• Childhood Kidney Diseases
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• 제5권2호
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• pp.206-209
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• 2001

Clq 신증은 면역형광검사상 특징적인 Clq 침착이 있고 전자현미경 검사상 전자 고밀도 침착을 보이는 면역 복합체 사구체 신염이다. 주로 스테로이드 저항성 신증후군으로 발현되나 때로는 중등도의 단백뇨와 혈뇨를 동반하는 지속적인 사구체 신염, 신염-신증후군, 또는 반월상 사구체 신염으로 발현된 증례가 보고된 바 있다. 문헌상 34례의 보고가 있고 국내에서는 성인에서 2례가 보고되어 있다. 저자들은 스테로이드 의존성 신증후군 소아에서 신생검으로 진단된 Clq 신증 1례를 경험하였기에 보고하는 바이다.

• Childhood Kidney Diseases
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• 제26권2호
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• pp.101-106
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• 2022

Nephrotic syndrome (NS) is a hypercoagulable state in which children are at risk of venous thromboembolism. A higher risk has been reported in children with steroid-resistant NS than in those with steroid-sensitive NS. The mortality rate of cerebral venous sinus thrombosis (CVST) is approximately 10% and generally results from cerebral herniation in the acute phase and an underlying disorder in the chronic phase. Our patient initially manifested as a child with massive proteinuria and generalized edema. He was treated with albumin replacement and diuretics, angiotensin-converting enzyme inhibitor, and deflazacort. Non-contrast computed tomography showed areas of hyperattenuation in the superior sagittal sinus when he complained of severe headache and vomiting. Subsequent magnetic resonance imaging revealed empty delta signs in the superior sagittal, lateral transverse, and sigmoid sinuses, suggesting acute CVST. Immediate anticoagulation therapy was started with unfractionated heparin, antithrombin III replacement, and continuous antiproteinuric treatment. The current report describes a life-threatening CVST in a child with steroid-dependent NS, initially diagnosed by contrast non-enhanced computed tomography and subsequently confirmed by contrast-enhanced magnetic resonance imaging, followed by magnetic resonance venography for recanalization, addressing successful treatment.

• Clinical and Experimental Pediatrics
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• 제58권8호
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• pp.275-282
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• 2015

While the incidence of nephrotic syndrome (NS) is decreasing in Korea, the morbidity of difficult-to-treat NS is significant. Efforts to minimize treatment toxicity showed that prolonged treatment after an initial treatment for 2-3 months with glucocorticosteroids was not effective in reducing frequent relapses. For steroid-dependent NS, rituximab, a monoclonal antibody against the CD20 antigen on B cells, was proven to be as effective, and short-term daily low-dose steroids during upper respiratory infections reduced relapses. Steroid resistance or congenital NS are indications for genetic study and renal biopsy, since the list of genes involved in NS is lengthening.

• 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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• 제23권1호
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• pp.1-6
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• 2019

Nephrotic syndrome (NS) is the most common glomerular disorder in childhood, and a vast majority of cases are idiopathic. The precise cause of this common childhood disease is not fully elucidated despite significant advancements in our understanding of podocyte biology. Idiopathic NS has been considered "a disorder of T-cell function" mediated by a circulating factor that alters podocyte function resulting in massive proteinuria since the last four decades. Several circulatory factors released from T-cells are considered to be involved in pathophysiology of NS; however, a single presumptive factor has not been defined yet. Extended evidence obtained by advances in the pathobiology of podocytes has implicated podocytes as critical regulator of glomerular protein filtration and podocytopathy. The candidate molecules as pathological mediators of steroid-dependent NS are CD80 (also known as B7-1), hemopexin, and angiopoietin-like 4. The "two-hit" hypothesis proposes that the expression of CD80 on podocytes and ineffective inhibition of podocyte CD80 due to regulatory T-cell dysfunction or impaired autoregulation by podocytes results in NS. Recent studies suggest that not only T cells but also other immune cells and podocytes are involved in the pathogenesis of MCNS.

• 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.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.72-75
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• 2016

Eosinophilic gastroenteritis is a rare disease characterized by prominent eosinophilic tissue infiltration of the gastrointestinal tract. Here, we report a case of eosinophilic gastroenteritis in an 18-year-old patient with prolonged nephrotic syndrome who presented with abdominal pain and peripheral hypereosinophilia. During the previous 2 years, he had visited local Emergency Department several times because of epigastric pain and nausea. He had been treated with steroid-dependent nephrotic syndrome since 3 years of age. Tests ruled out allergic and parasitic disease etiologies. Gastroduodenoscopy with biopsy revealed marked eosinophilic infiltration in the duodenum. Renal biopsy findings indicated minimal change disease spectrum without eosinophilic infiltration. The oral deflazacort dosage was increased, and the patient was discharged after abdominal pain resolved. To our knowledge, this is the first report of eosinophilic gastroenteritis in a patient with minimal change disease.

• 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.

• 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.

• Childhood Kidney Diseases
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• 제3권1호
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• pp.11-19
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• 1999

목 적 : 소아의 미세변화형 신증후군은 원발성 신증후군의 $85\%$이상을 차지하며, 스테로이드 치료로 완전관해가 잘오는 예후가 양호한 질환이다. 그러나 이와 같은 미세변화형 신증후군의 전형적 임상양상은 2-10세에서 주로 나타나며 임상증상과 치료반응이 발병연령이 증가하면서 변화를 보이는데 성인기에는 원발성 신증후군중 비교적 낮은 빈도를 보이며 소아기에 비해 치료에 대한 예후가 불량한 것으로 알려져 있다. 따라서, 본 연구에서는 소아기와 청소년기 미세변화형 신증후군의 임상양상의 차이를 비교하여 청소년기 미세변화 신증후군 환자의 치료에 도움이 되고자 하였다. 방 법 : 미세변화형 신증후군으로 치료받은 216명의 환아를 대상으로 발병연령에 따라 12세미만인 환아군(소아군) 188명과 12세이상 18세미만인 환아군(청소년군)28명으로 구분하여 두 군간의 임상양상의 차이를 비교하였다. 결 과 : 1) 대상기간중 전체 원발성 신증후군은 소아군이 245명, 청소년군이 55명으로 총 300례 였다. 소아군에서는 MCNS 188명($77\%$), FSGS 30명($12\%$), MsPGN 4명($1.6\%$)이었고, 청소년군에서는 MCNS 28명($51\%$), FSGS 12명($22\%$)으로 두 군에서 청소년군에서 MCNS의 빈도가 감소한 반면 FSGS의 빈도는 증가하였다. 2) 평균발병연령은 소아군이 $4.7{\pm}2.7$세, 청소년군이 $14.7{\pm}2.0$세였고 두 군간의 남녀비는 소아군 3.8:1, 청소년군 2.5:1로 차이가 없었다. 3) 혈뇨가 동반된 경우는 소아군 $17\%$, 청소년군 $39.3\%$로 유의한 차이가 있었고(P=0.005),고혈압은 각각 $0.5\%,\;7\%$로 차이가 있었으나 통계학적으로는 의미있는 차이를 보이지는 않았다. 4) 검사소견상 발병당시 24시간 뇨단백량, SPI, albumin, BUN, cholesterol치는 두 군간에 유의한 차이가 없었다. 5) 소아군의 $11.7\%$, 청소년군의 $14.7\%$가 초기 스테로이드 치료로 재발없이 관해가 유지되었으며, 빈발재발형은 각각 $23.9\%,\;14.7\%$, 스테로이드 의존형은 각각 $21.8\%,\;28.6\%$. 스테로이드 저항형은 각각 $13.3\%,\;14.7\%$로 나타났으나 의미 있는 차이는 없었다. 6) 스테로이드 이외의 면역 억제제가 필요했던 경우는 소아군은 $57\%$, 청소년군은 $65\%$였다. 7) 평균재발횟수($0.8{\pm}0.84$회/6개월 Vs $0.8{\pm}0.79$회/6개월) 및 관해에서 첫 재발까지의 기간은($7.7{\pm}8.4$개월 Vs $4.9{\pm}5.8$개월) 두 군간에 의미 있는 차이는 없었다. 결 론 : 소아 미세변화형 신증후군의 임상양상의 비교에서 혈뇨의 빈도는 청소년 환자군에서 의미 있게 높았다. 스테로이드 의존형과 빈발재발형의 비율, 재발의 빈도는 청소년환자군에서 비교적 높게 나타나 치료에 대한 반응성이 떨어지는 양상을 보이므로 청소년기 미세변화형 치료에 있어서 이러한 문제들이 인식되어야 하고 보다 적극적인 면역억제제의 선택이 고려되어야 할 것이다.