• Childhood Kidney Diseases
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• v.25 no.2
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• pp.92-111
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• 2021

Purpose: Nephrotic syndrome (NS) is the most common form of glomerulopathy in children. Most pediatric patients respond to glucocorticosteroid treatment (steroid-sensitive NS, SSNS), while approximately 10-15% will remain unresponsive or later become steroid-resistant. There has been a long-standing effort to find biomarkers that may predict steroid responsiveness. Methods: We systematically reviewed current studies which investigated clinically relevant biomarkers for predicting steroid responsiveness in pediatric NS. We performed a PubMed and EMBASE search to identify eligible articles. We collected data on urinary markers, blood/serum markers (including cellular phenotypes and mRNA expression), genotypes and HLA allele frequency. Results: A total of 659 articles were identified following electronic and manual searches. After reviewing the titles, abstracts, and full texts, 72 eligible articles were finally included. Vitamin D-binding protein (VDBP) seemed to be significantly elevated in SRNS than in SSNS, in both serum and urine specimen, although further validation is required. Conclusions: The present paper narratively illustrates current understandings of potential biomarkers that may help predict steroid responsiveness. Further investigation and collaboration involving a larger number of patients are necessary.

• Childhood Kidney Diseases
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• v.26 no.2
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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.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.92-99
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• 2007

The posterior reversible encephalopathy syndrome(PRES) is characterized clinically by a combination of acute or subacute confusion, lethargy, visual disturbance, and seizures. PRES has been described in various clinical settings, including severe hypertension, chemotherapy, eclampsia, and seizure. We report a case of a 7-year-old girl who had taken cyclosporine for steroid resistant nephrotic syndrome. Twenty one days after the cyclosporine therapy, she was admitted due to generalized tonic clonic seizure and headache. Her blood pressure was 170/90 mmHg. Magnetic resonance(MR) imaging showed necrotic/cystic lesions involving the bilateral parieto-occipital region. After discontinuation of cyclosporine, and control of blood pressure, she had no more seizure and headache. The follow-up MR examination which was performed 6 months later showed the decreased extent of the lesion.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.84-91
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• 2009

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene and G the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.

• The Korean Journal of Nuclear Medicine
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• v.26 no.2
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• pp.318-326
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• 1992

Ga-67 citrate scan has been requested for detection or follow-up of inflammatory or neoplastic disease. Visualization of Ga-67 citrate in the kidneys at 48 and 72 hr post injection is usually interpreted as evidence of renal pathology. But precise mechanisms of abnormal Ga-67 uptake in kidneys were unknown. We undertook a study to determine the clinical value of Ga-67 citrate imaging of the kidneys in 68 patients with primary or secondary nephropathy confirmed by renal biopsy and 66 control patients without renal disease. Renal uptake in 48 to 72 hr images was graded as follows: Grade 0=back-ground activity:1=faint uptake greater than background;2=definite uptake, but less than lumbar vertebrae; 3=same uptake as lumbar vertebrae, but less than liver; 4=same or higher uptake than liver. The results were as follows. 1) 42 of 65 (62%) patients with noninfectious nephritis showed grade 2 or higher Ga-67 renal uptake but only 10 percent of control patients showed similar uptake. 2) In 14 patients with systemic lupus erythematosus, 8 of 9 (89%) patients with lupus nephritis exhibited marked renal uptake 3) 36 of 41 patients (88%) with combined nephrotic syndrome showed Grade 2 or higher renal uptake. 4) Renal Ga-67 uptake was correlated with clinical severity of nephrotic syndrome determined by serum albumin level, 24 hr urine protein excretion and serum lipid levels. 5) After complete remission of nephrotic syndrome, renal uptake in all 8 patients who were initially Grade 3 or 4, decreased to Grade 1 or 0. In conclusion, we think that the mechanism of renal Ga-67 uptake in nephrotic syndrome might be related to the pathogenesis of nephrotic syndrome. In systemic lupus erythematosus, Ga-67 citrate scan is useful in predicting renal involvement.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.250-255
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• 2004

Wilson's disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson's disease. However, nephrotoxicity can occur after D-penlcillamlne treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syn,frome in a 3-year-old'girl with Wilson's disease 3 weeks after initiation of D-penicillamine.

• Journal of Haehwa Medicine
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• v.14 no.2
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• pp.133-136
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• 2005

Objective : There are no Clinical reports on Nephrotic syndromes Which the patients improved by only oriental medical treatment. Method : We had administered Sin Youm-Bang for three months, and that time the patient didn't take the western medicine anymore by her own(steroid). And we observe edema, fatigue, headache, dizziness, lumbago and 24'h proteinuria, when we use only Sin Youm Bang. Result : We use Sin Youm-Bang during 3 month, edema, fatigue, headache, dizziness, lumbago was disappeared. And also 24'hr proteinuria was decreased(520mg/day to 28mg/day). Conclusion : Accordingly, We think, Sin Youm-Bang is used to Nephrotic syndromes and from now on we need more study at protecting proteinuria mechanism and immunological mechanism of Sin Youm Bang.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.244-249
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• 2004

Obesity-associated focal segmental glomeruloscleropis(OB-FSGS) has been known to progress into advanced renal insufficiency, and its clinicopathological features Include obesity, FSGS lesions with glornerulomegaly and, nephrotic-range proteinuria without edema. A 14 year old girl with Prader-Willi syndrome showed nephrotic-range proteinuria without hypoalbuminemia or edema. The renal biopsy revealed focal segmental glomerulosclerosis together with glomerular hypertrophy and an increased mesangial matrix. We report here a case of OB-FSGS as one of the renal problems of Pradel-Willi syndrome, and we came to the conclusion that Prader-Willi syndrome is one of the Possible disease entities that can lead to renal insufficiency through obesity.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.1-10
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• 1999

Purpose: Up to date there is no nationwide survey on epidemiological or clinical data of nephrotic syndrome, so we investigated about age of onset, sex, result of renal biopsy, treatment method, its results of treatment, its responsiveness, time of response to treatment and pattern of relapse in Korea. Methods: Between 1987 and 1997, 2193 patients with primary nephrotic syndrome diagnosed at 38 university hospital and general hospital in Korea were included. Of these 1655 were male and 538 were female. Incidence peaked at 1-5 years of age. Results: Results were as follows; 1) Among 2193 cases, male was 1655($75.5\%$), female was 538 cases($24.5\%$) and male to female ratio was about 3:1. Among 1752 patients with MCNS, male was 1338, female was 414 and male to female ratio was about 3.23:1. The most prevalent age group was 1-5 years of age. 2) Renal biopsy was done in 942 cases($43\%$), pathologic findings were as follows; MCNS 646 cases($68.6\%$), FSCS 149 cases($15.8\%$). 3) Regimen of treatment were as follows; prednisolone 1191 cases, Calcort 192 cases, cyclophosphamide 251 cases, cyclosporin A 223 cases, MPD pulse therapy 120 cases.4) Complete response to treatment were noted in 1597 cases($82.2\%$, n=1944). 5) Responsiveness according to result of renal biopsy were significantly different between MCNS and FSGS. Complete response were noted in $86.5\%$ among patients with MCNS, $35.8\%$ in patients with FSGS. 6) Time of response to treatment were noted between 1 and 4 weeks after treatment in 879 cases($67.4\%$, n=1305). 7) 994 cases($73.1\%$) relapsed during follow up, most frequently between 2 months and 6 months after response. Conclusion : Nationwide survey of epidemiological and clinical data were performed in childhood primary nephrotic syndrome. Most of the clinical and epidemiological data were similar to other reports from U.S.A. and from Europe, however male to female ratio is higher in Korean nephrotic syndrome(3:1 in contrast to 2:1).

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.9-15
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• 2007

Purpose : Edema is one of the cardinal features of nephrotic syndrome. Although the pathogenesis of edema is not entirely understood, it is caused by hypovolemia or hypervolemia by different mechanisms. Accordingly it is important to evaluate the volume status of patients in order to treat the edema, but it is difficult to evaluate the patient's volume status only by clinical parameters. The quotient of urine sodium and potassium excretion $U_K/(U_{Na}+U_K)$ is introduced as a more useful way to evaluate volume status. In this study we will propose the usefulness of $U_K/(U_{Na}+U_K)$ in evaluating the volume status of children with nephrotic syndrome. Methods : Primary nephrotic syndrome patients at Yeungnam University Hospital since January 1995 to June 2005, were included in the study. We analyzed clinical parameters such as tachycardia, cardiomegaly, pleural effusion, blood chemistry and urinalysis prospectively. We defined hypovolemia when $U_K/(U_{Na}+U_K)$ exceeded 60%. Intravenous albumin and diuretics were administered to hypovolemic edematous patients. On the other hand, hypervolemic edematous patients were treated only with diuretics. Results : There were 50 cases of primary nephrotic syndrome patients(hypervolemia: 29 vs hypovolemia: 21). There were no significant differences in clinical symptoms and laboratory findings except for FeNa While $F_eNa$ and $U_K/(U_{Na}+U_K)$ had a significant negative correlation, BUN and $U_K/(U_{Na}+U_K)$ had a significant positive correlation. Urine output after edema treatment was effective and there were no treatment-related side effects in both groups. Conclusion : FeNa, BUN and $U_K/(U_{Na}+U_K)$ are a useful parameters for evaluating volume status of edematous nephrotic syndrome patients. We could suggest a therapeutic option for using albumin and/or diuretics according to volemic status by means of measured $U_K/(U_{Na}+UK)$.