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Recurrent glomerulonephritis

Paola Bordi, Marcello Tiseo, Giorgio Baldari, Sebastiano Buti
BACKGROUND: Patients undergone kidney transplantation present higher risk of Urothelial Carcinoma (UC) development and represent a subgroup of special interest. To date, vinflunine is the only drug approved in Europe for the treatment of advanced UC after failure of platinum-based chemotherapy. However, to our knowledge, no data on the concomitant administration of vinflunine and immunosuppressive agents are available. CASE PRESENTATION: The patient, a 45 years old Caucasian male, presented poorly differentiated UC of the bladder recurred after initial cystectomy with abdominal lymphadenopathies evidenced by FDG-PET/CT...
2016: BMC Cancer
Sepideh Darougar, Farin Rashid Farokhi, Shaghayegh Tajik, Nooshin Baghaie, Mehrdad Amirmoini, Bahram Bashardoust, Seyed Karen Hashemitari, Seyed Alireza Mahdaviani
Chronic granulomatous disease is a rare primary immunodeficiency disorder, which leads to increased susceptibility to recurrent infections and severe inflammatory manifestations.  There have been reports regarding different aspects of genitourinary involvement in chronic granulomatous disease, some of which are hydronephrosis, granulomatous cystitis, and glomerulonephritis, but among these complications, amyloidosis is rather rare. We report a patient with chronic granulomatous disease that developed amyloidosis later in the course of the disease...
July 2016: Iranian Journal of Kidney Diseases
Rui Fernandes, Sara Freitas, Pedro Cunha, Gloria Alves, Jorge Cotter
BACKGROUND: Goodpasture's syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described. CASE PRESENTATION: The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours...
2016: Journal of Medical Case Reports
Eun Jung Kim, Dong Ho Shin, Hee Jung Jeon, So Yon Rhee, Eun Sook Nam, Ji Young Park, Jieun Oh
POEMS syndrome is a rare paraneoplastic syndrome and there are few reports of polyneuropathy and monoclonal gammopathy associated with kidney dysfunction. Here, we report a case of POEMS syndrome with recurrent acute kidney injury (AKI). A 52-year-old man presented with bilateral aggravating paresthesia and latermotor weakness of the lower extremities accompanied by repeated elevation of serum creatinine. The patient was finally diagnosed with POEMS syndrome on the basis of fulfilling the two mandatory major criteria (polyneuropathy and monoclonal gammopathy), one other major criterion (sclerotic bone lesion), and several minor criteria...
June 2016: Electrolyte & Blood Pressure: E & BP
Fahad Edrees, Robert M Black, Laszlo Leb, Helmut Rennke
Kidney injury associated with lymphoproliferative disorders is rare, and the exact pathogenetic mechanisms behind it are still poorly understood. Glomerular involvement presenting as a nephrotic syndrome has been reported, usually secondary to membranoproliferative glomerulonephritis. We report a case of a 63-year-old male who presented with bilateral leg swelling due to nephrotic syndrome and acute kidney injury. A kidney biopsy showed minimal change disease with light chain deposition; however, no circulating light chains were present...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
Kamel El-Reshaid, Jozsef Varro, John P Madda
Lymph node involvement as a part of systemic vasculitis is rare. We report a case of women with rapidly progressive renal disease associated with recurrent epigastric pain, weight loss, and massive hilar as well as para-aortic lymphadenopathy. Ultrasound-guided biopsy of her scarred kidneys revealed antineutrophil cytoplasmic antibody-negative crescentic glomerulonephritis and that of lymph nodes showed severe necrotizing vasculitis. Biopsy of the lymph nodes and the failing kidney established the diagnosis of this rare presentation and ruled out lymphoma and tuberculosis...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
Mitsutoshi Shindo, Susumu Ookawara, Kiyonori Ito, Taisuke Kitano, Sawako Goto, Haruhisa Miyazawa, Yuichiro Ueda, Yoshio Kaku, Keiji Hirai, Taro Hoshino, Honami Mori, Izumi Yoshida, Kaoru Tabei, Yoshiyuki Morishita
A 68-year-old Japanese man was diagnosed with left otitis media with effusion and left uveitis more than 5 months before admission. He was urgently admitted to our hospital for progressive deterioration of his renal function [serum creatinine(Cr) 7.59 mg/dL] with proteinuria and urinary red blood cell casts, inflammation, and anemia. Additionally, his serum proteinase 3 antinuclear antibody (PR3-ANCA) level, determined by using the chemiluminescence enzyme immunoassay method, had increased to more than 3,500 U/mL...
2016: Nihon Jinzo Gakkai Shi
Oliver R Mikse, Jeremy H Tchaicha, Esra A Akbay, Liang Chen, Roderick T Bronson, Peter S Hammerman, Kwok-Kin Wong
Recurrent fusion of the v-myb avian myelobastosis viral oncogene homolog (MYB) and nuclear factor I/B (NFIB) generates the MYB-NFIB transcription factor, which has been detected in a high percentage of individuals with adenoid cystic carcinoma (ACC). To understand the functional role of this fusion protein in carcinogenesis, we generated a conditional mutant transgenic mouse that expresses MYB-NFIB along with p53 mutation in tissues that give rise to ACC: mammary tissue, salivary glands, or systemically in the whole body...
May 31, 2016: Oncotarget
Nobuko Sakai, Yukihiko Kawasaki, Tomoko Waragai, Tomoko Oikawa, Masatoshi Kaneko, Tomoko Sato, Kazuhide Suyama, Mitsuaki Hosoya
Immunoglobulin A nephropathy (IgAN) is the most common form of chronic glomerulonephritis worldwide. In Japan, the treatment for use as an initial therapy was established in Guidelines for the Treatment of Childhood IgA nephropathy; however, no rescue therapy for recurrent or steroid-resistant pediatric IgAN was established. We report here a 15-year-old boy with severe IgAN, who was treated with combination therapy involving prednisolone, mizoribine, warfarin, and dilazep dihydrochloride for 2 years. The response to the combination therapy was good and both proteinuria and hematuria disappeared...
June 8, 2016: Fukushima Journal of Medical Science
S Morkhandikar, P S Priyamvada, B H Srinivas, S Parameswaran
Thrombosis of the cerebral venous sinuses (CVT) is described in nephrotic syndrome. A 13-year-old girl was admitted with acute post-infectious glomerulonephritis (APIGN). Subsequently she developed recurrent seizures with focal neurological deficits. On evaluation, she was found to have CVT. To the best of our knowledge, this is the first report of CVT in APIGN. Identifying this complication is imperative, as timely diagnosis and treatment could be lifesaving.
May 2016: Indian Journal of Nephrology
Beth Vogt
Although kidney disease is much less common in children than in adults, clinicians must remain alert for the renal conditions that occur in this population because prompt diagnosis and management are essential. Acute postinfectious glomerulonephritis occurs as an uncommon sequela of streptococcal and other infections. Management is focused on providing supportive measures, including management of fluid-electrolyte imbalance and hypertension, until the patient recovers. Immunoglobulin (Ig) A nephropathy is a primary glomerulonephritis related to abnormal IgA glycosylation...
May 2016: FP Essentials
Danveer Bhadu, Puneet Kumar, Kiran Preet Malhotra, Aarti Sharma, Meha Sharma, Durgesh Srivastava
Microscopic polyangiitis is a small vessel vasculitis, associated with myeloperoxidaseantineutrophil cytoplasmic antibody. It rarely occurs in children. Central nervous system involvement in pediatric microscopic polyangiitis is not a well known entity with perhaps only five cases till date. We hereby present a 14-year-old girl with arthralgia, seizure, leukocytoclastic vasculitis, interstitial lung disease secondary to recurrent pulmonary hemorrhage, pauci-immune glomerulonephritis and high titers of MPO-ANCA, hence diagnostic of microscopic polyangiitis...
February 18, 2016: Acta Reumatológica Portuguesa
Ying Chen, Jinquan Wang, Feng Xu, Caihong Zeng, Zhihong Liu
AIMS: Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment. MATERIALS AND METHODS: Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively. RESULTS: The patients consisted of 27 males and 2 females, with an average age of 35...
June 2016: Clinical Nephrology
Ryan J Goldberg, Francis L Weng, Praveen Kandula
Allograft dysfunction after a kidney transplant is often clinically asymptomatic and is usually detected as an increase in serum creatinine level with corresponding decrease in glomerular filtration rate. The diagnostic evaluation may include blood tests, urinalysis, transplant ultrasonography, radionuclide imaging, and allograft biopsy. Whether it occurs early or later after transplant, allograft dysfunction requires prompt evaluation to determine its cause and subsequent management. Acute rejection, medication toxicity from calcineurin inhibitors, and BK virus nephropathy can occur early or later...
May 2016: Medical Clinics of North America
Kerstin Ebefors, Peidi Liu, Emelie Lassén, Johannes Elvin, Emma Candemark, Kristina Levan, Börje Haraldsson, Jenny Nyström
BACKGROUND: IgA nephropathy (IgAN) is the most common glomerulonephritis in the world, affecting close to a million people. Circulating galactose-deficient IgA (gd-IgA), present in patients with IgAN, form immune complex deposits in the glomerular mesangium causing local proliferation and matrix expansion. Intriguing though, individuals having gd-IgA deposits in the kidneys do not necessarily have signs of glomerular disease. Recurrence of IgAN only occurs in less than half of transplanted patients with IgAN, indicating that gd-IgA is not the only factor driving the disease...
April 5, 2016: BMC Nephrology
Brian T Brinkerhoff, Donald C Houghton, Megan L Troxell
Renal injury in hematopoietic cell transplant recipients may be related to a combination of factors including chemotherapy, radiation, infection, immunosuppressive agents, ischemia, and graft-versus-host disease, and can involve glomerular, tubulointerstitial, and vascular structures. We reviewed renal pathology from 67 patients at a single institution (2009-2014), including 14 patients with biopsy for clinical dysfunction, 6 patients with surgical kidney resection for other causes, and 47 autopsy patients...
June 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Maria Messina, Ester Gallo, Alberto Mella, Fabiola Pagani, Luigi Biancone
Focal segmental glomerulosclerosis (FSGS) represents one of the most severe glomerular diseases, with frequent progression to end-stage renal disease and a high rate of recurrence in renal allografts (30%-50%). Recurrent FSGS portends a negative outcome, with the hazard ratio of graft failure being two-fold higher then that of other glomerulonephritis. Two patterns of clinical presentations are observed: Early recurrence, which is characterized by massive proteinuria within hours to days after implantation of the renal graft, and late recurrence, which occurs several months or years after the transplantation...
March 24, 2016: World Journal of Transplantation
Rohit Tewari, Kusum Joshi, Ashwani Kumar, C S Rayat, Rajaram Iyer, Vinay Sakhuja, Mukut Minz
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMIDs) is a clinico-pathologic entity, the recurrence of which in the renal allograft has only recently been described. A 55-year-old male presented with rapid deterioration of renal function. Light microscopy showed membranoproliferative glomerulonephritis with kappa light chain restriction and only one sub-class of IgG. He subsequently underwent renal transplant. Two months later, he developed acute graft dysfunction. Renal biopsy showed a recurrence of the disease...
March 2016: Saudi Journal of Kidney Diseases and Transplantation
Ji Hae Nahm, Seung Hwan Song, Yu Seun Kim, Hae-Il Cheong, Beom Jin Lim, Beom Seok Kim, Hyeon Joo Jeong
C3 glomerulonephritis (C3GN) is a recently described, rare glomerular disease characterized by predominant or sole glomerular C3 deposits. Morphologic features of C3GN are similar to those of dense deposit disease (DDD); however, ribbon-like intramembranous electron-dense deposits are absent in the former. We report a case of de novo C3GN in a renal allograft with morphologic transformation to DDD. A 6-year-old boy presented with congenital left renal agenesis and right ureteropelvic junction obstruction. The patient underwent pyeloplasty but experienced recurrent urinary tract infections...
2016: Ultrastructural Pathology
Megan L Troxell, Donald C Houghton
BACKGROUND: Anti-glomerular basement membrane (anti-GBM) disease classically presents with aggressive necrotizing and crescentic glomerulonephritis, often with pulmonary hemorrhage. The pathologic hallmark is linear staining of GBMs for deposited immunoglobulin G (IgG), usually accompanied by serum autoantibodies to the collagen IV alpha-3 constituents of GBMs. METHODS: Renal pathology files were searched for cases with linear anti-GBM to identify cases with atypical or indolent course...
April 2016: Clinical Kidney Journal
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