• Childhood Kidney Diseases
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• 제9권1호
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• pp.108-111
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• 2005

세균성 복막염은 신증후군 환아의 생명을 위협하는 중요한 감염성 합병증으로 초기에 광범위한 항생제 병용치료가 이루어져야한다. 저자들은 신증후군 진단 후 치료가 지연된 환아에서 발생한 복막염 및 복부농양 증례를 경험하였기에 보고하는 바이다.

• Childhood Kidney Diseases
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• 제10권2호
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• pp.238-243
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• 2006

Nephrotic syndrome in childhood is known to be associated with a hypercoagulable state and thromboembolic complications, among which cerebral venous thrombosis is a very rare and serious one, with only a few isolated reports in the literature. A 9-year-old boy with known nephrotic syndrome was admitted due to a relapse with massive proteinuria and generalized edema. He complained of a prolonged frontal headache. The enhanced brain magnetic resonance imaging(MRI) showed a high signal in the region of the superior sagittal sinus and right transverse sinus consistent with a thrombus. He was managed with steroids, cyclosporine and warfarin. His headache subsided 2 weeks later and proteinuria resolved 1 month later. An MRI 2 months later was normal. We describe this case and review the literature to emphasize the importance of recognizing this potentially life threatening complication and initiating anticoagulation therapy.

• Childhood Kidney Diseases
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• 제27권1호
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• pp.1-10
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• 2023

Pediatric nephrotic syndrome (NS) is a clinical syndrome characterized by massive proteinuria, hypoalbuminemia, and generalized edema. Most childhood NS cases are idiopathic (with an unknown etiology). Traditional therapeutic approaches based on immunosuppressive agents largely support the key role of the immune system in idiopathic NS (INS), especially in the steroid-sensitive form. Although most previous studies have suggested the main role of T cell dysfunction and/or the abnormal secretion of certain glomerular permeability factors, recent studies have emphasized the role of B cells since the therapeutic efficacy of B cell depletion therapy in inducing and/or maintaining prolonged remission in patients with INS was confirmed. Furthermore, several studies have detected circulating autoantibodies that target podocyte proteins in a subset of patients with INS, suggesting an autoimmune-mediated etiology of INS. Accordingly, a new therapeutic modality using B cell-depleting drugs has been attempted, with significant effects in a subset of patients with INS. Currently, INS is considered an immune-mediated disorder caused by a complex interplay between T cells, B cells, soluble factors, and podocytes, which may vary among patients. More in-depth investigations of the pathogenic pathways of INS are required for an effective personalized therapeutic approach and to define precise targets for therapeutic intervention.

• Childhood Kidney Diseases
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• 제3권2호
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• pp.145-152
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• 1999

목적 : 최근 학동기 소아의 집단 신체검사에 소변검사가 포함된 후 소아 신장질환에 대한 관심이 높아지고 있으나 우리나라에서 소아에서의 신증후군을 포함한 신질환의 유병율은 잘 알려져 있지않으며 과거에 비해 변화가 있는지도 확실하지 않다. 저자들은 지난 13년동안 대전시와 인근 지역으로부터 입원한 모든 신증후군 환아의 연도별 발생수와 입원환아에 대한 비율 등을 알아보고 임상 및 신조직병리학적 소견에 따라 원인질환을 분석하므로써 단일 병원에서 조사된 결과가 지금까지 알려진 소아 신증후군의 연구결과와 얼마나 근접하는지 알아보고 신증후군의 유병률의 변화를 짐작하는데 도움을 받고자 본 연구를 시행하였다. 방법 : 1986년 5월부터 1998년 12월까지 13년동안 입원한 신증후군 환아 96명을 대상으로 입원 및 외래기록지를 후향적으로 분석하였다. 소아 신증후군의 발생빈도는 대전시내 3개의 종합병원의 도움을 얻었다. 결과 : 연도별 환아수는 1987년 8명, 1993년 8명, 1998년 6명으로 평균 환아수는 $7.4{\pm}2.1$명이었으며, 총입원환아에 대한 비율은 1987년 0.40%, 1993년 0.27%, 1998년 0.13%으로 평균 $0.30{\pm}0.11%$를 보였다. 15세 이하 소아 10만명당 발생빈도는 1988턴 5.6명, 1993년 5.5명 및 1998년 4.8명이었다. 초발시 연령은 평균 $6.8{\pm}3.5$세로 $1{\sim}4$세 26명(27.1%), $5{\sim}7$세 29명(30.2%), $8{\sim}10$세 25명(26.0%), 11세 이상 16명(16.7%)이었다. 일차성 신증후군의 평균연령는 $6.4{\pm}3.3$세, 이차성 신증후군의 평균연령은 $11.6{\pm}3.4$세이었다. 남아가 75명으로 남녀비는 3.6:1을 보였다. 임상병리학적 분류상 일차성 신증후군이 89명(92.7%), 이차성 신증후군이 7명(7.3%)이었으며 일차성 신증후군에서 minimal change nephrotic syndrome이 71명으로 79.8%, focal segmental glomerulosclerosis 11.2%, mesangial proliferation 4.5%, membranoprolifrative glomeulonephritis 3.4%, membranous nephropathy 1명 1.1%이었으며 2차성 신증후군은 $Henoch-Sch\"{o}nlein$ nephritis가 3례로 가장 많았다. 미세변화 신증후군 71명 중 비재발군이 16명으로 22.5%, 비빈발 재발군 49.3%, 빈발 재발군 18.3%, 스테로이드 의존군 9.9%를 보였다. 결론 : 대전시에서의 신증후군 환아의 발생빈도는 15세이하 소아 10만명당 약 5명으로 추정되었으며 10여 년전과 비교하여 큰 변화를 보이지는 않았다. 또한 저자들의 임상병리학적 연구결과가 다른 문헌에서 보고된 소아 신증후군의 연구결과와 큰 차이를 보이지 않음을 알 수 있었다.

• 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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• 제10권2호
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• pp.132-141
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• 2006

Purpose : This study was aimed to determine the predictive risk factors for the treatment response and relapse rate in children diagnosed with idiopathic nephrotic syndrome. Methods : We analyzed the medical records of children who were diagnosed and treated for childhood idiopathic nephrotic syndrome from November 1991 to May 2005. Variables selected in this study were age at onset, sex, laboratory data, concomitant bacterial infections, days to remission, and interval to first relapse. Results : There were 46 males and 11 females, giving a male:female ratio of 4.2:1. The age($mean{\pm}SD$) of patients was $5.8{\pm}4.1$ years old. Of all patients who were initially given corticosteroids, complete remission(CR) was observed in 54(94.7%). Of the 54 patients who showed CR with initial treatment, 40(70.2%) showed CR within 2 weeks and 14(24.6%) showed CR after 2 weeks. The levels of serum IgG were lower in the latter group who showed CR after 2 weeks(P=0.036). Of the 54 patients who showed CR with initial treatment, 47(82.5%) relapsed. Of these patients, 35.1% were frequent relapsers and 43.9% were infrequent relapsers. There was no significant correlation between the frequency of relapse and the following variables : sex, days to remission, and laboratory data. However, age at onset and interval to first relapse had a negative correlation with the frequency of relapse(Pearson's coefficient=-0.337, -0.433, P<0.012, P<0.01). Conclusion : The age at onset and the interval to first relapse were found to be predictive clinical parameters for the relapse rate, while the levels of serum IgG at initial presentation were a predictive laboratory factor for treatment response in childhood idiopathic nephrotic syndrome.

• Childhood Kidney Diseases
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• 제3권1호
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• pp.100-103
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• 1999

Focal Segmental Glomerulosclerosis(FSGS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. There is no ultimate curative treatment except renal transplantation. We have experienced a case of infantile nephrotic syndrome In a 10 month old boy who presented with proteinuria and hematuria. His elder brother also suffered from nephrotic syndrome and died at the age of 18 months due to sepsis. We have diagnosed this patient with clinical manifestations, laboratory data and pathologic findings which was done by open renal biopsy. The patient expired 54 days after admission because or progressive uremia and sepsis.

• Childhood Kidney Diseases
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• 제26권2호
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• pp.74-79
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• 2022

Purpose: Children with nephrotic syndrome may experience disease relapse or aggravation triggered by various viral infections. Limited studies on the clinical implications of the coronavirus disease 2019 (COVID-19) pandemic in children with nephrotic syndrome have been published worldwide. Therefore, this study aimed to investigate the effects of COVID-19 on the clinical course of nephrotic syndrome in children. Methods: The medical records of 59 patients with idiopathic nephrotic syndrome who visited our hospital between February and June 2022 were retrospectively analyzed. Results: Twenty of the total 59 patients with nephrotic syndrome were diagnosed with COVID-19 during the study period. The mean age at the time of the diagnosis of nephrotic syndrome and COVID-19 in all 20 patients was 4.6±3.5 and 8.9±3.9 years, respectively. Three patients (15%) were diagnosed with nephrotic syndrome relapse during COVID-19 and the relapse rate was similar to them without COVID-19 (20.5%, 8/39 patients). At the time of the COVID-19 diagnosis, fever (85%) and cough (40%) were the most common symptoms. After the diagnosis of COVID-19, all patients showed improvement with symptomatic treatment, including antipyretic analgesics and cold medicine. None of the critical patients required hospitalization or oral antiviral medications. Conclusions: Despite the use of immunosuppressants, the clinical manifestations of COVID-19 in children with nephrotic syndrome were not severe and are expected to be similar to that in the general population. The relapse rate of nephrotic syndrome in children with COVID-19 was also not different from them without COVID-19.

• Childhood Kidney Diseases
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• 제23권2호
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• pp.86-92
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• 2019

Steroid-resistant nephrotic syndrome (SRNS) has long been a challenge for clinicians due to its poor responsiveness to immunosuppressants, and rapid progression to end-stage renal disease. Identifying a monogenic cause for SRNS may lead to a better understanding of podocyte structure and function in the glomerular filtration barrier. This review focuses on genes associated with slit diaphragm, actin cytoskeleton, transcription factors, nucleus, glomerular basement membrane, mitochondria, and other proteins that affect podocyte biology.

• Childhood Kidney Diseases
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• 제27권2호
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• pp.117-120
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• 2023

Here, we present the case of a 2-month-old male infant with hyponatremic hypertensive syndrome resulting from stenosis of the right proximal and mid-renal arteries. The patient exhibited nephrotic-range proteinuria, low serum albumin, increased serum creatinine, and elevated renin and aldosterone levels. Doppler ultrasonography and computed tomography angiography revealed decreased vascular flow in the small right renal artery. Following a successful percutaneous balloon angioplasty, the patient experienced a decrease in blood pressure and normalization of serum electrolyte levels within a few days. However, it took 3 months for the proteinuria to resolve completely. This case is significant as it represents the first reported instance of a neonate presenting with clinical features resembling congenital nephrotic syndrome caused by renal artery stenosis that was successfully treated with percutaneous renal angioplasty.