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C3 nephropathy

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https://www.readbyqxmd.com/read/29775444/-influence-of-intensity-localization-and-type-of-deposits-in-renal-biopsy-for-disease-symptoms-and-follow-up-in-children-with-iga-nephropathy
#1
Karolina Cichoń-Kawa, Małgorzata Mizerska-Wasiak, Jadwiga Małdyk, Agnieszka Turczyn, Agnieszka Rybi-Szumińska, Anna Wasilewska, Agnieszka Firszt-Adamczyk, Roman Stankiewicz, Beata Bieniaś, Przemysław Sikora, Katarzyna Gadomska-Prokop, Ryszard Grenda, Małgorzata Pańczyk-Tomaszewska
IgA nephropathy is the most common glomerulonephritis in the world. For diagnosis kidney biopsy is necessary. AIM: The aim of the study was assessment the significance of IgA, C3 and IgG deposits intensity and location in kidney childhood IgA nephropathy (IgAN) for the symptoms of the disease and the follow up. MATERIALS AND METHODS: Study population consisted of 81 children, average 11,45±3,99 years. IgAN was recognized based on renal biopsy, performed 1,2±1,84, median 0,5 years after the onset...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29759419/circulating-complement-factor-h-related-protein-5-levels-contribute-to-development-and-progression-of-iga-nephropathy
#2
Li Zhu, Wei-Yi Guo, Su-Fang Shi, Li-Jun Liu, Ji-Cheng Lv, Nicholas R Medjeral-Thomas, Hannah J Lomax-Browne, Matthew C Pickering, Hong Zhang
IgA nephropathy (IgAN) is a disease associated with activation of the complement system. But the factors influencing complement activation in IgAN are not fully understood. Complement factor H (FH) is an essential negative regulator of complement C3 activation. Complement factor H-related protein (FHR)-5 shares high sequence similarity with factor H. However, unlike factor H, on binding to activated C3 it enables further activation to proceed. Previously, we reported the contribution of rare variants of the CFHR5 gene to IgAN susceptibility...
May 11, 2018: Kidney International
https://www.readbyqxmd.com/read/29703839/the-complexity-and-heterogeneity-of-monoclonal-immunoglobulin-associated-renal-diseases
#3
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/29701808/the-lectin-pathway-in-renal-disease-old-concept-and-new-insights
#4
Mariana Gaya da Costa, Felix Poppelaars, Stefan P Berger, Mohamed R Daha, Marc A Seelen
The complement system is composed of a network of at least 40 proteins, which significantly contributes to health and disease. The lectin pathway (LP) is one of three pathways that can activate the complement system. Next to protection of the host against pathogens, the LP has been shown to play a crucial role in multiple renal diseases as well as during renal replacement therapy. Therefore, several complement-targeted drugs are currently being explored in clinical trials. Among these complement inhibitors, specific LP inhibitors are also being tested in renal abnormalities such as in immunoglobulin A nephropathy and lupus nephritis...
April 26, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29625558/predominant-but-silent-c1q-deposits-in-mesangium-on-transplanted-kidneys-long-term-observational-study
#5
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/29578397/fibrillary-glomerulonephritis-in-an-hiv-patient-without-concurrent-hepatitis-c-infection-case-report-and-review-of-the-literature
#6
Lian Zhang, John M Carson, M Scott Lucia
The most common renal disease associated with human immunodeficiency virus infection (HIV) is HIV-associated nephropathy (HIVAN), especially in the African American patient population. However, various patterns of glomerulonephritis (GN) are not uncommon, collectively accounting for nearly half of the renal biopsies performed in HIV patients. Most GNs that occur in HIV patients are immune complex mediated, often with concurrent infections such as hepatitis B or C. Fibrillary glomerulonephritis (FGN), a rare primary glomerular disease, has only been reported in 2 HIV patients, and both patients had concurrent hepatitis C (HCV) infection...
May 2018: Clinical Nephrology
https://www.readbyqxmd.com/read/29536803/invasive-fungal-infections-in-colombian-patients-with-systemic-lupus-erythematosus
#7
Y Santamaría-Alza, J Sánchez-Bautista, J F Fajardo-Rivero, C L Figueroa
Introduction Systemic lupus erythematosus is an autoimmune disease with multi-organ involvement. Complications, such as invasive fungal infections usually occur in patients with a greater severity of the disease. Objective The objective of this study was to determine the prevalence and risk variables associated with invasive fungal infections in a Colombian systemic lupus erythematosus population. Materials and methods A cross-sectional, retrospective study that evaluated patients with systemic lupus erythematosus for six years...
June 2018: Lupus
https://www.readbyqxmd.com/read/29523959/the-light-at-the-end-of-the-tunnel-an-unusual-case-of-acute-kidney-injury-in-a-pediatric-patient-answers
#8
Miriam Steinberg, Joseph P Gaut, Stanley Paul Hmiel, Aadil Kakajiwala
Monoclonal gammopathies are a rare diagnosis in pediatric patients. A 19-year-old female patient with past medical history of hypogammaglobulinemia and natural killer cell deficiency and stage III follicular lymphoma, in remission, presented with a right-sided pneumonia, noted to have acute kidney injury and proteinuria. Complement C3 and C4 levels were normal. Anti-double-stranded DNA antibodies, antinuclear antibodies, anti-extractable nuclear antigen antibodies, and antineutrophil cytoplasmic antibodies were negative...
March 9, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29523638/complement-c3-and-risk-of-diabetic-microvascular-disease-a-cohort-study-of-95202-individuals-from-the-general-population
#9
Katrine Laura Rasmussen, Børge Grønne Nordestgaard, Sune Fallgaard Nielsen
BACKGROUND: Whether the complement system is involved in the development of diabetic microvascular disease is unknown. We tested the hypothesis that high concentrations of complement C3 are associated with increased risk of diabetic retinopathy, nephropathy, and neuropathy in individuals from the general population. METHODS: We studied 95202 individuals from the general population with baseline measurements of complement C3, genotyped for rs1065489, rs429608, and rs448260 determining concentrations of complement C3, and enrolled in the Copenhagen General Population Study from 2003 through 2013, following them until April 10, 2013...
March 9, 2018: Clinical Chemistry
https://www.readbyqxmd.com/read/29518610/zhen-wu-tang-protects-against-podocyte-injury-in-rats-with-iga-nephropathy-via-ppar%C3%AE-nf-%C3%AE%C2%BAb-pathway
#10
Bihao Liu, Yu He, Ruirui Lu, Jie Zhou, Lixia Bai, Peichun Zhang, Shufang Ye, Junbiao Wu, Chungling Liang, Yuan Zhou, Jiuyao Zhou
Zhen-wu-tang (ZWT) has been widely applied in chronic kidney diseases. However, the mechanism of ZWT remains unclear. Peroxisome proliferator-activated receptors-γ (PPARγ) is known as a protective factor for podocyte and kidney function. This study is aimed to investigate the protective effects of ZWT on IgA nephropathy (IgAN) in rats against podocyte injury and the underlying mechanism related to PPARγ. IgAN model rats were induced by administering bovine serum albumin, lipopolysaccharide, and carbon tetrachloride...
May 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29497040/podocyte-specific-rac1-deficiency-ameliorates-podocyte-damage-and-proteinuria-in-stz-induced-diabetic-nephropathy-in-mice
#11
Zhimei Lv, Mengsi Hu, Minghua Fan, Xiaobing Li, Jiangong Lin, Junhui Zhen, Ziyang Wang, Haijun Jin, Rong Wang
Activation of Ras-related C3 botulinum toxin substrate 1 (Rac1) has been implicated in diverse kidney diseases, yet its in vivo significance in diabetic nephropathy (DN) is largely unknown. In the present study, we demonstrated a podocyte-specific Rac1-deficient mouse strain and showed that specific inhibition of Rac1 was able to attenuate diabetic podocyte injury and proteinuria by the blockade of Rac1/PAK1/p38/β-catenin signaling cascade, which reinstated the integrity of podocyte slit diaphragms (SD), rectified the effacement of foot processes (FPs), and prevented the dedifferentiation of podocytes...
March 1, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29450785/the-role-of-the-alternative-pathway-of-complement-activation-in-glomerular-diseases
#12
REVIEW
Emilia Łukawska, Magdalena Polcyn-Adamczak, Zofia I Niemir
The complement system (CS) has recently been recognized as a bridge between innate and adaptive immunity that constitutes a very complex mechanism controlling the clearance of pathogens, cellular debris, and immune complexes. Out of three known pathways of complement activation, the alternative pathway (AP) plays a critical role in host defense by amplifying the complement response, independently of initiation pathway and continuously maintaining low-level activity in a process called 'thick-over.' A key molecule of the CS is C3, in which the AP is constantly activated...
February 15, 2018: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/29312858/-de-novo-glomerular-diseases-after-renal-transplantation-how-is-it-different-from-recurrent-glomerular-diseases
#13
REVIEW
Fedaey Abbas, Mohsen El Kossi, Jon Kim Jin, Ajay Sharma, Ahmed Halawa
The glomerular diseases after renal transplantation can occur de novo, i.e ., with no relation to the native kidney disease, or more frequently occur as a recurrence of the original disease in the native kidney. There may not be any difference in clinical features and histological pattern between de novo glomerular disease and recurrence of original glomerular disease. However, structural alterations in transplanted kidney add to dilemma in diagnosis. These changes in architecture of histopathology can happen due to: (1) exposure to the immunosuppression specifically the calcineurin inhibitors (CNI); (2) in vascular and tubulointerstitial alterations as a result of antibody mediated or cell-mediated immunological onslaught; (3) post-transplant viral infections; (4) ischemia-reperfusion injury; and (5) hyperfiltration injury...
December 24, 2017: World Journal of Transplantation
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
#14
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/29241200/atypical-hemolytic-uremic-syndrome-associated-with-complement-factor-h-mutation-and-iga-nephropathy-a-case-report-successfully-treated-with-eculizumab
#15
Hironori Nakamura, Mariko Anayama, Mutsuki Makino, Yasushi Makino, Katsuhiko Tamura, Masaki Nagasawa
We present a rare case of IgA nephropathy in a patient who developed atypical hemolytic uremic syndrome (aHUS) associated with a complement factor H (CFH) gene mutation, and who was successfully treated with eculizmab. A 76-year-old man was admitted as the patients had thrombotic microangiopathies findings. The patient was treated with plasma exchange, hemodialysis and methylprednisolone. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 level was not decreased. Light microscopy findings were consistent with hemolytic uremic syndrome and immunofluorescence analysis revealed IgA and C3 were detected...
2018: Nephron
https://www.readbyqxmd.com/read/29237409/iga-nephropathy-featuring-massive-wire-loop-like-deposits-in-two-patients-with-alcoholic-cirrhosis
#16
Daisuke Takada, Keiichi Sumida, Akinari Sekine, Ryo Hazue, Masayuki Yamanouchi, Tatsuya Suwabe, Noriko Hayami, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Yoshifumi Ubara
BACKGROUND: Various renal manifestations are known to develop in patients with liver disease, including chronic hepatitis and cirrhosis. CASE PRESENTATION: We evaluated renal disease in two 47-year-old Japanese men with liver cirrhosis and chronic alcoholism for 34 years and 27 years, respectively. Renal biopsy demonstrated massive wire loop-like deposits in the subendothelial space of the glomerular basement membrane and in the mesangium. However, immunofluorescence was only positive for IgA and C3, and electron microscopy did not reveal any organized structures in the electron-dense deposits...
December 13, 2017: BMC Nephrology
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
#17
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/29178575/serum-iga-c3-and-glomerular-c3-staining-predict-severity-of-iga-nephropathy
#18
Yukihiko Kawasaki, Ryo Maeda, Shinichiro Ohara, Kazuhide Suyama, Mitsuaki Hosoya
BACKGROUND: The aim of this study was to determine whether serum immunoglobulin A/complement factor 3 (IgA/C3) ratio and glomerular C3 staining predict outcome in IgA nephropathy. METHODS: We collected data for 44 IgA nephropathy children treated with multi-drug combination therapy. The children were retrospectively divided into four groups based on serum IgA/C3 ratio and glomerular C3 staining: group A, IgA/C3 ratio >2.68 (median) and glomerular C3 staining ≥2...
February 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29114042/deletion-variants-of-cfhr1-and-cfhr3-associate-with-mesangial-immune-deposits-but-not-with-progression-of-iga-nephropathy
#19
Perrine Jullien, Blandine Laurent, Guillaume Claisse, Ingrid Masson, Miriana Dinic, Damien Thibaudin, Francois Berthoux, Eric Alamartine, Christophe Mariat, Nicolas Maillard
Activation of complement through the alternative pathway has a key role in the pathogenesis of IgA nephropathy (IgAN). Large, international, genome-wide association studies have shown that deletion of complement factor H-related genes 1 and 3 ( CFHR3,1Δ ) is associated with a reduced risk of developing IgAN, although the prognostic value of these deletions in IgAN remains unknown. Here, we compared the renal outcomes of patients with IgAN according to their CFHR3,1Δ genotype. This retrospective, monocentric cohort study included 639 white patients with biopsy-proven IgAN since 1979 (mean age at diagnosis, 40...
February 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29043511/implication-of-decreased-serum-complement-3-in-patients-with-diabetic-nephropathy
#20
Junlin Zhang, Yiting Wang, Rui Zhang, Hanyu Li, Qianqian Han, Ruikun Guo, Tingli Wang, Li Li, Fang Liu
AIMS: The serum complement 3 (C3) level was reduced in many patients with type 2 diabetes mellitus (T2DM) and diabetic nephropathy (DN). However, the clinical implications of such change are still less understood. This study was aimed to explore the association between C3 level and the baseline clinicopathological characteristics and the prognosis of T2DM patients with DN. METHODS: A total of 171 T2DM patients with biopsy-proven DN who received follow-up for at least 1 year were recruited...
January 2018: Acta Diabetologica
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