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Nuntawan Piyaphanee, Chompoonut Ananboontarick, Suroj Supavekin, Achra Sumboonnanonda
BACKGROUND: Rapidly progressive glomerulonephritis (RPGN), a clinical diagnosis defined as acute nephritic syndrome with dramatic loss of renal function within few months, is associated with crescentic glomerulonephritis (CresGN), which requires ≥50% crescents in pathology. The percentage of crescents in children with RPGN may vary, however, determining disease characteristics and renal outcome. METHODS: To evaluate the renal outcomes and factors associated to end-stage renal disease (ESRD), this retrospective cohort study assessed children aged ≤15 years with RPGN at a tertiary medical center...
August 20, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Lauren E Neidig, Michael A Owston, Erin Ball, Edward J Dick
BACKGROUND: Crescentic glomeruli are the hallmark finding in rapidly progressive glomerulonephritis (RPGN) and are characterized by disruption and proliferation of the glomerular capsule and an influx of cells into Bowman's space. Pauci-immune-type RPGN is identified by a lack of immunoglobulins and immune complexes in the glomerular basement membrane. METHODS: Complete necropsy and histology were performed on the affected chimpanzee. Electron microscopy was performed on kidney sections...
August 10, 2016: Journal of Medical Primatology
M O Faroque, K M Hadiuzzaman, S F Islam, R M Hossain, M N Islam, A H Ahmed, P I Ahmed, M R Alam
Over a period of 3 years (January 2011 to December 2013) 100 cases of Lupus nephritis patients admitted in nephrology department of Bangabandhu Sheikh Mujib Medical University (BSMMU) were evaluated. Their clinical characteristics, biochemical parameters, renal histology according to WHO classification were categorized and their treatment modalities and outcome was observed. Among 100 patients, 84 were female and 16 were male, with F:M ratio 5:1. Mean age of female were 23±4 years and male were 29±4 years, mean BP in male was systolic 135±8 mmHg, diastolic 80±9mmHg and in female systolic was 130±7mmHg, diastolic 75±6 mmHg, mean Serum Creatinine for male was 180±12μmol/L and mean serum creatinine in female was 170±20μmol/L...
April 2016: Mymensingh Medical Journal: MMJ
Pui Shan Julia Chan, Moon Ho Leung
We report a case of 63-year-old Chinese man, having a history of anti-myeloperoxidase (MPO) antibody anti-neutrophil cytoplasmic antibody (ANCA)-associated pulmonary-renal syndrome 9 years ago, presented with second episode of rapidly progressive glomerulonephritis (RPGN) and alveolar haemorrhage compatible with anti-glomerular basement membrane (GBM) disease. In first presentation, his anti-GBM antibody was negative. This time, anti-MPO antibody was negative, but anti-GBM antibody was positive. The long interval of sequential development of anti-GBM disease after ANCA-associated vasculitis in this patient may provide clues to the potential immunological links between these two distinct conditions...
April 2016: Oxford Medical Case Reports
Ken-Ei Sada, Masayoshi Harigai, Koichi Amano, Tatsuya Atsumi, Shouichi Fujimoto, Yukio Yuzawa, Yoshinari Takasaki, Shogo Banno, Takahiko Sugihara, Masaki Kobayashi, Joichi Usui, Kunihiro Yamagata, Sakae Homma, Hiroaki Dobashi, Naotake Tsuboi, Akihiro Ishizu, Hitoshi Sugiyama, Yasunori Okada, Yoshihiro Arimura, Seiichi Matsuo, Hirofumi Makino
OBJECTIVE: To compare disease severity classification systems for six-month outcome prediction in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: Patients with newly diagnosed AAV from 53 tertiary institutions were enrolled. Six-month remission, overall survival, and end-stage renal disease (ESRD)-free survival were evaluated. RESULTS: According to the European Vasculitis Study Group (EUVAS)-defined disease severity, the 321 enrolled patients were classified as follows: 14, localized; 71, early systemic; 170, generalized; and 66, severe disease...
September 2016: Modern Rheumatology
Wisit Cheungpasitporn, Claudia C Zacharek, Fernando C Fervenza, Lynn D Cornell, Sanjeev Sethi, Loren P Herrera Hernandez, Samih H Nasr, Mariam P Alexander
Anti-glomerular basement membrane (anti-GBM) disease is a major cause of rapidly progressive glomerulonephritis (RPGN). On the other hand, fibrillary glomerulonephritis (GN) typically presents as proteinuria, hematuria and renal insufficiency, but rarely as RPGN. Without electron microscopy, the diagnosis of fibrillary GN can be missed. We report a 68-year-old white woman who presented with RPGN with kidney biopsy demonstrating diffuse crescentic GN on light microscopy. By immunofluorescence, there was bright linear staining of the GBMs and smudgy mesangial staining for immunoglobulin G, C3, and kappa and lambda light chain...
February 2016: Clinical Kidney Journal
Soumita Bagchi, Parmod Mittal, Geetika Singh, Sanjay Kumar Agarwal, Lavleen Singh, Dipankar Bhowmik, Sandeep Mahajan, Amit Dinda
BACKGROUND: An aging population is an important demographic issue in India. The knowledge base about kidney diseases among the elderly Indians is inadequate. We aim to delineate the clinical profile and spectrum of biopsy-proven kidney disease in elderly patients. METHODS: Records of all elderly patients (≥60 years) who had undergone kidney biopsy in the nephrology department from January 2010 to December 2014 were reviewed. Their clinical details and laboratory investigations at the time of biopsy were noted...
April 2016: International Urology and Nephrology
Dahai Wang, Fang Wang, Jie Ding, Huijie Xiao, Xuhui Zhong, Xiaoyu Liu
OBJECTIVE: IgA nephropathy is the most common type of glomerulonephritis in the world. Its clinical and pathological manifestations vary. A few of the patients with IgA nephropathy present with rapidly progressive glomerulonephritis (RPGN) and/or crescent formation. Their conditions are serious and acute, but there are few reports on their characteristics, treatment and outcome. This study aimed to analyze the clinicalopathological features, treatment and prognosis of primary IgA nephropathy in children, to provide a reference for clinical diagnosis and treatment...
September 2015: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Rafeel Syed, Amina Rehman, Gautam Valecha, Suzanne El-Sayegh
Rapidly progressive glomerulonephritis (RPGN) is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic term crescentic glomerulonephritis (CGN). From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG), anti-GBM GN, and immune complex GN...
2015: BioMed Research International
Jimmy A Thomas, Dmitri Vasin, Mercury Lin, Arthur E Anderson, Charles E Alpers
Fibrillary glomerulonephritis (FGN) is a rare cause of rapidly progressive glomerulonephritis (RPGN). We report a case of FGN in which the patient presented with a clinical pulmonary-renal syndrome and whose kidney biopsy showed > 90% crescents on light microscopy. Immunofluorescence microscopy showed pseudo-linear IgG and C3 staining of the glomerular capillary walls resulting in an initial diagnosis of crescentic glomerulonephritis of anti-glomerular basement membrane (anti-GBM) antibody etiology. Electron microscopy showed fibrillary deposits permeating the glomerular capillary walls, characteristic of FGN...
February 2016: Clinical Nephrology
Ya-ting Ge, Jin-lan Liao, Wei Liang, Zu-ying Xiong
BACKGROUND: Anti-glomerular basement membrane disease (anti-GBM disease) is an autoimmune glomerulonephritis disease that is characterized by IgG linear deposition along the non-collagen domain of a3 chains of type IV collagen on the GBM. Although anti-GBM disease accompanied with IgA linear deposition along GBMs was discussed previously in some papers, anti-GBM disease combined with IgA granular deposition in the mesangial area, especially complicated with reversible posterior leukoencephalopathy syndrome (RPLS), was rarely reported...
2015: American Journal of Case Reports
Kiyoki Kitagawa, Kengo Furuichi, Akihiro Sagara, Yasuyuki Shinozaki, Shinji Kitajima, Tadashi Toyama, Akinori Hara, Yasunori Iwata, Norihiko Sakai, Miho Shimizu, Shuichi Kaneko, Takashi Wada
BACKGROUND: The prevention of relapse and infection complications during remission maintenance therapy is required to improve the prognosis of patients with microscopic polyangiitis (MPA) showing rapidly progressive glomerulonephritis (RPGN). The clinicopathological characteristics of patients with ANCA-positive MPA were examined to determine the risk factors for relapse or infectious complications after remission induction therapy. PATIENTS AND METHODS: The study population consisted of 52 patients diagnosed as ANCA-positive MPA showing RPGN from 2002 to 2012, after publication of the Japanese guideline for RPGN...
October 2016: Clinical and Experimental Nephrology
Gonzalo de Luna, Dominique Chauveau, Julien Aniort, Pierre-Louis Carron, Pierre Gobert, Alexandre Karras, Sylvain Marchand-Adam, François Maurier, Pierre-Yves Hatron, Alexandre Mania, Guillaume le Guenno, Stéphane Bally, Boris Bienvenu, Eric Cardineau, Tiphaine Goulenok, Noémie Jourde-Chiche, Maxime Samson, Antoine Huart, Jacques Pourrat, Aurélien Tiple, Olivier Aumaitre, Xavier Puéchal, Farhad Heshmati, Claire le Jeunne, Luc Mouthon, Loïc Guillevin, Benjamin Terrier
The use of plasma exchanges (PLEX) in systemic necrotizing vasculitides (SNV) still need to be codified. To describe indications, efficacy and safety of PLEX for the treatment of SNV, we conducted a multicenter retrospective study on patients with ANCA-associated vasculitis (AAV) or non-viral polyarteritis nodosa (PAN) treated with PLEX. One hundred and fifty-two patients were included: GPA (n = 87), MPA (n = 56), EGPA (n = 4) and PAN (n = 5). PLEX were used for rapidly progressive glomerulonephritis (RPGN) in 126 cases (86%), alveolar hemorrhage in 64 cases (42%), and severe mononeuritis multiplex in 23 cases (15%)...
December 2015: Journal of Autoimmunity
Yoo Hyung Kim, Hae Ri Kim, Young Rok Ham, Jae Woong Jeon, Sarah Chung, Dae Eun Choi, Kang Wook Lee, Ki Ryang Na
Idiopathic membranous glomerulonephritis (IMGN) is commonly diagnosed in adults with proteinuria. Rapid deterioration of renal function is a rare complication of IMGN, except when accompanied by renal vein thrombosis, malignant hypertension, or other underlying disease, including lupus nephritis. Here, we present a case of rapid deterioration of renal function in a patient with MGN superimposed with anti-neutrophil cytoplasmic antibody (ANCA)-associated rapidly progressive crescentic glomerulonephritis (RPGN)...
August 2015: Chonnam Medical Journal
Amr El-Husseini, Juan-Carlos Aycinena, Bennet George, Stuart Jennings, Virgilius Cornea, B Peter Sawaya
Fibrillary glomerulonephritis (FGN) is a rare disorder with poor renal prognosis. It is a heterogeneous disease associated with significant risk of end-stage renal disease (ESRD). Its etiology and pathogenesis have not been clearly identified. We report a case of a patient presenting with hypertensive crisis, nephrotic range proteinuria, and rapidly progressive glomerulonephritis (RPGN). The kidney biopsy demonstrates crescentic GN on light microscopy (LM) and strong pseudo-linear/globular glomerular basement membrane (GBM) staining for immunoglobulin G on immunofluorescence (IF), suggestive of anti-GBM disease...
October 2015: Clinical Nephrology
Rahul Mannan, Pramela Anthony Singh, Vatsala Misra, Mamta Singh, Ravi Mehrotra, Sneh Lata Tewarson, Arvind Gupta, Mridu Manjari
OBJECTIVES: Pauci-immune glomerulonephritis is the commonest cause of rapidly progressive glomerulonephritis (RPGN) which is associated with increased mortality and morbidity. More than 90% of these patients have serological presence of either antineutrophil cytoplasmic antibodies (ANCA), of cytoplamic (C) or perinuclear (P) type. "Immunofluoresence studies" exhibiting minimal or no fluorescence is diagnostic in all such cases. The present study aims to study the differences between renal biopsies of serologically ANCA negative versus ANCA positive individuals...
April 2015: Journal of Clinical and Diagnostic Research: JCDR
Carole Henique, Guillaume Bollee, Olivia Lenoir, Neeraj Dhaun, Marine Camus, Anna Chipont, Kathleen Flosseau, Chantal Mandet, Masayuki Yamamoto, Alexandre Karras, Eric Thervet, Patrick Bruneval, Dominique Nochy, Laurent Mesnard, Pierre-Louis Tharaux
Necrotizing and crescentic rapidly progressive GN (RPGN) is a life-threatening syndrome characterized by a rapid loss of renal function. Evidence suggests that podocyte expression of the transcription factor peroxisome proliferator-activated receptor γ (PPARγ) may prevent podocyte injury, but the function of glomerular PPARγ in acute, severe inflammatory GN is unknown. Here, we observed marked loss of PPARγ abundance and transcriptional activity in glomerular podocytes in experimental RPGN. Blunted expression of PPARγ in podocyte nuclei was also found in kidneys from patients diagnosed with crescentic GN...
January 2016: Journal of the American Society of Nephrology: JASN
George H B Greenhall, Alan D Salama
Rapidly progressive glomerulonephritis (RPGN) results from severe crescentic damage to glomeruli and leads to irreversible kidney failure if not diagnosed and managed in a timely fashion. Traditional treatment has relied on glucocorticoids and cyclophosphamide, with additional plasmapheresis for certain conditions. Here we describe updates in the management of RPGN, according to the underlying renal pathology. However, there remains a paucity of trials that have enrolled patients with more advanced renal disease, dialysis dependence or with RPGN, and we are therefore still reliant on extrapolation of data from studies of patients with a less severe form of disease...
April 2015: Clinical Kidney Journal
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No abstract text is available yet for this article.
2015: Nihon Jinzo Gakkai Shi
S Taghipour Zahir, E Hosseini
Kidneys are complex organs with multiple vital functions. They are an essential part of the urinary system and also are necessary for regulation of body homeostasis like electrolytes, acid base balance and blood pressure. Diagnosis of renal injuries is based on clinical and histopathologic features. In a descriptive cross-sectional study conducted in Shahid Sadoughi Hospital, Yazd, Iran, pathology reports of all renal biopsies, by light microscopic examination, immunofluorecence and electron microscopy (EM) were perused...
October 2014: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
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