• 대한한의학회지
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• 제21권1호
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• pp.20-28
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• 2000

The purpose of this research is to investigate the synergistic effect of herb medicines with hydrocortisone and the regulation effect on the immune system of Onbitang and Dangguijakyaksan at the supernatant of PHA-P stimulated PBMC in the patients with minimal change nephrotic syndrome(MCNS). From the measurement of the concentration rate of IL-4, sCD23 and IL-13, the experiment yielded the following results : The Onbitang group showed a greater tendency to suppress IL-4 and IL-13 levels in MCNS group with no statistical significance. It showed very strong suppression in soluble CD23 compared with control group in MCNS group. The Dangguijakyaksan group, though not statistically significant, was inclined to suppress IL-4 level in MCNS group. It shows stronger suppression in sCD23 and IL-13 levels than these of control group in MCNS group. As for the synergistic effect, the group of hydrocortisone with herb medicines(Onbitang or Danguijakyaksan) produced more suppressive effect to IL-13 level in MCNS group than that of hydrocortisone-only group. They also tended to suppress sCD23 and IL-4 levels, though no statistical significance can be given. As to the suppressive effect of 1L-13 level, the group of Onbitang with hydrocortisone showed an increase of 22.6%, while the group of Dangguijakyaksan with hydrocortisone showed 14.7%. So Onbitang is more effective than Dangguijakyaksan. From the above results, a combinative treatment(herb medicines with hydrocortisone) can be an alternative method to substitute for steroid therapy. It can be a more effective therapy than steroid-only therapy because it is expected to reduce side effects and it shows more special effect to suppress IL-13 level. Based on the present results, further investigation concerning the serum IgE elevation is needed.

• Kidney Research and Clinical Practice
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• 제37권4호
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• pp.347-355
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• 2018

Background: Nephrotic syndrome (NS) is the most common glomerulopathy in children. Acute kidney injury (AKI) is a common complication of NS, caused by severe intravascular volume depletion, acute tubular necrosis, interstitial nephritis, or progression of NS. However, the incidence and risk factors of childhood-onset NS in Korea are unclear. Therefore, we studied the incidence, causes, and risk factors of AKI in hospitalized Korean patients with childhood-onset NS. Methods: We conducted a retrospective review of patients with childhood-onset NS who were admitted to our center from January 2015 to July 2017. Patients with decreased renal function or hereditary/secondary NS, as well as those admitted for management of other conditions unrelated to NS, were excluded. Results: During the study period, 65 patients with idiopathic, childhood-onset NS were hospitalized 90 times for management of NS or its complications. Of these 90 cases, 29 met the Kidney Disease Improving Global Outcomes criteria for AKI (32.2%). They developed AKI in association with infection (n = 12), NS aggravation (n = 11), dehydration (n = 3), and intravenous methylprednisolone administration (n = 3). Age ${\geq}9$ years at admission and combined use of cyclosporine and renin-angiotensin system inhibitors were risk factors for AKI. Conclusion: AKI occurred in one-third of the total hospitalizations related to childhood-onset NS, owing to infection, aggravation of NS, dehydration, and possibly high-dose methylprednisolone treatment. Age at admission and use of nephrotoxic agents were associated with AKI. As the AKI incidence is high, AKI should be considered during management of high-risk patients.

• Kidney Research and Clinical Practice
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• 제36권3호
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• pp.257-263
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• 2017

Background Rituximab (RTX) can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS). However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children. Methods Eighteen consecutive child patients with SDNS who were treated with three or more cycles of RTX for one year or longer were recruited, and their medical records were retrospectively reviewed. Results The patients were followed for $4.7{\pm}1.9years$ and received $5.2{\pm}2.3cycles$ of RTX over $2.8{\pm}1.1years$. Approximately 70% of the additional RTX cycles were administered due to recovery of B-cells without relapse. The relapse rate decreased from $3.4{\pm}2.0per$ year initially to $0.4{\pm}0.8per$ year at the third year after RTX treatment. Approximately 10% of the RTX infusions were accompanied by mild infusion reactions. Eight patients showed sustained remission without any oral medication after the last cycle of RTX, while 10 patients had one or more episodes of relapse after the last cycle of RTX. The relapse rate in the latter group decreased from $2.8{\pm}1.5per$ year before RTX treatment to $1.3{\pm}0.8per$ year after cessation of RTX treatment. No significant differences in clinical parameters were found between the two groups. Conclusion This retrospective study showed that pre-emptive and long-term, repeated RTX treatment is relatively effective and safe in children with SDNS. However, well-designed prospective studies are needed to confirm these findings.

• Journal of Korean Neurosurgical Society
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• 제66권6호
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• pp.735-742
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• 2023

Nephrotic syndrome (NS) is associated with cerebral venous sinus thrombosis (CVST), which is a rare cerebrovascular disorder in children. Systemic anticoagulation with heparin is the standard therapy for CVST, and mechanical thrombectomy (MT) has been described as a salvage treatment for adult anticoagulant refractory CVST, However, it has never been reported in children. We describe a case of MT for refractory CVST in a child with NS. A 13-year-old boy with newly diagnosed NS presented to an emergency department with acute headache. A head computed tomography showed acute thrombus in the superior sagittal sinus, straight sinus and transverse sinus. The child was started on heparin therapy, but clinically deteriorated and became unresponsive. In view of the rapid deterioration of the condition after anticoagulation treatment, the patient received intravascular treatment. Several endovascular technologies, such as stent retriever and large bore suction catheter have been adopted. After endovascular treatment, the patient's neurological condition was improved within 24 hours, and magnetic resonance venography of the head demonstrated that the CVST was reduced. The child recovered with normal neurological function at discharge. This case highlights the importance of considering MT for refractory CVST, and we suggest that MT may be considered for refractory CVST with NS in children.

• Child Health Nursing Research
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• 제5권2호
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• pp.125-135
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• 1999

The purpose of this study was to identity the level of burden and quality of life of the subjects. The subjects of this study were 68 mothers of nephrotic syndrome patients who children hospitalized in 2 Pediatric wards of University Hospital in Seoul. The data were collected through a questionnaires and the period of the data collection was from August 1st to September 30th ,1998. The instruments for this study were Burden Measurement Instrument developed by Montgomery et. al (1985) and Quality of life scale designed by Ro, Yoo JA(1988). The statistical analysis was used by SPSS, t-test, ANOVA and Pearson correlation coefficient. The results of were as follows. : 1. The level of burden showed a mean score 54.47 and the level quality of life, a mean score 140.20. 2. The level of burden differed according to s religion, pt's purpose of admission and perceived patient's condition by mothers. 3. The level of quality of life differed according to perceived pt's condition by mothers. 4. There was a negative correlation between burden and quality of lifer =-3.97, p<.001).

• Childhood Kidney Diseases
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• 제13권2호
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• pp.189-196
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• 2009

목적 : 신증후군에서 사구체 단백 투과성이 증가하는 것은 전신적인 순환 인자와 관련되어 있는 것으로 여겨진다. 전신적인 순환 인자의 사구체 외 기관에서의 영향과 관련하여, 본 연구에서는 복막 투석 중인 steroid resistant nephrotic syndrome (SRNS) 환자에서의 복막투석을 통한 단백 소실 정도를 파악하고자 하였다. 방법 : 2001년부터 2009년까지 복막 투석 중인 신증후군 환자 12명(SRNS)과 대조군 14명을 대상으로 후향적 환자-대조군 연구를 시행하였다. 성별, 투석 시작 시 연령, 체중, 신장, 투석 방법, 투석양, 투석 시작 시 검사 소견(혈액 요소 질소, 혈청 크레아티닌, 혈청 단백, 혈청 알부민, 24시간 투석액 부피, 24시간 투석액 단백, Kt/V)과 1년 뒤 투석 시 검사 소견(24시간 투석액 단백)을 조사하였다. 단백질 섭취 정도를 평가하기 위해 nPNA (normalized protein equvalent of total nitrogen appearance)를 측정하였다. 결과 : SRNS군과 대조군은 nPNA를 비롯한 다른 지표에 의미 있는 차이를 보이지 않았으나, SRNS군에서 혈청 알부민이 $3.7{\pm}0.3$ g/dL로 대조군($4.0{\pm}0.4$ g/dL, P=0.021)보다 낮았다. 복막액을 통한 단백 소실량은 SRNS군에서 대조군보다 통계학적으로 의미 있게 높았으며($3,044.4{\pm}837.6\;mg/m^2$/day vs. $1,791.6{\pm}1,244.0\;mg/m^2$/day, P=0.007), 혈장 단백에 대한 복막의 투과성은 SRNS군에서 대조군보다 2배 가량 높았다($1.06{\pm}0.46%$ vs. $0.58{\pm}0.43%$, P=0.010). SRNS군과 대조군 모두 초기와 1년 뒤 복막 단백 소실량이 증가하였으나 대조군에서 더 큰 차이를 보였으며 복막 단백 소실량의 시간 경과에 따른 변화는 두 군(SRNS군 vs. 대조군)간에 의미있게 차이가 있었다(P=0.023). 결론 : 신증후군을 앓고 있는 소아 환자에서 복막 투석을 시행할 때는 이들의 영양상태, 성장 및 발달에 평가가 보다 적극적으로 이루어져야 하고, 복막을 통한 단백 소실을 모니터링하여 이를 보충하려는 노력 또한 필요하리라 생각된다.

• 한국임상약학회지
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• 제14권1호
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• pp.11-23
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• 2004

Although most children with idiopathic nephrotic syndrome respond to corticosteroid therapy, many responders show steroid dependency and frequent relapse. In these children, one of the major problems is the serious side effects resulting from continuous steroid therapy. Thus, this study was conducted to assess the therapeutic efficacy and safety of six-month cyclosporine treatment with the low-dose deflazacort therapy in children with nephrotic syndrome. Thirty children with steroid dependence (SD), frequent relapse (FR) and steroid resistance (SR) were enrolled in this study. They were treated with 6-month oral cyclosporine $(Cypol-N^{(R)})$ plus the low-dose deflazacort $(Calcort^{(R)})$ therapy at Samsung Medical Center from September 2002. The dosage of cyclosporine was started at 5 mg/kg/day and was monthly adjusted to maintain clinical remission and/or a trough blood level, while deflazacort dosage was reduced gradually. Clinical evaluation and monitoring of cyclosporine toxicity were performed every $2\sim4$ weeks. Outcomes were compared to the latest sir-month period of steroid only therapy before cyclosporine treatment. Student's t-test and ANOVA were used for statistical analysis. Out of 28 children with SD and FR, 23 $(82.1\%)$ sustained remission, and 5 $(17.9\%)$ experienced 1 or 2 relapses during therapy. Out of 2 children with SR, 1 child sustained remission, and 1 child showed no response. The mean duration of remission and occurrence of relapse were significantly improved (p <.0001). In addition, the mean dosage of steroid was significantly reduced (p=.003). Although a number of adverse effects occurred in this study, they were not so serious as to necessitate discontinuation of the therapy. No nephrotoxicity was observed. Twenty out of the 28 children who had been in remission relapsed after withdrawal of cyclosporine. Fifteen of these children showed relapse within a month. These results demonstrated that the combination of cyclosporine with the low-dose deflazacort was efficient and safe in children with SD and FR during the six-month treatment. However, further studies are necessary in order to resolve the problem of high relapse rate after discontinuation of cyclosporine.

• Childhood Kidney Diseases
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• 제1권1호
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• pp.13-16
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• 1997

스테로이드에 의한 골다공증은 특히 성장하는 소아 신증후군에서 심각한 문제이다. 이러한 경우에 활성형 비타민 D원알파, 일성신약)를 1년간 투여하고 그 효과를 보기 위하여 여러가지 골대사 지표를 치료 전후에 검사하여 보았다. 대상 환자는 40명의 빈회재발형 신증후군 환아이며, 본 연구를 시작하기전까지 환자의 스테로이드 치료기간은 $50{\pm}29$ 개월 이었다. 성인에서 골형성지표로서 잘알려진 혈청 osteocalcin(ng/ml)은 치료전 $7.75{\pm}3.34$, 치료후 $9.38{\pm}5.06$으로 증가 되었고, 골 흡수 지표로 장 알려진 소변 pyridinoline(nmol/mmol Cr)은 치료전 $417.26{\pm}250.98$, 치료후 $462.59{\pm}265.15$로 증가되어 소아 에서 골대사 지표로서 유의하지 않았다. 그러나 골밀도는 $0.71{\pm}0.016$에서 $0.73{\pm}0.015$로 의미 있게 증가하였다(p<0.05). 스테로이드에 의한 골다공증때 활성형 비타민의 투여로 골다공증을 예방할 수 있을것으로 사료되며, 골 형성 및 골 흡수의 지표로서 성인에서 많이 쓰이는 검사법은 소아의 경우는 예민하지 않았으며, 골밀도 검사만이 유의한 것으로 나타났다.

• Childhood Kidney Diseases
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• 제11권1호
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• pp.16-23
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• 2007

목적 : 저감마글로불린혈증은 모든 신증후군에서 관찰되고 있으나 그 병태생리는 알려져 있지 않다. 미세 변화 신증후군 환아들의 혈청 IgG와 알부민 값 간의 상관관계를 분석하고자 하였다. 방법 : 신증후군으로 진단된 46명의 환아들(단백뇨 $>40mg/m^2/h$, 혈청 알부민 값 <2.5g/dL의 초발 입원 시의 IgG, 알부민 및 층 콜레스테롤 값을 취하고, 각 지표간의 상관관계를 통계학적으로 분석하였다. 결과 : 신증후군 환아들의 평균 알부민, IgG 및 총 콜레스테롤 값은 각각 $1.7{\pm}0.3g/dL,\;368{\pm}143mg/dL,\;431{\pm}78mg/dL$이었다. 혈청 알부민과 총 콜레스테롤 값의 관계에서, 두 지표는 서로 반비례를 보였다(r=0.58, P=0.0001), 한편 알부민과 IgG 값들 간의 관계에서 정비례 관계를 보였다. (r=0.37, P=0.01). 결론 : 신증후군에서 IgG 값은 알부민 값과 연관이 있으며, 소변 내 단백 손실의 정도를 반영한다. 이러한 현상을 밝히기 위한 추가 연구가 필요 할 것으로 보인다.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.123-129
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• 1997

목적 : 소아의 스테로이드 저항성 신증후군은 예후가 불량함에도 불구하고 지금까지 효과적인 치료방법이 없는 병이었으나 1990년 Mendoza등이 Methylprednisolone 충격 요법이 스테로이드 저항성 신증후군에서 효과적이라는 것을 보고하였다. 하지만 2년 뒤 Waldo 등은 Methylprednisolone 충격 요법이 Mendoza 등의 보고와는 다르게 스테로이드 저항성 신증후군에 효과가 적었다고 보고하여 이에 저자는 한국 소아에서 스테로이드 저항성 신증후군이 발생한 경우 Methylprednisolone 충격 요법의 효과를 알아보고 위의 보고의 서로 다른 결과를 확인하고자 본 연구를 시행하였다. 방법 : 1990년부터 1995년까지 만 5년간 서울대병원 소아과에 스테로이드 저항성 신증후군으로 입원한 소아를 대상으로 Methylprednisolone 충격 요법을 시행하여 $30{\pm}11$개월동안 추적 관찰하였다. 결과 : 1) Methylprednisolone 충격 요법 치료로 신증후군이 완해가 유도된 환아는 20명중 9명으로 45%이었다. 2) Methylprednisolone 충격 요법 치료후 신증후군의 완해가 유지된 환아는 20명중 9명으로 45%이었다. 3) Methylprednisolone 충격 요법에 반응이 없었던 환아중 만성 신부전으로 이행된 경우는 5례로서 25%이었다. 결론 : Methylprednisolone 충격 요법은 스테로이드 저항성 신증후군 소아의 45%에서 신증후군의 완해를 유도하고 유지시키는 효과적인 치료 방법이었다.