• 대한간호학회지
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• 제27권2호
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• pp.303-314
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• 1997

This is descripitive study conducted to identify educational needs of mothers of nephrotic syndrome patients and tie degree of nerses' educational performances perceived by mothers who look after mainly nephrotic syndrome patients. The study subjects were composed of 74 mothers of nephrotic syndrome patients whose children were hospitalized in 2 Pediatric wards of University Hospital in Seoul and 1 in Pen from June in 1996 to Januaryin 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. Pearson correlation was done to measure relations between general characterictics of subjects and their educational needs and Stepwise Multiple Regression was done to test a variable affecting educational needs. The results were as follows. 1. Mean score of educational needs of subjects was 137.06(Maximum 176). The score of the educational needs of home care was the highes, but the question numbers(of that category) are smaller than others. So, the educational need of the diagnosis art treatment was regarded as the highest in contents. 2. The mean score of nurses' educational performances was very low, 74.91(Maximum 176). Nurse's educational performances score in the diagnosis and treatment of the disease ranked as the highest Burt that score in the care during hoapitalization was the highest in contents as the educational needs was. 3. The number of children excepting the patient (r²=.215289. p=.006) and the age of patient(r²=.23770, p=.001) were emerged as important variables affecting the degree of mother's educational need.

• Childhood Kidney Diseases
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• 제18권1호
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• pp.47-50
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• 2014

신증후군 환아에서 감염은 매우 중요한 사망원인이 된다. 독감 바이러스는 매번 겨울철마다 유행하며, 독감 바이러스의 치명률은 건강한 소아에서 호흡기세포융합바이러스의 사망률과 비슷하므로 독감에 의한 감염도 신증후군 환아들에게는 매우 치명적일 수 있다. 독감에 의한 사망률에는 폐렴으로 인한 사망이 많은 부분을 차지한다. 하지만, 독감은 예방접종과 항바이러스 치료제가 존재하므로 치료 및 예방이 가능하다. 그러므로, 적극적인 독감 예방접종과 항바이러스 치료는 신증후군 환자들에게서 치명률을 낮출 수 있을 것으로 생각된다. 저자들은 신증후군 치료중에 A형 독감(H1N1)에 의한 폐렴에 걸린 7세 남아를 경험하였기에 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제5권2호
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• pp.206-211
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• 2002

윌슨병의 치료제로 구리 흡착제인 D-penicillamine이 주로 사용되고 있으나, 심각한 부작용이 발생할 경우 투약을 중단하고 trientine 등을 대체약으로 사용할 수 있다. 저자들은 윌슨병으로 D-penicillamine 사용 도중 6개월만에 미세변화형 신증후군이 발생한 후 trientine으로 치료약을 대체 후 관해가 유도된 1례를 경험하였기에 보고하는 바이다.

• Childhood Kidney Diseases
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• 제3권2호
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• pp.217-220
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• 1999

Kimura disease is a chronic benign disorder, primarily seen in asians male during the second and third decades of life, which presents itself as a tumour like lesion with a predilection for the head and neck region. There is high prevalence of associated renal disease. We report two cases of nephrotic syndrome associated with Kimura disease, and this is the first report of Kimura disease with renal involvement in Korean children.

• 대한한방내과학회지
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• 제22권1호
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• pp.103-108
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• 2001

Nephrotic syndrome(NS) is characterized albuminuria, hypoalbuminemia, hyperlipidemia and edema. The thromboembolic complications of NS are reasonably common and have a broad range of clinical manifestations. The treatment of NS is empirical and symptomatic because the etiology and pathogenesis are unknown. We report treatment of patient who has been afflicted with NS for 20 years. He complained edema, dyspnea, Rt. hemiparesis, aphasia. We treated him with converting enzyme inhibitor, steroid, three kinds of herb-medicine for 45 days. The patient improved in clinical symptoms and routine chemistry, urine analysis test.

• Clinical and Experimental Pediatrics
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• 제56권10호
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• pp.456-458
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• 2013

Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy has increased, and some previously unobserved disease associations are now seen in patients with CF. It is important to follow patients with CF for possible abnormalities that may accompany CF. In this paper, we present two rare cases of CF accompanied by nephrotic syndrome.

• Clinical and Experimental Pediatrics
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• 제58권8호
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• pp.275-282
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• 2015

While the incidence of nephrotic syndrome (NS) is decreasing in Korea, the morbidity of difficult-to-treat NS is significant. Efforts to minimize treatment toxicity showed that prolonged treatment after an initial treatment for 2-3 months with glucocorticosteroids was not effective in reducing frequent relapses. For steroid-dependent NS, rituximab, a monoclonal antibody against the CD20 antigen on B cells, was proven to be as effective, and short-term daily low-dose steroids during upper respiratory infections reduced relapses. Steroid resistance or congenital NS are indications for genetic study and renal biopsy, since the list of genes involved in NS is lengthening.

• Clinical and Experimental Pediatrics
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• 제54권8호
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• pp.317-321
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• 2011

Children who suffer from steroid-resistant nephrotic syndrome (SRNS) require aggressive treatment to achieve remission. When intravenous high-dose methylprednisolone fails, calcineurin inhibitors, such as cyclosporine and tacrolimus, are used as the first line of treatment. A significant number of patients with SRNS progress to end-stage renal disease if remission is not achieved. For these children, renal replacement therapy can also be problematic; peritoneal dialysis may be accompanied by significant protein loss through the peritoneal membrane, and kidney allograft transplantation may be complicated by recurrence of SRNS. Plasmapheresis and rituximab were initially used for treatment of recurrent SRNS after transplantation; these are now under consideration as rescue therapies for refractory SRNS. Although the prognosis of SRNS is complicated and unfavorable, intensive treatment in the early stages of the disease may achieve remission in more than half of the patients. Therefore, timely referral of pediatric SRNS patients to pediatric nephrology specialists for histological and genetic diagnosis and treatment is highly recommended.

• 동아시아식생활학회지
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• 제9권4호
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• pp.427-434
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• 1999

This study was conducted to investigate the effects of vitamin I supplementation on renal function in high fat diet and adriamycin (ADR) induced experimental nephrotic syndrome in model rats. The effects of vitamin E supplementation on renal function in high fat diet and ADR treated rats were as follows. Kidney weight was decreased by vitamin E supplementation. Serum total protein was increased by the excess supplementation. Blood urea nitrogen(BUN) was decreased by the high supplementation. However, serum albumin and creatinine showed no significant differences between groups. Urinary volume tended to increase by vitamin I supplementation. Urinary urea-N tended by vitamin I supplementation. Particularly glomerular filtration rate(GFR) was significantly decreased by vitamin E supplementation. These results suggested that vitamin E supplementation could alleviate the adverse effects caused in renal function by highfatdiet and ADR treatments.

• 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.