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Serkan Feyyaz Yalın, Necmi Eren, Ayse Sinangil, Vural Taner Yilmaz, Erhan Tatar, Ali Riza Ucar, Mustafa Sevinc, Özgür Can, Alp Gurkan, Nurol Arik, Sabahat Alisir Ecder, Murathan Uyar, Murat Yasar, Sibel Gulcicek, Meral Mese, Hamad Dheir, Ulkem Cakir, Şimal Köksal Cevher, Kultigin Turkmen, Bahtisen Guven, Dilek Guven Taymez, Banu Erkalma Senates, Tevfik Ecder, Huseyin Kocak, Adam Uslu, Erol Demir, Taner Basturk, Melike Betul Ogutmen, Can Kinalp, Belda Dursun, Zerrin Bicik Bahcebasi, Savas Sipahi, Fatih Dede, Meric Oruc, Yasar Caliskan, Ahmed Genc, Berna Yelken, Mehmet Rıza Altıparmak, Aydin Turkmen, Nurhan Seyahi
BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from lack of alpha-galactosidase A (AGALA) activity in lysosomes. OBJECTIVE: In this multicenter study, we aimed to evaluate the prevalence of FD in renal transplant (Tx) recipients in Turkey. We also screened dialysis patients as a control group. METHODS: All Tx and dialysis patients were screened regardless of the presence of a primary disease. We measured the AGALA activity in all male patients as initial analysis...
February 8, 2019: Nephron
Tainá Veras de Sandes-Freitas, Juliana Gomes Ramalho de Oliveira, Gilberto Loiola de Alencar Dantas, Lucas Lobo Mesquita, Eliana Régia Barbosa de Almeida, Ronaldo de Matos Esmeraldo
BACKGROUND: In several countries, patients with end-stage renal disease who are ineligible for dialysis are considered urgency priority (UP) for kidney transplantation (KT) through distinct allocation rules. There are scarce published data on clinical features and outcomes after KT of these patients. METHODS: We retrospectively reviewed and compared demographic and clinical pretransplant characteristics and outcomes after KT of all patients transplanted under UP allocation in a single Brazilian transplant center from January 10 to March 16 (n = 74) and 1: 1 patients transplanted under standard allocation in the same period (n = 74)...
February 7, 2019: Nephron
Akira Maeda, Naoshi Fukushima, Naoshi Horiba, Hiroko Segawa, Ken-Ichi Miyamoto
BACKGROUND: Hyperphosphatemia is a major accelerator of complications in chronic kidney disease and dialysis, and phosphate (Pi) binders have been shown to regulate extracellular Pi levels. Research on hyperphosphatemia in mouse models is scarce, and few models display hyperphosphatemia induced by glomerular injury, despite its relevance to human glomerular disease conditions. In this study, we investigated the involvement of hyperphosphatemia in kidney disease progression using a mouse model in which hyperphosphatemia is induced by focal segmental glomerulosclerosis (FSGS)...
February 7, 2019: Nephron
Fatma Yazılıtaş, Fatma Zehra Oztek-Celebi, Özcan Erel, Evrim Kargın Çakıcı, Murat Alışık, Mehmet Bülbül
BACKGROUND: There is a growing body of evidence showing that there is an association between oxidative damage and kidney injury in patients with nephrotic syndrome (NS). Dynamic thiol/disulphide homeostasis (DTDH) is an important antioxidant system. The aim of this study was to determine if DTDH could be used as a biomarker of antioxidant status in pediatric NS patients using a novel automated method. METHODS: The study included 39 patients with NS and 40 healthy controls...
February 6, 2019: Nephron
Javad Savoj, Brian Becerra, Jin K Kim, Maria Fusaro, Maurizio Gallieni, Dawn Lombardo, Wei Ling Lau
Cardiovascular disease is prevalent in patients with chronic kidney disease (CKD) and responsible for approximately half of all CKD-related deaths. Unfortunately, the presence of CKD can lead to a challenging interpretation of cardiac biomarkers essential in accurate diagnosis and prompt management of heart failure and acute coronary syndrome. There is growing interest in novel cardiac biomarkers that may improve diagnostic accuracy reflecting myocardial injury, inflammation, and remodeling. Interpretation of these biomarkers in CKD can be complicated, since elevated levels may not reflect myocardial injury or wall tension but rather decreased urinary clearance with retention of solutes and/or overall CKD-associated chronic inflammation...
February 6, 2019: Nephron
Hayley J Fryer, Gavin I Welsh
An infant is described as "preterm" if they are born before 37 weeks of gestation. As nephrogenesis does not complete until 36 weeks of gestation, premature infants are often born with underdeveloped kidneys. Current evidence strongly suggests that this leaves those born prematurely more susceptible to developing kidney-related conditions later in life. Although the incidence of premature birth steadily increases each year in the United Kingdom, reports of perinatal death following preterm labour are falling...
February 6, 2019: Nephron
Nancy S Krieger, Marc Grynpas, Amy VandenEynde, John R Asplin, Kevin K Frick, Min Ho Kim, Felix M Ramos, Ignacio Granja, David A Bushinsky
BACKGROUND: Urine (u) calcium (Ca) excretion is directly dependent on dietary sodium (Na) intake leading to the recommendation for Na restriction in hypercalciuric kidney stone formers. However, there is no direct evidence that limiting Na intake will reduce recurrent stone formation. MATERIALS AND METHODS: We used genetic hypercalciuric stone-forming (GHS) rats, which universally form Ca phosphate (P) kidney stones, fed either a low Na (LNa, 0.05%) or normal Na (NNa, 0...
February 6, 2019: Nephron
Senji Okuno, Masaaki Inaba, Eiji Ishimura, Shinya Nakatani, Hidenori Chou, Shigeichi Shoji, Tomoyuki Yamakawa
BACKGROUND/AIM: Cinacalcet, an allosteric modulator of the calcium (Ca) sensing receptor, reduces the level of parathyroid hormone (PTH) in serum as well as parathyroid gland volume following administration in hemodialysis patients with secondary hyperparathyroidism, though long-term results are yet to be reported. METHODS: Serum parameters (n = 23), together with total parathyroid gland volume (n = 18), were determined in Japanese hemodialysis patients given cinacalcet treatment for 8 years...
February 1, 2019: Nephron
Ryo Matsuura, Kent Doi, Yohei Komaru, Yoshihisa Miyamoto, Teruhiko Yoshida, Takuya Isegawa, Kohei Yoshimoto, Tetsushi Yamashita, Eisei Noiri, Masaomi Nangaku
Backgrounds/Objectives: Elevated erythropoietin (EPO) is observed in human acute kidney injury (AKI). Whether blood EPO level is associated with mortality or other organ dysfunction in critically ill patients is unknown. METHODS: A prospective observational cohort study of 162 AKI patients requiring renal replacement therapy (RRT) was conducted in our intensive care unit (ICU) during October 2013 through October 2016. We evaluated the relation with plasma EPO at RRT initiation and 90-day mortality, hemoglobin, urine output, and sequential organ failure assessment (SOFA) score until day 7 or discharge from the ICU...
February 1, 2019: Nephron
Sabrina Milan Manani, Mitchell H Rosner, Grazia Maria Virzì, Anna Giuliani, Sonia Berti, Carlo Crepaldi, Claudio Ronco
BACKGROUND: Peritoneal dialysis (PD) is an ideal model for testing remote monitoring (RM). In this study, we evaluated the RM application longitudinally in stable patients undergoing automated PD (APD). METHODS: This was an observational study, comparing outcomes in patients with (current patients) and without (historical data) exposure of RM. We analyzed cost-effectiveness of RM-APD measuring the number of night alarms, number of hospital visits, direct and indirect costs...
January 30, 2019: Nephron
Mamdouh A Oraby, Mohammed F El-Yamany, Marwa M Safar, Naglaa Assaf, Hamdy A Ghoneim
BACKGROUND: Early prediction and clinical intervention are extremely important in order to delay or hinder diabetic nephropathy (DN) progression. OBJECTIVES: This study aimed to detect early signs of DN and study the potential ameliorating effect of the dipeptidyl peptidase-4 inhibitor, linagliptin, on some early markers for DN in fructose-streptozotocin (Fr-STZ)-induced diabetic rats. METHOD: Fr-STZ rats were treated with either linagliptin (3 mg/kg p...
January 30, 2019: Nephron
Yuan-Yuan Zhang, Wan-Ning Wang, Sen-Sen Su, Bin Chen, Wei-Xia Sun, Hao Wu, Yan-Li Cheng, Zhong-Gao Xu
BACKGROUND: 12-Lipoxygenase (12-LO) and angiotensin II (Ang II) are involved in the development of diabetic renal hypertrophy, in which cyclin-kinase inhibitors, p21 and p27 play pivotal roles. Here, we study the effects of 12-LO and its interaction with Ang II on glomerular p21 and p27 expression in diabetic conditions. METHODS: Models used in the current study include glomerular mesangial cells (MCs); and glomeruli from (1) type 2 diabetic db/db mice; (2) type 2 diabetic rats induced by high-fat diet feeding followed by streptozotocin injection; (3) 12-LO knockout (12-LOKO) mice; and (4) normal rats infused with Ang II or 12(S)-hydroxyeicosatetraenoic acid (12[S]-HETE, arachidonic acid metabolite of 12-LO)...
January 24, 2019: Nephron
Sergey Moiseev, Victor Fomin, Kirill Savostyanov, Alexander Pushkov, Alexey Moiseev, Andrey Svistunov, Leyla Namazova-Baranova
AIM: To evaluate the prevalence and clinical features of Fabry disease in patients with end-stage renal disease (ESRD) undergoing chronic hemodialysis. METHODS: α-Galactosidase A activity was measured in the dried blood spots by tandem mass spectrometry in 5,572 dialysis patients (63.7% males). Diagnosis of Fabry disease was confirmed by sequencing of the GLA gene and by evaluating the globotriaosylsphingosine level in the dried blood spots. RESULTS: Fabry disease was diagnosed in 20 (0...
January 24, 2019: Nephron
Li Yuan, Ping-Ping Liao, Hai-Cheng Song, Jia-Hui Zhou, Hai-Chen Chu, Lin Lyu
AIMS: Hyperbilirubinemia is associated with postoperative acute kidney injury in patients undergoing cardiac surgeries. A high concentration of bilirubin could induce oxidative stress and cell apoptosis. The aim of this study was to investigate whether hyperbilirubinemia aggravated the renal tubule cells injury and the pro-apoptotic potential of bilirubin on renal ischemia reperfusion injury (RIRI). METHODS: The human proximal tubular epithelial cell line HK-2 cells were challenged with a gradient concentration of bilirubin for 24 h...
January 23, 2019: Nephron
Nicholas M Selby
International criteria for describing the presence and severity of acute kidney injury (AKI) based on changes in serum creatinine concentration and/or degree of oliguria are now widely accepted. Subject of Review: Three recent articles have debated the definition and diagnosis of AKI, offering conflicting opinions. On one side [Lancet 2017; 389: 779-781 and Nephrology Times 2018] an argument is made that a focus on creatinine-based staging has de-emphasised the traditional clinical approach of determining cause of AKI (pre-renal, renal or post-renal), and that any classification system based on serum creatinine is inherently flawed...
January 18, 2019: Nephron
Mira Choi, Yoland-Marie Anistan, Kai-Uwe Eckardt, Maik Gollasch, Peter Nickel
Microscopic hematuria is a common feature of patients with Alport syndrome, a familial nephropathy due to mutations in COL4A3, COL4A4 or COL4A5. These genes encode for α3, α4, and α5 type IV collagen polypeptide chains (collagen IV α345), crucial for the structural component of the glomerular basement membrane. Even patients with mild phenotype, namely isolated microhematuria (X-linked females with thin basement membrane on electron microscopy or heterozygous carriers of COL4A3 or COL4A4 mutations), can potentially progress to proteinuria and to end-stage renal disease...
January 18, 2019: Nephron
Mona Boaz, Odile Azoulay, Idit F Schwartz, Doron Schwartz, Suhair Assady, Batya Kristal, Sydney Benshitrit, Noa Yanai, Talia Weinstein
BACKGROUND: Malnutrition is a frequently observed disorder in patients with chronic kidney disease (CKD) receiving hemodialysis (HD). While variously defined, malnutrition is a frequently observed condition among patients on HD. Prevalence estimates of malnutrition among Israeli HD patients have not been reported. OBJECTIVES: To survey nutrition intake in Israeli HD patients; estimate malnutrition risk prevalence; identify risk factors, and characterize malnutrition risk in HD patients...
January 17, 2019: Nephron
Andraž Stožer, Radovan Hojs, Jurij Dolenšek
BACKGROUND: Beta cells are central in the pathophysiology of diabetes, since their functional adaptation maintains euglycemia in insulin-resistant individuals and beta cell dysfunction is required for the clinical picture of frank diabetes. The pathophysiological mechanisms driving compensation and decompensation are incompletely understood and little is known about the influence of chronic kidney disease (CKD) on beta cell function. SUMMARY: In compensated insulin resistance, beta cells enhance their function at all stages in the stimulus-secretion coupling cascade, from the most proximal membrane depolarization to the intermediate increase in intracellular calcium concentration and the most distal granule fusion...
January 16, 2019: Nephron
Liad Hinden, Joseph Tam
Diabetic nephropathy (DN), a distinct manifestation of diabetic kidney disease, affects approximately 30% of patients with diabetes. While most attention has been focused on glomerular changes related to DN, there is growing evidence that tubulopathy is a key feature in the pathogenesis of this disease. The renal proximal tubule cells (RPTCs) are particularly sensitive to the deleterious effect of chronic hyperglycemia. However, the cellular changes that control the dysfunction of the RPTCs are not fully understood...
January 11, 2019: Nephron
Suwasin Udomkarnjananun, Natavudh Townamchai, Kroonpong Iampenkhae, Aisawan Petchlorlian, Nattachai Srisawat, Pisut Katavetin, Methee Sutherasan, Apirak Santingamkun, Kearkiat Praditpornsilpa, Somchai Eiam-Ong, Yingyos Avihingsanon
BACKGROUND: Delayed graft function (DGF) could worsen early and long-term outcomes of kidney transplantation (KT). DGF is caused by several pre-transplantation and perioperative factors in both donors and recipients. At present, there are no biomarkers or tests during the immediate post-KT period that can accurately predict the development of DGF. MATERIALS AND METHODS: This prospective study was conducted in deceased donor KT (DDKT) at King Chulalongkorn Memorial Hospital, Thailand...
January 11, 2019: Nephron
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