• Childhood Kidney Diseases
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• 제2권1호
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• pp.73-76
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• 1998

Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

• Childhood Kidney Diseases
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• 제9권1호
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• pp.91-96
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• 2005

CsA 유발성 중추 신경독성은 신증후군 환자에서 드물게 보고되고 있다. 저자들은 신 증후군환아에서 CsA 치료 중에 뇌 자기공명 영상으로 확진한 급성 백색질 뇌증 1례를 경험하였기에 보고하는 바이다.

• Childhood Kidney Diseases
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• 제9권2호
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• pp.245-250
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• 2005

저자들은 정맥 주사용 스테로이드 충격요법과 경구 cyclophosphamide 치료를 받던 신증후군 환아에서 가역성 후백질뇌병증 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제7권1호
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• pp.91-95
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• 2003

저자들은 4세에 신증후군으로 진단받고 스테로이드를 투여하며 재발을 수 회 반복하던 환자가 갑작스런 복통과 구토를 주소로 입원하여 시행한 검사 상 급성 무결석 담낭염으로 진단받고 금식, 비 위관 배액, 충분한 수액 공급, 그리고 항생제치료로 완치된 경우를 경험하였기에 이를 보고하는 바이다.

• Childhood Kidney Diseases
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• 제14권2호
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• pp.218-222
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• 2010

최근의 유행한 신종 H1N1 S-OIV는 전세계적으로 퍼지고 있고, 이에 동반된 호흡기 질환등의 여러 합병증들이 보고되었다. 신증후군은 간염바이러스 등의 감염 후에 발현될 수 있는 것으로 알려져 있으나 S-OIV 감염 후에 발생한 예는 보고된 적이 없는 실정이다. 저자들은 S-OIV 감염으로 확진된 환아에서 발생한 신증후군이 발생하였으나 스테로이드 치료로 쉽게 치료된 경험을 하였기에 이를 보고하는 바이다.

• 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.

• Childhood Kidney Diseases
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• 제7권2호
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• pp.197-203
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• 2003

다양한 신경학적 이상을 동반한 소두증과 조기발현 신증군을 보이는 Galloway-Mowat 증후군은 치료반응이 불량하며 신기능의 저하가 진행되 조기에 사망하는 드문 유전성 질환이다. 임상적, 조직학적 다양성을 보이는 소두증과 신증후군을 동반한 일련의 질환군에 대해 아직 그 견해가 정립되지 않았고 향후 지속적인 연구가 이어질 것으로 생각되며 유전성 및 예후 등을 고려할 때 주목해야 할 필요가 있다고 생각되어 저자들이 경험한 소두증과 신경학적 이상을 동반한 조기발현 신증후군 1례를 보고하는 바이다.

• Childhood Kidney Diseases
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• 제2권2호
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• pp.183-186
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• 1998

Steroid Resistant Nephrotic Syndrome(SRNS) in children has poor prognosis and no effective therapy. In 1994, Ravi Elhence have reported that IV cyclophosphamide therapy was effective against SRNS of children. So, we evaluated the efficacy of IV cyclophosphamide in 3 children with biopsy proven steroid-resistant MCNS. And the result was the rapeutic failure. In conclusion, IV cyclophosphamide therapy wass not effective against SRNS of children.

• Childhood Kidney Diseases
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• 제3권2호
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• pp.217-220
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• 1999

Kimura disease is a chronic benign disorder, primarily seen in asians male during the second and third decades of life, which presents itself as a tumour like lesion with a predilection for the head and neck region. There is high prevalence of associated renal disease. We report two cases of nephrotic syndrome associated with Kimura disease, and this is the first report of Kimura disease with renal involvement in Korean children.

• Childhood Kidney Diseases
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• 제23권1호
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• pp.7-21
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• 2019

Nephrotic syndrome (NS) is a common chronic glomerular disease in children characterized by significant proteinuria with resulting hypoalbuminemia, edema, and hyperlipidemia. Renal biopsy findings of diffuse foot processes effacement on electron microscopy and minimal change disease, focal segmental glomerulosclerosis (FSGS), or diffuse mesangial proliferation on light microscopy. It has been speculated that circulating permeability factors would be implicated in the pathogenesis of NS because they have been reportedly detected in the sera of patients and in experimental models of induced proteinuria. Moreover, a substantial portion of the patients with primary FSGS recurrence shortly after transplantation. This report reviews the current knowledge regarding the role of circulating permeability factors in the pathogenesis of proteinuria in NS and suggests future targeted therapeutic approaches for NS.