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https://www.readbyqxmd.com/read/29775446/-chronic-kidney-disease-in-children-up-to-2-years-of-age-a-single-center-data-analysis
#1
Kinga Musiał, Marta Sałamacha, Sylwia Gralec, Anna Pers, Łukasz Jacheć, Danuta Zwolińska
Although anomalies in the urinary tract are the leading cause of chronic kidney disease (CKD) in the pediatric population, there are few studies focusing on etiological discrepancies between younger and older children. AIM: The aim of the study was to perform a comparative analysis of etiology of CKD in children hospitalized in the Department of Pediatric Nephrology at the Wrocław Medical University, with reference to the patients' age and gender. MATERIALS AND METHODS: The retrospective analysis considered medical records of 174 patients aged 0-18 years, diagnosed with CKD, hospitalized in our Department in the years 2011-2017...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29775445/the-clinical-pattern-of-nephrotic-syndrome-in-children-has-no-effect-on-the-concentration-of-soluble-urokinase-receptor-supar-in-serum-and-urine
#2
Agnieszka Ochocińska, Wioletta Jarmużek, Roman Janas
Concentration of soluble urokinase receptor (suPAR) was regarded as viable marker to differentiate the focal segmental glomerulosclerosis (FSGS) from other glomerulopathies and also as predictive parameter for progression of renal disease. AIM: The aim of this study was to evaluate serum and urine (s)(u)suPAR concentration in steroid-sensitive and steroid-resistant nephrotic children treated with different (double and triple-drug) regimens. MATERIALS AND METHODS: Overall 43 children were evaluated including 14 patients with steroid-resistant nephrotic syndrome (SRNS) aged 9±6 years and 29 with steroid-sensitive nephrotic syndrome (SSNS) aged 9±5 years, as well as control group (n=59)...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29766469/proteinuric-glomerulopathy-in-an-adolescent-with-a-distal-partial-trisomy-chromosome-1
#3
Takaya Sasaki, Masahiro Okabe, Takeshi Tosaki, Yu Honda, Masahiro Ishikawa, Nobuo Tsuboi, Takashi Yokoo
We report a case of distal partial trisomy 1 from q32.1 to 41 that have exhibited proteinuric glomerulopathy. The patient was a 17-year-old adolescent with clinical features of low birth weight, mild mental retardation and mild deafness, from the birth. He exhibited non-nephrotic range proteinuria with the mild obesity since the age of sixteen. Image studies did not reveal morphological abnormalities of the kidneys. Renal biopsy findings showed no definitive evidence of primary glomerular diseases, and were characterized by a very low glomerular density, glomerulomegaly and focal effacement of podocyte foot processes...
May 16, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29750317/mycophenolate-mofetil-for-sustained-remission-in-nephrotic-syndrome
#4
Uwe Querfeld, Lutz T Weber
The clinical application of mycophenolate mofetil (MMF) has significantly widened beyond the prophylaxis of acute and chronic rejections in solid organ transplantation. MMF has been recognized as an excellent treatment option in many immunologic glomerulopathies. For children with frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS) experiencing steroid toxicity, MMF has been recommended as a steroid-sparing drug. Uncontrolled studies in patients with FRNS and SDSN have shown that many patients can achieve sustained remission of proteinuria with MMF monotherapy...
May 11, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29731056/clinical-significance-of-macrophage-polarization-in-antibody-mediated-rejection-of-renal-allograft
#5
J Kim, S-E Choi, B J Lim, Y S Kim, K H Huh, J Lee, S I Kim, M S Kim, H J Jeong
BACKGROUND: The significance of proinflammatory M1 (classically activated) and profibrotic M2 (alternatively activated) macrophages in antibody-mediated rejection (ABMR) after kidney transplantation has not been investigated. METHODS: Fifty-five biopsy-confirmed ABMR samples were stained with MRP 8/14 (a marker of M1 macrophages) and CD163 (a marker of M2 macrophages), and positive cells were counted in glomeruli and the tubulointerstitium, respectively. Patients were classified into M1 and M2 polarization groups according to the glomerular and tubulointerstitial M1:M2 ratio, and the results were compared with Banff scores, serum creatinine level, estimated glomerular filtration rate (eGFR), and graft survival...
May 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29729982/c3-glomerulopathy-associated-with-monoclonal-ig-is-a-distinct-subtype
#6
Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, Sanjeev Sethi
Monoclonal immunoglobulins (MIg) may play a causal role in C3 glomerulopathy (C3G) by impairing regulation of the alternative pathway of complement. Ninety-five patients with C3G were tested for MIg of which 36 were positive. Their mean age at diagnosis was 60 years and among patient 50 years and older, 65.1% had a MIg. At presentation, median serum creatinine and proteinuria were 1.9 mg/dL and 3.0 g/24 hours. Hematuria was present in 32 (88.9%) patients. Twelve (34.3%) patients had low C3 levels. C3 nephritic factor was detected in 45...
May 2, 2018: Kidney International
https://www.readbyqxmd.com/read/29728434/focal-segmental-glomerulosclerosis-in-a-patient-with-prefibrotic-primary-myelofibrosis
#7
Gopal Krishana Bohra, Durga Shankar Meena, Nitin Bajpai, Abhishek Purohit
We report a case of 56-year-old man presented to us with chief complaints of frothy urine and leg swelling. A urinalysis revealed nephrotic-range proteinuria. Haematological investigations revealed thrombocytosis, leucocytosis and peripheral blood smear showed a leucoerythroblastic picture. JAK 2 mutation was positive. To confirm the diagnosis of myeloproliferative neoplasm, bone marrow biopsy was done, which was suggestive of primary myelofibrosis. The patient underwent kidney biopsy due to rapidly declining renal function and persistent proteinuria, which was suggestive of focal segmental glomerulosclerosis...
May 4, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29725655/iga-dominant-glomerulopathy-and-thrombotic-microangiopathy-after-chemotherapy
#8
Zeljko Dvanajscak, Bethany E Karl, Amber P Sanchez, Vighnesh Walavalkar
No abstract text is available yet for this article.
March 2018: KI Reports
https://www.readbyqxmd.com/read/29724182/favorable-effect-of-bortezomib-in-dense-deposit-disease-associated-with-monoclonal-gammopathy-a-case-report
#9
Shuma Hirashio, Ayaka Satoh, Takahiro Arima, Kouichi Mandai, Tadasuke Awaya, Kumi Oshima, Shigeo Hara, Takao Masaki
BACKGROUND: Complement component 3 (C3) glomerulopathy, which includes dense deposit disease (DDD) and C3 glomerulonephritis, is caused by dysregulation of the alternative complement pathway. In most cases, C3 glomerulopathy manifests pathologically with membranoproliferative glomerulonephritis-like features. An association between C3 glomerulopathy and monoclonal gammopathy was recently reported in several cases, raising the possibility that C3 glomerulopathy is the underlying pathological process in monoclonal gammopathy of renal significance...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29724174/eculizumab-as-salvage-therapy-for-recurrent-monoclonal-gammopathy-induced-c3-glomerulopathy-in-a-kidney-allograft
#10
Philipp Moog, Philipp J Jost, Maike Büttner-Herold
BACKGROUND: Monoclonal gammopathy causes several kinds of renal pathology. A rare and special form is monoclonal gammopathy-induced C3 glomerulopathy (MG-C3G). Like idiopathic C3G, MG-C3G frequently leads to end-stage renal disease. MG-C3G frequently recurs after renal transplantation, leading to graft failure in most of the patients. While there is some evidence for successful treatment of recurrent idiopathic C3 glomerulopathy with eculizumab after renal transplantation, nothing is known about its efficacy in the setting of recurrent MG-C3G...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29713256/primary-glomerulonephritis-with-unique-c4d-deposition-and-concurrent-non-infectious-intermediate-uveitis-a-case-report-and-literature-review
#11
Jong Man Park, Harin Lee, Sangheon Song, Eun Young Seong, Ihm Soo Kwak, Sung Who Park, Young-Keum Kim, Nari Shin, Mee Young Sol
C4 glomerulopathy is a recently introduced entity that presents with bright C4d staining and minimal or absent immunoglobulin and C3 staining. We report a case of a 62-year-old man with C4 glomerulonephritis (GN) and uveitis. He presented to the nephrology department with proteinuria and hematuria. The patient also had intermediate uveitis along with proteinuria and hematuria. A kidney biopsy that was performed in light of continuing proteinuria and hematuria showed a focal proliferative, focal sclerotic glomerulopathy pattern on light microscopy, absent staining for immunoglobulin or C3 by immunofluorescence microscopy, with bright staining for C4d on immunohistochemistry, and electron-dense deposits on electron microscopy...
April 30, 2018: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29706017/distribution-of-exogenous-complement-factor-h-in-mice-in-vivo
#12
Aino R Koskinen, Zhu-Zhu Cheng, Matthew C Pickering, Kalevi Kairemo, Taru Meri, H Terence Cook, Seppo Meri, T Sakari Jokiranta
Factor H is an important regulator of complement activation in plasma and on cell surfaces in both humans and mice. If FH function is compromised, inappropriate complement activation on self-surfaces can have disastrous effects as seen in the kidney diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy. Since FH constructs have been proposed to be used in treatment for these diseases, we studied the distribution of exogenous FH fragments in mice. Full-length mFH, mFH1-5 and mFH18-20 fragments were radiolabeled and their distribution was examined in WT, FH-/- and FH-/- C3-/- mice in vivo...
April 29, 2018: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29703839/the-complexity-and-heterogeneity-of-monoclonal-immunoglobulin-associated-renal-diseases
#13
Sanjeev Sethi, S Vincent Rajkumar, Vivette D D'Agati
Monoclonal gammopathies are characterized by the overproduction of monoclonal Ig (MIg) detectable in the serum or urine resulting from a clonal proliferation of plasma cells or B lymphocytes. The underlying hematologic conditions range from malignant neoplasms of plasma cells or B lymphocytes, including multiple myeloma and B-cell lymphoproliferative disorders, to nonmalignant small clonal proliferations. The term MGUS implies presence of an MIg in the setting of a "benign" hematologic condition without renal or other end organ damage...
April 27, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29672702/the-disappointing-contribution-of-anti-human-leukocyte-antigen-donor-specific-antibodies-characteristics-for-predicting-allograft-loss
#14
Maxime Courant, Jonathan Visentin, Gabriel Linares, Valérie Dubois, Sébastien Lepreux, Gwendaline Guidicelli, Olivier Thaunat, Pierre Merville, Lionel Couzi, Jean-Luc Taupin
Background: Pathogenicity of donor-specific antibodies (DSAs) can be assessed using the single-antigen flow beads (SAFB) assays through mean fluorescence intensity (MFI) with or without serum ethylenediaminetetraacetic acid (EDTA) treatment, measurement of C1q or C3d binding and/or their intragraft detection [graft-bound donor-specific antibody (gDSA)]. We aimed to investigate which of these markers best associates with antibody-mediated rejection (ABMR) and kidney allograft loss at the time of a for-cause biopsy...
April 17, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29670616/overactivity-of-alternative-pathway-convertases-in-patients-with-complement-mediated-renal-diseases
#15
Marloes A H M Michels, Nicole C A J van de Kar, Marcin Okrój, Anna M Blom, Sanne A W van Kraaij, Elena B Volokhina, Lambertus P W J van den Heuvel
Overactivation of the alternative pathway of the complement system is associated with the renal diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). C3 nephritic factors (C3NeF) play an important role in C3G pathogenesis by stabilizing the key enzymatic complex of complement, the C3 convertase. However, the reliability of assays detecting these autoantibodies is limited. Therefore, in this study, we validated and optimized a prototype hemolytic method for robust detection and characterization of factors causing convertase overactivity in large patient cohorts...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29660004/renal-transplant-outcomes-and-de-novo-donor-specific-anti-human-leukocyte-antigen-antibodies-a-systematic-review
#16
Ankit Sharma, Joshua R Lewis, Wai H Lim, Suetonia Palmer, Giovanni Strippoli, Jeremy R Chapman, Stephen I Alexander, Jonathan C Craig, Germaine Wong
Background: Pre-transplant donor-specific anti-human leukocyte antigen antibodies (DSAs) are known risk factors for acute rejection and reduced graft survival after kidney transplantation. DSAs may also develop de novo DSAs (dnDSAs) after transplantation but the clinical implications of these antibodies remain uncertain. Methods: We undertook a systematic review of observational studies that examined the association between dnDSAs and graft and patient outcomes (through August 2017) with the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system of reporting used to assess the quality of evidence available...
April 11, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29657219/paraneoplastic-glomerulopathies-associated-with-hematologic-malignancies
#17
Jeyachandran Dhanapriya, Thanigachalam Dineshkumar, Ramanathan Sakthirajan, Dhanikachalam Surendar, Natarajan Gopalakrishnan, T Balasubramaniyan
Paraneoplastic glomerulopathy (PG) includes a group of glomerular lesions that are not directly related to tumor burden, invasion, or metastasis, but induced by products from tumor cells such as hormones, growth factors, cytokines, and tumor antigens. PG was first described by Galloway in 1922, in which nephrotic syndrome (NS) secondary to Hodgkin's lymphoma was reported. They can present before, at the time of diagnosis, during remission, or at relapse of the hematological malignancies. We present three cases of paraneoplastic NS associated with hematological malignancies...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657200/complementary-bodybuilding-a-potential-risk-for-permanent-kidney-disease
#18
Wael El-Reshaid, Kamel El-Reshaid, Shaikha Al-Bader, Ahmad Ramadan, John Patrick Madda
We report our experience of renal disease associated with bodybuilders who had been on high-protein diet, anabolic androgenic steroids (AASs), and growth hormone (GH) for years. A total of 22 adult males who volunteered information about use of high protein diet and AAS or GH were seen over a six-year period with renal disease. Kidney biopsy revealed focal segmental glomerulosclerosis (FSGS) in eight, nephroangiosclerosis in four, chronic interstitial nephritis in three, acute interstitial nephritis in two, nephrocalcinosis with chronic interstitial nephritis in two, and single patients with membranous glomerulopathy, crescentic glomerulopathy, and sclerosing glomerulonephritis...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29649973/clinical-and-histological-evolution-after-de-novo-donor-specific-anti-human-leukocyte-antigen-antibodies-a-single-centre-retrospective-study
#19
Yassine Bouatou, Olivia Seyde, Solange Moll, Pierre-Yves Martin, Jean Villard, Sylvie Ferrari-Lacraz, Karine Hadaya
BACKGROUND: Donor-specific anti-human leukocyte antigen (HLA) antibodies (DSA) can be preformed or de novo (dn). Strategies to manage preformed DSA are well described, but data on the management and outcomes of dnDSA are lacking. METHODS: We performed a retrospective analysis of data from a single centre of the management and outcomes of 22 patients in whom a dnDSA was identified with contemporary and follow up biopsies. RESULTS: Evolution from baseline to follow up revealed a statistically significant loss of kidney function (estimated glomerular filtration rate: 45...
April 12, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29620516/fibronectin-glomerulopathy-caused-by-the-y973c-mutation-in-fibronectin-a-case-report-and-literature-review
#20
Chao Li, Yu-Bing Wen, Hang Li, Ming-Xi Li, Xue-Wang Li, Xue-Mei Li
Fibronectin glomerulopathy is a rare autosomal dominant inherited glomerular disease associated with massive deposition of fibronectin. We recently diagnosed fibronectin glomerulopathy in a 29-year-old woman presenting nephrotic syndrome. Genetic analysis of fibronectin 1 gene showed heterozygosity for the Y973C mutation. However, this mutation was not found in her parents. She had stable renal function but persistent nephrotic proteinuria after one-year follow-up.
March 30, 2018: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
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