• 대한유전성대사질환학회지
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• 제20권1호
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• pp.29-35
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• 2020

쥬버트 증후군(JS, Joubert syndrome)은 대부분 상염색체 열성으로 유전되는 유전성 대사질환으로 임상증상은 신생아 시기부터 발현된다. 저자들은 신생아기부터 특징적인 임상 증상이 순차적으로 발현되어 임상적으로 JS를 의심하였으나 특징적인 뇌 MRI 소견인 molar tooth sign (MTS)이 늦게 나타난 후 전장엑솜분석(WES, whole exome sequencing)으로 확진 된, 말기 신부전을 동반한 JS 1례를 경험하였기에 보고하고자 한다. 14세 남자 환자는 출생 직후 반복적인 무호흡과 과호흡으로 치료받은 병력이 있으며, 생후 8개월때부터 전반적 발달 지연과 관련되어 처음 병원을 방문하여 기본적인 발달 지연에 관한 검사를 시행하였으나 특이 소견 없었고, 이후 15개월 때 근육생검을 포함한 여러 검사를 통해 사립체(mitochondrial) 질환으로 진단 되었었다. 이후 물리 치료만 하며 관찰 하던 중 안구진탕과 망막질환이 확인되었다. 생후 7세 8개월에는 처음 발작이 있었으며, 말기 신부전이 있어 8세부터 혈액투석을 시작한 후, 혈액 투석 직후 수차례 발작이 있었으나 전해질 불균형으로 인한 발작으로 진단하여 항뇌전증 약물 치료는 하지 않았다. 9세 4개월 때 고혈압으로 인한 뇌출혈로 치료 받았으며, 이때 시행한 뇌 CT상 MTS가 처음 의심되었다. 13세 10개월에 시행한 뇌 MRI 검사상 MTS가 명확히 확인되었고, 전장엑솜 분석으로 JS의 CEP290 mutation (c.6012-12T>A)이 확인되었다. 환자는 신생아기부터 발현된 특징적인 임상 소견과 말기 신부전 상태, 뇌 CT 또는 MRI소견, 그리고 전장엑솜분석 검사로 JS로 확진하였다. JS는 임상 양상이 다양할 뿐만 아니라 진단에 중요한 MTS 소견이 초기에 보이지 않더라도, 임상적으로 의심된다면 확진을 위해서 전장엑솜분석을 시행하는 것이 필요하다.

• 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.

• 대한임상독성학회지
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• 제16권2호
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• pp.157-160
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• 2018

Chronic silica nephropathy has been associated with tubulointerstitial disease, immune-mediated multisystem disease, chronic kidney disease, and end-stage renal disease. On the other hand, acute intentional exposure is extremely rare. The authors' experienced a 44-year-old man who took rapid cement hardener (sodium silicate) in a suicide attempt whilst in a drunken state. He visited the emergency department approximately 1 hour after ingestion. Information on the material was obtained after 3 L gastric lavage. The patient complained of a sore throat, epigastric pain, and swollen to blood tinged vomitus. Proton pump inhibitors, hemostats, steroid, and fluids were administered. Nine hours after ingestion, he was administered 200 mL hematochezia. Immediately after, a gas-troenterologist performed an endoscopic procedure that revealed diffuse hyperemic mucosa with a color change and variable sized ulceration in the esophagus, whole stomach, and duodenal $2^{nd}$ portion. Approximately 35 hours later, persistent oligouria and progressive worsening of the renal function parameters (BUN/Cr from 12.2/1.2 to 67.5/6.6 mg/dL) occurred requiring hemodialysis. The patient underwent 8 sessions of hemodialysis for 1 month and the BUN/Cr level increased to 143.2/11.2 mg/dL and decreased to 7.6/1.5 mg/dL. He was discharged safely from the hospital. Follow up endoscopy revealed a severe esophageal stricture and he underwent endoscopic bougie dilatation. Acute cement hardener (sodium silicate) intoxication can cause renal failure and strong caustic mucosal injury. Therefore, it is important to consider early hemodialysis and treatment to prevent gastrointestinal injury and remote esophageal stricture.

• 한국전산유체공학회지
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• 제16권4호
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• pp.84-91
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• 2011

Hemodialysis is essential for patients with end stage renal failure. It is important to improve the patency rate and to minimize occurrence of the stenosis. Also, the blood flow to the artificial kidney can affect the blood flow characteristics through arteriovenous graft. Thus, the delivered dose are important factors for analyzing hemodynamic characteristics during hemodialysis access. In this study, the numerical analysis was performed for the effect of the delivered dose during hemodialysis access on the blood flow through the graft. As a result, The adverse pressure gradient occurred in case of a larger delivered dose through a catheter than standard dose and the flow instability increased. Also the circulation flow appeared largely at anastomotic site of the vein when the delivered dose was exceeded about half blood flow of inlet blood flow.

• 한국전산유체공학회:학술대회논문집
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• 한국전산유체공학회 2011년 춘계학술대회논문집
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• pp.469-476
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• 2011

Hemodialysis is essential for patients with end stage renal failure. It is important to improve the patency rate and to minimize occurrence of the stenosis. Also, the blood flow to the artificial kidney can affect the blood flow characteristics though arteriovenous graft. Thus, the delivered dose are important factors for analyzing hemodynamic characteristics during hemodialysis access. In this study, the numerical analysis was performed for the effect of the delivered dose during hemodialysis access on the blood flow through the graft. As a result, The adverse pressure gradient occurred in case of a larger delivered dose through a catheter than standard dose and the flow instability increased. Also the circulation flow appeared largely at anastomotic site of the vein when the delivered dose was exceeded about half blood flow of inlet blood flow.

• Childhood Kidney Diseases
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• 제1권1호
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• pp.17-23
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• 1997

목적 : IgA 신병증은 다양한 임상 소견을 보일 수 있으며 초기에는 대부분 예후가 양호한 것으로 알려졌었으나 현재는 발병 20년후 20-30%가 말기 신부전증을 초래한다고 알려져 있다. IgA 신병증의 예후를 예측하기 위한 여러 조사에서 발병 초기의 심한 단백뇨, 고혈압, 조직 병리학 상의 심한 변화 등이 있을 경우 예후가 나쁘다고 하였다. 1996년 Yagame등은 IgA 신병증에서 새로운 조직 병리학적 분류를 하였고 WHO 분류 방법이 사구체 병변을 중심으로 한데 비해 세뇨관과 간질의 변화까지 포함하고 있어 이들의 분류 방법이 더 우수하며 추적관찰 결과 만성 신부전의 예후 인자로써 유용하다고 발표하였다. IgA 신병증에서 임상 증세, 검사 소견, WHO 및 Yagame 등의 조직 병리학적 분류 등과 추적 관찰시 만성 신부전과의 상관 관계를 알아보고자 하였다. 방법 : 1984년 1월부터 1996년 2월까지 소아과에 입원하여 신생검상 메산지움에 IgA가 현저히 침착되어 있어 IgA 신병증으로 진단된 환아 79명을 대상으로 평균 27개월의 추적관찰시 신 기능이 정상인군(73례)과 만성 신부전으로 진행한 군(6례)을 서로 비교하여 IgA 신병증에서의 예후 예측 인자에 대하여 알아보고자 하였다. 결과 : 1) 성별, 나이, 증상기간 등은 양군간 차이가 없었으나 내원시 고혈압이 있을 경우 만성 신부전으로의 진행이 많았다. 2) 내원시에 여러 임상 검사중 심한 단백뇨가 만성 신부전으로의 진행이 많았다. 3) WHO 및 Yagame 등의 조직 병리학적 분류 모두 소아 환자에서는 예후와의 관련을 찾을 수 없었다. 결론 : 이상의 결과로 보아 IgA 신병증 환아에서 내원시에 심한 단백뇨, 고혈압이 있을 경우 추적 관찰 결과 예후가 좋지 않았다. 그러나 본 연구의 경우 치료에 대한 평가가 이루어지지 않았고 대상 환아가 적었기에 향후 이를 포함하는 광범위한 연구가 진행되어야 할 것으로 사료된다.

• 대한본초학회지
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• 제31권5호
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• pp.71-78
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• 2016

Objectives : Diabetic nephropathy is one of the most common chronic complications of diabetes and a leading cause of end-stage renal failure in the world. Mesangial cell proliferation is known as the major pathologic features such as glomerulosclerosis and renal fibrosis. Wiryeongtang (WRT) is a well-known traditional herbal formula as therapeutic agents for chronic edema and dysuresia of renal homeostasis. In the present study, we investigated whether WRT inhibits high glucose (HG)-induced renal dysfunction by TGF-β/Smads signal regulation in cultured mesangial cells.Methods : Inhibitory effect of WRT (10-50 ㎍/ml) on HG-stimulated mesangial cells proliferation and dysfunction were evaluated by [³H]-thymidine incorporation, Western blot, and RT-qPCR.Results : WRT significantly decreased HG-accelerated thymidine incorporation in human renal mesangial cell in a dose-dependent levels. WRT induced down-regulation of cyclins/CDKs and up-regulation of CDK inhibitor, p21waf1/cip1 and p27kip1 expression. In addition, HG enhanced expression of dysfunction biomarker such as collagen IV and CTGF, which was markedly attenuated by WRT. WRT decreased TGF-β1 and Smad-2/Smad-4 expression, whereas increased Smad-7 expression under HG. Furthermore, WRT inhibited HG-induced inflammatory factors level such as ICAM-1 and MCP-1 as well as NF-κB p65 nuclear translocation and intracellular ROS production.Conclusions : These results suggested that WRT may alleviate mesangial proliferation and inflammation possibly involved in renal fibrotic process, further diabetic nephropathy through disturbing TGF-β1/Smad signaling and NF-κB/ROS pathway. Thus, WRT might prove to be effective in the treatment of renal dysfunction leading to diabetic nephropathy.

• Journal of Hospice and Palliative Care
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• 제23권3호
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• pp.162-165
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• 2020

Purpose: The coronavirus disease 2019 (COVID-19) pandemic has brought the world to a standstill and has exposed the lack of preparedness of most nations' health care systems. Even in usual times, palliative care has not received its fair share of recognition as an important component of patient care; instead, the emphasis is often placed on aggressive patient management. Now, with the entire medical community and decision-making committees focussed on intensive patient care, end-of-life care has taken a backseat. Methods: This article is a brief communication. Results: COVID 19 infection has been shown to lead to greater mortality and morbidity in patients with pre-existing illnesses such as hypertension, diabetes, renal failure, and cancer. Patients typically in need of end-of-life care, such as those with late-stage cancer or heart failure, are therefore at a higher risk of both contracting COVID-19 and suffering a more severe disease course. The strict nationwide lockdowns being imposed in most countries have deterred patients from seeking medical attention or hospice care. Every day new research is coming to light regarding COVID 19. This has helped significantly in creating awareness and limiting the spread of disease. However, misinformation is also rampant, leading to discrimination and mistreatment of infected patients. Conclusion: This pandemic has been a terrifying ordeal for all and has exposed our entire population physically, psychologically, emotionally, and financially to unimaginable stresses. In the present scenario, EOL care is as much a necessity as intensive care and should be given at least a fraction of its importance.

• 대한전기학회논문지:시스템및제어부문D
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• 제51권11호
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• pp.521-527
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• 2002

The Hemodialysis system is the device for the patients who have suffered from end stage renal failure as the kidney which removes the waste products in a human body. The existing hemodialysis is based on a 8-bit micro-controller and it is not a touch-screen type but a manual type. This paper is focused on hemodialysis system based on high control and expension embedded system. The whole system consists of main control unit and sub control unit(dialysis control unit, blood control unit, monitoring control unit, networking unit). The dialysis control unit, blood control unit, monitoring control unit are processed by 3 microcontrollers and network unit is for monitoring a renal failure patient's condition. For the evaluation of the system performance, the saline was pured into blood unit and then water removal rate, conductivity and temperature of hemodialysis liquid were measured 10 times in an each state suing the UF pump in the fluid unit varing the quantity of saline to 1000cc, 2000cc, 3000cc and 4000cc. As a result, the rates of water removal are 98.6% in condition of 000cc saline, 96.9% in 2000cc, 98.9% in 3000cc and 98.3% in 4000cc. The conductivities of hemodialysis liquid are 99.6% in the first to third condition and 99.7% in the forth condition. The temperatures of hemodialysis liquid are 99.8% in the first to third condition and 99.6% in th forth condition.

• Journal of Chest Surgery
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• 제57권2호
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• pp.184-194
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• 2024

Background: Left ventricular assist devices (LVADs) are widely employed as a therapeutic option for end-stage heart failure. We evaluated the outcomes associated with centrifugal-flow LVAD implantation, comparing 2 device models: the Heartmate 3 (HM3) and the Heartware Ventricular Assist Device (HVAD). Methods: Data were collected from patients who underwent LVAD implantation between June 1, 2015 and December 31, 2022. We analyzed overall survival, first rehospitalization, and early, late, and LVAD-related complications. Results: In total, 74 patients underwent LVAD implantation, with 42 receiving the HM3 and 32 the HVAD. A mild Interagency Registry for Mechanically Assisted Circulatory Support score was more common among HM3 than HVAD recipients (p=0.006), and patients receiving the HM3 exhibited lower rates of preoperative ventilator use (p=0.010) and extracorporeal membrane oxygenation (p=0.039). The overall early mortality rate was 5.4% (4 of 74 patients), with no significant difference between groups. Regarding early right ventricular (RV) failure, HM3 implantation was associated with a lower rate (13 of 42 [31.0%]) than HVAD implantation (18 of 32 [56.2%], p=0.051). The median rehospitalization-free period was longer for HM3 recipients (16.9 months) than HVAD recipients (5.3 months, p=0.013). Furthermore, HM3 recipients displayed a lower incidence of late hemorrhagic stroke (p=0.016). In the multivariable analysis, preoperative use of continuous renal replacement therapy (odds ratio, 22.31; p=0.002) was the only significant predictor of postoperative RV failure. Conclusion: The LVAD models (HM3 and HVAD) demonstrated comparable overall survival rates. However, the HM3 was associated with a lower risk of late hemorrhagic stroke.