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https://www.readbyqxmd.com/read/29792392/hemizygous-fabry-disease-associated-with-membranous-nephropathy-a-rare-case-report
#1
Wenyan Zhou, Zhaohui Ni, Minfang Zhang
BACKGROUND: Fabry disease may coexist with various glomerular diseases, including IgA nephropathy, focal segmental glomerulosclerosis, etc. In this study, we report a rare case of Fabry disease associated with membranous nephropathy (MN). CASE PRESENTATION: A 30-year-old man with nephrotic proteinuria, normal renal function, and no other extrarenal manifestations underwent a renal biopsy in February 2017. Light microscopy and immunofluorescence indicated MN (stage 1)...
May 24, 2018: Clinical Nephrology
https://www.readbyqxmd.com/read/29792176/anti-neutrophil-cytoplasmic-antibody-associated-glomerulonephritis-with-detection-of-myeloperoxidase-and-phospholipase-a-2-receptor-in-membranous-nephropathy-lesions-report-of-two-patients-with-microscopic-polyangiitis
#2
Kenta Tominaga, Takahiro Uchida, Toshihiko Imakiire, Kenji Itoh, Hideyuki Shimazaki, Kuniaki Nakanishi, Hiroo Kumagai, Naoki Oshima
BACKGROUND: Podocyte phospholipase A2 receptor (PLA2 R) is a major target antigen in idiopathic adult membranous nephropathy (MN). Histological PLA2 R staining in the renal tissue has proven to be useful for the detection of idiopathic MN. However, glomerular PLA2 R deposits have also been recently observed in several patients with secondary MN, such as hepatitis B virus-associated, hepatitis C virus-associated, and neoplasm-associated MN. Certain inflammatory environments have been suggested to lead to abnormal expression of PLA2 R epitopes, with the resulting production of PLA2 R autoantibodies...
May 23, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29792170/liddle-s-like-syndrome-associated-with-nephrotic-syndrome-secondary-to-membranous-nephropathy-the-first-case-report
#3
Eriko Yamaguchi, Kazuhiro Yoshikawa, Izaya Nakaya, Karen Kato, Yoshikazu Miyasato, Terumasa Nakagawa, Yutaka Kakizoe, Masashi Mukoyama, Jun Soma
BACKGROUND: Liddle's syndrome is a rare monogenic form of hypertension caused by truncating or missense mutations in the C termini of the epithelial sodium channel (ENaC) β or γ subunits. Patients with this syndrome present with early onset of hypertension, hypokalemia, metabolic alkalosis, hyporeninemia and hypoaldosteronism, and a potassium-sparing diuretics (triamterene or amiloride) can drastically improves the disease condition. Although elderly patients having these characteristics were considered to have Liddle's syndrome or Liddle's-like syndrome, no previous report has indicated that Liddle's-like syndrome could be caused by nephrotic syndrome of primary glomerular disease, which is characterized by urinary excretion of > 3 g of protein/day plus edema and hypoalbuminemia, or has explained how the activity function of ENaC could be affected in the setting of high proteinuria...
May 23, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29792169/use-of-synthetic-adrenocorticotropic-hormone-in-patients-with-iga-nephropathy
#4
Bhanu Prasad, Shelley Giebel, Michelle C E McCarron, Nelson Leung
BACKGROUND: Synthetic adrenocorticotropic hormone (ACTH) has been demonstrated to be effective in patients with membranous nephropathy, minimal change disease and some histological subtypes of focal segmental glomerulosclerosis. Its clinical impact in patients with IgA nephropathy is currently unclear. CASE PRESENTATION: In this report, we describe the clinical use of ACTH in patients with IgA nephropathy. Three female patients (24-44 years) with overt proteinuria received intramuscular (IM) ACTH for varying time periods (8-14 months)...
May 23, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29791050/a-novel-clcn5-pathogenic-mutation-supports-dent-disease-with-normal-endosomal-acidification
#5
Yohan Bignon, Alexi Alekov, Nadia Frachon, Olivier Lahuna, Carine Jean-Baptiste Doh-Egueli, Georges Deschênes, Rosa Vargas-Poussou, Stéphane Lourdel
Dent disease in an X-linked recessive renal tubular disorder characterized by low-molecular-weight proteinuria, hypercalciuria, nephrolithiasis, nephrocalcinosis and progressive renal failure. Inactivating mutations of CLCN5, the gene encoding the 2Cl- /H+ exchanger ClC-5 have been reported in patients with Dent disease 1. In vivo studies in mice harboring an artificial mutation in the "gating glutamate" of ClC-5 (c.632A > C, p.Glu211Ala) and mathematical modeling suggest that endosomal chloride concentration could be an important parameter in endocytosis, rather than acidification as earlier hypothesized...
May 23, 2018: Human Mutation
https://www.readbyqxmd.com/read/29790391/neutrophil-exocytosis-induces-podocyte-cytoskeletal-reorganization-and-proteinuria-in-experimental-glomerulonephritis
#6
Dawn J Caster, Erik A Korte, Min Tan, Michelle T Barati, Shweta Tandon, T Michael Creed, David J Salant, Jessica L Hata, Paul N Epstein, Hui Huang, David W Powell, Kenneth R McLeish
Acute glomerulonephritis is characterized by rapid glomerular neutrophil recruitment, proteinuria, and glomerular hypercellularity. The current study tested the hypothesis that release of neutrophil granule contents plays a role in both loss of filtration barrier leading to proteinuria and the increase in glomerular cells. Inhibition of neutrophil exocytosis with a peptide inhibitor prevented proteinuria and attenuated podocyte and endothelial cell injury, but had no effect on glomerular hypercellularity in an experimental acute glomerulonephritis model in mice...
May 23, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29789969/identification-of-risk-factors-for-toxicity-in-patients-with-hormone-receptor-positive-advanced-breast-cancer-treated-with-bevacizumab-plus-letrozole-a-calgb-40503-alliance-correlative-study
#7
Daneng Li, Linda M McCall, Olwen M Hahn, Clifford A Hudis, Harvey J Cohen, Hyman B Muss, Aminah Jatoi, Jacqueline M Lafky, Karla V Ballman, Eric P Winer, Debu Tripathy, Bryan Schneider, William Barry, Maura N Dickler, Arti Hurria
BACKGROUND: In hormone receptor-positive advanced breast cancer, a progression-free survival benefit was reported with addition of bevacizumab to first-line letrozole. However, increased toxicity was observed. We hypothesized that functional age measures could be used to identify patients at risk for toxicity while receiving letrozole plus bevacizumab for hormone receptor-positive advanced breast cancer. METHODS: CALGB 40503 was a phase III trial that enrolled patients with hormone receptor-positive advanced breast cancer randomized to letrozole with or without bevacizumab...
May 22, 2018: Breast Cancer Research and Treatment
https://www.readbyqxmd.com/read/29789431/clinical-and-pathology-findings-associate-consistently-with-larger-glomerular-volume
#8
Aleksandar Denic, Jerry Mathew, Venkata V Nagineni, R Houston Thompson, Bradley C Leibovich, Lilach O Lerman, John C Lieske, Mariam P Alexander, Joshua J Augustine, Walter K Kremers, Andrew D Rule
Background Glomerular volume increases when demand exceeds nephron supply, which may lead to glomerulosclerosis. It is unclear if determinants of glomerular volume are consistent between populations that differ by severity of comorbidities. Methods We studied kidney biopsy specimens from living kidney donors ( n =2453) and patients who underwent radical nephrectomy for a renal tumor ( n =780). We scanned specimen sections into high-resolution digital images, manually traced glomerular profiles, and calculated mean glomerular volumes using the Weibel-Gomez stereologic formula (separately for nonsclerosed glomeruli and globally sclerosed glomeruli)...
May 22, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29789348/gestational-low-protein-intake-enhances-whole-kidney-mir-192-and-mir-200-family-expression-and-epithelial-to-mesenchymal-transition-in-rat-adult-male-offspring
#9
Letícia B Sene, Victor Hugo Gonçalves Rizzi, José A R Gontijo, Patricia A Boer
Studies have shown that adult offspring of mothers fed a protein-restricted diet during pregnancy present a pronounced reduction of nephron number associated with decreased fractional urinary sodium excretion and arterial hypertension. Additionally, recent advances in our understanding of the molecular pathways that govern the association of gestational nutritional restriction, intrauterine growth retardation and inflammation with impaired nephrogenesis, nephron underdosing and kidney fibrosis point to the epithelial to mesenchymal transition (EMT) as a common factor...
May 22, 2018: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29786187/-steroid-resistant-focal-segmental-glomerulosclerosis-treated-with-cascade-plasmafiltration-and-rituximab
#10
Paola Tatangelo, Francesco Londrino, Giorgio Di Vasta, Giuliana Guido, Alessia Centi, Sara Dominijanni, Eleonora Bernabei, Maria Stella Caramiello, Damiano Di Franco, Roberto Palumbo
A 39-year man with primary steroid resistant focal segmental glomerulosclerosis (FSGS) was treated with mycophenolate mofetil and ACE-inhibitors. After six months a different therapeutics approach was mandatory due to the worsening of renal function and the relapse of proteinuria at the nephrotic range. The combination of cascade plasmafiltration and single dose of rituximab (375 mg/m²) achieved clinical remission and improved renal function in six months follow up. Cascade plasmafiltration in association with rituximab can be considered as a salvage method for primary steroid-resistant FSGS...
May 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29786183/-hyperkalemia-as-a-limiting-factor-in-the-use-of-drugs-that-block-the-renin-angiotensin-aldosterone-system-raas
#11
Antonio Santoro, Marcora Mandreoli
Angiotensin-converting enzyme (ACE-I) inhibitors and ARBs have shown real efficacy in reducing blood pressure, proteinuria, in slowing the progression of chronic kidney disease (MRC) and in clinical improvement. in patients with heart failure, diabetes mellitus and ischemic heart disease. However, their use is limited by some side effects such as the increase in serum potassium (K), which can be particularly severe in patients with renal insufficiency. In the 23,000 patients followed by the PIRP project of the Emilia-Romagna Region, hyperkalaemia at the first visit (K> 5...
May 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29786155/is-home-blood-pressure-monitoring-in-hypertensive-disorders-of-pregnancy-consistent-with-clinic-recordings
#12
Erkan Kalafat, Iffat Mir, Helen Perry, Basky Thilaganathan, Asma Khalil
OBJECTIVES: To assess the agreement of home blood-pressure monitoring (HBPM) and office blood pressure measurements in a cohort of pregnant women with Hypertensive Disorders of Pregnancy (HDP). METHODS: This was a cohort study at St George's Hospital, University of London conducted between years 2016 and 2017. The inclusion criteria were chronic hypertension, gestational hypertension or high risk of developing preeclampsia, no significant proteinuria and no hematological or biochemical abnormalities...
May 22, 2018: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29786038/a-predictive-model-for-estimation-risk-of-proliferative-lupus-nephritis
#13
Dong-Ni Chen, Li Fan, Yu-Xi Wu, Qian Zhou, Wei Chen, Xue-Qing Yu
Background: Lupus nephritis (LN) is classified by renal biopsy into proliferative and nonproliferative forms, with distinct prognoses, but renal biopsy is not available for every LN patient. The present study aimed to establish an alternate tool by building a predictive model to evaluate the probability of proliferative LN. Methods: In this retrospective cohort with biopsy-proven LN, 382 patients in development cohort, 193 in internal validation cohort, and 164 newly diagnosed patients in external validation cohort were selected...
June 5, 2018: Chinese Medical Journal
https://www.readbyqxmd.com/read/29785115/management-and-outcomes-of-pregnancy-with-or-without-lupus-nephritis-a-systematic-review-and-meta-analysis
#14
REVIEW
Jiayue Wu, Jinghang Ma, Wei-Hong Zhang, Wen Di
Background: Although it is well established that systemic lupus erythematosus (SLE) negatively affects pregnancy outcomes, there is insufficient evidence on the effect of lupus nephritis (LN) on antenatal management and pregnancy outcomes. We performed a systematic review and meta-analysis to determine the association of LN with management and pregnancy outcomes in SLE patients. Methods: Embase, Medline, Cochrane, and ClinicalTrials.gov were carefully searched for relevant English and Chinese language studies...
2018: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/29784613/cryoglobulinemic-glomerulonephritis-associated-with-nodal-and-renal-infiltration-by-t-cell-lymphoma-of-t-follicular-helper-phenotype-a-case-report
#15
Jennifer Li, Marille Umakanathan, Chow Heok P'ng, Winny Varikatt, Fiona Kwok, Ming-Wei Lin, Mirna Vucak-Dzumhur
We present a unique case of cryoglobulinemic glomerulonephritis associated with nodal and renal infiltration by T-cell lymphoma of T-follicular helper phenotype. The patient presented with transient neurologic symptoms, severe nephritic syndrome with nephrotic-range proteinuria, and acute kidney injury. He had elevated double-stranded DNA levels, low complement levels, detectable cryoglobulin, and detectable immunoglobulin M (IgM) paraprotein. The kidney biopsy showed cryoglobulinemic glomerulonephritis with a membranoproliferative pattern and diffuse interstitial infiltrates on light microscopy; IgM, C3 but weak IgG, C1q, and negative C4d staining on immunofluorescence; and deposits with organized substructures on electron microscopy...
May 18, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29784612/risk-of-progression-of-nonalbuminuric-ckd-to-end-stage-kidney-disease-in-people-with-diabetes-the-cric-chronic-renal-insufficiency-cohort-study
#16
Digsu N Koye, Dianna J Magliano, Christopher M Reid, Christopher Jepson, Harold I Feldman, William H Herman, Jonathan E Shaw
BACKGROUND: Reduced glomerular filtration rate (GFR) in the absence of albuminuria is a common manifestation of chronic kidney disease (CKD) in diabetes. However, the frequency with which it progresses to end-stage kidney disease (ESKD) is unknown. STUDY DESIGN: Multicenter prospective cohort study. SETTING & PARTICIPANTS: We included 1,908 participants with diabetes and reduced GFR enrolled in the Chronic Renal Insufficiency Cohort (CRIC) Study in the United States...
May 18, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29783931/short-term-sequelae-of-preeclampsia-a-single-center-cohort-study
#17
Michael Girsberger, Catherine Muff, Irene Hösli, Michael Jan Dickenmann
BACKGROUND: Data on the prevalence of persistent symptoms in the first year after preeclampsia are limited. Furthermore, possible risk factors for these sequelae are poorly defined. We investigated kidney function, blood pressure, proteinuria and urine sediment in women with preeclampsia 6 months after delivery with secondary analysis for possible associated clinical characteristics. METHODS: From January 2007 to July 2014 all women with preeclampsia and 6-months follow up at the University Hospital Basel were analyzed...
May 21, 2018: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/29782856/sirt1-activation-prevents-anti-thy-1-1-mesangial-proliferative-glomerulonephritis-in-the-rat-through-the-nrf2-are-pathway
#18
Kaipeng Huang, Ruiming Li, Wentao Wei
Mesangial proliferative glomerulonephritis (MsPGN) is characterized by glomerular mesangial cells proliferation and extracellular matrix deposition in mesangial area, which develop into glomerulosclerosis. Both silent information regulator 2-related protein 1 (Sirt1) and nuclear factor erythroid 2-related factor 2/ anti-oxidant response element (Nrf2/ARE) pathway had remarkable renoprotective effects. However, whether Sirt1 and Nrf2/ARE pathway can regulate the pathological process of MsPGN remains unknown...
May 18, 2018: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29782634/long-term-follow-up-of-biomarkers-of-podocyte-damage-and-renal-function-in-patients-with-and-without-preeclampsia
#19
Ylbe Palacios de Franco, Karina Velazquez, Natalia Segovia, Gladys Sandoval, Estefania Gauto, Ylbe V Franco Palacios, Carlos R Franco Palacios
INTRODUCTION: preeclampsia can be associated with future renal disease. OBJECTIVES: To measure changes in renal function overtime in patients with preeclampsia. METHODS: urine and serum samples from eleven patients with preeclampsia and eight patients with a normal pregnancy were obtained during pregnancy, postpartum, and 3 years after delivery. Urine podocalyxin, protein, and serum creatinine were measured. RESULTS: after 3 years, there were no significant differences in urinary podocalyxin in patients with or without preeclampsia: 4...
May 17, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29782633/macrothrombocytopenia-renal-dysfunction-and-nephrotic-syndrome-in-a-young-male-patient-a-case-report-of-myh9-related-disease
#20
Gabriela Sevignani, Giovana Memari Pavanelli, Sibele Sauzem Milano, Bianca Ramos Ferronato, Maria Aparecida Pachaly, Hae Ii Cheong, Mauricio de Carvalho, Fellype Carvalho Barreto
MYH9-related disease is an autosomal dominant disorder caused by mutations of the MYH9 gene, which encodes the non-muscle myosin heavy chain IIA on chromosome 22q12. It is characterized by congenital macrothrombocytopenia, bleeding tendency, hearing loss, and cataracts. Nephropathy occurs in approximately 30% of MYH9-related disease in a male patient carrier of a de novo missense mutation in exon 1 of the MYH9 gene [c.287C > T; p.Ser(TCG)96(TTG)Leu]. He presented all phenotypic manifestations of the disease, but cataracts...
May 17, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
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