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https://www.readbyqxmd.com/read/28092354/use-of-defibrotide-to-treat-transplant-associated-thrombotic-microangiopathy-a-retrospective-study-of-the-paediatric-diseases-and-inborn-errors-working-parties-of-the-european-society-of-blood-and-marrow-transplantation
#1
L Yeates, M A Slatter, S Bonanomi, F L W I Lim, S Y Ong, A Dalissier, W Barberi, A Shulz, M Duval, C Heilmann, A Willekens, W H Y Hwang, C Uderzo, P Bader, A R Gennery
No abstract text is available yet for this article.
January 16, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28089409/the-italian-registry-of-therapeutic-apheresis-2015
#2
REVIEW
Giustina De Silvestro
Data collection on apheresis activities in Italy throughout 2015 including techniques, clinical indications and adverse effects was performed by means of a SidEM model questionnaire (aggregate data). These data provided by 67 Apheresis Units from 17 Italian regions, albeit rough, are sufficiently informative, mainly in comparison with previous surveys on these statistics. PEx has been the most frequent technique for plasma removal/treatment (11787 procedures) followed by LDL-apheresis (927 procedures). ECP/photopheresis has been the most frequent cytapheresis procedure: 6,606 session, mainlyby using the off-line technique...
December 29, 2016: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28078430/blood-urea-nitrogen-to-serum-creatinine-ratio-is-an-accurate-predictor-of-outcome-in-diarrhea-associated-hemolytic-uremic-syndrome-a-preliminary-study
#3
Werner Keenswijk, Jill Vanmassenhove, Ann Raes, Evelyn Dhont, Johan Vande Walle
: Diarrhea-associated hemolytic uremic syndrome (D+HUS) is a common thrombotic microangiopathy during childhood and early identification of parameters predicting poor outcome could enable timely intervention. This study aims to establish the accuracy of BUN-to-serum creatinine ratio at admission, in addition to other parameters in predicting the clinical course and outcome. Records were searched for children between 1 January 2008 and 1 January 2015 admitted with D+HUS. A complicated course was defined as developing one or more of the following: neurological dysfunction, pancreatitis, cardiac or pulmonary involvement, hemodynamic instability, and hematologic complications while poor outcome was defined by death or development of chronic kidney disease...
January 11, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28075427/evaluation-of-sudomotor-function-in-adult-patients-with-long-lasting-type-1-diabetes
#4
Agnieszka Gandecka, Aleksandra Araszkiewicz, Stanisław Piłaciński, Bogna Wierusz-Wysocka, Dorota Zozulińska-Ziółkiewicz
INTRODUCTION    The function of sweat glands appears to be impaired in patients with diabetic complications.  OBJECTIVES    The aim of the study was to evaluate the sudomotor function in adult patients with type 1 diabetes (DM1) and healthy controls and its relationship with metabolic control and diabetic complications. PATIENTS AND METHODS    The study included 404 adult patients with DM 1 (194 women) aged 41 years (IQR: 32-51), disease duration 23 years (IQR: 18-31). Electrochemical skin conductance (ESC) was measured with SUDOSCAN+ on feet and hands in the study group and in the control group of 84 healthy volunteers...
January 10, 2017: Polskie Archiwum Medycyny Wewnętrznej
https://www.readbyqxmd.com/read/28072696/cerebroretinal-microangiopathy-with-calcifications-and-cysts-a-case-report
#5
Wenrui Xu, Jiuliang Zhao, Yicheng Zhu, Weihong Zhang
RATIONAL: Cerebroretinal microangiopathy with calcifications and cysts (CRMCC) is believed to be an autosomal recessive genetic disease, with disorders in multisystem organs. Its characteristic neurological disorders manifested on neuroimaging are a triad of leukoencephalopathy, intracranial calcifications, and parenchymal cysts. In this paper, we report a CRMCC patient with multisystem involvement, focusing on the neuroimaging features, to get a better understanding of the rare disease and improve our diagnostic ability...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28072480/free-fatty-acids-induce-autophagy-and-lox-1-upregulation-in-cultured-aortic-vascular-smooth-muscle-cells
#6
Cheng-I Cheng, Yueh-Hong Lee, Po-Han Chen, Yu-Chun Lin, Ming-Huei Chou, Ying-Hsien Kao
Elevation of free fatty acids (FFAs) is known to affect microvascular function and contribute to obesity-associated insulin resistance, hypertension, and microangiopathy. Proliferative and synthetic vascular smooth muscle cells (VSMCs) increase intimal thickness and destabilize atheromatous plaques. This study aimed to investigate whether saturated palmitic acid (PA) and monounsaturated oleic acid (OA) modulate autophagy activity, cell proliferation, and vascular tissue remodeling in an aortic VSMC cell line...
November 5, 2016: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28071761/plasma-heme-induced-renal-toxicity-is-related-to-a-capillary-rarefaction
#7
Nahid Tabibzadeh, Céline Estournet, Sandrine Placier, Joëlle Perez, Héloïse Bilbault, Alexis Girshovich, Sophie Vandermeersch, Chantal Jouanneau, Emmanuel Letavernier, Nadjib Hammoudi, François Lionnet, Jean-Philippe Haymann
Severe hypertension can lead to malignant hypertension (MH) with renal thrombotic microangiopathy and hemolysis. The role of plasma heme release in this setting is unknown. We aimed at evaluating the effect of a mild plasma heme increase by hemin administration in angiotensin II (AngII)-mediated hypertensive rats. Prevalence of MH and blood pressure values were similar in AngII and AngII + hemin groups. MH rats displayed a decreased renal blood flow (RBF), increased renal vascular resistances (RVR), and increased aorta and interlobar arteries remodeling with a severe renal microcirculation assessed by peritubular capillaries (PTC) rarefaction...
January 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28065883/down-regulation-of-proangiogenic-microrna-126-and-microrna-132-are-early-modulators-of-diabetic-cardiac-microangiopathy
#8
Shruti Rawal, Pujika Emani Munasinghe, Amol Shindikar, Jono Paulin, Vicky Cameron, Patrick Manning, Michael J A Williams, Gregory T Jones, Richard Bunton, Ivor Galvin, Rajesh Katare
AIM: Microangiopathy due to endothelial dysfunction is a major contributing factor to the development of diabetes-induced cardiovascular disease (CVD). Dysregulation of endothelial-specific microRNAs (miRs) is correlated with impaired angiogenesis and cell survival. We investigated the profile of two angiomiRs, miR-126, and miR-132, in the plasma of type 2 diabetic individuals without any known history of CVD as well as in the cardiac tissues collected from diabetics undergoing cardiac surgery...
January 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28064326/diabetic-patients-are-at-a-higher-risk-of-lacunar-infarction-and-dyslipidemia-results-of-a-comparative-pilot-study-from-king-fahad-hospital-of-the-university-saudi-arabia
#9
Azra Zafar
OBJECTIVE: To describe the various risk factors and identify the characteristics of ischemic stroke in diabetic patients. METHODS: A retrospective study carried out at King Fahd Hospital of the University in Al-Khobar, kingdom of Saudi Arabia from February 2010 to December 2015. Statistical analysis was performed by the Statistical Package for the Social Sciences (version 22.0, SPSS Inc, Chicago, IL, USA). RESULTS: One hundred and twenty-seven diabetic patients with ischemic stroke were compared with 127 non-diabetics...
January 2017: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/28060108/diagnostic-utility-of-complement-immunohistochemical-studies-in-post-stem-cell-transplant-intestinal-thrombotic-microangiopathy-case-report
#10
Jenna E Rossoff, Jennifer Schneiderman, Sonali Chaudhury, Nicoleta C Arva
Thrombotic complications are a significant source of morbidity and mortality following hematopoietic stem cell transplants. Among them, transplant-associated thrombotic microangiopathy (TA-TMA) is a well-recognized syndrome that can affect various organ systems. Its etiology is related to endothelial injury accompanied by complement activation. As many of the signs and symptoms of the disease are also encountered in other complications following hematopoietic stem cell transplant, it can often be difficult to establish the diagnosis based on clinical data alone...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28057640/murine-systemic-thrombophilia-and-hemolytic-uremic-syndrome-from-a-factor-h-point-mutation
#11
Yoshiyasu Ueda, Imran Mohammed, Delu Song, Damodar Gullipalli, Lin Zhou, Sayaka Sato, Yuan Wang, Shuchi Gupta, Zhongjian Cheng, Hong Wang, Jialing Bao, Yingying Mao, Lawrence Brass, X Long Zheng, Takashi Miwa, Matthew Palmer, Joshua Dunaief, Wen-Chao Song
Complement plays a key role in host defense but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic uremic syndrome, a type of thrombotic microangiopathy (TMA) that causes renal failure in patients. We describe here that disruption of FH function on the cell surface can also lead to disseminated complement-dependent macrovascular thrombosis...
January 5, 2017: Blood
https://www.readbyqxmd.com/read/28050002/pulmonary-tumor-thrombotic-microangiopathy-caused-by-a-parotid-tumor-early-antemortem-diagnosis-and-long-term-survival
#12
Haruka Toyonaga, Michiko Tsuchiya, Chikara Sakaguchi, Hitomi Ajimizu, Yosuke Nakanishi, Seiya Nishiyama, Noboru Morikawa, Yasuyuki Hayashi, Yukio Nagasaka, Hiroshi Yasui
Pulmonary tumor thrombotic microangiopathy (PTTM) is a high-mortality disease that is difficult to diagnose clinically. Our patient was an 80-year-old woman who came to us due to symptoms of increasing dyspnea. A clinical evaluation showed that she had hypoxemia and pulmonary arterial hypertension without any abnormalities in the major pulmonary arteries, bronchi, or alveoli. A lung perfusion scan showed multiple wedge-shaped perfusion defects. Further examination revealed adenocarcinoma in her right parotid gland with metastasis to the submandibular lymph nodes...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28036112/protocol-of-glucose-control-safety-and-efficacy-in-type-2-diabetes-a-network-meta-analysis-glucose-dinet-protocol-rational-and-design
#13
REVIEW
Guillaume Grenet, Audrey Lajoinie, Shams Ribault, Gia Bao Nguyen, Thomas Linet, Augustin Metge, Catherine Cornu, Michel Cucherat, Philippe Moulin, François Gueyffier
The aim of this study is to propose a ranking of the currently available antidiabetic drugs, regarding vascular clinical outcomes, in type 2 diabetic patients, through a network meta-analysis approach. Randomized clinical trials, regardless of the blinding design, testing contemporary antidiabetic drugs and considering clinically relevant outcomes in patients with type 2 diabetes mellitus will be included. The primary outcomes of this analysis will be overall mortality, cardiovascular mortality, and major cardiovascular events...
December 30, 2016: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/28035470/the-clinical-and-laboratory-features-of-chinese-han-anti-factor-h-autoantibody-associated-hemolytic-uremic-syndrome
#14
Di Song, Xiao-Rong Liu, Zhi Chen, Hui-Jie Xiao, Jie Ding, Shu-Zhen Sun, Hong-Yan Liu, Wei-Yi Guo, Su-Xia Wang, Feng Yu, Ming-Hui Zhao
BACKGROUND: Anti-complement factor H (CFH) autoantibody-associated hemolytic uremic syndrome (HUS) is a severe sub-type of HUS. METHODS: We assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric patients with this sub-type of HUS. Thirty-three consecutive patients with acute kidney injury who tested positive for serum anti-CFH autoantibodies were enrolled in this study. RESULTS: All of the eight patients who underwent renal biopsies presented with changes typical of thrombotic microangiopathy, especially changes in chronic characteristics...
December 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28034515/-splenic-late-infarction-after-laparoscopic-gastrectomy-a-case-report
#15
Víctor Soriano-Giménez, David Ruiz de Angulo-Martín, Vicente Munítiz-Ruiz, María de Los Ángeles Ortiz-Escandell, Luisa Fernanda Martínez-de Haro, Pascual Parrilla-Paricio
INTRODUCTION: Laparoscopic gastrectomy has emerged in recent years as an effective technique for the treatment of morbid obesity due to low mortality morbidity rates. Its complications include dehiscence suture line, and others such as splenic infarction. We discuss a case of splenic infarction after laparoscopic gastrectomy. CLINICAL CASE: 45 year old male with a BMI of 37.8 kg/m(2), diabetes-II for 15 years, the last five in treatment with insulin, a fasting blood glucose around 140mg/dl, HbA1c of 7...
December 26, 2016: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/28033278/delayed-onset-of-posterior-reversible-encephalopathy-syndrome-in-a-case-of-scleroderma-renal-crisis-with-maintenance-hemodialysis-case-report-and-literature-review
#16
Ching-Yang Chen, Shin-Yuan Hung, Yi-Jer Lee, Yi-Chan Lin, Chu-Cheng Pai
INTRODUCTION: In some cases, scleroderma renal crisis (SRC) is not easily distinguishable from other thrombotic microangiopathies such as thrombotic thrombocytopenic purpura, especially when the presentation includes neurological or extra-renal manifestations. Here, we present a case of SRC who developed a rare neurotoxic complication, posterior reversible encephalopathy syndrome (PRES).A 36-year-old man with a history of diffuse cutaneous systemic sclerosis developed SRC and acute-on-chronic renal failure and ultimately required maintenance hemodialysis...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28033115/quantification-of-differences-between-nailfold-capillaroscopy-images-with-a-scleroderma-pattern-and-normal-pattern-using-measures-of-geometric-and-algorithmic-complexity
#17
Samuel George Urwin, Bridget Griffiths, John Allen
This study aimed to quantify and investigate differences in the geometric and algorithmic complexity of the microvasculature in nailfold capillaroscopy (NFC) images displaying a scleroderma pattern and those displaying a 'normal' pattern. 11 NFC images were qualitatively classified by a capillary specialist as indicative of 'clear microangiopathy' (CM), i.e. a scleroderma pattern, and 11 as 'not clear microangiopathy' (NCM), i.e. a 'normal' pattern. Pre-processing was performed, and fractal dimension (FD) and Kolmogorov complexity (KC) were calculated following image binarisation...
December 29, 2016: Physiological Measurement
https://www.readbyqxmd.com/read/28029267/the-pathological-spectrum-associated-with-the-ultrastructural-finding-of-thin-glomerular-basement-membrane-a-tertiary-medical-city-experience-and-review-of-the-literature
#18
Hala Kfoury, Maria Arafah
BACKGROUND: Thin glomerular basement membrane (GBM) has been noted in several glomerular diseases including IgA nephropathy, focal segmental glomerulosclerosis (FSGS), Fabry's disease, and Alport's syndrome. We conducted this study to investigate the pathological ultrastructural spectrum of thin GBMs, to identify associated diseases, and to measure the GBM thickness in thin GBMs in our adult population. MATERIALS AND METHODS: All renal biopsies with thin GBM, diagnosed between 2010 and 2016, were retrieved and reviewed...
December 28, 2016: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28028259/-an-autopsy-case-of-pulmonary-tumor-thrombotic-microangiopathy-that-progressed-slowly-during-chemotherapy-for-advanced-gastric-cancer
#19
Nobuyasu Suzuki, Ayaka Azami, Yukitoshi Todate, Takeshi Tada, Mitsuru Waragai, Michitaka Honda, Atai Sato, Yoshinao Takano, Tsuyoshi Abe, Yasushi Teranishi
A man in his 50s presented to our hospital for anemia and was diagnosed with advanced gastric cancer with para-aortic lymph node metastases. He underwent gastrojejunostomy and received postoperative chemotherapy. Despite stable disease with chemotherapy, he complained of cough and respiratory discomfort and was subsequently admitted with progressive respiratory distress. Pulmonary hypertension and right-sided heart failure developed, and he died of sudden cardiopulmonary arrest 9 days after admission. An autopsy revealed widespread tumor metastasis, and he was diagnosed with pulmonary tumor thrombotic microangiopathy(PTTM)associated with gastric cancer...
December 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28025630/adjustment-of-eculizumab-dosage-pattern-in-patients-with-atypical-hemolytic-uremic-syndrome-with-suboptimal-response-to-standard-treatment-pattern
#20
Camino García Monteavaro, Carmen Peralta Roselló, Borja Quiroga, José María Baltar Martín, Lorena Castillo Eraso, Fernando de Álvaro Moreno, Alberto Martínez Vea, María Teresa Visus-Fernández de Manzanos
In patients with atypical hemolytic uremic syndrome (aHUS), complement blocking by eculizumab rapidly halts the process of thrombotic microangiopathy and it is associated with clear long-term hematologic and renal improvements. Eculizumab treatment consists of a 4-week initial phase with weekly IV administration of 900 mg doses, followed by a maintenance phase with a 1,200 mg dose in the fifth week and every 14 ± 2 days thereafter. We present three patients with aHUS and suboptimal response to eculizumab treatment at the usual administration dosage who showed hematologic and renal improvements after an adjustment in the eculizumab treatment protocol...
2016: Case Reports in Nephrology
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