• 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.

• Clinical and Experimental Pediatrics
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• 제48권10호
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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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• 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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• 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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• 제13권2호
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• pp.161-169
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• 2009

저자들은 1세 이하에 발병한 신증후군 환아 7명의 진단 및 치료를 경험하였기에 보고하는 바이다. 1996년부터 2007년까지 본원에서 1세 이전에 신증후군으로 진단 받은 환아 7명을 대상으로 병록 고찰을 통해 후향적으로 조사하였다. 선천성 신증후군은 3명, 영아형 신증후군은 4명 이었고, 남아가 1명, 여아가 6명으로 여아가 많았다. 6명에서 신생검을 시행하였고, 2명에서 미만성 메산지움 경화증, 2명에서 국소분절사구체경화증, 1명에서 Finnish형 신증후군 그리고 나머지 1명에서는 미세 변화형으로 확인되었다. 4명의 환아에서 NPHS2, PLCE1, 그리고 WT1의 유전자 분석을 시행하였고 2예에서 WT1 변이가 확인되었다. 선천성 신증후군으로 진단받은 3명 중 1명은 Finnish형 신증후군, 2명은 DDS로 진단받았고, 3명 모두 패혈증으로 사망하였다. 영아형 신증후군으로 진단받은 4명 중 2명은 사망하였고, 다른 1명은 관해가 되어 치료되었고, 나머지 1명은 치료 4개월 경 말기 신부전으로 진행하여 복막투석을 하고 있다. 1세 이전에 발병하는 선천성 신증후군은 소아 신증후군에 비하여 예후가 나쁘지만, 병력의 세밀한 검토, 임상소견 및 조직검사, 유전자 분석을 통한 정확한 진단이 빠르게 이루어져 조기 투석 및 신이식 등 적극적인 치료를 한다면 생존률의 향상을 기대할 수 있을 것이다. 대부분의 1세 이전 진단받는 신증후군은 유전적 신질환으로 추후 이들 환아들에 대한 유전형-표현형의 상관관계에 대한 추가 연구가 필요하다.

• Journal of Genetic Medicine
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• 제17권2호
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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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• 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.

• 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.

• Clinical and Experimental Pediatrics
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• 제62권3호
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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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• 제3권2호
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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.