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https://www.readbyqxmd.com/read/28093526/cathepsin-k-deficiency-ameliorates-systemic-lupus-erythematosus-like-manifestations-in-faslpr-mice
#1
Yi Zhou, Huimei Chen, Li Liu, Xueqing Yu, Galina K Sukhova, Min Yang, Vasileios C Kyttaris, Isaac E Stillman, Bruce Gelb, Peter Libby, George C Tsokos, Guo-Ping Shi
Cysteinyl cathepsin K (CatK) is expressed in osteoclasts to mediate bone resorption, but is also inducible under inflammatory conditions. Fas(lpr) mice on a C57BL/6 background develop spontaneous systemic lupus erythematosus-like manifestations. Although normal mouse kidneys expressed negligible CatK, those from Fas(lpr) mice showed elevated CatK expression in the glomeruli and tubulointerstitial space. Fas(lpr) mice also showed elevated serum CatK levels. CatK deficiency in Fas(lpr) mice reduced all tested kidney pathologies, including glomerulus and tubulointerstitial scores, glomerulus complement C3 and IgG deposition, chemokine expression and macrophage infiltration, and serum autoantibodies...
January 16, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#2
Emmanuel Estève, Pauline Krug, Aurélie Hummel, Jean-Baptiste Arnoux, Olivia Boyer, Anais Brassier, Pascale de Lonlay, Vincent Vuiblet, Stéphanie Gobin, Rémi Salomon, Christine Piètrement, Jean-Paul Bonnefont, Aude Servais, Louise Galmiche
Lysinuric protein intolerance (LPI) is a rare autosomal recessive disease caused by mutations in the SLC7A7 gene encoding the light subunit of a cationic amino acid transporter. Symptoms mimic primary urea cycle defects but dysimmune symptoms are also described. Renal involvement in LPI was first described in the 1980's. In 2007, it appeared that it could concern as much as 75% of LPI patients and could lead to end stage renal disease. The most common feature is proximal tubular dysfunction and nephrocalcinosis but glomerular lesions are also reported...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28064541/the-role-of-ifi35-in-lupus-nephritis-and-related-mechanisms
#3
Lihua Zhang, Honglin Zhu, Yisha Li, Xiaodan Dai, Bin Zhou, Quanzhen Li, Xiaoxia Zuo, Hui Luo
OBJECTIVES: It's reported that multiple genes in the IFN-γ/STAT1 pathway were hypomethylated and associated with the pathogenesis of lupus nephritis (LN). Our previous study using microarray analysis suggested that interferon induced 35-kDa protein (IFI35) was hypomethylated and increased in LN. However, the role of IFI35 in LN and related mechanism remains to be elucidate. METHODS: The expressions of IFNγR, STAT1, IFI35 and MBD2 in the human kidneys tissues was detected by real-time PCR and western blot...
January 9, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28063327/serum-levels-of-fibroblast-growth-factor-23-are-elevated-in-patients-with-active-lupus-nephritis
#4
Aline L Resende, Rosilene M Elias, Myles Wolf, Luciene M Dos Reis, Fabiana G Graciolli, Geuza D Santos, Cristiane B Dias, Vanda Jorgetti, Viktoria Woronik, Rosa M A Moysés
BACKGROUND: Fibroblast growth factor 23 (FGF23), a phosphate-regulating hormone is an established cardiovascular risk factor. Recently, FGF23 has been related to inflammation. Lupus is an inflammatory disease, and whether FGF23 is associated with Lupus nephritis (LN) activity is unknown. MATERIALS AND METHODS: We studied 15 pre-menopausal patients with recent LN diagnose (⩽2months) and compared them to 1:1 age-matched healthy control group. We measured serum levels of intact FGF23, interleukin-6 (IL-6), tumor necrosis factor α (TNFα), and urinary levels of monocyte chemotactic protein (MCP1)...
January 4, 2017: Cytokine
https://www.readbyqxmd.com/read/28063160/elevated-semaphorin5a-in-systemic-lupus-erythematosus-is-in-association-with-disease-activity-and-lupus-nephritis
#5
Yan Du, Xinyu Wu, Mo Chen, Wenwen Wang, Weihong Xv, Lv Ye, Di Wu, Jing Xue, Wenjia Sun, Judong Luo, Huaxiang Wu
OBJECTIVE: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by extensive immune response including over-activation of T and B cells development of pathogenic autoantibodies, organ damage induced by the formation and deposition of immune complex, and the abnormal elevation of type I interferon. Semaphorin5A (Sema5A), is essentially involved in immune cell regulation and also implicated in the pathogenesis of autoimmune disorders. We aimed to evaluate the role of Sema5A in patients with SLE...
January 7, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28060446/successful-treatment-of-donor-derived-hepatitis-c-viral-infection-in-three-transplant-recipients-from-a-donor-at-increased-risk-for-bloodborne-pathogens
#6
Ashesh P Shah, Andrew Cameron, Pooja Singh, Adam M Frank, Jonathan M Fenkel
We report here the successful treatment of hepatitis C virus (HCV) transmitted from a nucleic acid testing (NAT)-negative donor to 3 HCV-negative recipients - 2 renal transplants and 1 liver. Both renal recipients underwent standard deceased-donor renal transplantation with immediate graft function. The liver recipient underwent standard orthotopic liver transplantation and recovered uneventfully. The donor was a 39-year-old woman with a terminal serum creatinine of 0.7 mg/dL. She was high risk for bloodborne pathogens, based upon a history of sexual contact with an HCV-infected male partner...
January 6, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28059018/outcome-in-biopsy-proven-lupus-nephritis-evaluation-of-biopsies-from-the-norwegian-kidney-biopsy-registry
#7
G E Norby, G Mjøen, R Bjørneklett, B E Vikse, H Holdaas, E Svarstad, K Aasarød
Objective To evaluate long-term mortality and end-stage renal disease (ESRD) in a cohort of Norwegian patients with biopsy-proven lupus nephritis (LN). Methods Renal biopsies were obtained from 178 patients with LN from 1988 until 2007. Mortality rate and death causes were provided by Statistics Norway and ESRD data were provided by the Norwegian Renal Registry. Risk factors for all-cause mortality were evaluated by Cox regression. Standardized mortality ratio (SMR) was compared to observed deaths in a matched general population sample...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28057010/update-on-lysinuric-protein-intolerance-a-multi-faceted-disease-retrospective-cohort-analysis-from-birth-to-adulthood
#8
Wladimir Mauhin, Florence Habarou, Stéphanie Gobin, Aude Servais, Anaïs Brassier, Coraline Grisel, Célina Roda, Graziella Pinto, Despina Moshous, Fahd Ghalim, Pauline Krug, Nelly Deltour, Clément Pontoizeau, Sandrine Dubois, Murielle Assoun, Louise Galmiche, Jean-Paul Bonnefont, Chris Ottolenghi, Jacques de Blic, Jean-Baptiste Arnoux, Pascale de Lonlay
BACKGROUND: Lysinuric protein intolerance (LPI) is a rare metabolic disease resulting from recessive-inherited mutations in the SLC7A7 gene encoding the cationic amino-acids transporter subunit y(+)LAT1. The disease is characterised by protein-rich food intolerance with secondary urea cycle disorder, but symptoms are heterogeneous ranging from infiltrative lung disease, kidney failure to auto-immune complications. This retrospective study of all cases treated at Necker Hospital (Paris, France) since 1977 describes LPI in both children and adults in order to improve therapeutic management...
January 5, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28056333/-recommendation-for-the-prevention-and-treatment-of-non-steroidal-anti-inflammatory-drug-induced-gastrointestinal-ulcers-and-its-complications
#9
(no author information available yet)
Non-steroidal anti-inflammatory drugs (NSAIDs) are a broad class of non glucocorticoid drugs which are extensively used in anti-inflammatory, analgesic, and antipyretic therapies. However, NSAIDs may cause many side effects, most commonly in gastrointestinal(GI) tract. Cardiovascular system, kidney, liver, central nervous system and hematopoietic system are also involved. NSAID-induced GI side effects not only endanger the patients' health, increase mortality, but also greatly increase the cost of medical care...
January 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27999645/serum-alpha-actinin-antibody-status-in-systemic-lupus-erythematosus-and-its-potential-in-the-diagnosis-of-lupus-nephritis
#10
Mansour Babaei, Zahra Rezaieyazdi, Nayereh Saadati, Massoud Saghafi, Maryam Sahebari, Bahram Naghibzadeh, Habibollah Esmaily
BACKGROUND: In lupus nephritis (LN), deposition of pathogenic autoantibodies in the glomeruli is mediated via cross-reactivity with alpha-actinin. Association of serum alpha-actinin antibody (AαA) with LN has been shown in a few studies but the results are controversial. METHODS: Eighty patients into entered the study. The diagnosis of SLE was confirmed according to the American College of Rheumatology criteria and LN was diagnosed by proteinuria ≥ 500 mg/24 hour and kidney biopsy...
2016: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/27994909/assessment-of-ten-year-long-results-of-kidney-biopsies-performed-on-children-in-the-thrace-region-of-turkey
#11
Neşe Özkayın, Gökçe Çıplak, Ufuk Usta, Hakan Gençhellaç, Osman Temizöz
BACKGROUND: Many children with kidney diseases can be diagnosed and treated without a biopsy. However, biopsy is a valuable method for the diagnostic and prognostic evaluation of children with kidney diseases. AIMS: To evaluate the clinical and pathological profiles of the kidney biopsies in our department to provide epidemiological data for clinical practice. STUDY DESIGN: Retrospective cross-sectional study. METHODS: Kidney biopsies and patient's charts in pediatric patients performed between May 2005 and February 2015 at the Pediatric Nephrology Department, Trakya University School of Medicine were assessed retrospectively...
November 2016: Balkan Medical Journal
https://www.readbyqxmd.com/read/27973968/serum-soluble-tumour-necrosis-factor-receptor-2-stnfr2-as-a-biomarker-of-kidney-tissue-damage-and-long-term-renal-outcome-in-lupus-nephritis
#12
I Parodis, H Ding, A Zickert, L Arnaud, A Larsson, E Svenungsson, C Mohan, I Gunnarsson
OBJECTIVES: We investigated the performance of soluble tumour necrosis factor receptor-2 (sTNFR2) as a biomarker of renal activity, damage, treatment response, and long-term outcome in lupus nephritis (LN). METHOD: Serum sTNFR2 levels were assessed in 64 LN patients (52 proliferative, 12 membranous) before and after induction treatment, and in 314 non-lupus controls. In LN patients, renal biopsies were performed at baseline and post-treatment. Patients with ≥ 50% reduced proteinuria, normal or improved estimated glomerular filtration rate (eGFR) by ≥ 25%, and inactive urinary sediment were considered clinical responders (CRs)...
December 15, 2016: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/27958636/intrauterine-growth-restriction-in-pregnant-women-after-kidney-transplantation-as-a-marker-of-preeclampsia
#13
Anna Cyganek, Filip Andrzej Dabrowski, Bronislawa Pietrzak, Zoulikha Jabiry-Zieniewicz, Barbara Grzechocinska, Anna Madej, Miroslaw Wielgos
OBJECTIVES: Delayed motherhood is associated with an increasing number of comorbidities such as glomerulonephritis, systemic lupus erythematosus, and diabetic nephropathy. Women after renal transplant belong to the group of patients who require a highly individualized approach to treatment and diagnosis. The aim of the study was to validate the commonly used diagnostic criteria for preeclampsia which seem to be irrelevant in patients with chronic renal insufficiency. MATERIAL AND METHODS: The course of pregnancy and delivery were retrospectively analyzed in 48 renal transplant patients...
2016: Ginekologia Polska
https://www.readbyqxmd.com/read/27927880/serum-osteoprotegrin-opg-in-subclinical-atherosclerosis-in-systemic-lupus-erythematosus
#14
A N Kiani, P Aukrust, T Ueland, I Hollan, E Barr, L S Magder, M Petri
INTRODUCTION: Osteoprotegerin (OPG) is a member of the tumor necrosis factor (TNF) receptor family. It has recently been demonstrated that OPG is produced by a variety of tissues, including the cardiovascular system (heart, arteries, veins), lung, kidney, immune tissues, and bone. The OPG-RANKL signaling pathway is strongly related to vascular calcification. We determined the association of this biomarker with subclinical atherosclerosis in systemic lupus erythematous (SLE). METHODS: We measured OPG and markers of subclinical atherosclerosis (coronary artery calcium (CAC), carotid intima-media thickness (cIMT) carotid plaque) in 166 SLE patients (91% female, 64% Caucasian, 31% African American, 5% others, mean age 45 years)...
December 6, 2016: Lupus
https://www.readbyqxmd.com/read/27927780/genomic-analysis-of-kidney-allograft-injury-identifies-hematopoietic-cell-kinase-as-a-key-driver-of-renal-fibrosis
#15
Chengguo Wei, Li Li, Madhav C Menon, Weijia Zhang, Jia Fu, Brian Kidd, Karen L Keung, Christopher Woytovich, Ilana Greene, Wenzhen Xiao, Fadi Salem, Zhengzi Yi, John Cijiang He, Joel T Dudley, Barbara Murphy
Renal fibrosis is the common pathway of progression for patients with CKD and chronic renal allograft injury (CAI), but the underlying mechanisms remain obscure. We performed a meta-analysis in human kidney biopsy specimens with CAI, incorporating data available publicly and from our Genomics of Chronic Renal Allograft Rejection study. We identified an Src family tyrosine kinase, hematopoietic cell kinase (Hck), as upregulated in allografts in CAI. Querying the Kinase Inhibitor Resource database revealed that dasatinib, a Food and Drug Administration-approved drug, potently binds Hck with high selectivity...
December 7, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27923701/prognostic-significance-of-repeat-biopsy-in-lupus-nephritis-histopathologic-worsening-and-a-short-time-between-biopsies-is-associated-with-significantly-increased-risk-for-end-stage-renal-disease-and-death
#16
Cristina Arriens, Sixia Chen, David R Karp, Ramesh Saxena, Kamalanathan Sambandam, Eliza Chakravarty, Judith A James, Joan T Merrill
BACKGROUND/PURPOSE: Approximately half of patients with systemic lupus erythematosus (SLE) develop lupus nephritis (LN), a major cause of morbidity and early mortality in that disease. Prolonged renal inflammation is associated with irreversible kidney damage which confers a 30% risk of end stage renal disease (ESRD), making early, aggressive treatment mandatory. Failure to achieve therapeutic response or recurrence of renal flare often prompts repeat biopsy. However, the role of repeat biopsy in determining long-term renal prognosis remains controversial...
December 3, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27921164/m-type-phospholipase-a2-receptor-pla2r-glomerular-staining-in-pediatric-idiopathic-membranous-nephropathy
#17
Shoichiro Kanda, Shigeru Horita, Takeshi Yanagihara, Akira Shimizu, Motoshi Hattori
BACKGROUND: Identifying M-type phospholipase A2 receptor (PLA2R) is a landmark breakthrough for understanding adult idiopathic membranous nephropathy (iMN). However, potential roles for PLA2R in pediatric iMN have not been well investigated. METHODS: A total of 34 pediatric iMN patients who underwent kidney biopsy between 1972 and 2015 were enrolled in this study. The study cohort consisted of 15 children aged from 3 to 9 years and 19 aged from 10 to 15 years...
December 5, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27914701/il-2-anti-il-2-complexes-ameliorate-lupus-nephritis-by-expansion-of-cd4-cd25-foxp3-regulatory-t%C3%A2-cells
#18
Ji-Jing Yan, Jae-Ghi Lee, Joon Young Jang, Tai Yeon Koo, Curie Ahn, Jaeseok Yang
Adoptive transfer of regulatory T cells (Tregs) can delay disease progression and reduce mortality in lupus-prone mice. Here, we tested whether complex (IL-2C) consisting of IL-2 and anti-IL-2 monoclonal antibody (JES6-1) ameliorates lupus nephritis by expanding Tregs as an alternative to problematic Treg infusion therapy. IL-2C treatment of NZB/W F1 mice induced an effective and sustained expansion of CD4(+)CD25(+)Foxp3(+) Tregs in both the kidneys and spleen along with decreased renal infiltration of T cells, B cells, and innate immune cells...
November 30, 2016: Kidney International
https://www.readbyqxmd.com/read/27913748/a-3-year-follow-up-of-a-patient-with-acute-renal-failure-caused-by-thrombotic-microangiopathy-related-to-antiphospholipid-syndrome-case-report
#19
X-J Zhou, M Chen, S-X Wang, F-D Zhou, M-H Zhao
BACKGROUND: Microvascular manifestations of antiphospholipid antibody syndrome in the kidneys include acute renal failure, thrombotic microangiopathy and hypertension. Therapy has been largely empiric. CASE REPORT: A 49-year-old Chinese man presented with anuric acute renal failure without abundant proteinuria and heavy haematuria, but markedly low levels of urinary sodium, potassium and chlorine upon admission. On day 1 of hospitalization, his thrombocytopenia, anaemia and renal failure showed rapid progression...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27900972/failure-to-thrive-and-nephrocalcinosis-due-to-distal-renal-tubular-acidosis-a-rare-presentation-of-pediatric-lupus-nephritis
#20
Madhumita Nandi, Mrinal Kanti Das, Sukanta Nandi
A 9-year-old female child was initially diagnosed of having nephrocalcinosis with distal renal tubular acidosis (dRTA) while investigating for short stature. She later on developed features of nephrotic syndrome (NS) while on treatment for RTA. Investigation for the cause of NS revealed very strong serological evidence in favor of systemic lupus erythematosus (SLE). Histopathological confirmation could not be done due to bilateral severely contracted kidneys. There are a few case reports of dRTA as the presentation of SLE, but nephrocalcinosis with dRTA with subsequent manifestation of SLE has hitherto not been reported in literature...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
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