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Pankaj Beniwal, Lalit Pursnani, Sanjeev Sharma, R K Garsa, Mohit Mathur, Prasad Dharmendra, Vinay Malhotra, Dhanajai Agarwal
Studies published from centers across India have reported different and contradicting patterns of glomerular disease. In this retrospective study, we report our experience from a Tertiary Care Center in Northwest India. A total of 702 renal biopsies performed between 2008 and 2013 were reviewed of which 80 were excluded from the study because of having insufficient records or if the biopsies were taken from an allograft. The study included 411 males (66.1 %) and 211 females (33.9%) with an age range of 12-70 years (mean 30...
September 2016: Saudi Journal of Kidney Diseases and Transplantation
Imen Gorsane, I Helal, I Yacoub, F Ben Hamida, E Abderrahim, T Ben Abdallah
Focal and segmental glomerulosclerosis (FSGS) is a heterogeneous entity. Previous few studies have evaluated the efficacy of calcineurin inhibitors in primary FSGS and have suggested positive benefit. In this single-center, retrospective study (1975-2014), we report our experience in Tunisian adults with primary FSGS treated with cyclosporine A (CsA). It includes patients histologically proven FSGS and managed in the Charles Nicolle Hospital at Tunis, Tunisia. The dose of CsA was adjusted to maintain a whole blood trough level of 80-150 ng/mL...
September 2016: Saudi Journal of Kidney Diseases and Transplantation
Rutger J Maas, Jeroen K Deegens, Bart Smeets, Marcus J Moeller, Jack F Wetzels
Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are the key histological findings in patients with idiopathic nephrotic syndrome (INS). Although MCD and idiopathic FSGS are often considered to represent separate entities based on differences in their presenting characteristics, histology and outcomes, little evidence exists for this separation. We propose that MCD and idiopathic FSGS are different manifestations of the same progressive disease. The gradual development of FSGS in patients with non-remitting or relapsing INS has been well documented...
October 17, 2016: Nature Reviews. Nephrology
Beom Jin Lim, Jae Won Yang, Woo Sung Do, Agnes B Fogo
Focal segmental glomerulosclerosis (FSGS) is characterized by focal and segmental obliteration of glomerular capillary tufts with increased matrix. FSGS is classified as collapsing, tip, cellular, perihilar and not otherwise specified variants according to the location and character of the sclerotic lesion. Primary or idiopathic FSGS is considered to be related to podocyte injury, and the pathogenesis of podocyte injury has been actively investigated. Several circulating factors affecting podocyte permeability barrier have been proposed, but not proven to cause FSGS...
October 16, 2016: Journal of Pathology and Translational Medicine
Channa Jayasumana, Carlos Orantes, Raul Herrera, Miguel Almaguer, Laura Lopez, Luis Carlos Silva, Pedro Ordunez, Sisira Siribaddana, Sarath Gunatilake, Marc E De Broe
Increase in the prevalence of chronic kidney disease (CKD) is observed in Central America, Sri Lanka and other tropical countries. It is named chronic interstitial nephritis in agricultural communities (CINAC). CINAC is defined as a form of CKD that affects mainly young men, occasionally women. Its aetiology is not linked to diabetes, hypertension, glomerulopathies or other known causes. CINAC patients live and work in poor agricultural communities located in CINAC endemic areas with a hot tropical climate, and are exposed to toxic agrochemicals through work, by ingestion of contaminated food and water, or by inhalation...
October 13, 2016: Nephrology, Dialysis, Transplantation
Anna Kelly Krislane de Vasconcelos Pedrosa, Luiz Fernando Oliveira Torres, Ana Corina Brainer Amorim da Silva, Adrianna Barros Leal Dantas, Káthia Liliane da Cunha Ribeiro Zuntini, Lia Cordeiro Bastos Aguiar
Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case...
July 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Hideki Yokoi, Masashi Mukoyama
Renal fibrosis is characterized by glomerulosclerosis and tubulointerstitial fibrosis. Transforming growth factor-β (TGF-β) is postulated to play a central role in the development of both fibrotic processes. Extracellular matrix proteins, particularly type I collagen and fibronectin, accumulate in the tissue during renal fibrogenesis. CCN2, also known as connective tissue growth factor (CTGF), is increased in the setting of fibrosis and modulates a number of downstream signaling pathways involved in the fibrogenic properties of TGF-β...
2017: Methods in Molecular Biology
Yuka Sugawara, Kenjiro Honda, Daisuke Katagiri, Motonobu Nakamura, Takahisa Kawakami, Ryo Nasu, Akimasa Hayashi, Yukako Shintani, Akihiro Tojo, Eisei Noiri, Mineo Kurokawa, Masashi Fukayama, Masaomi Nangaku
The development of nephrotic syndrome (NS) after umbilical cord transplantation (UBT) has been reported in only four cases to date. We herein report the case of a 50-year-old woman who developed NS 94 days after UBT. She fell into oliguria and required dialysis. A kidney biopsy revealed focal and segmental glomerulosclerosis. Although glucocorticoid monotherapy did not improve her condition, the addition of low-density lipoprotein (LDL) apheresis resulted in remission of NS, a drastic improvement in her renal function, and withdrawal from dialysis...
2016: Internal Medicine
Ricardo Brandina, Katia Ramos Moreira Leite, Emerson Pereira Gregório, Karen Barros Parron Fernandes, Miguel Srougi
OBJECTIVES: To assess the association of histopathological parameters in non-neoplastic renal parenchyma with development of new onset chronic kidney disease (CKD) after radical nephrectomy. METHODS: Data were extracted from 222 patients who underwent radical nephrectomy. The Modification of Diet in Renal Disease (MDRD) formula was used. The study end point was development of CKD, defined as an estimated glomerular filtration rate (eGFR) of less than 60ml/minute/1,73m(2)...
October 7, 2016: Urology
Anna Julie Peired, Alessandro Sisti, Paola Romagnani
Mesenchymal stem cells form a population of self-renewing, multipotent cells that can be isolated from several tissues. Multiple preclinical studies have demonstrated that the administration of exogenous MSC could prevent renal injury and could promote renal recovery through a series of complex mechanisms, in particular via immunomodulation of the immune system and release of paracrine factors and microvesicles. Due to their therapeutic potentials, MSC are being evaluated as a possible player in treatment of human kidney disease, and an increasing number of clinical trials to assess the safety, feasibility, and efficacy of MSC-based therapy in various kidney diseases have been proposed...
2016: Stem Cells International
Frederico F C T Freitas, Gilberto Araujo, Marcella L Porto, Flavia P S Freitas, Jones B Graceli, Camille M Balarini, Elisardo C Vasquez, Silvana S Meyrelles, Agata L Gava
Increased blood pressure variability (BPV), which can be experimentally induced by sinoaortic denervation (SAD), has emerged as a new marker of the prognosis of cardiovascular and renal outcomes. Considering that increased BPV can lead to organ-damage, the goal of the present study was to evaluate the effects of SAD on renal function in an experimental model of chronic kidney disease (CKD). SAD was performed in male Wistar rats 2 weeks before 5/6 nephrectomy and the animals were evaluated 4 weeks after the induction of CKD...
2016: Frontiers in Physiology
Jie Ni, Sujin Bao, Ruth I Johnson, Bingbing Zhu, Jianhua Li, Justin Vadaparampil, Christopher M Smith, Kirk N Campbell, Florian Grahammer, Tobias B Huber, John C He, Vivette D D'Agati, Andrew Chan, Lewis Kaufman
MAGI-1 is a multi-domain cytosolic scaffolding protein that in kidney is specifically located at the podocyte slit diaphragm, a specialized junction that is universally injured in proteinuric diseases. There it interacts with several essential molecules including nephrin and neph1, which are required for slit diaphragm formation and as an intracellular signaling hub. Here, we show that diminished MAGI-1 expression in cultured podocytes reduced nephrin and neph1 membrane localization and weakened tight junction integrity...
October 5, 2016: Journal of Biological Chemistry
Le Zhang, Siyi He, Fan Yang, Hua Yu, Wei Xie, Qian Dai, Di Zhang, Xiaoqin Liu, Shiwen Zhou, Kebin Zhang
The purpose of this study was to investigate the therapeutic effects of hyperoside (Hyp) on glomerulosclerosis in diabetic nephropathy and its underlying mechanisms. Blood glucose, kidney mass, and renal function of mice were measured. Renal morphology was observed using hematoxylin and eosin, periodic acid - Schiff's, and Masson's trichrome stain. Fibronectin (FN) and collagen IV (COL IV) in kidney were determined by Western blot and immunohistochemical studies. Matrix metalloproteinases (MMP)-2 and -9 and tissue inhibitors of metalloproteinase (TIMP)-1 in renal tissues were detected on both the mRNA and protein levels...
July 19, 2016: Canadian Journal of Physiology and Pharmacology
Julia Hoefele, Markus J Kemper, Ulf Schoenermarck, Susanna Mueller, Hanns-Georg Klein, Anja Lemke
About 30% of children with nephrotic syndrome (NS) have inherited forms. Among them, mutations in Wilms tumor suppressor gene 1 (WT1) are a well characterized cause associated with steroid-resistant NS, Wilms tumor, and urogenital malformation in males. However, the role of WT1 mutations in adult-onset focal segmental glomerulosclerosis (FSGS) is unclear. We report the case of a 38-year-old female with FSGS. She had been diagnosed with streak ovaries during diagnostic workup for infertility. Mutational analysis identified the heterozygous mutation c...
October 5, 2016: Nephron
Xiangling Wang, Franca Anglani, Lada Beara-Lasic, Anila J Mehta, Lisa E Vaughan, Loren Herrera Hernandez, Andrea Cogal, Steven J Scheinman, Gema Ariceta, Robert Isom, Lawrence Copelovitch, Felicity T Enders, Dorella Del Prete, Giuseppe Vezzoli, Fabio Paglialonga, Peter C Harris, John C Lieske
BACKGROUND AND OBJECTIVES: Dent disease is a rare X-linked disorder characterized by low molecular weight proteinuria and often considered a renal tubular disease. However, glomerulosclerosis was recently reported in several patients. Thus, Dent disease renal histopathologic features were characterized and assessed, and their association with kidney function was assessed. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Clinical renal pathology reports and slides (where available) were collected from 30 boys and men in eight countries who had undergone clinical renal biopsy between 1995 and 2014...
October 3, 2016: Clinical Journal of the American Society of Nephrology: CJASN
Jaime A Campbell, John R Krause
Prolonged immunosuppression in solid organ transplant recipients has been considered a risk for developing opportunistic infections and malignancies. Acute leukemia is a rare complication. We report a case of acute promyelocytic leukemia (APL) (FAB M3) after cadaveric renal transplant for focal segmental glomerulosclerosis in a 24-year-old woman. Her immunosuppressive therapy included tacrolimus, mycophenolate mofetil, and prednisone. Approximately 2 years after transplant, she became pancytopenic, prompting administration of filgrastim...
October 2016: Proceedings of the Baylor University Medical Center
Angelika Url, Verena Krutak, Anna Kübber-Heiss, Sonja Chvala-Mannsberger, Nadia Robert, Nora Dinhopl, Peter Schmidt, Chris Walzer
According to previous studies in captive cheetah ( Acinonyx jubatus ) populations, one of the most threatening diseases besides amyloidosis, myelopathy, veno occlusive disease, and gastritis, is renal failure. Contrary to captive cheetahs in North America and South Africa, morphological data concerning renal lesions in the cheetah European Endangered Species Program (EEP) are lacking. This study details the histological characterization as well as immunohistochemical and morphometrical analysis of nephropathies in 35 captive cheetahs from the EEP, which were necropsied between 1985 and 2003...
September 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
Yasser Gamal, Ahlam Badawy, Salwa Swelam, Mostafa S K Tawfeek, Eman Fathalla Gad
Glucocorticoids are primary therapy of idiopathic nephrotic syndrome (INS). However, not all children respond to steroid therapy. We assessed glomerular glucocorticoid receptor expression in fifty-one children with INS and its relation to response to steroid therapy and to histopathological type. Clinical, laboratory and glomerular expression of glucocorticoid receptors were compared between groups with different steroid response. Glomerular glucocorticoid expression was slightly higher in controls than in minimal change early responders, which in turn was significantly higher than in minimal change late responders...
October 3, 2016: Fetal and Pediatric Pathology
Fatemeh Yaghoubi, Maliheh Yarmohammadi, Mohammad Vasei
We report a 55-year-old man presented with anemia and weakness, history of flank pain, hematuria and nephrotic syndrome. Spiral abdominopelvic computerized tomography (CT) scan showed multiloculated cystic mass (120 ×100 ×80 mm) in lower portion of left kidney with internal enhancing solid components and coarse peripheral calcifications. Radical nephrectomy of left kidney was done and biopsy confirmed renal cell carcinoma (RCC), papillary type, sarcomatoid foci, Fuhrman grade III. We assumed that, presence of nephrotic syndrome and paraneoplastic glomerulopathy leaded to heavy proteinuria in this case...
2016: Journal of Renal Injury Prevention
Olga Balafa, Georgios Liapis, Paraskevi Pavlakou, Georgios Baltatzis, Rigas Kalaitzidis, Moses Elisaf
Kimmelstiel and Wilson originally described nodular glomerulosclerosis as the pathognomonic lesion of diabetic nephropathy. Nevertheless, nodular glomerulosclerosis pattern can rarely occur in non-diabetic patients. In such cases, the differential diagnosis includes membranoproliferative glomerulonephritis, light or heavy chain deposition disease, amyloidosis, fibrillary and immunotactoid glomerulonephritis and chronic hypoxic or ischemic conditions. In cases that the above entities cannot be proven, the term idiopathic nodular glomerulosclerosis is given...
September 22, 2016: Pathology, Research and Practice
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