difference in the incidence of renal involvement or nephrotic syndrome among patients receiving different doses of steroids. Conclusion: In this study, the epidemiologic features of HSP in children were similar to those described in previous studies, but GI and joint symptoms manifested more frequently. It is essential to carefully monitor renal involvement and progression to chronic renal disease in patients ${\geq}7$ years old and in patients affected by severe GI symptoms. It can be assumed that there is no direct association between early doses of steroids and prognosis.

• 대한한방소아과학회지
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• 제34권3호
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• pp.1-17
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• 2020

Objectives The purpose of this study is to analyze clinical studies on effectiveness of herbal medicine in refractory nephrotic syndrome (RNS). Methods We searched the randomized controlled trials (RCTs) with herbal medicine treatment on RNS from the Pubmed, CNKI, OASIS, NDSL, J-stage, and CiNii. The demographic data, duration of illness, intervention, treatment period, outcome, adverse events, and composition of herbal medicine were analyzed for this study. Results 11 RCT studies were selected and analyzed. The children in the control group were given western medicine therapy, and the treatment group was given herbal medicine along with the same western medicine of the control group. The most commonly used herbal medicines were the prescriptions to treat 'Kidney Deficiency with Blood Stasis (腎虛兼瘀血)' which composed of 'Promoting blood circulation (化瘀)', 'Diuresis-inducing (利水)', or 'Heating Yang (溫陽)' medicine based on 'Replenishing Kidney or Spleen (補腎, 補脾)' medicine. In the treatment group, proteinuria and serum lipid was significantly decreased, serum albumin was significantly increased, and total effective rate was significantly higher than the control group. Hypercoagulation and relapse rate was also significantly reduced. Adverse events were significantly lower in the treatment group. Conclusions Herbal medicine treatment on pediatric RNS can be suggested as a new treatment for children who have less response to the conventional therapy. It can also supplement the limitations of the western medicine by reducing adverse events from the steroids and immuno-suppressive agents, and lower the relapse rate as well.