• Clinical and Experimental Pediatrics
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• 제59권4호
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• pp.202-204
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• 2016

A 15-year-old boy, who was diagnosed with Alport syndrome and end-stage renal disease, received a renal transplant from a living-related donor. On postoperative day 1, his daily urine output was 10,000 mL despite normal graft function. His laboratory findings including urine, serum osmolality, and antidiuretic hormone levels showed signs similar to central diabetes insipidus, so he was administered desmopressin acetate nasal spray. After administering the desmopressin, urine specific gravity and osmolality increased abruptly, and daily urine output declined to the normal range. The desmopressin acetate was tapered gradually and discontinued 3 months later. Graft function was good, and urine output was maintained within the normal range without desmopressin 20 months after the transplantation. We present a case of a massive polyuria due to transient deficiency of antidiuretic hormone with the necessity of desmopressin therapy immediately after kidney transplantation in a pediatric patient.

• Healthcare Informatics Research
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• 제24권4호
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• pp.292-299
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• 2018

Objectives: The aim of the present study was to assess the frequency of use, and preferences regarding information and communication technologies (ICTs) among Ecuadorian patients with chronic kidney disease (CKD) undergoing hemodialysis. Methods: We conducted an anonymous cross-sectional survey-based study from January 2016 to April 2017, involving 393 patients with end-stage renal disease from 9 hemodialysis centers, in which they rated their use and preferences of various ICTs through a modified version of the Michigan Questionnaire. The questionnaire collected information regarding demographics, patients' interest in obtaining health-related information through ICTs, and interest in using ICTs as a potential way to communicate with their healthcare providers. A chi-square test for association and adjusted regression analyses were performed. Results: Among all patients who participated, 64.3% reported owning a cellphone, with less than a third reporting active Internet connection. The most used ICT for obtaining information about CKD and/or hemodialysis was web-based Internet, followed by YouTube. SMS was rated the highest to receive and seek health-related information, followed by Facebook. Younger age and higher levels of education were associated with a higher overall usage of ICTs. Finally, more than half of the patients reported interest in using WhatsApp for communicating with their healthcare providers. Conclusions: Understanding the preferences of ICTs among patients with CKD undergoing hemodialysis could help to improve their outcomes through the potential uses and benefits of ICTs. Further research is needed to assess their role in improving the care of patients with chronic diseases.

• Advances in Traditional Medicine
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• 제5권2호
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• pp.75-91
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• 2005

Great efforts have been made to improve both the quality of life and life expectancy of diabetes by treating problems associated with chronic complications such as neuropathy, retinopathy and nephropathy. In particular, diabetes is an increased risk of developing several types of kidney disease, and the predominant cause of end-stage renal disease in patients with this disorder is diabetic nephropathy. Therefore, prevention of the occurrence and progression of diabetes and its complications has become a very important issue. The scientific observations of an animal model of streptozotocin-induced diabetes, spontaneously occurring diabetes and diabetic nephropathy in this study suggest that one of the Kampo prescriptions, Hachimi-jio-gan comprising eight constituents, is a novel therapeutic agent.

• 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.

• Childhood Kidney Diseases
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• 제8권1호
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• pp.51-56
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• 2004

고혈압성 뇌병증은 혈압상승과 함께 두통, 오심, 구토, 시력장애, 경련, 의식 변화 등을 특징으로 하는 급성 신경 증후군으로 소아에서는 대부분 급성 신장염이나 신혈관성 고혈압에 의해 발생하고 지금까지 요로 결석과 동반된 고혈압상 뇌병증의 보고는 없었다. 저자들은 요관 결석과 동반되어 발생한 고혈압성 뇌병증 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Microbiology and Biotechnology
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• 제17권12호
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• pp.2005-2017
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• 2007

Diabetic nephropathy remains a major cause of morbidity and mortality in the diabetic population and is the leading cause of end-stage renal failure. Despite current therapeutics including intensified glycemic control and blood pressure lowering agents, renal disease continues to progress relentlessly in diabetic patients, albeit at a lower rate. Since synthetic drugs for diabetes are known to have side effects, fungal mushrooms as a natural product come into preventing the development of diabetes. Our previous report showed the hypoglycemic effect of extracellular fungal polysaccharides (EPS) in streptozotocin (STZ)-induced diabetic rats. In this study, we analyzed the differential expression patterns of rat kidney proteins from normal, STZ-induced diabetic, and EPS-treated diabetic rats, to discover diabetes-associated proteins in rat kidney. The results of proteomic analysis revealed that up to 500 protein spots were visualized, of which 291 spots were differentially expressed in the three experimental groups. Eventually, 51 spots were statistically significant and were identified by peptide mass fingerprinting. Among the differentially expressed renal proteins, 10 were increased and 16 were decreased significantly in diabetic rat kidney. The levels of different proteins, altered after diabetes induction, were returned to approximately those of the healthy rats by EPS treatment. A histopathological examination showed that EPS administration restored the impaired kidney to almost normal architecture. The study of protein expression in the normal and diabetic kidney tissues enabled us to find several diabetic nephropathy-specific proteins, such as phospholipids scramblase 3 and tropomyosin 3, which have not been mentioned yet in connection with diabetes.

• Journal of Korean Medical Science
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• 제33권53호
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• pp.341.1-341.11
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• 2018

Background: The converging epidemics of tuberculosis (TB) and end-stage renal disease (ESRD) have generated a significant public health burden, however, previous studies have been limited to a small number of patients. This nationwide cohort study aimed to assess the rate of developing active TB among patients receiving dialysis for ESRD. Methods: The Korean national health insurance database was used to identify patients receiving dialysis for new-onset ESRD during 2004-2013, who were propensity score matched to an equivalent number of non-dialysis subjects from the general population. The incidences of active TB in the ESRD and control cohorts were calculated for 2004-2013, and multivariable Cox proportional hazards model was used to evaluate the ESRD-related risk of active TB. Results: During 2004-2013, 59,584 patients received dialysis for newly diagnosed ESRD. In the dialysis and control cohorts, 457 (0.8%) and 125 (0.2%) cases of active TB were detected, respectively. Patients with ESRD were associated with a significantly higher risk of active TB compared to the controls (incidence rate ratio, 4.80). The ESRD cohort had an independently elevated risk of active TB (adjusted hazard ratio, 4.39; 95% confidence interval, 3.60-5.37). Conclusion: We found that patients receiving dialysis for ESRD had an elevated risk of active TB. These results highlight the need for detailed and well-organised guidelines for active TB screening among patients with ESRD.

• Childhood Kidney Diseases
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• 제11권2호
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• pp.178-184
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• 2007

목 적 : 1990년 Mendoza등이 스테로이드 저항성 FSGS에서 Mendoza protocol에 의한 장기간의 MP 충격 요법이 효과적임을 보고한 이후 이 치료법의 효과에 대한 보고가 있어 왔다. 본 연구에서는 cyclosporin이나 cyclophosphamide를 먼저 사용하지 않은 스테로이드 저항성 FSGS에서 Mendoza protocol 에 의한 장기간의 MP 충격 요법의 치료 효과를 알아보고자 하였다. 방 법 : 1986년부터 2006년 사이에 세브란스병원 소아과에서 스테로이드 저항성 FSGS를 진단 받고 MP 충격 요법을 시행했던 10명의 환아를 후향적으로 분석하였다. 결 과 : 평균 발병 연령은 2.6세였으며 치료 시작시의 평균 연령은 5.7세였다. 치료 종료 후 평균 추적 관찰 기간은 35개월이었다 치료 종료 후 5명에서 완전 관해가 유도되었으며(50%) 2명에서 부분 관해가 유도되었으나(20%) 이 중 1명은 후에 재발하였다. 3명은 치료에 반응하지 않았으며(30%) 이 중 2명은 치료 중 말기 신부전으로 진행하여 신이식을 시행하였다. 결 론 : Mendoza protocol에 의한 장기간의 MP 충격 요법은 현재까지의 연구 결과에서는 cyclosporin A, cyclophosphamide를 먼저 투여하지 않고 최초 치료로 사용할 경우 그 치료 효과가 좋지 않았으나 본 연구에서는 50% 정도로 효과를 보이고 있으므로 스테로이드 저항성 FSGS 환아의 초기 치료에 사용할 수 있을 것으로 생각된다. 또한 cyclosporin A 또는 cyclophosphamide를 먼저 사용하고 효과가 없는 경우 MP 충격 요법을 시작하는 경우에도 치료 효과는 좋을 것으로 생각 할 수 있다.

• Childhood Kidney Diseases
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• 제6권1호
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• pp.120-122
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• 2002

IgA신병증으로 말기 신부전에 도달한 B형 혈액형의 9세 남아가 O형 혈액형의 아버지로부터 신이식을 시행받았다. 환아는 16개월 간 복막 투석 중이었으며 이전에 수혈받은 병력은 없었다. 이식 9일째 혈관내 용혈이 발견되었으며 항 B 항체 형성이 확인되었다. 저자들은 공여자의 신장과 함께 이동한 림프구가 일으킨 자가 면역 용혈성 빈혈로 진단하고 사이클로스포린 투여를 중단하였다. 용혈성 빈혈은 회복되었으나 이식 18일째 이식 신 기능 저하가 발견되어 이식 신생검을 시행하여 급성 거부를 확인하고 스테로이드 충격 요법을 시행하였다. 저자들은 ABO 부적합 신이식 후 발생한 용혈성 빈혈 1례를 겅험하여 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제52권11호
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• pp.1260-1266
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• 2009

목 적 : 특발성 신증후군은 소아의 가장 흔한 일차성 사구체 질환 중의 하나이다. 신증후군은 초기 경구 스테로이드 치료에 대한 반응에 따라서 임상적으로 스테로이드 반응성 신증후군과 스테로이드 저항성 신증후군으로 분류될 수 있다. 그러나 현재까지 신증후군에서 스테로이드의 정확한 작용 기전은 알려져 있지 않다. 신증후군 환자를 대상으로 여러 가지 유전자 다형성을 분석함으로써 스테로이드 치료에 대한 반응의 차이를 설명하려는 여러 시도들이 있어왔다. 방 법 : 본 연구에서는 190명의 신증후군 환자를 대상으로 NR3C1 유전자 다형성(ER22/23EK, N363S, BclI)을 확인하여 유전형과 임상-병리 양상의 연관성에 대해서 분석하였다. 결 과 : 신증후군 환자의 평균 연령은 4.95세였고 남아가 134명이었다. 11명의 환자는 신증후군의 가족력이 있었다. 그러나 이 환자들을 대상으로 NPHS2, WT1, ACTN4, TRPC6 유전자 분석을 시행한 결과 이상 소견은 발견되지 않았다. 80명의 환자(42.1%)는 초기 스테로이드 저항성이었고 그 중 31명의 환자는 말기 신질환으로 진행하였다. 신장 조직 검사는 113명의 환자를 대상으로 시행되었고 그 중 36명의 환자(31.9%)는 미세변화 신증후군이었고 77명의 환자(68.1%)는 초점성 분절성 사구체 경화증이었다. BclI 유전형을 비교하였을 때 G allele 빈도는 환자군과 대조군에서 차이가 없었다. ER22/23EK과 N363S 유전형은 각각 ER/ER과 NN으로 환자군과 대조군에서 동일한 양상을 보였다. BclI 유전형은 신증후군의 발병 나이, 초기 스테로이드 반응 여부, 신장의 병리학적 소견, 말기 신질환으로의 진행여부와 연관성을 보이지 않았다. 결 론 : 한국 신증후군 환아를 대상으로 한 이 연구 결과는 NR3C1 유전자의 ER22/23EK, N363S 및 BclI 유전자 다형성이 신증후군의 발병, 초기 스테로이드 치료에 대한 반응, 신장의 조직학적 소견 및 신 기능의 저하에 영향을 미치지 않음을 보여준다.