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Membranoproliferative

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https://www.readbyqxmd.com/read/28317619/membranoproliferative-glomerulonephritis-in-pregnancy
#1
Devika Nair, Laura Kidd, N Kevin Krane
BACKGROUND: Membranoproliferative glomerulonephritis (MPGN) is an uncommon form of glomerulonephritis and it can be particularly difficult to predict outcomes and manage women with this disorder during pregnancy. MATERIALS AND METHODS: The management of 3 successful pregnancies in women with MPGN from 1 center and previously described cases from the world literature are reviewed. This includes a number of large studies of pregnancy in women with underlying glomerular disease as well as small case series and individual reports...
April 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28299015/endogenous-nocardial-endophthalmitis-in-an-immunosuppressed-patient-a-serious-warning-of-an-underlying-life-threatening-and-blinding-disorder
#2
Hemant Trehan, Jaya Kaushik, Vaibhav Kumar Jain, Jitendra Kumar Singh Parihar, Abhijit Avasthi
PURPOSE: To report a case of bilateral endogenous nocardial endophthalmitis with central nervous system involvement in an immunocompromised individual with an extremely poor outcome. CASE REPORT: A 35-year-old man with a history of long-term, prescribed oral steroid use for membranoproliferative glomerulonephritis presented with profound bilateral vision loss. Patient's diagnosis of bilateral endogenous nocardial endophthalmitis was delayed. Nocardia was finally isolated from a brain biopsy after a repeat magnetic resonance imaging revealed a brain abscess...
January 2017: Journal of Ophthalmic & Vision Research
https://www.readbyqxmd.com/read/28291506/immunotactoid-glomerulopathy-leading-to-the-discovery-of-poems-syndrome%C3%A2
#3
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...
March 14, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28260442/nephrotic-syndrome-after-hematopoietic-stem-cell-transplant-outcomes-in-iran
#4
Fereshteh Saddadi, Ali Alidadi, Monir Hakemi, Babak Bahar
OBJECTIVES: Patients undergoing hematopoietic stem cell transplant have an elevated incidence of acute renal failure. However, the incidence of nephritic syndrome due to graft-versus-host disease is growing and is independently associated with chronic renal disease after this procedure. MATERIALS AND METHODS: We conducted a prospective study to examine the risk of chronic kidney disease in glomerulopathy patients following hematopoietic stem cell transplant with a follow-up of 10 years...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28243475/kinetics-of-rituximab-excretion-into-urine-and-peritoneal-fluid-in-two-patients-with-nephrotic-syndrome
#5
Klaus Stahl, Michelle Duong, Anke Schwarz, A D Wagner, Hermann Haller, Mario Schiffer, Roland Jacobs
Clinical observations suggest that treatment of Rituximab might be less effective in patients with nephrotic range proteinuria when compared to nonnephrotic patients. It is conceivable that the reason for this is that significant amounts of Rituximab might be lost in the urine in a nephrotic patient and that these patients require a repeated or higher dosage. However, this has not been systematically studied. In this case report we describe two different patients with nephrotic range proteinuria receiving Rituximab...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28226323/immune-complex-membranoproliferative-glomerulonephritis-associated-with-transjugular-intrahepatic-portosystemic-shunts-in-alcohol-cirrhosis-two-case-reports
#6
Julio Hernández-Jaras, Jordi Espí-Reig, Rafael Alis, Ana-Maria García-Martínez, Diego Rodríguez-Ortega, Lourdes Roca-Argente, Jose-Luis Moll-Guillen
OBJECTIVE: To report two cases with alcoholic cirrhosis, that were treated with transjugular intrahepatic portosystemic shunt (TIPS) placement. CLINICAL PRESENTATION AND INTERVENTION: The two patients had a history of alcoholic cirrhosis and TIPS surgery was performed on them. In both cases, 4 months after TIPS placement, proteinuria was observed along with histological alterations characteristic of immune complex-MPGN. CONCLUSION: The TIPS in one patient was successful without immediate complication while the other patient was referred for combined liver-kidney transplant...
February 22, 2017: Medical Principles and Practice: International Journal of the Kuwait University, Health Science Centre
https://www.readbyqxmd.com/read/28210841/rare-genetic-variant-in-the-cfb-gene-presenting-as-atypical-hemolytic-uremic-syndrome-and-immune-complex-diffuse-membranoproliferative-glomerulonephritis-with-crescents-successfully-treated-with-eculizumab
#7
Khalid Alfakeeh, Mohammed Azar, Majid Alfadhel, Alsuayri Mansour Abdullah, Nourah Aloudah, Khaled O Alsaad
BACKGROUND: Complement factor B gene (CFB) is an important component of the alternate pathway of complement activation that provides an active subunit that associates with C3b to form the C3 convertase, which is an essential element in complement activation. Among the complement-associated disorders, mutations and pathogenic variants in the CFB gene are relatively rare phenomena. Moreover, mutated CFB affiliation with immune-complex diffuse membranoproliferative glomerulonephritis (IC-MPGN) and atypical hemolytic uremic syndrome (aHUS) are considered a highly rare occurrence...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28207635/kidney-transplantation-rates-across-glomerulonephritis-subtypes-in-the-united-states
#8
Michelle M O'Shaughnessy, Sai Liu, Maria E Montez-Rath, Richard A Lafayette, Wolfgang C Winkelmayer
BACKGROUND: Whether kidney transplantation rates differ by glomerulonephritis (GN) subtype remains largely unknown. METHODS: Using the US Renal Data System, we identified all adult patients with ESRD attributed to 1 of 6 GN subtypes who initiated dialysis in the US (1996-2013). Patients with diabetic nephropathy (DN) and autosomal-dominant polycystic kidney disease (ADPKD) served as "external" non-GN comparators. Using Cox proportional hazards regression, with death considered a competing risk, we estimated hazard ratios [HRs (95% confidence intervals)] for first kidney transplantation, controlling for year, demographics, comorbidities, socioeconomic factors, and Organ Procurement Organization (OPO)...
February 15, 2017: Transplantation
https://www.readbyqxmd.com/read/28098115/a-novel-fibrillin-1-mutation-in-an-egyptian-marfan-family-a-proband-showing-nephrotic-syndrome-due-to-focal-segmental-glomerulosclerosis
#9
Mohammad Al-Haggar, Ashraf Bakr, Yahya Wahba, Paul J Coucke, Fatma El-Hussini, Mona Hafez, Riham Eid, Abdel-Rahman Eid, Amr Sarhan, Ali Shaltout, Ayman Hammad, Sohier Yahia, Ahmad El-Rifaie, Dina Abdel-Hadi
Marfan syndrome (MFS), the founding member of connective tissue disorder, is an autosomal dominant disease; it is caused by a deficiency of the microfibrillar protein fibrillin-1 (FBN1) and characterized by involvement of three main systems; skeletal, ocular, and cardiovascular. More than one thousand mutations in FBN1 gene on chromosome 15 were found to cause MFS. Nephrotic syndrome (NS) had been described in very few patients with MFS being attributed to membranoproliferative glomerulonephritis secondary to infective endocarditis...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28096309/anti-factor-b-and-anti-c3b-autoantibodies-in-c3-glomerulopathy-and-ig-associated-membranoproliferative-gn
#10
Maria Chiara Marinozzi, Lubka T Roumenina, Sophie Chauvet, Alexandre Hertig, Dominique Bertrand, Jérome Olagne, Marie Frimat, Tim Ulinski, Georges Deschênes, Stephane Burtey, Michel Delahousse, Bruno Moulin, Christophe Legendre, Véronique Frémeaux-Bacchi, Moglie Le Quintrec
In C3 glomerulopathy (C3G), the alternative pathway of complement is frequently overactivated by autoantibodies that stabilize the C3 convertase C3bBb. Anti-C3b and anti-factor B (anti-FB) IgG have been reported in three patients with C3G. We screened a cohort of 141 patients with C3G and Ig-associated membranoproliferative GN (Ig-MPGN) for anti-FB and anti-C3b autoantibodies using ELISA. We identified seven patients with anti-FB IgG, three patients with anti-C3b IgG, and five patients with anti-FB and anti-C3b IgG...
January 17, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#11
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/28058212/complement-related-kidney-diseases-recurrence-after-transplantation
#12
REVIEW
Maurizio Salvadori, Elisabetta Bertoni
The recurrence of renal disease after renal transplantation is becoming one of the main causes of graft loss after kidney transplantation. This principally concerns some of the original diseases as the atypical hemolytic uremic syndrome (HUS), the membranoproliferative glomerulonephritis (MPGN), in particular the MPGN now called C3 glomerulopathy. Both this groups of renal diseases are characterized by congenital (genetic) or acquired (auto-antibodies) modifications of the alternative pathway of complement...
December 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/28008340/fractional-excretion-of-sodium-in-hepatorenal-syndrome-clinical-and-pathological-correlation
#13
Ali A Alsaad, Hani M Wadei
AIM: To determine the accuracy of fractional excretion of sodium (FeNa) in the diagnosis of hepatorenal syndrome (HRS). METHODS: Eighty-eight liver transplantation candidates with renal dysfunction and/or proteinuria were included in the study sample. The baseline characteristics of the patients were obtained. All the 88 patients underwent iothalamate glomerular filtration rate testing, 24-h urine collection for urinary sodium and protein excretions, random urine for sodium and creatinine testing, and percutaneous kidney biopsy...
December 8, 2016: World Journal of Hepatology
https://www.readbyqxmd.com/read/28003788/multimodal-imaging-of-membranoproliferative-glomerulonephritis-type-ii
#14
Zafer Cebeci, Serife Bayraktar, Merih Oray, Nur Kir
To report the fundus findings on multimodal imaging of a membranoproliferative glomerulonephritis type II (MPGNII) patient. A 41-year-old woman had a medical history of renal transplantation for MPGNII 5 years prior to presentation to our clinic for ophthalmic examination. Fundus examination showed macular retinal pigment epithelial changes and large peripapillary atrophy in the right eye, and yellowish bilateral drusen-like deposits peripherally. Fundus fluorescein angiography and indocyanine green angiography revealed multifocal hyperfluorescence in the peripheral retina with accompanying deposits...
October 2016: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/27994856/c3-glomerulopathy-in-adults-a-distinct-patient-subset-showing-frequent-association-with-monoclonal-gammopathy-and-poor-renal-outcome
#15
Isaac E Lloyd, Alexander Gallan, Hunter K Huston, Kalani L Raphael, Dylan V Miller, Monica P Revelo, Mazdak A Khalighi
BACKGROUND: C3 glomerulopathy (C3G) includes both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD) and is defined by C3-dominant deposits on immunofluorescence. Dysfunction of the alternative pathway (AP) of complement is central to the pathophysiology of C3G and young patients often harbor genetic alterations of AP mediators. Recently, a link between C3G and paraproteinemia has been established. We performed this study to better characterize older patients with C3G where this association is more frequently seen...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27904866/sj%C3%A3-gren-syndrome-related-membranous-glomerulonephritis-progressing-to-membranoproliferative-glomerulonephritis
#16
Junko Yabuuchi, Tatsuya Suwabe, Toshiharu Ueno, Junichi Hoshino, Akinari Sekine, Noriko Hayami, Masahiko Oguro, Kyohei Kunisawa, Masahiro Kawada, Masayuki Yamanouchi, Keiichi Sumida, Hiroki Mizuno, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Kenichi Ohashi, Takeshi Fujii, Yoshifumi Ubara
We report a case of glomerulopathy in a 36-year-old Japanese woman with primary Sjögren syndrome (pSS). The first renal biopsy suggested membranous glomerulonephritis. However, repeat biopsy was performed after 16 years because of increased proteinuria, revealing membranoproliferative glomerulonephritis with mesangial deposits, subendothelial deposits, and subepithelial deposits. Immunofluorescent studies showed predominant deposition of IgG2 and IgG4. This patient was positive for antinuclear antibody and anti-SS-A antibody...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27866967/cryofibrinogen-associated-glomerulonephritis
#17
Sanjeev Sethi, Ralph Yachoui, David L Murray, Jai Radhakrishnan, Mariam P Alexander
Cryofibrinogen is an under-recognized cryoprotein. Cryofibrinogen is a cryoprecipitate that develops following plasma refrigeration, but does not occur in cold serum. People with cryofibrinogenemia may be asymptomatic, but this cryoprotein can be associated with thromboembolic disease, particularly affecting the skin. Kidney manifestations are relatively uncommon, but are likely underestimated. We describe clinical features and kidney biopsy results in 2 patients with cryofibrinogen-related kidney disease. Both patients presented with proteinuria and hematuria...
February 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27817791/-advances-in-clinical-research-on-c1q-nephropathy
#18
REVIEW
Juan Yang, Bi-Li Zhang
C1q nephropathy is a rare type of glomerulonephritis manifested as the deposition of C1q in the glomerular mesangium during immunofluorescent staining. Systemic lupus erythematosus and type I membranoproliferative glomerulonephropathy need to be excluded in the diagnosis of C1q nephropathy. C1q nephropathy has various manifestations under a light microscope, mainly including minimal change disease, focal segmental glomerulosclerosis, and proliferative glomerulonephritis. This disease is mainly manifested as persistent proteinuria or nephrotic syndrome and occurs more frequently in boys...
November 2016: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/27812262/membranoproliferative-glomerulonephritis-secondary-to-a-low-grade-lymphoproliferative-disorder-a-rare-cause-of-renal-dysfunction
#19
Tushar Sehgal, Nidhi Jain, Gaurav Prakash, Ritambhra Duseja, Neelam Varma
Chronic lymphocytic leukemia is a B-cell neoplasm with an indolent clinical course. Most patients are asymptomatic and are diagnosed incidentally on a routine blood count. The malignant cells of this low-grade neoplasm infiltrate various organs and tissues. However, the resultant end organ damage is a rare phenomenon. Here we describe a case of chronic lymphocytic leukemia that presented with an unusual cause of acute renal dysfunction. The patient had deranged renal parameters with a nephrotic range proteinuria...
December 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27778453/successful-recovery-of-acute-renal-transplant-failure-in-recurrent-hepatitis-c-virus-associated-membranoproliferative-glomerulonephritis
#20
E Schrezenmeier, K Wu, F Halleck, L Liefeldt, S Brakemeier, F Bachmann, S Kron, K Budde, M Duerr
Recurrence of hepatitis C virus (HCV)-associated membranoproliferative glomerulonephritis (MPGN) in the kidney transplant may lead to continuous graft deterioration and the need for further renal replacement therapy. The novel direct-acting antiviral agents (DAAs) allow a highly effective and interferon-free treatment option for chronic HCV-infected patients. Data on the therapeutic safety and efficacy in HCV-infected renal transplant patients are sparse, especially for patients with severe renal impairment...
March 2017: American Journal of Transplantation
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