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https://www.readbyqxmd.com/read/28443261/diabetes-mellitus-caused-by-secondary-hemochromatosis-after-multiple-blood-transfusions-in-2-patients-with-severe-aplastic-anemia
#1
Hyun Jin Kim, Yoon-Myung Kim, Eungu Kang, Beom Hee Lee, Jin-Ho Choi, Han-Wook Yoo
Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT) at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9...
March 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28442888/new-developments-in-the-treatment-of-multiple-myeloma-clinical-utility-of-daratumumab
#2
REVIEW
Cian McEllistrim, Janusz Krawczyk, Michael E O'Dwyer
Multiple myeloma is a clonal disorder of plasma cells that is currently considered incurable. CD38 is a 46 kDa type II transmembrane glycoprotein that is highly expressed on myeloma cells. Daratumumab is a first in-class human IgG1 monoclonal antibody that targets CD38, and has antimyeloma effects through several mechanisms. Single-agent trials show surprising activity in heavily pretreated myeloma patients. Trials in the relapsed setting, where daratumumab is added to lenalidomide and dexamethasone or bortezomib and dexamethasone, have demonstrated significantly improved progression-free survival with acceptable toxicity...
2017: Biologics: Targets & Therapy
https://www.readbyqxmd.com/read/28421539/%C3%AE-synuclein-aggregates-with-%C3%AE-amyloid-or-tau-in-human-red-blood-cells-correlation-with-antioxidant-capability-and-physical-exercise-in-human-healthy-subjects
#3
Simona Daniele, Deborah Pietrobono, Jonathan Fusi, Caterina Iofrida, Lucia Chico, Lucia Petrozzi, Annalisa Lo Gerfo, Filippo Baldacci, Fabio Galetta, Gabriele Siciliano, Ubaldo Bonuccelli, Gino Santoro, Maria Letizia Trincavelli, Ferdinando Franzoni, Claudia Martini
Neurodegenerative disorders (NDs) are characterized by abnormal accumulation/misfolding of specific proteins, primarily α-synuclein (α-syn), β-amyloid1-42 (Aβ), and tau, in both brain and peripheral tissue. In addition to homo-oligomers, the role of α-syn interactions with Aβ or tau has gradually emerged. The altered protein accumulation has been related to both oxidative stress and physical activity; nevertheless, no correlation among the presence of peripheral α-syn hetero-aggregates, antioxidant capacity, and physical exercise has been discovered as of yet...
April 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28417072/atypical-juvenile-presentation-of-gm2-gangliosidosis-ab-in-a-patient-compound-heterozygote-for-c-259g%C3%A2-%C3%A2-t-and-c-164c%C3%A2-%C3%A2-t-mutations-in-the-gm2a-gene
#4
Carla Martins, Catherine Brunel-Guitton, Anne Lortie, France Gauvin, Carlos R Morales, Grant A Mitchell, Alexey V Pshezhetsky
GM2-gangliosidosis, AB variant is an extremely rare autosomal recessive inherited disorder caused by mutations in the GM2A gene that encodes GM2 ganglioside activator protein (GM2AP). GM2AP is necessary for solubilisation of GM2 ganglioside in endolysosomes and its presentation to β-hexosaminidase A. Conversely GM2AP deficiency impairs lysosomal catabolism of GM2 ganglioside, leading to its storage in cells and tissues. We describe a 9-year-old child with an unusual juvenile clinical onset of GM2-gangliosidosis AB...
June 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28413720/direct-imaging-of-app-proteolysis-in-living-cells
#5
Niccoló Parenti, Ambra Del Grosso, Claudia Antoni, Marco Cecchini, Renato Corradetti, Francesco S Pavone, Martino Calamai
Alzheimer's disease is a multifactorial disorder caused by the interaction of genetic, epigenetic and environmental factors. The formation of cytotoxic oligomers consisting of Aβ peptide is widely accepted as being one of the main key events triggering the development of Alzheimer's disease. Aβ peptide production results from the specific proteolytic processing of the amyloid precursor protein (APP). Deciphering the factors governing the activity of the secretases responsible for the cleavage of APP is still a critical issue...
2017: PeerJ
https://www.readbyqxmd.com/read/28409830/magnesium-for-treating-sickle-cell-disease
#6
REVIEW
Nan Nitra Than, Htoo Htoo Kyaw Soe, Senthil K Palaniappan, Adinegara Bl Abas, Lucia De Franceschi
BACKGROUND: Sickle cell disease is an autosomal recessive inherited haemoglobinopathy which causes painful vaso-occlusive crises due to sickle red blood cell dehydration. Vaso-occlusive crises are common painful events responsible for a variety of clinical complications; overall mortality is increased and life expectancy decreased compared to the general population. Experimental studies suggest that intravenous magnesium has proven to be well-tolerated in individuals hospitalised for the immediate relief of acute (sudden onset) painful crisis and has the potential to decrease the length of hospital stay...
April 14, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28404591/intravital-imaging-of-the-kidney-in-a-rat-model-of-salt-sensitive-hypertension
#7
Bradley T Endres, Ruben M Sandoval, George J Rhodes, Silvia B Campos-Bilderback, Malgorzata M Kamocka, Christopher McDermott-Roe, Alexander Staruschenko, Bruce A Molitoris, Aron M Geurts, Oleg Palygin
Hypertension is one of the most prevalent diseases worldwide, and a major risk factor for renal failure and cardiovascular disease. The role of albuminuria, a common feature of hypertension and robust predictor of cardiorenal disorders, remains incompletely understood. The goal of this study was to investigate the mechanisms leading to albuminuria in the kidney of a rat model of hypertension, the Dahl salt-sensitive (SS) rat. To determine the relative contributions of the glomerulus and proximal tubule (PT) to albuminuria, we applied intravital two-photon-based imaging to investigate the complex renal physiological changes that occur during salt-induced hypertension...
April 12, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28400547/anemia-in-thyroid-diseases
#8
Ewelina Szczepanek-Parulska, Aleksandra Hernik, Marek Ruchała
Anemia is a frequent, although often underestimated, clinical condition accompanying thyroid diseases. In spite of the fact that anemia and thyroid dysfunction often occur simultaneously, the causative relationship between these two disorders remains ambiguous. Thyroid hormones stimulate erythrocytes precursors proliferation directly, as well as via erythropoietin production enhancement, whereas iron-deficient anemia negatively influences thyroid hormonal status. Thus, different forms of anemia might emerge in the course of thyroid dysfunction...
March 28, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/28390315/glutathione-inhibits-antibody-and-complement-mediated-immunologic-cell-injury-via-multiple-mechanisms
#9
Zhen Zhang, Xiling Zhang, Xin Fang, Manabu Niimi, Yanru Huang, Honglan Piao, Shan Gao, Jianglin Fan, Jian Yao
Antioxidant glutathione (GSH) plays an important role in the regulation of immunity. However, little is known about its effects on humoral immunity, especially its action on effector molecules like antibody and complement. Given that these molecules contain abundant disulfide bonds, we speculated that GSH might influence the action of these proteins via its thiol function. Using a model of a glomerular mesangial cell (MC) lysis induced by antibodies plus complement, we addressed this hypothesis. Exposure of rat MCs to anti-Thy-1 antibody plus complement or anti-MC rabbit serum caused a complement-dependent cell lysis, which was completely blocked by GSH...
March 31, 2017: Redox Biology
https://www.readbyqxmd.com/read/28389280/in-vitro-safety-and-efficacy-evaluations-of-a-complex-botanical-mixture-of-eugenia-dysenterica-dc-myrtaceae-prospects-for-developing-a-new-dermocosmetic-product
#10
Larissa Cleres Moreira, Renato Ivan de Ávila, Danillo Fabrini Maciel Costa Veloso, Tatiana Nascimento Pedrosa, Emerson Silva Lima, Renê Oliveira do Couto, Eliana Martins Lima, Aline Carvalho Batista, José Realino de Paula, Marize Campos Valadares
In the context of developing a new natural product-based cosmetic, the in vitro efficacy and safety evaluations of a complex botanical mixture based on Eugenia dysenterica leaf hydroalcoholic extract (EDE) (2.5-1000μg/mL) were carried out. Chromatographic analysis demonstrated the presence of the tannin (ellagic acid) and flavonoids (quercetin and gallic acid) which characterize the EDE as a polyphenol-rich mixture. Using HFF-1 fibroblasts, it was shown that EDE promoted cell regeneration after UVA exposure...
April 4, 2017: Toxicology in Vitro: An International Journal Published in Association with BIBRA
https://www.readbyqxmd.com/read/28385336/association-between-pancreatitis-and-immune-mediated-haemolytic-anaemia-in-cats-a-cross-sectional-study
#11
A Zoia, M Drigo
In most cases, the underlying causes of feline pancreatitis and feline immune-mediated haemolytic anaemia (IMHA) remain unknown. Following the observation that some cats present with both diseases concurrently, the aim of this cross-sectional study was to investigate if the two disorders were associated statistically in this species. A residency case log of all cases seen at the Small Animal Hospital, University of Glasgow, Glasgow, UK, between July 2004 and December 2007, was used as the database for this study and an association between these two disorders was assessed by Fisher's exact test...
April 3, 2017: Journal of Comparative Pathology
https://www.readbyqxmd.com/read/28382694/quantitative-analysis-of-hyperpolarized-129-xe-gas-transfer-mri
#12
Ziyi Wang, Scott Haile Robertson, Jennifer Wang, Mu He, Rohan S Virgincar, Geoffry M Schrank, Elianna A Bier, Sudarshan Rajagopal, Yuh Chin Huang, Thomas G O'Riordan, Craig R Rackley, H Page McAdams, Bastiaan Driehuys
PURPOSE: Hyperpolarized (129) Xe magnetic resonance imaging (MRI) using Dixon-based decomposition enables single-breath imaging of (129) Xe in the airspaces, interstitial barrier tissues, and red blood cells (RBCs). However, methods to quantitatively visualize information from these images of pulmonary gas transfer are lacking. Here, we introduce a novel method to transform these data into quantitative maps of pulmonary ventilation, and (129) Xe gas transfer to barrier and RBC compartments...
April 6, 2017: Medical Physics
https://www.readbyqxmd.com/read/28382682/cerebrospinal-fluid-analysis-in-recumbent-adult-dairy-cows-with-or-without-spinal-cord-lesions
#13
D Achard, D Francoz, C Grimes, A Desrochers, S Nichols, M Babkine, G Fecteau
BACKGROUND: Diagnosis of central nervous system (CNS) lesions in recumbent dairy cattle (RDC) is challenging because neurologic examination is limited and medical imaging often is challenging or unrewarding. Cerebrospinal fluid (CSF) analysis is useful in the diagnosis of CNS disorders in cattle. However, its utility in identifying spinal cord lesions in RDC remains to be evaluated. HYPOTHESIS/OBJECTIVES: We hypothesized that CSF analysis would discriminate between RDC with and without spinal cord lesions...
April 6, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28381149/two-unusual-cases-of-oral-lichen-planus-arising-after-oral-squamous-cell-carcinoma
#14
Davide Bartolomeo Gissi, Sofia Asioli, Andrea Gabusi
Autoimmune diseases occur when the immune system fails to recognize self-antigens expressed on the body's own cells and attacks them. Oral lichen planus (OLP) is a chronic autoimmune mucocutaneous disease of the oral cavity characterized by white/red lesions. Considered a potentially malignant disorder, OLP evolution into oral squamous cell carcinoma (OSCC) is still a matter of debate. While chronic autoimmune inflammation is considered a potential risk factor for malignant transformation in many solid tumors, the opposite idea that cancer may trigger autoimmune responses remains controversial...
March 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28375637/development-of-a-general-aza-cope-reaction-trigger-applied-to-fluorescence-imaging-of-formaldehyde-in-living-cells
#15
Kevin J Bruemmer, Ryan R Walvoord, Thomas F Brewer, Guillermo Burgos-Barragan, Niek Wit, Lucas B Pontel, Ketan J Patel, Christopher J Chang
Formaldehyde (FA) is a reactive signaling molecule that is continuously produced through a number of central biological pathways spanning epigenetics to one-carbon metabolism. On the other hand, aberrant, elevated levels of FA are implicated in disease states ranging from asthma to neurodegenerative disorders. In this context, fluorescence-based probes for FA imaging are emerging as potentially powerful chemical tools to help disentangle the complexities of FA homeostasis and its physiological and pathological contributions...
April 4, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28374203/association-between-exposure-to-pesticides-and-disorder-on-hematological-parameters-and-kidney-function-in-male-agricultural-workers
#16
Nagah M Hassanin, Osama M Awad, Sourya El-Fiki, Reda A I Abou-Shanab, Ahmed R A Abou-Shanab, Ranya A Amer
Pesticides occupy a critical position among many chemicals to which man can be exposed; their diffusion into the environment causes killing and damaging of some forms of life. The lack of highly selective pesticide action represents risk both for man and other desirable forms of life present in the environment. The present study was designed to evaluate the relation between exposure to pesticides and disorder on hematological parameters and kidney function of male agricultural workers with the mean age 37.11 ± 9...
April 3, 2017: Environmental Science and Pollution Research International
https://www.readbyqxmd.com/read/28369973/persistent-symptomatic-parvovirus-b19-infection-with-severe-thrombocytopenia-transmitted-by-red-blood-cell-transfusion-containing-low-parvovirus-b19-dna-levels
#17
Keiki Nagaharu, Yuka Sugimoto, Yuji Hoshi, Takanori Yamaguchi, Ryugo Ito, Keiji Matsubayashi, Keiki Kawakami, Kohshi Ohishi
BACKGROUND: Transfusion-mediated human parvovirus B19 (PVB19) infection is rare but often causes severe hematologic disorders. In Japan, routine blood donor screening for PVB19 antigen (detection sensitivity, 10(6.4) IU/mL) using a chemiluminescent enzyme immunoassay (CLEIA) was introduced in 2008. However, there is no consensus on the minimal infectious dose of PVB19 permissible for red blood cells (RBCs). CASE REPORT: A 64-year-old man, who had received hemodialysis for diabetic nephropathy for 5 years, underwent an RBC transfusion for anemia caused by hemorrhagic enterocolitis...
April 2, 2017: Transfusion
https://www.readbyqxmd.com/read/28369932/successful-management-of-passenger-lymphocyte-syndrome-in-an-abo-compatible-nonidentical-isolated-bowel-transplant-a-case-report-and-review-of-the-literature
#18
Daniel Foell, Sabina Glasmeyer, Norbert Senninger, Heiner Wolters, Daniel Palmes, Ralf Bahde
BACKGROUND: Passenger lymphocyte syndrome (PLS), a subtype of graft-versus-host disease, is a rare disorder encountered mainly in ABO-mismatched hematopoietic stem cell transplantation and infrequently in all types of ABO-mismatched solid organ transplantation. We here report the fifth case of PLS in small bowel transplantation (SBTx) and the first one describing the successful management of PLS in a cadaveric, isolated SBTx. CASE REPORT: A 60-year-old Caucasian female with blood group A D+ suffering from short bowel syndrome received a small bowel transplant from a 32-year-old Caucasian female with blood group O D+ (HLA mismatch 2/6)...
March 28, 2017: Transfusion
https://www.readbyqxmd.com/read/28367656/prevalence-of-anemia-and-hemoglobin-disorders-among-school-children-in-myanmar
#19
Saw Thu Wah, Yoon Shwe Yi, Aye Aye Khin, Chotiros Plabplueng, Pornlada Nuchnoi
The prevalence of anemia is high in the population of Myanmar and hypochromic microcytic anemia (HMA) is predominant. The objective of our study was to determine the prevalence of anemia and causes of HMA among school children. A cross-sectional study was conducted on 239 children from Thanlyin and Insein Townships, Yangon Region, Myanmar. Complete blood count (CBC) and blood film morphology was examined on venous blood samples. Hypochromic microcytic anemia cases were subsequently analyzed for serum ferritin and cellulose acetate hemoglobin (Hb) electrophoresis...
April 2, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28349897/3d-printing-scaffold-coupled-with-low-level-light-therapy-for-neural-tissue-regeneration
#20
Wei Zhu, Jonathan K George, Volker J Sorger, Lijie Grace Zhang
3D printing has shown promise for neural regeneration by providing customized nerve scaffolds to structurally support and bridge the defect gap as well as deliver cells or various bioactive substances. Low-level light therapy (LLLT) exhibits positive effects on rehabiliation of degenerative nerves and neural disorders. With this in mind, we postulate that 3D printed neural scaffold coupling with LLLT will generate a new strategy to repair neural degeneration. To achieve this goal, we applied red laser light to stimualte neural stem cells on 3D printed scaffolds and investigated the subsequent cell response with respect to cell proliferation and differentiation...
April 12, 2017: Biofabrication
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