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idiopathic membranous

Rossana Malatesta-Muncher, Karen W Eldin, Laurence H Beck, Mini Michael
BACKGROUND: Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab...
March 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Motohiro Tamiya, Hidekazu Suzuki, Takayuki Shiroyama, Ayako Tanaka, Naoko Morishita, Norio Okamoto, Kenichi Sakai, Hironori Shigeoka, Kunimitsu Kawahara, Tomonori Hirashima
Background Bevacizumab (Bev) is generally well-tolerated, and Bev-associated intestinal perforation (BAP) is a rare albeit serious side effect in cases of non-small cell lung cancer (NSCLC). Therefore, the present study aimed to identify clinical predictors of BAP to help predict and manage the development of life-threatening intestinal complications among patients receiving Bev. Methods This retrospective study evaluated demographic, clinical, and treatment factors for patients with NSCLC who were treated with Bev between February 2010 and August 2015 at our center...
March 14, 2018: Investigational New Drugs
Ping Xu, Yu-di He, Ze-Ming Yu, Kai Luo, Huai-Ya Xie, Pei-Mei Zou, Xiao Gu, Shi-Rui Wang, Jian-Fang Cai, Qun Xu, Hang Li, Xue-Wang Li
Objective To compare the efficacy and safety of tacrolimus with those of cyclosporine in treating idiopathic membranous nephropathy (IMN) via network meta-analysis. Methods Databases including PubMed,Embase,CENTRAL (Cochrane),Wanfang Database,CNKI,and VIP citation database were searched for relevant studies according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Package Meta 4.5.0 and Gemtc 0.8.1 in R 3.3.1 were used to analyze the included studies. Results In this network meta-analysis,the complete remission rate (RR=0...
February 28, 2018: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
Irem Serifoglu, Balam Er Dedekarginoglu, Ebru Hatice Ayvazoglu Soy, Gaye Ulubay, Mehmet Haberal
OBJECTIVES: Hemoptysis is a symptom that can be caused by airway disease, pulmonary parenchymal disease, or pulmonary vascular disease, or it can be idiopathic. Infection is the most common cause of hemoptysis, accounting for 60% to 70% of cases. Hemoptysis is also an initial symptom of diffuse alveolar hemorrhage syndrome, although it may be absent at presentation in one-third of patients. Diffuse alveolar hemorrhage is characterized by disruption of the alveolar-capillary basement membranes because of either injury or inflammation of the arterioles, venules, or capillaries, resulting in bleeding in alveolar spaces...
March 2018: Experimental and Clinical Transplantation
Ilan Rozenberg, Andres Kotliroff, Tania Zahavi, Sydney Benchetrit
BACKGROUND: Idiopathic membranous nephropathy (IMN) is one of the most common causes of nephrotic syndrome (NS) in Caucasian adults. Most patients have good renal prognosis, but 30-40% may progress to end stage renal disease (ESRD). OBJECTIVES: To evaluate the efficacy and safety of immunosuppressive treatment (IST) in high-risk patients. METHODS: All IMN patients diagnosed by kidney biopsy from 2004-2010 were included. Clinical and laboratory data were collected at each follow-up visit...
March 2018: Israel Medical Association Journal: IMAJ
Eunjin Bae, Sung Woo Lee, Seokwoo Park, Dong Ki Kim, Hajeong Lee, Hyuk Huh, Ho Jun Chin, Shina Lee, Dong-Ryeol Ryu, Ji In Park, Sejoong Kim, Dong Jun Park, Shin-Wook Kang, Yon Su Kim, Yun Kuy Oh, Yong Chul Kim, Chun Soo Lim, Jung Tak Park, Jung Pyo Lee
Idiopathic membranous nephropathy (MN) is the most common glomerulonephritis in elderly patients showing nephrotic syndrome. However, little is known about its treatment options and outcomes in elderly MN patients at long term follow-up. We retrospectively enrolled patients with biopsy-proven MN between April 1990 and December 2015 from eight tertiary hospitals in Korea. Among them, we excluded patients who had secondary causes of MN and subnephrotic-range proteinuria. We evaluated the presenting features and clinical outcomes and analyzed the all-cause mortality, renal outcomes, infection, and remission with respect to age...
March 3, 2018: Archives of Gerontology and Geriatrics
Jinglan Zhang, Jiakai Lu, Xiaorui Zhou, Xuefeng Xu, Qing Ye, Qitan Ou, Yanna Li, Jiapeng Huang
OBJECTIVES: The mortality of pregnant women with idiopathic pulmonary arterial hypertension (PAH) is very high. There are limited data on the management of idiopathic PAH during pregnancy. The authors aimed to examine systematically the characteristics of parturient women with idiopathic PAH, to explore the adverse effects of idiopathic PAH on pregnancy outcomes, and to report the multidisciplinary perioperative management strategy from the largest comprehensive cardiac hospital in China...
March 7, 2018: Journal of Cardiothoracic and Vascular Anesthesia
Y F Dong, L W Sun, B Zhang, X Y Kuang, X L Niu, Y L Kang, S Hao, P Wang, Z Li, G H Zhu, W Y Huang, Y Wu
Objective: To explore the clinical features and expression of PLA(2)R in renal tissue of children with idiopathic membranous nephropathy. Methods: Retrospective study was performed in patients with membranous nephropathy diagnosed through renal biopsy and the follow-up time was at least half a year in Shanghai Children ' s Hospital from January 2010 to February 2017. We compared their clinicopathological and pathological findings of IMN. Indirect immunofluorescence assay was used to detect glomerular PLA(2)R expression...
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Caspar Geenen, Declan C Murphy, Maria T Sandinha, Jon Rees, David H W Steel
PURPOSE: To investigate the association between the vertical elevation of the external limiting membrane (ELM) and visual outcome in patients undergoing surgery for idiopathic full-thickness macular hole. METHODS: Retrospective observational study of a consecutive cohort of patients undergoing vitrectomy to treat macular hole. The greatest vertical height of the central ELM above the retinal pigment epithelium (ELM height) was measured on spectral domain optical coherence tomography preoperatively...
March 5, 2018: Retina
Suhwan Lee, June-Gone Kim
PURPOSE: To investigate the impact of preserving the foveal flap on surgical outcomes of full-thickness macular hole (MH) with foveal flaps. METHODS: We retrospectively reviewed patients with Stages 2 and 3 idiopathic MH, who underwent pars plana vitrectomy by a single surgeon at Asan Medical Center from November 2011 to November 2016. In the study group, we included eyes with MH and a foveal flap on preoperative spectral domain optical coherence tomography and successfully preserved the flap during surgery...
March 5, 2018: Retina
Agnieszka Pozdzik, Isabelle Brochériou, Cristina David, Fahd Touzani, Jean Michel Goujon, Karl Martin Wissing
The discovery of circulating antibodies specific for native podocyte antigens has transformed the diagnostic workup and greatly improved management of idiopathic membranous nephropathy (iMN). In addition, their identification has clearly characterized iMN as a largely autoimmune disorder. Anti-PLA2R1 antibodies are detected in approximately 70% to 80% and anti-THSD7A antibodies in only 2% of adult patients with iMN. The presence of anti-THSD7A antibodies is associated with increased risk of malignancy. The assessment of PLA2R1 and THSD7A antigen expression in glomerular immune deposits has a better sensitivity than measurement of the corresponding autoantibodies...
2018: BioMed Research International
Jiannan Li, Zhao Cui, Jianyan Long, Wei Huang, Jinwei Wang, Hong Zhang, Haibo Wang, Luxia Zhang, Pierre Ronco, Ming-Hui Zhao
Background: Primary glomerular nephropathy (PGN) is a leading cause of end-stage renal disease in developing countries, however, nationwide studies investigating the spectrum of PGN are limited. Methods: To evaluate the composition of various pathological types of PGN in a comprehensive geographic coverage in China, a national inpatient database covering 43.7 million patients was used in this analysis. Diagnoses were extracted from the discharge diagnoses. Results: The percentage of patients with PGN in hospitalized patients decreased from 0...
March 2, 2018: Nephrology, Dialysis, Transplantation
Jingli Guo, WenYi Tang, Xiaofeng Ye, Haixiang Wu, Gezhi Xu, Wei Liu, Yongjin Zhang
BACKGROUND: To evaluate the structural changes associated with visual acuity (VA) in patients with idiopathic macular telangiectasia (MT) type 1 using multimodal imaging modalities. METHODS: A retrospective study of 14 patients with MT type 1 and of 10 eyes from 10 healthy individuals as age-matched controls was conducted. The medical records of patients who had undergone colour fundus photography, spectral domain optical coherence tomography (OCT), fluorescein angiography and OCT angiography were reviewed...
March 5, 2018: BMC Ophthalmology
Tomohiko Akahori, Takeshi Iwase, Kentaro Yamamoto, Eimei Ra, Kenichi Kawano, Yasuki Ito, Hiroko Terasaki
PURPOSE: To determine whether the macula is displaced after vitrectomy with internal limiting membrane (ILM) peeling in eyes with an idiopathic macular hole. DESIGN: A retrospective, observational case series METHODS: Optical coherence tomography angiography (OCTA) was used to obtain 3 mm × 3 mm en face images before and 2, 4, and 8 weeks after the vitrectomy with ILM peeling for 20 eyes of 20 patients with an idiopathic macular hole. The displacements of easily identifiable retinal vessel bifurcations were measured relative to the fovea and the optic disc...
March 2, 2018: American Journal of Ophthalmology
Yanfang Duan, Jing Qian, Kun Chen, Zhuo Zhang
RATIONALE: Pigmented villonodular synovitis (PVNS) is an idiopathic, proliferative disorder lesion of synovial tissue, which is regarded as a benign disease, but has a local invasion. Up to now, these are no consensus about the etiology and pathogenesis of PVNS. Because of the lack of typical clinical features, misdiagnosis and delayed diagnosis are not uncommon, magnetic resonance imaging (MRI) can assist diagnosis and histopathological examination is recognized as the gold standard for the final diagnosis...
January 2018: Medicine (Baltimore)
Roland Hetzer, Mariano Francisco Del Maria Javier, Eva Maria Delmo Walter
Background: While heart transplantation has gained recognition as the gold standard therapy for advanced heart failure, the scarcity of donor organs has become an important concern. The evolution of surgical alternatives such as ventricular assist devices (VADs), allow for recovery of the myocardium and ensure patient survival until heart transplantation becomes possible. This report elaborates the role of VADs as a bridge to heart transplantation in infants and children (≤18 years old) with end-stage heart failure...
January 2018: Annals of Cardiothoracic Surgery
Felix Claverie-Martin, Jorge Trujillo-Suarez, Hilaria Gonzalez-Acosta, Cristina Aparicio, Maria L Justa Roldan, Blanka Stiburkova, Kimiyoshi Ichida, Maria A Martín-Gomez, Maria Herrero Goñi, Marta Carrasco Hidalgo-Barquero, Victoria Iñigo, Ricardo Enriquez, Elizabeth Cordoba-Lanus, Victor M Garcia-Nieto
BACKGROUND: Renal hypouricemia (RHUC), a rare inherited disorder characterized by impaired uric acid (UA) reabsorption in the proximal tubule, is caused by mutations in SLC22A12 or SLC2A9. Most mutations have been identified in Japanese patients, and only a few have been detected in Europeans. METHODS: We report clinical, biochemical and genetics findings of fourteen Spanish patients, six Caucasians and eight of Roma ethnia, diagnosed with idiopathic RHUC. Two of the patients presented exercise-induced acute renal failure and another one had several episodes of nephrolithiasis and four of them had progressive deterioration of renal function, while the rest were asymptomatic...
February 24, 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
Weng Hon Lee, Adrian Tey, Kevin Gales, Hardeep Singh Mudhar, Francisco C Figueiredo
AIM: To describe a cohort of patients with irreversible unilateral bullous keratopathy (BK) of undetermined aetiology. METHOD: Retrospective, single-centre case series in a tertiary corneal referral centre. RESULTS: Eleven consecutive patients (nine females; mean age 71.7 years) presented from 1999 to 2009 with acute onset unilateral visual loss. At presentation, the best-corrected visual acuity of the affected eyes was 6/9 or worse with mean central corneal thickness (CCT) of 684 (SD 66) μm...
February 26, 2018: British Journal of Ophthalmology
Vigdis Andersen Eidsvaag, Hans-Arne Hansson, Kjell Heuser, Erlend A Nagelhus, Per Kristian Eide
AIM: Idiopathic intracranial hypertension (IIH) is characterized by symptoms indicative of increased intracranial pressure (ICP), such as headache and visual impairment. We have previously reported that brain biopsies from IIH patients show patchy astrogliosis and increased expression of the water channel aquaporin-4 (AQP4) at perivascular astrocytic endfeet. METHODS: The present study was undertaken to investigate for ultrastructural changes of the cerebral capillaries in individuals with IIH...
February 22, 2018: Brain Research
Junguee Lee, Dong Hyun Oh, Ki Cheol Park, Ji Eun Choi, Jong Beom Kwon, Jongho Lee, Kuhn Park, Hae Joung Sul
Primary cilia are solitary, non-motile, axonemal microtubule-based antenna-like organelles that project from the plasma membrane of most mammalian cells and are implicated in transducing hedgehog signals during development. It was recently proposed that aberrant SHH signaling may be implicated in the progression of idiopathic pulmonary fibrosis (IPF). However, the distribution and role of primary cilia in IPF remains unclear. Here, we clearly observed the primary cilia in alveolar epithelial cells, fibroblasts, and endothelial cells of human normal lung tissue...
February 23, 2018: Molecules and Cells
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