• Childhood Kidney Diseases
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• v.23 no.2
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• pp.67-76
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• 2019

Kidney disease is a major global health issue. Hemodialysis and kidney transplantation have been used in the clinic to treat renal failure. However, the dialysis is not an effective long-term option, as it is unable to replace complete renal functions. Kidney transplantation is the only permanent treatment for end-stage renal disease (ESRD), but a shortage of implantable kidney tissues limits the therapeutic availability. As such, there is a dire need to come up with a solution that provides renal functions as an alternative to the current standards. Recent advances in cell-based therapy have offered new therapeutic options for the treatment of damaged kidney tissues. Particularly, cell secretome therapy utilizing bioactive compounds released from therapeutic cells holds significant beneficial effects on the kidneys. This review will describe the reno-therapeutic effects of secretome components derived from various types of cells and discuss the development of efficient delivery methods to improve the therapeutic outcomes.

• Journal of Dental Rehabilitation and Applied Science
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• v.40 no.2
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• pp.46-54
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• 2024

Purpose: The purpose of this study was to compare the periodontal status of end-stage renal disease patients undergoing dialysis and referred for intraoral evaluation prior to renal transplantation surgery with those having normal kidney function. Materials and Methods: Patients who had been undergoing dialysis for end-stage renal disease and been referred to the Dental Clinic Center by the Department of Nephrology at University Hospital for intraoral evaluation prior to kidney transplantation surgery. For comparison of periodontal status, subjects without abnormalities in kidney function were matched with the patients by age and gender and selected as healthy controls. The patients' age, gender, comorbidities, type of dialysis received, and duration of dialysis were investigated by reference to their medical records, and data on their periodontal status were analyzed via the relevant periodontal records. Results: A total of 102 patients, including 51 dialyzed patients and 51 healthy control group subjects, participated in this study. In the patients with end-stage renal disease undergoing dialysis with periodontal probing depth of 5 mm or more, percentage of sites with clinical attachment level of 4 mm or more, percentage of teeth with bleeding on probing, number of missing teeth, and ratio of moderate to severe periodontitis were all significantly greater than in the healthy controls. Conclusion: The periodontal status of end-stage renal disease patients undergoing dialysis and referred for intraoral evaluation prior to kidney transplantation was worse than that of healthy controls.

• Clinical and Molecular Hepatology
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• v.24 no.4
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• pp.351-357
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• 2018

The advent of novel, direct-acting antiviral (DAA) regimens for hepatitis C virus (HCV) infection has revolutionized its treatment by producing a sustained virologic response of more than 95% with few side effects and no comorbidities in the general population. Until recently, ideal DAA regimens have not been available to patients with severe renal impairment and end-stage renal disease because there are limited data on the pharmacokinetics, safety, and efficacy of treatment in this unique population. In a hemodialysis context, identifying patients in need of treatment and preventing HCV transmission may also be a matter of concern. Recently published studies suggest that a combination of paritaprevir/ritonavir/ombitasvir and dasabuvir, elbasvir/grazoprevir, or glecaprevir/pibrentasvir successfully treats HCV infection in chronic kidney disease stage 4 or 5 patients with or without hemodialysis.

• Journal of Genetic Medicine
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• v.11 no.2
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• pp.74-78
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• 2014

Sotos syndrome (SS, OMIM 117550) is characterized by prenatal and postnatal overgrowth with multiple congenital anomalies. However, there have been few cases of growth retardation caused by renal failure from infancy. We report a case of dysplasia of the bilateral kidneys with renal failure and poor postnatal growth. A 2-month-old boy visited the emergency room owing to poor oral intake and abdominal distension. He was born at the gestational age of 38 weeks with a birth weight of 4,180 g. After birth, he had feeding difficulty and abdominal distension. Upon physical examination, his height and weight were in less than the 3rd percentile, while his head circumference was in the 50th percentile on the growth curve. He also showed a broad and protruding forehead and high hairline. Blood laboratory tests showed severe azotemia; emergent hemodialysis was needed. Abdominal ultrasonography revealed bilateral renal dysplasia with multiple cysts and diffuse bladder wall thickening. A posterior urethral valve was suggested based on vesicoureterography and abdominal magnetic resonance findings. Results of a colon study to rule out congenital megacolon did not reveal any specific findings. The conventional karyotype of the patient was 46, XY. Array comparative genomic hybridization study revealed a chromosome 5q35 microdeletion including the NSD1 gene, based on which SS was diagnosed. We describe a case of SS presenting with end stage renal disease due to posterior urethral valve. The typical somatic overgrowth of SS in the postnatal period was not observed due to chronic renal failure that started in the neonatal period.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.165-168
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• 2017

Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.91-97
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• 2020

Purpose: Alport syndrome (AS) is one of the most common inherited renal diseases caused due to mutations of genes encoding specific proteins of the type IV collagen family, and its major clinical manifestations include progressive renal failure, sensorineural deafness, and ocular abnormalities. We investigated the clinical characteristics and long-term prognosis of AS in Korean pediatric and adult populations. Methods: We conducted a retrospective review of medical records of 33 children and adults who had been diagnosed or treated with AS from 1985 to 2019. Results: The mean age of the 33 patients diagnosed with AS was 16.2±13.6 years, and the male-to-female ratio was 2:1. At the first visit, recurrent gross hematuria was the most common initial symptom. In 10 of 33 patients (30.3%), sensorineural hearing loss (SNHL) was diagnosed, but none had ophthalmic problems. Moreover, 11 of 33 patients (33.3%) had advanced to end-stage renal disease (ESRD), and a significant difference was observed in the age of the patients who progressed to ESRD based on the presence or absence of SNHL (P=0.035). Conclusion: SNHL in AS can be an important prognostic factor for long-term deterioration of renal function. Further investigation is required to confirm the clinical course and the genetic characteristics of AS in Korea through prospective national cohort studies.

• Biomolecules & Therapeutics
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• v.32 no.3
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• pp.291-300
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• 2024

Autosomal dominant polycystic kidney disease (ADPKD), a congenital genetic disorder, is a notable contributor to the prevalence of chronic kidney disease worldwide. Despite the absence of a complete cure, ongoing research aims for early diagnosis and treatment. Although agents such as tolvaptan and mTOR inhibitors have been utilized, their effectiveness in managing the disease during its initial phase has certain limitations. This review aimed to explore new targets for the early diagnosis and treatment of ADPKD, considering ongoing developments. We particularly focus on cell polarity, which is a key factor that influences the process and pace of cyst formation. In addition, we aimed to identify agents or treatments that can prevent or impede the progression of renal fibrosis, ultimately slowing its trajectory toward end-stage renal disease. Recent advances in slowing ADPKD progression have been examined, and potential therapeutic approaches targeting multiple pathways have been introduced. This comprehensive review discusses innovative strategies to address the challenges of ADPKD and provides valuable insights into potential avenues for its prevention and treatment.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.23-29
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• 2008

Kidney allograft transplantation is the most effective method of renal replacement for end stage renal disease patients. Still, it is another kind of 'disease', requiring immunosuppression to keep the allograft from rejection(allograft immune reaction). Immune system of the allograft recipient recognizes the graft as a 'pathogen (foreign or danger)', and the allograft-recognizing commanderin-chief of adaptive immune system, T cell, recruits all the components of immune system for attacking the graft. Proper activation and proliferation of T cell require signals from recognizing proper epitope(processed antigen by antigen presenting cell) via T cell receptor, costimulatory stimuli, and cytokines(IL-2). Thus, most of the immunosuppressive agents suppress the process of T cell activation and proliferation.

• Proceedings of the PSK Conference
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• 2003.10b
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• pp.145.1-145.1
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• 2003

Oxidative stress has been implicated in a range of disease states, including end-stage renal failure treated with hemodialysis [Westhuyzen J. et al, 2003]. Free radicals react with biological molecules and destroy the structure of cells, which eventually causes free-radical induced disease such as cancer, renal failure, aging, etc. Exogenous or endogenously produced nitric oxide (NO) inhibits superoxide-stimulated urea permeability. In the inner medulla, superoxide generation by local oxidases may stimulate urea transport, and the role of endogenous No may be to dampen this effect by decreasing superoxide levels ［Zimpelmann J. et al, 2003 (Epub ahead of print)］. (omitted)

• Journal of Chest Surgery
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• v.35 no.9
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• pp.680-683
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• 2002

Infective endocarditis that involves the right side of the heart has been estimately 5% of all cases of infective endocarditis. It has been shown that about 70% of right-sided heart infective endocarditis cases have preexisting congenital heart disease or acquired valvular lesion. It would occur in intravenous drug users or end-stage renal disease patients with indwelling venous dialysis catheter. Antibiotic therapy is more effective in the right and, when it fails, the consequence of valve disruption and emboli are less. Patients receiving long-term hemodialysis are a unique population with regard in the risk of bacteremia and subsequent infective endocarditis. We experienced one case of the active infective endocarditis with right atrial vegetation without tricuspid or pulmonary valve involvement in patient with end-stage renal disease receiving long-term hemodialysis, who needed surgical correction after medical treatment failure. Then we reported it with references that right-sided heart infective endocarditis is rare, but difficult to diagnose, life-threatening because of delayed medical treatment.