• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.1
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• pp.29-35
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• 2020

Joubert syndrome (JS) is a rare genetic disorder that is characterized by ataxia, hypotonia, developmental delay, respiratory abnormalities such as apnea-hyperpnea, and abnormal eye movements. The pathognomonic diagnostic finding is the "molar tooth sign" (MTS) on brain magnetic resonance imaging (MRI), described as cerebellar vermis hypoplasia or dysplasia, thick and horizontally oriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. JS is characterized by genetic heterogeneity: pathogenic variants in over 30 genes have been identified to date. The CEP290 protein, which is on chromosome 12q21.3, is most frequently mutated in patients with JS, especially with renal involvement. Here, we report a case of JS in a 14-year-old male patient with end-stage renal disease. To the best of our knowledge, this is the first Korean report of a patient with JS due to CEP290 mutation (c.6012-12T> A) whose diagnosis was confirmed after repetitive MRI. We suggest consultation with an experienced neuro-radiologist and follow-up MRI studies to detect a "hidden" MTS if clinical findings suggest a diagnosis of JS. Furthermore, even in the absence of an MTS, whole exome sequencing should be considered.

• Journal of Microbiology and Biotechnology
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• v.17 no.12
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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 The Korean Society of Clinical Toxicology
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• v.16 no.2
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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.

• Journal of computational fluids engineering
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• v.16 no.4
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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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• 2011.05a
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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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• v.1 no.1
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• pp.17-23
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• 1997

After the first description of IgA nephropathy by Berger in 1968, the prognosis of this disease was considered favourable. However recent studies have revealed that IgA nephropathy result in end stage renal desease in 25-30% by 20 years. Heavy proteinuria, hypertension, histological high class are regarded as poor prognostic factors. In 1996, Yagame et al reported the new histopathologic grading with a strong correlation between the grading, heavy proteinuria, high s-Cr level and renal survival. The aims of this study are to determine whether the pathological grading and other clinical parameters could contribute to predicting the outcome of this disease eventhough pediatric patients. Seventy nine patients (59 males, 20 females) with IgA nephropathy were examined. Patients were 2.08-15.17 years of age ($9.85{\pm}2.83$). The mean follow-up duration were $27{\pm}28$ months. Six of seventy nine patients progressed to chronic renal failure during the follow-up periods. High 24h urinary protein excretion at diagnosis were significantly higher in chronic renal failure patients (p<0.05). Hypertension at diagnosis were the significant associated factors in progression of chronic renal failure (p<0.05). Histological changes of IgA nephropathy in light microscopy were classified into five classes by WHO classification, four grades in Yagame's gradings. Among the seventy nine patients, 24 were as class 1, 30 as class 2, 23 as class 3; 4 as class 4, 0 as class 5 by WHO classification. 23 were classified grade 1, 31 as grade 2, 24 as grade 3, 1 as grade 4 by Yagame's grading. Among six patients who progressed to chronic renal failure, 1 clssified as class 1, 1 as class 2, 3 as class 3, 1 as class 4, 0 as class 5 by WHO Classification. 1 patients were classified as grade 1, 1 as grade 2, 3 as grade 3, 1 as grade 4 by Yagame's grading. (p>0.05) In conclusion, hypertension and heavy proteinuria at initial presentation were significantly associated with progression of chronic renal failure. The classification of WHO & Yagame's grading has no significant association with the progression of chronic renal failure in pediatric patients.

• The Korea Journal of Herbology
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• v.31 no.5
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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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• v.23 no.3
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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.

• The Transactions of the Korean Institute of Electrical Engineers D
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• v.51 no.11
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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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• v.57 no.2
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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.