• 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.

• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1033-1037
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• 2005

Pediatric nephrotic syndrome is a well-known illness for its high relapsing rate. If we can predict the relapsing rate and the responses to the steroid therapy of individual patients with nephrotic syndrome, the predictability will be helpful in building a therapeutic plan. Here is my review of research articles on the risk factors for the prediction of relapsing 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.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.13 no.2
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• pp.161-169
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• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Journal of Genetic Medicine
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• v.17 no.2
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• pp.97-101
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• 2020

The combination of central nervous system abnormalities and renal impairment is a notable characteristic of Galloway-Mowat syndrome (GAMOS), a disease which often accompanies microcephaly, developmental delay, and nephrotic syndrome. Many subtypes exist having various phenotypes and genotypes, and many genetic causes are still being identified. An 18-month-old boy first visited our clinic for seizure, delayed development, and microcephaly. During follow-up visits he developed proteinuria and nephrotic syndrome at the age of 6. Nephrotic syndrome became refractory to treatment. These phenotypes were suggestive of GAMOS. Next generation sequencing was performed for genetic analysis and revealed novel compound heterozygous variants in the WDR4 gene: c.494G>A (p.Arg165Gln) and c.540C>G (p.Ile180Met). This is the first case in Korea of GAMOS involving the WDR4 gene.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.218-222
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• 2010

Nephrotic syndrome is a clinical syndrome characterized by heavy proteinuria, hypoalbuminemia, edema and hyperlipidemia. Causes of idiopathic nephrotic syndrome include minimal change nephrotic syndrome (MCNS), focal segmental glomerulosclerosis (FSGS) and mesangial proliferation. Other causes of nephrotic syndrome are rare genetic disorders and secondary diseases associated with drugs, infections, or neoplasia. Since February 2009, a swine-origin H1N1 influenza virus (S-OIV) from Mexico has been spread among humans in unexpected rapidity. S-OIV is markedly different from seasonal influenza, in that many of those affected are previously healthy young people. While pulmonary complications of S-OIV infection have been frequently documented, renal complications have not been as widely recognized. We report a case of 4 year-old boy who had developed nephrotic syndrome after S-OIV infection with good response after steroid treatment.

• 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.

• Clinical and Experimental Pediatrics
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• v.62 no.3
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• pp.85-89
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• 2019

Purpose: We compared thyroid hormone profiles in children with nephrotic syndrome (NS) during the nephrotic phase and after remission. Methods: This study included 31 pediatric NS patients. The thyroid hormone profiles included serum levels of triiodothyronine (T3), thyroxine (T4), thyroid-stimulating hormone (TSH), and free T4. Results: Of the 31 patients, 16 (51.6%) showed abnormal thyroid hormone profiles: 6 had overt hypothyroidism, 8 had subclinical hypothyroidism, and 2 had low T3 syndrome. The mean serum T3, T4, and free T4 levels in the nephrotic phase and after remission were $82.37{\pm}23.64$ and $117.88{\pm}29.49ng/dL$, $5.47{\pm}1.14$ and $7.91{\pm}1.56{\mu}g/dL$, and $1.02{\pm}0.26$ and $1.38{\pm}0.23ng/dL$, respectively; the levels were significantly lower in the NS nephrotic phase (P=0.0007, P<0.0001, and P=0.0002). The mean serum TSH levels during the nephrotic phase and after remission were $8.05{\pm}3.53$ and $4.08{\pm}2.05{\mu}IU/mL$, respectively; they were significantly higher in the nephrotic phase (P=0.0005). The urinary protein/creatinine ratio during the nephrotic phase was significantly correlated with serum T3, T4, and free T4 levels (r=-0.5995, P=0.0032; r=-0.5797, P=0.0047; r=-0.5513, P=0.0078) as well as with TSH levels (r=0.5022, P=0.0172). A significant correlation was found between serum albumin and serum T3 levels during the nephrotic phase (r=0.5385, P=0.0018) but not between serum albumin and T4, TSH, or free T4 levels. These significant correlations all disappeared after remission. Conclusion: Abnormal thyroid hormone profile findings were observed in 51.6% of pediatric patients with NS. Thyroid hormone levels normalized after remission, regardless of levothyroxine therapy.

• Child Health Nursing Research
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• v.3 no.2
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• pp.169-177
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• 1997

This is descripitive study conducted to identify educational needs of mothers of nephrotic syndrome patients and the degree of the importance of educational activity perceived by nurses who look after nephrotic syndrome patients. The study subjects were composed of 60 mothers & 63 nurses of nephrotic syndrome patients whose children were hospitalized in 2 Pediatric wards of University Hospital in Seoul and 1 in Pusan from Mar. in 1997 to July 1997. A questionaire for this study was item Kikert type 5 point scale, developed on the basis of previous literature and researcher's clinical experience and the reliability of the used instruments was α=.97. The data analysis was done by SAS. t-test, and ANOVA were done to determine the effect of general characteristics of subjects on their educational needs. T-test was done to measure relations between the degree of educational needs and importance of educational activity. The results were as follows. 1. The Educational needs of mothers ranked as the highest in home care. 2. In character of mothers, the degree of education, economic state, the number of child and occupation was significant in statistically. 3. The importance of educational activity of nurses ranked the highest in diagnosis and treatment of the disease. 4. The educational needs of nephrotic syn. children' mother and importance of nurses were different in diagnosis and treatment of the disease and home care. On the basis of these results, we suggest as follows. 1. It is proposed that nurses use these results of the study actively for the educational program for Nephrotic Syndrome patients and their mothers. 2. It is suggested to make a comparative study of the degree of nurses' understanding of the importance on educational items with the instruments of this study.