• 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 Medicine and Life Science
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• 제19권2호
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• pp.66-69
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• 2022

Patients with vesicoureteral reflux (VUR), the retrograde flow of urine from the bladder to the kidney, are known to experience renal scarring; this results in the worsening of renal function. Reflux nephropathy is a cause of chronic kidney disease, and VUR has also been observed in dialysis patients. VUR is a major underlying precursor condition of urinary tract infection (UTI) and is sometimes accompanied by hydronephrosis. However, there are no guidelines for the management of UTI due to VUR-associated hydronephrosis in patients with end-stage kidney disease. Herein, we report a case of UTI caused by VUR-associated hydronephrosis in a dialysis patient treated with percutaneous nephrostomy.

• 한국생물공학회:학술대회논문집
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• 한국생물공학회 2003년도 생물공학의 동향(XIII)
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• pp.347-350
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• 2003

본 연구에서는 신장세포를 이용하여 신장을 재생하는 조직공학적인 신장 재생 방법을 개발하기 위해 신생 rat으로부터 분리한 신장세포를 피브린 고분자와 혼합하여 신부전 rat의 신장에 주사 이식하였고 4주 후에 신장 구조의 형성 및 개선된 BUN, creatinine 수치를 확인하였다. 이는 이식된 세포에 의해 신부전의 증상이 완화(치료)된 것으로, 앞으로 이에 대한 추가적인 장기간의 실험이 필요하다.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.143-148
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• 2013

신경섬유종은 드문 전신 질환으로 여러 장기를 침범하며 특히 다양한 크기의 혈관을 침범하여 혈관병을 발생시켜 대동맥 협착, 모야모야병, 신동맥 협착 등을 일으킨다. 이로 인하여 약 0.4-6.4%의 환자에서 고혈압 증상이 있으며 이의 원인으로 신혈관 협착이 가장 흔하다. 특히 약물에도 조절되지 않는 고혈압은 혈관 협착 등이 원인이 될 수 있기 때문에 도플러 초음파 검사나 전산화 혈관 조영술 등의 정밀 검사가 필요하다. 이에 대한 치료는 약물 치료, 피부경유 혈관경유혈관성형술, 수술적 치료 등의 병합 요법으로 이루어지며 재협착 발생률이 높기 때문에 주의 깊은 추적 관찰이 필요하다. 본 저자들은 제 1형 신경섬유종증에 합병된 모야모야병에서 신장동맥 협착을 동반한 고혈압을 진단받고 치료한 1례를 경험하였기에 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제50권10호
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• pp.931-937
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• 2007

The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however, renal function recovers in most of them. About 10% of children with HUS do not reveal preceding diarrheal illness, and is referred to as D- HUS or atypical HUS. Atypical HUS comprises a heterogeneous group of thrombomicroangiopathy (TMA) triggered by non-enteric infection, virus, drug, malignancies, transplantation, and other underlying medical condition. Emerging data indicate dysregulation of alternative complement pathway in atypical HUS, and genetic analyses have identified mutations of several regulatory genes; i.e. the fluid phase complement regulator Factor H (CFH), the integral membrane regulator membrane cofactor protein (MCP; CD46) and the serine protease Factor I (IF). The uncontrolled activation of the complement alternative pathway results in the excessive consumption of C3. Plasma exchange or plasma infusion is recommended for treatment of, and has dropped the mortality rate. However, overall prognosis is poor, and many patients succumb to end-stage renal disease. Clinical presentations, response to plasma therapy, and outcome after renal transplantation are influenced by the genotype of the complement regulators. Thrombotic thrombocytopenic purpura (TTP), another type of TMA, occurs mainly in adults as an acquired disease accompanied by fever, neurologic deficits and renal abnormalities. However, less frequent cases of congenital or hereditary TTP associated with ADAMTS-13 (a disintegrin and metalloprotease, with thrombospondin 1-like domains 13) gene mutations have been reported, also. Recent advances in molecular genetics better allow various HUS to be distinguished on the basis of their pathogenesis. The genetic analysis of HUS is important in defining the underlying etiology, predicting the genotype-related outcome and optimizing the management of the patients.

• Journal of Korean Neurosurgical Society
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• 제43권5호
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• pp.237-238
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• 2008

Myoclonus is a rare side effect of gabapentin (GBP) and has been reported in patients with preexisting myoclonus, mental retardation, chronic static encephalopathy, diffuse brain damage, impaired renal function, or end stage renal disease. We report a case of myoclonus in a patient with normal renal function and no previous disorders. A 69-year-old female underwent diskectomy and foraminotomy at the left L4-L5 level. Post-operatively, she complained of paresthesia in her left leg, which was thought to be due to root manipulation during surgery. To relieve the paresthesia, she was given tramadol, an oral opioid agonist, and GBP. One week after GBP was increased to 900 mg per day, myoclonus developed, which severely impaired her normal activity. Her symptoms resolved 2 days after discontinuation of GBP. The coadministration of tramadol and GBP may mutually enhance the myoclonic potential of each drug. The causal relationship between GBP and myoclonus was suggested by cessation of myoclonus after GBP discontinuation despite continued therapy with tramadol.

• 생명과학회지
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• 제27권3호
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• pp.267-274
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• 2017

본 연구는 말기 신부전 환자의 체내에서 증가되는 시안산이 신경학적 합병증의 원인으로 작용하는지 알아보고자 시안산 처리에 따른 신경아교종 세포인 C6 세포의 변화를 관찰하였다. 또한, 시안산에 의해 발현되는 세포자멸사 관련 인자를 알아보기 위하여 western blot 및 유전자 발현의 변화를 검색하기 위하여 cDNA 유전자 미세배열분석을 하였다. 시안산의 처리 농도가 0, 1, 5, 10, 20, 40 mM 증가할수록 신경아교종 세포의 생존율이 유의하게 감소하였고 세포자멸사에 주된 역할을 하는 caspase-8는 증가되었고 procaspase-3는 감소하였다. 그러나 caspase-8에 의해 활성화되는 Bax 단백질은 시안산의 처리 농도가 증가할수록 caspase-8의 증가에도 감소하였고, 세포자멸사를 조절하는 단백질인 Bcl-2와 IAP은 명확히 확인할 수 없었다. cDNA 유전자 미세배열 분석 결과, 총 1,099 종의 유전자 중에서 934 개의 유전자가 감소하였고 증가된 것은 165 개였다. 세포자멸사 관련 유전자에서도 감소한 것은 16 개였고, 증가된 6 개 유전자 가운데 heat shock 70 kD protein 1A가 현저한 증가를 나타내었다. 이상의 결과로 보아, 시안산은 신경아교종 세포에서 caspase-8 및 caspase-3와 관련된 세포자멸사를 유발시키며, 신경아교종 세포의 유전자들의 발현을 감소시키는 것으로 생각된다. 따라서 체내에서 증가된 시안산이 신경아교종 세포에 영향을 미쳐 말기 신부전 환자의 뇌병증에도 영향을 주는 것이라 생각된다.

• 대한불안의학회지
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• 제2권2호
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• pp.128-135
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• 2006

Objective : In this study, we investigated the prevalence of depression and its impact on the healthrelated Quality of life (HRQoL) of the patients with End-Stage Renal Disease (ESRD) on Hemodialysis. Method : The Quality of life (QOL) of patients was evaluated by HRQoL Questionnaire, "Medical Outcome Survey 36-Item Short Form Health Survey Korean Version (SF-36-K)". The patients on Hemodialysis in ESRD, were chosen from the hemodialytic room at Wonkwang University and Jeongeup-Asan Hospital. The number of patients was 95 (64 from Wonkwang University Hospital and 31 from Jeongeup-Asan Hospital) and all of them were above 19 years old. We performed various investigations to find a statistical correlations between HRQoL and physical & psychosocial factors such as the demographic characteristics, clinical characteristics (hemoglobin level and albumin level etc), and the score of Beck's depressive inventory (BDI). Results : The HRQoL value of patients on hemodialysis in ESRD is far poorer than the HRQoL reference value of Koreans and Americans, who are in normal healthly. The prevalence of depressive symptoms by BDI of the ESRD patients on hemodialysis is 68.6%, and age and depression have negative correlations with HRQoL of the patients. However, education level, serum albumin level, and social support have positive correlations with HRQoL. The patient group with depression has significantly poorer HRQoL than the group without depression. Conclusion : The HRQoL of ESRD patients on hemodialysis is not good in both physical and mental aspects. The prevalence of depression is very high and depression has negative impact of HRQoL of patients. Based on our study, it is essential to accompany with therapeutic Strategy to improve the HRQoL of ESRD patients on hemodialysis.

• 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.