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https://www.readbyqxmd.com/read/29680473/rituximab-in-the-management-of-pediatric-steroid-resistant-nephrotic-syndrome-a-systematic-review
#1
Manel Jellouli, Rim Charfi, Bayen Maalej, Abdelmajid Mahfoud, Sameh Trabelsi, Tahar Gargah
OBJECTIVES: To evaluate the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome. STUDY DESIGN: A systematic review evaluating the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome was performed. Data from studies, performed before April 2017 were collected, from MEDLINE, Cochrane Library, Scopus, and Web of Science. Study eligibility criteria included clinical trials and observational studies with a minimal sample size of 5 patients, regarding treatment with rituximab in children with steroid-resistant nephrotic syndrome...
April 18, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29625558/predominant-but-silent-c1q-deposits-in-mesangium-on-transplanted-kidneys-long-term-observational-study
#2
Takahiro Kanai, Yuko Akioka, Kenichiro Miura, Masataka Hisano, Junki Koike, Yutaka Yamaguchi, Motoshi Hattori
BACKGROUND: C1q nephropathy (C1qN) was first described as glomerular disease characterized by predominant meangial C1q deposits in patients with proteinuria and no evidence of systemic lupus erythematosus. Several studies, however, revealed the clinical heterogeneity of C1qN, showing some cases with normal urinalysis. To confirm the existence of cases with predominant mesangial C1q deposits and negative or mild proteinuria and/or hematuria, we investigated renal graft biopsy specimens showing negative to mild proteinuria (less than or equal to 1+ by dip stick test) and/or hematuria...
April 6, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29523280/can-thrombophilia-worsen-maternal-and-perinatal-outcomes-in-cases-of-severe-preeclampsia
#3
Fernanda Spadotto Baptista, Maria Rita de Figueiredo Lemos Bortolotto, Fabiola Roberta Marim Bianchini, Vera Lucia Jornada Krebs, Marcelo Zugaib, Rossana Pulcinelli Vieira Francisco
OBJECTIVE: To evaluate whether thrombophilia worsens maternal and foetal outcomes among patients with severe preeclampsia (PE). METHOD: From October 2009 to October 2014, an observational retrospective cohort study was performed on pregnant women with severe PE diagnosed before 34 weeks of gestation and their newborns hospitalized at the Clinics Hospital, FMUSP. Patients who had no heart disease, nephropathies, pre-gestational diabetes, gestational trophoblastic disease, foetal malformation, or twin pregnancy and who underwent thrombophilia screening during the postnatal period were included...
January 2018: Pregnancy Hypertension
https://www.readbyqxmd.com/read/29477943/the-use-of-emergency-apheresis-in-the-management-of-plasma-cell-disorders
#4
REVIEW
Sevgi Kalayoglu-Besisik
Hyperviscosity syndrome (HVS) develops most commonly in Waldenström's macroglobulinemia (WM) and multiple myeloma (MM). Plasmapheresis is the immediate therapy and very effective at relieving symptoms by removing paraprotein. The most commonly used replacement fluid is 4%-5% human albumin in physiologic saline. FFP may be used in patients with coagulation abnormalities. Plasmapheresis should be continued until acute symptoms abate. Hyperviscosity impairs the circulation in the retina and causes hemorrhages around the small retinal vessels...
February 20, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29460894/-the-clinical-and-morphological-characteristics-of-c1q-glomerulopathy
#5
V A Varshavsky, A N Grishina, N V Chebotareva, E I Gudkova
C1q glomerulopathy is a rare variety of chronic glomerulonephritis manifested as C1q deposition revealed by immunofluorescence microscopy. The pathogenesis and etiology of the disease have not been studied. The paper deals with the results of clinical, morphological, immunofluorescence, and electron microscopic examinations in 13 patients with C1q glomerulopathy. Light microscopy more commonly revealed membranous nephropathy, mesangioproliferative glomerulonephritis, and nephrosclerosis. Immunofluorescence microscopy detected a C1q fraction in association with other deposits, more frequently IgM and IgG ones...
2018: Arkhiv Patologii
https://www.readbyqxmd.com/read/29395258/extra-criteria-manifestations-of-antiphospholipid-syndrome-risk-assessment-and-management
#6
Massimo Radin, Michelle Remião Ugolini-Lopes, Savino Sciascia, Danieli Andrade
OBJECTIVES: Extra-criteria manifestations of antiphospholipid syndrome (APS) might impact on prognosis and morbidity of the disease. In this study, we aimed to evaluate a population of patients with primary APS (PAPS) whether the extra-criteria manifestations were more frequently found in subjects with higher adjusted Global APS Score (aGAPSS) values when compared to patients with thrombotic and/or obstetric APS ("criteria" manifestations) only. METHODS: Clinical records were analyzed to retrieve extra-criteria manifestation of APS, cardiovascular risk factors and antiphospholipid antibodies profile...
January 5, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29320993/distribution-of-glomerular-diseases-in-taiwan-preliminary-report-of-national-renal-biopsy-registry-publication-on-behalf-of-taiwan-society-of-nephrology
#7
Hsien-Fu Chiu, Hung-Chun Chen, Kuo-Cheng Lu, Kuo-Hsiung Shu
BACKGROUND: Despite the development of biomarkers and noninvasive imaging tools, biopsy remains the only method for correctly diagnosing patients with unexplained hematuria, proteinuria and renal failure. Renal biopsy has been performed for several decades in Taiwan; however, a national data registry is still lacking until 2013. METHODS: The Renal Biopsy Registry Committee was established within the Taiwan Society of Nephrology in January 2013. A biopsy registry format, including basic demographic data, baseline clinical features, laboratory data, and clinical and pathological diagnosis was developed...
January 10, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29282041/a-novel-compound-heterozygous-mutation-of-the-smarcal1-gene-leading-to-mild-schimke-immune-osseous-dysplasia-a-case-report
#8
Shuaimei Liu, Mingchao Zhang, Mengxia Ni, Peiran Zhu, Xinyi Xia
BACKGROUND: Schimke immune-osseous dysplasia (SIOD, OMIM 242900) is characterized by spondyloepiphyseal dysplasia, T-cell deficiency, renal dysfunction and special facial features. SMARCAL1 gene mutations are determined in approximately 50% of patients diagnosed with SIOD. CASE PRESENTATION: The case presented here is that of a 6-year-old boy who was born at 33 weeks to healthy, non-consanguineous Chinese parents. He presented with short stature (95 cm; <3rd percentile) and proteinuria...
December 28, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/29243784/value-of-immunofluorescence-mediated-detection-of-ig-c1q-c3-and-fra-for-the-identification-and-diagnosis-of-atypical-membranous-nephropathy
#9
W-X Wang, C-Y Hu
OBJECTIVE: The present study was to investigate the value of immunofluorescence-mediated detection of Ig, C1q, C3, and FRA for the identification and diagnosis of atypical membranous nephropathy (AMN). PATIENTS AND METHODS: Fifty-five patients with AMN and 135 patients with idiopathic membranous nephropathy (IMN) diagnosed by renal biopsy in our hospital were consecutively selected. The positive expressions of Ig, C1q, C3, and FRA by immunofluorescence were analyzed...
December 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29225799/kidney-involvement-in-the-schnitzler-syndrome-a-rare-disease
#10
REVIEW
Carlo Basile, Luigi Rossi, Francesco Casucci, Annalisa Teutonico, Pasquale Libutti, Piero Lisi, Carlo Lomonte, Raffaele Manna
The Schnitzler syndrome (SS) is a rare and underdiagnosed entity that associates a chronic urticarial rash, monoclonal IgM (or sometimes IgG) gammopathy and signs and symptoms of systemic inflammation. During the past 45 years, the SS has evolved from an elusive little-known disorder to the paradigm of a late-onset acquired auto-inflammatory syndrome. Though there is no definite proof of its precise pathogenesis, it should be considered as an acquired disease involving abnormal stimulation of the innate immune system, which can be reversed by the interleukin-1 receptor antagonist anakinra...
December 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29208861/etiology-of-early-renal-allograft-dysfunction-and-comparison-between-dysfunction-and-function-group-a-single-center-study
#11
K M Hadiuzzaman, S F Islam, M O Faroque, R M Hossain, M Munirunnessa, S I Selim, M Morshed
Over a period of two years thirty five renal allograft recipients & donors were evaluated to find out the aetiology of early renal allograft dysfunction, in the Department of Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from March 2010 to February 2012. A comparison was made between dysfunction & functioning graft group. Mean age of recipients were (36.4±9.4) years, mean age of donors were (41.7±8.3) years, with a male and female ratio of 3:1. Fifty percent recipients showed one heliotype match, ninety percent recipients were anti CMV antibody IgG positive, few were anti CMV antibody IgM positive...
October 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/29191199/paraffin-immunofluorescence-for-detection-of-immune-complexes-in-renal-biopsies-an-efficient-salvage-technique-for-diagnosis-of-glomerulonephritis-in-dogs
#12
Akira Yabuki, Mariko Sawa, Moeko Kohyama, Takeshi Hamamoto, Osamu Yamato
BACKGROUND: Renal biopsy is an essential tool for the diagnosis of proteinuric kidney diseases in dogs, and evaluation of immune complexes (IC) by immunofluorescence (IF) of frozen sections (IF-F) is required for the diagnosis of IC-mediated glomerulonephritis (ICGN). However, the use of frozen sections from renal biopsies can have limitations. The aim of this study was to develop a reliable IF method using formalin-fixed and paraffin-embedded (FFPE) sections to detect ICs in dog ICGN...
December 1, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/29079181/de-novo-immune-complex-deposition-in-kidney-allografts-a-series-of-32-patients
#13
Isaac E Lloyd, Faris Ahmed, Monica P Revelo, Mazdak A Khalighi
Immune complex deposition in kidney allografts can include both recurrent and de novo processes. Recurrent glomerulonephritis is a well-recognized phenomenon and has been shown to be a common cause of allograft failure. De novo immune complex-mediated disease remains relatively poorly characterized, likely owing to the less frequent use of immunofluorescence and electron microscopy in the transplant setting. We performed a retrospective review of kidney allograft biopsies showing glomerular immune complex deposition...
January 2018: Human Pathology
https://www.readbyqxmd.com/read/29027319/pregnancy-in-immunoglobulin-m-nephropathy
#14
Meryem Hocaoğlu, Sabahat Alışır Ecder, Abdulkadir Turgut, Ateş Karateke
Immunoglobulin M nephropathy is an uncommon glomerular disease and a relatively less recognized clinico-immunopathological entity in the domain of glomerulonephritis, often thought to be a bridge between minimal change disease and focal segmental glomerulosclerosis. It is characterized by asymptomatic hematuria, episodes of macroscopic hematuria and proteinuria. Corticosteroids remain the mainstay of therapeutic strategies for such patients. We present what we believe to be the first known case of successful pregnancy in a 40-year-old woman with a 12-year history of underlying immunoglobulin M nephropathy...
January 2018: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28658762/role-of-immunofluorescence-in-adult-onset-nephrotic-syndrome-a-study-in-a-tertiary-care-centre-of-western-india
#15
Sharada Rane, Prerana Mutyal, Nicholas Dcunha, Mayur Parkhi, Meenal Jadhav
INTRODUCTION: Study of renal Immunofluorescence (IF) is an ancillary but essential technique in evaluation of renal biopsies in glomerulopathies and also it enlightens on the pathogenesis of nephrotic syndrome. AIM: To determine the role of IF in evaluating definite subtyping and diagnosis of adult onset nephrotic syndrome and attempting clinicopathological correlation. MATERIALS AND METHODS: A total of 52 patients of adult onset nephrotic syndrome were evaluated clinically and with pertinent investigations; and subjected to USG guided percutaneous renal biopsy which was processed and stained for light microscopy and for immunofluorescence by direct method (DIF) using antibodies against IgG, IgM, IgA and C3...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28578628/relationship-between-immunoglobulin-deposition-and-early-lesions-of-progressive-glomerulonephropathy-in-young-common-marmosets
#16
Naoaki Yamada, Nanako Hashimoto, Junichi Kamiie, Takuya Doi, Junko Sato, Takeshi Inoue, Kinji Shirota, Minoru Tsuchitani
The authors previously investigated progressive glomerulonephropathy in 2- to 11-year-old common marmosets and characterized age-related changes of the renal glomeruli and development of tubulointerstitial lesions. In this study, immunoglobulin deposition and ultrastructural changes of the glomeruli were investigated in 5 young marmosets from 6 months to 3 years of age with pre-onset or early glomerulonephropathy. In all animals, the foot processes of podocytes were effaced, and IgM was deposited into the glomeruli...
January 2018: Veterinary Pathology
https://www.readbyqxmd.com/read/28573371/frequency-of-familial-mediterranean-fever-mefv-gene-mutations-in-patients-with-biopsy-proven-primary-glomerulonephritis
#17
Can Huzmeli, Ferhan Candan, Gokhan Bagci, Demet Alaygut, Ali Yilmaz, Asim Gedikli, Binnur Bagci, Meryem Timucin, Ilhan Sezgin, Mansur Kayatas
Primary glomerulopathies are those disorders that affect glomerular structure, function, or both in the absence of a multisystem disorder. We aimed to evaluate the frequency of MEFV gene mutation to show possible coexistence of FMF in patients diagnosed with biopsy-proven primary glomerulonephritis (GN). A total of 64 patients with biopsy-proven primary GN were included in the study. MEFV gene mutations examined retrospectively. The mean age of patients was 39.6 ± 13.4 (range 18-69), 35 of patients were female and 29 of patients were male...
November 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28545410/combined-proximal-tubulopathy-crystal-storing-histiocytosis-and-cast-nephropathy-in-a-patient-with-light-chain-multiple-myeloma
#18
Chung-Kuan Wu, An-Hang Yang, Hung-Chih Lai, Bing-Shi Lin
BACKGROUND: The diagnosis of myeloma, a plasma dyscrasia, often results from the workup of unexplained renal disease. Persistent renal failure in myeloma is commonly caused by tubular nephropathy due to circulating immunoglobulins and free light chains. Myeloma cast nephropathy is characterized by crystalline precipitates of monoclonal light chains within distal tubules. Immunoglobulin crystallization rarely occurs intracellularly, within proximal tubular cells (light chain proximal tubulopathy) and interstitial histiocytes (crystal-storing histiocytosis)...
May 25, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28509128/a-case-of-rapid-amelioration-of-hepatitis-c-virus-associated-cryoglobulinemic-membranoproliferative-glomerulonephritis-treated-by-interferon-free-directly-acting-antivirals-for-hcv-in-the-absence-of-immunosuppressant
#19
Fumiaki Obata, Taichi Murakami, Junko Miyagi, Sayo Ueda, Taizo Inagaki, Masanori Minato, Hiroyuki Ono, Kenji Nishimura, Eriko Shibata, Masanori Tamaki, Sakiya Yoshimoto, Fumi Kishi, Seiji Kishi, Motokazu Matsuura, Kojiro Nagai, Hideharu Abe, Toshio Doi
Mixed cryoglobulinemic syndrome, which is a systemic vasculitis characterized by the immune complex deposition in small- and medium-sized arteries and most often due to chronic hepatitis C virus (HCV) infection, sometimes clinically manifests as refractory glomerulonephritis or nephritic syndrome. Patients with mixed cryoglobulinemic nephropathy who have a rapidly progressive glomerulonephritis should receive immunosuppressive therapy. After disease stabilization, patients should receive concurrent therapy for the underlying HCV infection...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28291506/immunotactoid-glomerulopathy-leading-to-the-discovery-of-poems-syndrome%C3%A2
#20
Carole Philipponnet, Jean-Louis Kemeny, Cyril Garrouste, Martin Soubrier, Anne-Elisabeth Heng
Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy. Histological analysis revealed membranoproliferative glomerulonephritis with IgG, IgM, κ, λ, and C3 deposits...
June 2017: Clinical Nephrology
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