• Childhood Kidney Diseases
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• v.9 no.1
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• pp.108-111
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• 2005

Nephrotic syndrome is a common chronic disease in childhood. Patients with nephrotic syndrome are at an increased risk of bacterial Infections due to immunological changes secondary to proteinuria. Primary bacterial peritonitis is one of the most serious and common infectious complications. The rate of peritonitis is, 2-6% and overwhelming infection still carries a mortality rate of 1.5%. We experienced a rare case of nephrotic syndrome complicated with severe peritonitis and Peritoneal empyema in a 10-year old girl after 2 months of medical neglect by parents. Here we emphasize thf: importance of early detection and treatment of peritoneal infection in nephrotic syndrome.

• Childhood Kidney Diseases
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• v.10 no.2
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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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• v.27 no.1
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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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• v.3 no.2
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• pp.145-152
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• 1999

Purpose: The prevalence of childhood renal diseases including the nephrotic syndrome in Korea has not been well established. In priority to this point, we analyzed ninety-six nephrotic children of the annual incidence, the ratio of them to all inpatients and estimated incidence of childhood nephrotic syndrome under 15 years of age in Taejon, Korea. Also we classified them by clinical and histopathological findings. Methods: we analyzed the admission and outpatient records retrospectively between June 1986 and December 1998. For the estimation of incidence, we were assisted with three other general hospitals in Taejon Results: The mean annual number and the ratio to inpatients were $7.4{\pm}2.1,\;0.3{\pm}0.11%$ respectively. The latter showed a tendency to decrease during the recent three years. The estimated annual incidences of childhood nephrotic syndrome in Taejon were 5.6 in 1988, 5.5 in 1993 and 4.8 in 1998 per 100,000 for children aged up to 15 years. The mean age of our patients was $6.8{\pm}3.5$ tears, 1-7 years of age was 55 cases(57.3%), and male to female ratio was 3.6:1. By the clinicopathologic classification, 89 cases(92.7%) were classified as primary nephrotic syndrome, and 7 cases(7.3%) as secondary nephrotic syndrome. Among the primary nephrotic syndrome, there was 79.8% of minimal change nephrotic syndrome, focal segmental glomerulosclerosis 11.2%, mesangial proliferation 4.5%, membranoproliferative glomerulonephritis 3.4%, and membranous nephropathy 1.1%. $Henoch-Sch\"{o}nlein$ nephritis was the most frequent of 3 cases in the secondary nephrotic syndrome. Respones to steroid therapy of 71 cases minimal change were classified as non-relapse 22.5%, non-frequent relapse 49.3%, frequent relapse 18.3% and steroid-dependence 9.9%. Conclusion: The estimated incidence of childhood nephrotic syndrome was about 5 per 100,000 for children aged up to 15 years and it showed little changes during 10 years in Taejon, Korea. Our results of clinicopathologic study was little difference from the results reported in other literatures.

• Childhood Kidney Diseases
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• v.23 no.1
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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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• v.10 no.2
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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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• v.3 no.1
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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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• v.26 no.2
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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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• v.23 no.2
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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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• v.27 no.2
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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.