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Glomerulonephrities

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https://www.readbyqxmd.com/read/28731904/monoclonal-gammopathy-of-undetermined-significance-after-kidney-transplantation-single-center-experience
#1
Gaetano Alfano, Francesco Fontana, Elisabetta Colaci, Andrea Messerotti, Francesca Bettelli, Leonardo Potenza, Mario Luppi, Gianni Cappelli
BACKGROUND: Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic premalignant plasma cell disorder. Prevalence and clinical outcomes of MGUS in kidney transplant (KT) recipients have been previously reported in few studies with conflicting results. METHODS: We conducted a retrospective study in a population of 548 KT recipients transplanted between 1998 and 2015. RESULTS: Thirty-nine subjects (8.1%) developed MGUS after KT...
July 21, 2017: Transplantation
https://www.readbyqxmd.com/read/28731865/-joining-the-spots-in-adults-and-young-tots-a-clinicopathological-study-of-henoch-sch%C3%A3-nlein-purpura-iga-vasculitis
#2
Priyanka P Kedia, Rajalakshmi Tirumalae, Divya Puttegowda, Meryl Antony
INTRODUCTION: Henoch-Schönlein Purpura (HSP; IgA vasculitis), the most common vasculitis of childhood, has a wide spectrum of clinical manifestations ranging from palpable purpura with abdominal pain and arthritis to the more morbid renal involvement. AIMS AND OBJECTIVES: To study and correlate the clinical presentation, laboratory values, skin and renal histopathology and immunofluoroscence findings in HSP. MATERIAL AND METHODS: A total of 44 cases of HSP from March 2011 to February 2014 were studied for the above features along with their clinical outcomes...
August 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28731667/-henoch-schonlein-purpura-involving-the-penis-a-case-report
#3
Borja Croche Santander, Elena Campos, Adela Sánchez, Laura Marcos, Isabel Díaz, Cristóbal Toro
Schonlein-Henoch purpura accounts for the majority of cases of systemic vasculitis in children. Classical presentation is characterized by palpable purpura, glomerulonephritis, arthralgias and abdominal pain. Although genitourinary manifestations, in form of testicular and scrotal involvement, have been widely described, penile involvement remains an extremely rare complication. We report a case of a 6-year-old boy who presented with purpuric rash on the glans, prepuce and penile shaft, with painful edema in the penile region...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28729970/severe-cyclophosphamide-related-hyponatremia-in-a-patient-with-acute-glomerulonephritis
#4
Pasquale Esposito, Maria Valentina Domenech, Nicoletta Serpieri, Marta Calatroni, Ilaria Massa, Alessandro Avella, Edoardo La Porta, Luca Estienne, Elena Caramella, Teresa Rampino
Cyclophosphamide is frequently used to treat cancer, autoimmune and renal diseases, such as rapidly progressive glomerulonephritis. Its side effects are well-known, including bone marrow depression, infections, alopecia, sterility, bladder malignancy and hemorrhagic cystitis. Moreover, in some cases cyclophosphamide use has been related to the onset of hyponatremia, by development of a syndrome of inappropriate antidiuresis. Indeed, severe hyponatremia has been previously reported in patients treated with high-dose or moderate-dose of intravenous cyclophosphamide, while only few cases have been reported in patients treated with low dose...
July 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28729648/a-haplotype-in-cfh-family-genes-confers-high-risk-of-rare-glomerular-nephropathies
#5
Yin Ding, Weiwei Zhao, Tao Zhang, Hao Qiang, Jianping Lu, Xin Su, Shuzhen Wen, Feng Xu, Mingchao Zhang, Haitao Zhang, Caihong Zeng, Zhihong Liu, Huimei Chen
Despite distinct renal lesions, a series of rare glomerular nephropathies are reportedly mediated by complement overactivation. Genetic variations in complement genes contribute to disease risk, but the relationship of genotype to phenotype has not been straightforward. Here, we screened 11 complement genes from 91 patients with atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G) and membranoproliferative glomerulonephritis type I (MPGN I), and identified the concomitant presence of three missense variations located within the human complement Factor H (CFH) gene cluster...
July 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28729035/a-novel-cfhr1-cfhr5-hybrid-leads-to-a-familial-dominant-c3-glomerulopathy
#6
Shambhuprasad K Togarsimalemath, Sidharth K Sethi, Rajan Duggal, Moglie Le Quintrec, Pranaw Jha, Régis Daniel, Florence Gonnet, Shyam Bansal, Lubka T Roumenina, Veronique Fremeaux-Bacchi, Vijay Kher, Marie-Agnes Dragon-Durey
The intrinsic similarity shared between the members of the complement factor H family, which comprises complement factor H and five complement factor H-related (CFHR) genes, leads to various recombination events. In turn these events lead to deletions of some genes or abnormal proteins, which are found in patients with atypical hemolytic uremic syndrome or C3 glomerulopathies. Here we describe a novel genetic rearrangement generated from a heterozygous deletion spanning 146 Kbp involving multiple CFHR genes leading to a CFHR1-R5 hybrid protein...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28722531/patterns-of-renal-disease-in-south-korea-a-20-year-review-of-a-single-center-renal-biopsy-database
#7
Ho Sik Shin, Dae Hyeon Cho, Soo Kyoung Kang, Hyun Jeong Kim, Soo Young Kim, Joung Wook Yang, Gyong Hoon Kang, Ye Na Kim, Yeonsoon Jung, Bong-Kwon Cheon, Hark Rim
BACKGROUND: Several registries and centers have reported the results of renal biopsies from different parts of the world. As there are few data regarding the epidemiology of glomerulonephritis (GN) in South Korea, we conducted this study on renal biopsy findings during the last 20 years from a single center. METHODS: Data for 818 patients who underwent renal biopsy at our center between 1992 and 2011 were collected retrospectively. All kidney specimens were examined with light microscopy (LM) and immunofluorescent microscopy (IF)...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28719617/microrna-155-deficiency-leads-to-decreased-autoantibody-levels-and-reduced-severity-of-nephritis-and-pneumonitis-in-pristane-induced-lupus
#8
Harald Leiss, Wilhelm Salzberger, Barbara Jacobs, Irina Gessl, Nicolas Kozakowski, Stephan Blüml, Antonia Puchner, Attila Kiss, Bruno K Podesser, Josef S Smolen, Georg H Stummvoll
OBJECTIVE: We herein examine the role of endogenous miR155 in the development of systemic manifestations in pristane induced lupus. MATERIALS AND METHODS: Systemic lupus in miR155-deficient and wild type mice was induced upon injection of pristane and analyzed after 8 months, PBS-injected mice served as controls. Glomerulonephritis and pneumonitis were quantified using the kidney biopsy score and a newly adapted histomorphometric image analysis system; lung tissue was further analyzed by tissue cytometry...
2017: PloS One
https://www.readbyqxmd.com/read/28717059/pharmacological-effects-of-a-vitamin-k1-2-3-epoxide-reductase-vkor-inhibitor-3-acetyl-5-methyltetronic-acid-on-cisplatin-induced-fibrosis-in-rats
#9
Masashi Uchida, Tomoya Miyoshi, Yohei Miyamoto
Cisplatin (CDDP) is a chemotherapeutic agent that is widely used in the treatment of lymphomas and solid malignancies. However, its clinical usage is limited by its severe side effects in the kidneys. Glomerular and tubular injuries in the kidneys commonly progress to interstitial fibrosis and, ultimately, the end stage of renal failure. We previously reported that 3-acetyl-5-methyltetronic acid (AMT) had inhibitory effects on rat renal vitamin K1 2,3-epoxide reductase (VKOR) in vitro and also suppressed mesangial cell proliferation and, consequently, the formation of fibrosis via the vitamin K-dependent activation of the growth arrest-specific 6 (Gas6)/Axl pathway in anti-Thy-1 glomerulonephritis (Thy-1 GN) in rats...
July 16, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#10
Takayuki Katsuno, Shige Mizuno, Masatsuna Mabuchi, Naotake Tsuboi, Atsushi Komatsuda, Shoichi Maruyama
BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the non-amyloid deposition of monoclonal immunoglobulin fragments in the basement membranes. Heavy chain deposition disease (HCDD) is a type of MIDD. HCDD is an extremely rare disease, and only three cases have been reported in Japan up to the present. The prognosis of HCDD is very poor, and optimal treatment has not been established. Only a few cases of HCDD with favorable long-term renal prognosis have been reported to date...
July 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28712854/c5-nephritic-factors-drive-the-biological-phenotype-of-c3-glomerulopathies
#11
Maria-Chiara Marinozzi, Sophie Chauvet, Moglie Le Quintrec, Morgane Mignotet, Florent Petitprez, Christophe Legendre, Mathilde Cailliez, Georges Deschenes, Michel Fischbach, Alexandre Karras, Francois Nobili, Christine Pietrement, Marie-Agnes Dragon-Durey, Fadi Fakhouri, Lubka T Roumenina, Veronique Fremeaux-Bacchi
C3 Glomerulopathies, which include Dense Deposit Disease and C3 Glomerulonephritis, are associated with genetic and acquired dysregulation of the C3 convertase alternative pathway of complement. The potential role of the activation of the C5 convertase has not been studied extensively. Here we analyzed IgG samples from patients with C3 Glomerulopathies to identify circulating autoantibodies that stabilize the C3 alternative pathway (C3 Nephritic Factors) as well as C5 convertases (C5 Nephritic Factors), thus preventing decay of these enzyme complexes...
July 13, 2017: Kidney International
https://www.readbyqxmd.com/read/28710302/a-rare-case-of-atypical-chronic-lymphocytic-leukaemia-presenting-as-nephrotic-syndrome
#12
Martina Soldarini, Lucia Farina, Augusto Genderini, Niccolo Bolli
Chronic lymphocytic leukaemia (CLL) is characterised by a lymphocytosis of mature-appearing clonal CD5+, CD23+ B lymphocytes. CLL cells arise from the bone marrow and infiltrate lymphoid tissues such as lymph nodes and spleen. Presentation is usually through discovery of lymphocytosis or lymphadenopathy. Unusual presentations, especially paraneoplastic syndromes are rare. Here, we describe a rare case presenting with severe nephrotic syndrome associated with the presence of a monoclonal protein in serum. Workup for suspected plasma cell dyscrasia led instead to the diagnosis of bone marrow infiltration by atypical CLL without lymphocytosis...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28709599/recurrence-of-glomerulonephritis-an-underestimated-and-unmet-medical-need
#13
Johan W de Fijter
Recurrent glomerulonephritis is increasingly recognized as an important contributor to premature renal allograft failure. The incidence of recurrent glomerulonephritis varies widely depending on original disease, renal biopsy policy, and time of observation. Biopsy-documented clinical recurrence rates underestimate the true prevalence. The impact of recurrent glomerulonephritis on graft survival parameters also widely varies and requires large collaborative efforts with more detailed and balanced evaluations, such as other major causes for chronic renal allograft dysfunction...
August 2017: Kidney International
https://www.readbyqxmd.com/read/28705055/ferrokinetics-is-associated-with-the-left-ventricular-mass-index-in-patients-with-chronic-kidney-disease
#14
Akihito Tanaka, Daijo Inaguma, Yu Watanabe, Eri Ito, Naoki Kamegai, Hiroya Shimogushi, Hibiki Shinjo, Kiyomi Koike, Yasuhiro Otsuka, Asami Takeda
Background Patients with chronic kidney disease (CKD) often have the complication of anaemia. Usage of an erythropoietin-stimulating agent accelerates iron deficiency because it promotes iron utilization. Recently, iron administration was reported to be effective for patients with cardiac failure. We examined the association between ferrokinetics and cardiac function in patients with CKD. Methods In this cross-sectional study, we examined 558 patients (424 men and 134 women; mean age, 68.9 ± 13.1 years) with CKD who were admitted to our hospital...
July 14, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28704582/autosomal-dominant-form-of-type-iv-collagen-nephropathy-exists-among-patients-with-hereditary-nephritis-difficult-to-diagnose-clinicopathologically
#15
Aya Imafuku, Kandai Nozu, Naoki Sawa, Eiko Hasegawa, Rikako Hiramatsu, Masahiro Kawada, Junichi Hoshino, Kiho Tanaka, Yasuo Ishii, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Kazumoto Iijima, Yoshifumi Ubara
AIM: Type IV collagen nephropathies include Alport Syndrome and thin basement membrane nephropathy (TBMN), which are caused by mutations in COL4A3/A4/A5 genes. Recently, the report of patients with heterozygous mutations in COL4A3/A4 have been increasing. The clinical course of these patients has a wide variety, and they are diagnosed as TBMN, autosomal dominant Alport syndrome (ADAS), or familial focal segmental glomerular sclerosis. However, diagnosis, frequency and clinicopathological manifestation of them remains unclear...
July 13, 2017: Nephrology
https://www.readbyqxmd.com/read/28699638/bcat1-controls-metabolic-reprogramming-in-activated-human-macrophages-and-is-associated-with-inflammatory-diseases
#16
Adonia E Papathanassiu, Jeong-Hun Ko, Martha Imprialou, Marta Bagnati, Prashant K Srivastava, Hong A Vu, Danilo Cucchi, Stephen P McAdoo, Elitsa A Ananieva, Claudio Mauro, Jacques Behmoaras
Branched-chain aminotransferases (BCAT) are enzymes that initiate the catabolism of branched-chain amino acids (BCAA), such as leucine, thereby providing macromolecule precursors; however, the function of BCATs in macrophages is unknown. Here we show that BCAT1 is the predominant BCAT isoform in human primary macrophages. We identify ERG240 as a leucine analogue that blocks BCAT1 activity. Selective inhibition of BCAT1 activity results in decreased oxygen consumption and glycolysis. This decrease is associated with reduced IRG1 levels and itaconate synthesis, suggesting involvement of BCAA catabolism through the IRG1/itaconate axis within the tricarboxylic acid cycle in activated macrophages...
July 12, 2017: Nature Communications
https://www.readbyqxmd.com/read/28692695/cancer-in-korean-patients-with-end-stage-renal-disease-a-7-year-follow-up
#17
Kyung Don Yoo, Jung Pyo Lee, Su Mi Lee, Jae Yoon Park, Hajeong Lee, Dong Ki Kim, Shin-Wook Kang, Chul Woo Yang, Yong-Lim Kim, Chun Soo Lim, Kwon Wook Joo, Yon Su Kim
BACKGROUND: The effectiveness of dialysis on the incidence of cancer in patients with end-stage renal disease (ESRD) remains to be clarified. In this study, we evaluated the incidence rate and type of cancer among patients with ESRD, compared to the general population, through a prospective 7-year follow-up. We also calculated the cumulative incidence rate of cancer associated with ESRD, with stratification to control for the competing risk of death. METHODS: This prospective observational cohort study was conducted using data from a nationwide study on patients with ESRD in Korea...
2017: PloS One
https://www.readbyqxmd.com/read/28690757/minimal-change-disease-in-horseshoe-kidney
#18
Yosr Chaabouni, Rahma Guesmi, Yosr Hentati, Khaoula Kammoun, Mohamed Ben Hmida, Zeineb Mnif, Tahya Boudawara, Jamil Hachicha
The horseshoe kidney is a frequent urological birth defect. The most frequent complications are urinary tract infections, stones and hydronephrosis. The occurrence of glomerular disease in horseshoe kidney is rare. Therefore, we report the first case of minimal change disease occurring in a patient with horseshoe kidney in literature. A 22-year-old Caucasian man without personal or family medical history admitted to the pneumology department for a pulmonary artery embolism. In presence of a generalized oedema, a biological assessment was performed yielding intense nephrotic syndrome with urine protein excretion 22g/day...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28689588/renal-manifestations-of-hepatitis-c-virus
#19
REVIEW
Clodoveo Ferri, Dilia Giuggioli, Michele Colaci
Hepatitis C virus (HCV) is a hepatotropic and lymphotropic virus responsible for hepatic and extrahepatic autoimmune and neoplastic disorders, including renal involvement, which is the consequence of immune-mediated organ damage due to glomerular deposition of immune-complex and/or anti-HCV IgG antibodies and complement. It can appear at any time during the natural history of HCV infection, more often as membranoproliferative glomerulonephritis, alone or in association with other HCV-related disorders. The presence of renal involvement should be investigated in HCV-infected individuals at the first referral and during clinical follow-up...
August 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28685101/end-stage-kidney-failure-in-oman-an-analysis-of-registry-data-with-an-emphasis-on-congenital-and-inherited-renal-diseases
#20
Intisar Al Alawi, Issa Al Salmi, Adhra Al Mawali, Yacoub Al Maimani, John A Sayer
Globally, end-stage kidney disease (ESKD) is a huge burden on health care systems. The aims of this study were to perform a comprehensive epidemiological and etiological report of ESKD patients commencing RRT in Oman with an emphasis on genetic causes and inherited kidney disease. All newly registered Omani patients with ESKD commencing RRT from 2001 until 2015 (n = 2,922) were analysed using the RRT register in Oman. All potentially genetic or inherited causes of ESKD were reviewed. In Oman, ESKD is more prevalent in males (57...
2017: International Journal of Nephrology
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