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https://www.readbyqxmd.com/read/28815969/-318c-t-polymorphism-of-the-ctla-4-gene-is-an-independent-risk-factor-for-rbc-alloimmunization-among-sickle-cell-disease-patients
#1
V B Oliveira, M R Dezan, F C A Gomes, S F Menosi Gualandro, J E Krieger, A C Pereira, J D Marsiglia, J E Levi, V Rocha, A Mendrone-Junior, E C Sabino, C L Dinardo
Cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) molecule is expressed on T-lymphocyte membrane and negatively influences the antigen-presenting process. Reduced expression of CTLA-4 due to gene polymorphisms is associated with increased risk of autoimmune disorders, whose physiopathology is similar to that of post-transfusion red blood cell (RBC) alloimmunization. Our goal was to evaluate if polymorphisms of CTLA-4 gene that affect protein expression are associated with RBC alloimmunization. This was a case-control study in which 134 sickle cell disease (SCD) patients and 253 non-SCD patients were included...
August 17, 2017: International Journal of Immunogenetics
https://www.readbyqxmd.com/read/28814778/in-vivo-and-in-vitro-sex-differences-in-the-dendritic-morphology-of-developing-murine-hippocampal-and-cortical-neurons
#2
Kimberly P Keil, Sunjay Sethi, Machelle D Wilson, Hao Chen, Pamela J Lein
Altered dendritic morphology is common in neurodevelopmental disorders (NDDs), many of which show sex biases in prevalence, onset and/or severity. However, whether dendritic morphology varies as a function of sex in juvenile mice or primary neuronal cell cultures is largely unknown even though both are widely used models for studying NDDs. To address this gap, we quantified dendritic morphology in CA1 pyramidal hippocampal and adjacent somatosensory pyramidal cortical neurons from male and female postnatal day (P)28 C57BL/6J mice...
August 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28809446/deferasirox-for-managing-iron-overload-in-people-with-thalassaemia
#3
REVIEW
Claudia Bollig, Lisa K Schell, Gerta Rücker, Roman Allert, Edith Motschall, Charlotte M Niemeyer, Dirk Bassler, Joerg J Meerpohl
BACKGROUND: Thalassaemia is a hereditary anaemia due to ineffective erythropoiesis. In particular, people with thalassaemia major develop secondary iron overload resulting from regular red blood cell transfusions. Iron chelation therapy is needed to prevent long-term complications.Both deferoxamine and deferiprone are effective; however, a review of the effectiveness and safety of the newer oral chelator deferasirox in people with thalassaemia is needed. OBJECTIVES: To assess the effectiveness and safety of oral deferasirox in people with thalassaemia and iron overload...
August 15, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28808446/neuron-specific-fluorescence-reporter-based-live-cell-tracing-for-transdifferentiation-of-mesenchymal-stem-cells-into-neurons-by-chemical-compound
#4
Do Won Hwang, Hyun Woo Kwon, Jaeho Jang, Hee Jung Jung, Kwang Rok Kim, Dong Soo Lee
Although transdifferentiation of mesenchymal stem cells (MSCs) into neurons increases the possibility of therapeutic use of MSCs for neurodevelopmental disorders, the use of MSCs has the limitation on differentiation efficiency to neuronal lineage and lack of an easy method to monitor the transdifferentiation. In this study, using time-lapse live cell imaging, we assessed the neuronal differentiation of MSCs induced by a small molecule "NHPDQC (N-hydroxy-2-oxo-3-(3-phenylprophyl)-1,2-dihydroquinoxaline-6-carboxamide, C18H17N3O3)...
2017: Stem Cells International
https://www.readbyqxmd.com/read/28803808/two-novel-mutations-p-ser160pro-and-p-arg472cys-causing-glucose-6-phosphate-isomerase-deficiency-are-associated-with-erythroid-dysplasia-and-inappropriately-suppressed-hepcidin
#5
Renata Mojzikova, Pavla Koralkova, Dusan Holub, Zuzana Saxova, Dagmar Pospisilova, Daniela Prochazkova, Petr Dzubak, Monika Horvathova, Vladimir Divoky
Glucose-6-phosphate isomerase (GPI) deficiency, a genetic disorder responsible for chronic nonspherocytic hemolytic anemia, is the second most common red blood cell glycolytic enzymopathy. We report three patients from two unrelated families of Czech and Slovak origin with macrocytic hemolytic anemia due to GPI deficiency. The first patient had 15% of residual GPI activity resulting from two new heterozygous missense mutations c.478T>C and c.1414C>T leading to substitutions p.(Ser160Pro) and p.(Arg472Cys)...
April 14, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28798838/simultaneous-measurement-of-erythrocyte-deformability-and-blood-viscoelasticity-using-micropillars-and-co-flowing-streams-under-pulsatile-blood-flows
#6
Yang Jun Kang
The biophysical properties of blood provide useful information on the variation in hematological disorders or diseases. In this study, a simultaneous measurement method of RBC (Red Blood Cell) deformability and blood viscoelasticity is proposed by evaluating hemodynamic variations through micropillars and co-flowing streams under sinusoidal blood flow. A disposable microfluidic device is composed of two inlets and two outlets, two upper side channels, and two lower side channels connected to one bridge channel...
January 2017: Biomicrofluidics
https://www.readbyqxmd.com/read/28789919/identification-of-long-and-very-long-chain-fatty-acids-plasmalogen-c16-0-and-phytanic-acid-as-new-lipid-biomarkers-in-tunisian-coronary-artery-disease-patients
#7
Samia Hadj Ahmed, Nadia Koubaa, Wafa Kharroubi, Amira Zarrouk, Amira Mnari, Fethi Batbout, Habib Gamra, Sonia Hammami, Gérard Lizard, Mohamed Hammami
Long and very long chain fatty acids (LCFAs and VLCFAs) may play an active role in coronary artery diseases (CAD) etiology. Our aim was to evaluate the associations between LCPUFAs (C20:4n-6; C20:5n-3 and C22:6n-3) and VLCSFAs (C22:0, C24:0; and C26:0), as well as markers of peroxisomal integrity evaluated by phytanic acid and plasmalogen-C16:0 (PL-C16:0) in addition to the markers of lipid peroxidation (malondialdehyde [MDA] and conjugated dienes [CD]) and inflammation (high sensitivity C-reactive protein [hs-CRP]) with vascular severity evaluated by Gensini score in order to determine their possible effects on CAD in Tunisian population...
August 5, 2017: Prostaglandins & Other Lipid Mediators
https://www.readbyqxmd.com/read/28770911/targeting-%C3%AE-cys93-in-hemoglobin-s-with-an-antisickling-agent-possessing-dual-allosteric-and-antioxidant-effects
#8
Tigist Kassa, Michael Brad Strader, Akito Nakagawa, Warren M Zapol, Abdu I Alayash
Sickle cell disease (SCD) is an inherited blood disorder caused by a β globin gene mutation of hemoglobin (HbS). The polymerization of deoxyHbS and its subsequent aggregation (into long fibers) is the primary molecular event which leads to red blood cell (RBC) sickling and ultimately hemolytic anemia. We have recently suggested that HbS oxidative toxicity may also contribute to SCD pathophysiology due to its defective pseudoperoxidase activity. As a consequence, a persistently higher oxidized ferryl heme is formed which irreversibly oxidizes "hotspot" residues (particularly βCys93) causing protein unfolding and subsequent heme loss...
August 3, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28769542/relationship-of-mean-platelet-volume-to-mdd-a-retrospective-study
#9
Liqiang Cai, Luoyi Xu, Lili Wei, Wei Chen
BACKGROUND: Results of numerous studies show that major depressive disorder (MDD) is associated with a chronic low-grade inflammation, but the underlying mechanism remains unclear. AIM: To compare the results of blood cell analysis of MDD patients with healthy controls, and explore the potential value of it as an indicator of immune-inflammation in MDD, especially the mean platelet volume. METHODS: 103 MDD patients and 106 healthy controls with matched age and gender were recruited...
February 25, 2017: Shanghai Archives of Psychiatry
https://www.readbyqxmd.com/read/28766840/high-birth-prevalence-of-sickle-cell-disease-in-northwestern-tanzania
#10
Emmanuela E Ambrose, Julie Makani, Neema Chami, Tulla Masoza, Rogatus Kabyemera, Robert N Peck, Erasmus Kamugisha, Alphaxard Manjurano, Neema Kayange, Luke R Smart
BACKGROUND: Worldwide, hemoglobinopathies affect millions of children. Identification of hemoglobin disorders in most sub-Saharan African countries is delayed until clinical signs of the disease are present. Limited studies have been conducted to understand their prevalence and clinical presentation among newborns in resource-limited settings. METHODOLOGY: This was a prospective cohort study. Newborns (aged 0-7 days) at two hospitals in Northwestern Tanzania were enrolled and followed prospectively for 6 months...
August 2, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28761130/lipodystrophy-and-obesity-are-associated-with-decreased-number-of-t-cells-with-regulatory-function-and-pro-inflammatory-macrophage-phenotype
#11
S Berger, G Ceccarini, G Scabia, I Barone, C Pelosini, F Ferrari, S Magno, A Dattilo, L Chiovato, P Vitti, F Santini, M Maffei
BACKGROUND/OBJECTIVES: In lipodystrophy (LD) adipose tissue function to store lipids is impaired, leading to metabolic syndrome, similar to that found in obesity. Emerging evidence links dysmetabolism with disorders of the immune system. Our aim is to investigate whether T-cell populations with regulatory function and monocyte-derived macrophages (MDMs) are affected by LD and obesity. SUBJECTS/METHODS: Blood was collected from 16 LD, 16 obese (OB, BMI>30 kg m(-2)) and 16 healthy normal-weight women (CNT)...
July 10, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28759763/trends-in-biomarkers-biotic-indices-and-fish-population-size-revealed-contrasting-long-term-effects-of-recycled-water-on-the-ecological-status-of-a-mediterranean-river
#12
Nicole Colin, Alberto Maceda-Veiga, Mario Monroy, Martí Ortega-Ribera, Mireia Llorente, Adolfo de Sostoa
Recycled water is important for maintaining river flow in semi-arid regions. However, it has ecological risk, as suggested by comparison of habitat and white and red blood cell count in two wild fish species (Barbus meridionalis and Squalius laietanus) before and after an input of recycled water in Ripoll River (NE Spain) in 2009. Due to the lack of normal ranges for blood variables in wild fish, we surveyed seasonally the same river reaches in 2013 to test if blood alterations from 2009 compromised the viability of the fish populations...
July 27, 2017: Ecotoxicology and Environmental Safety
https://www.readbyqxmd.com/read/28755192/newborn-screening-for-hereditary-tyrosinemia-type-i-in-qu%C3%A3-bec-update
#13
Yves Giguère, Marie-Thérèse Berthier
Hereditary tyrosinemia type I (HTI) is a rare autosomal recessive disorder caused by a fumarylacetoacetate hydrolase (FAH) deficiency. If untreated, its acute form is characterized by hepatic failure, renal dysfunction and neurological crisis, and may lead to death. Due to a genetic founder effect in the French-Canadian population, the prevalence of HTI is increased in the province of Quebec (1/19 819), with the IVS12 + 5G>A (1062 + 5G>A) splice site mutation responsible for more than 90% of mutated alleles...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28752030/syringaresinol-4-o-%C3%AE-d-glucoside-alters-lipid-and-glucose-metabolism-in-hepg2-cells-and-c2c12-myotubes
#14
Shuai Wang, Chongming Wu, Xin Li, Yue Zhou, Quanyang Zhang, Fuchao Ma, Jianhe Wei, Xiaopo Zhang, Peng Guo
Syringaresinol-4-O-β-d-glucoside (SSG), a furofuran-type lignan, was found to modulate lipid and glucose metabolism through an activity screen of lipid accumulation and glucose consumption, and was therefore considered as a promising candidate for the prevention and treatment of metabolic disorder, especially in lipid and glucose metabolic homeostasis. In this study, the effects of SSG on lipogenesis and glucose consumption in HepG2 cells and C2C12 myotubes were further investigated. Treatment with SSG significantly inhibited lipid accumulation by oil red O staining and reduced the intracellular contents of total lipid, cholesterol and triglyceride in HepG2 cells...
July 2017: Acta Pharmaceutica Sinica. B
https://www.readbyqxmd.com/read/28751561/exome-sequencing-identifies-recurrent-bcor-gene-alterations-and-the-absence-of-klf2-tnfaip3-and-myd88-mutations-in-splenic-diffuse-red-pulp-small-b-cell-lymphoma
#15
Laurent Jallades, Lucile Baseggio, Pierre Sujobert, Sarah Huet, Kaddour Chabane, Evelyne Callet-Bauchu, Aurélie Verney, Sandrine Hayette, Jean-Pierre Desvignes, David Salgado, Nicolas Levy, Christophe Béroud, Pascale Felman, Françoise Berger, Jean-Pierre Magaud, Laurent Genestier, Gilles Salles, Alexandra Traverse-Glehen
Splenic diffuse red pulp lymphoma is an indolent small B-cell lymphoma recognised as a provisional entity in the WHO 2008 classification. Its precise relationship with other related splenic B-cell lymphomas with frequent leukaemic involvement or other lymphoproliferative disorders remains undetermined. We performed whole-exome sequencing to explore the genetic landscape of 10 splenic diffuse red pulp lymphoma cases from paired tumour and normal samples. A selection of 109 somatic mutations was then evaluated in a cohort including 42 splenic diffuse red pulp lymphoma samples and compared to those identified in 46 splenic marginal zone lymphoma and 8 hairy-cell leukaemia samples...
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28750791/use-of-nandrolone-decanoate-in-treatment-of-pure-red-cell-aplasia-secondary-to-diclofenac-administration-a-case-report
#16
Paula Nassar de Marchi, André Nanny Le Sueur Vieira, José Francisco Antunes Ribeiro, Silvano Salgueiro Geraldes, Paulo Roberto Rodrigues Ramos, Alessandra Melchert, Priscylla Tatiana Chalfun Guimarães-Okamoto
Pure red cell aplasia (PRCA) is a disorder that leads to a nonregenerative anemia that results from erythroid precursors failing to reach maturity in the bone marrow, whereas the numbers of mature myeloid and megakaryocytic cells remain normal. PRCA can be induced by autoimmune processes, infections, drugs, toxins, and radiation, and is diagnosed by a bone marrow cytology examination after excluding the most common causes of nonregenerative anemia. Immunosuppressive therapies are used to treat PRCA, and usually involve the use of glucocorticoids, cyclosporin, or azathioprine...
March 2017: Topics in Companion Animal Medicine
https://www.readbyqxmd.com/read/28748217/mechanisms-and-applications-of-the-anti-inflammatory-effects-of-photobiomodulation
#17
Michael R Hamblin
Photobiomodulation (PBM) also known as low-level level laser therapy is the use of red and near-infrared light to stimulate healing, relieve pain, and reduce inflammation. The primary chromophores have been identified as cytochrome c oxidase in mitochondria, and calcium ion channels (possibly mediated by light absorption by opsins). Secondary effects of photon absorption include increases in ATP, a brief burst of reactive oxygen species, an increase in nitric oxide, and modulation of calcium levels. Tertiary effects include activation of a wide range of transcription factors leading to improved cell survival, increased proliferation and migration, and new protein synthesis...
2017: AIMS Biophysics
https://www.readbyqxmd.com/read/28745572/prevalence-of-thalassemia-traits-and-iron-deficiency-anemia-in-sindh-pakistan
#18
Abdul H Kandhro, Virapong Prachayasittikul, Chartchalerm Isarankura Na-Ayudhya, Pornlada Nuchnoi
Among microcytic hypochromic anemias, the most common disorders are iron deficiency anemia and co-pathological conditions such as α- or β-thalassemia (α- or β-thal) traits. The aim of the present study was to determine the frequency and prevalence of iron deficiency anemia and α- or β-thal traits based on clinical laboratory data across different ethnic groups in five districts of Sindh Province, Pakistan. The present retrospective study analyzed 3 years (2012-2015) of encoded and unlinked clinical laboratory data, and identified 3030 microcytic hypochromic anemia cases...
July 26, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28732547/crescentic-glomerular-nephritis-associated-with-rheumatoid-arthritis-a-case-report
#19
K Balendran, L D S U Senarathne, R D Lanerolle
BACKGROUND: Rheumatoid arthritis is a systemic disorder where clinically significant renal involvement is relatively common. However, crescentic glomerular nephritis is a rarely described entity among the rheumatoid nephropathies. We report a case of a patient with rheumatoid arthritis presenting with antineutrophil cytoplasmic antibody-negative crescentic glomerular nephritis. CASE PRESENTATION: A 54-year-old Sri Lankan woman who had recently been diagnosed with rheumatoid arthritis was being treated with methotrexate 10 mg weekly and infrequent nonsteroidal anti-inflammatory drugs...
July 21, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28731851/the-frequency-of-occurrence-of-fish-shaped-red-blood-cells-in-different-haematologic-disorders
#20
Christoph Robier, Carolin Körber, Franz Quehenberger, Manfred Neubauer, Albert Wölfler
BACKGROUND: Red blood cells (RBC) resembling the silhouette of a fish are rarely observed in peripheral blood (PB) smears. In this study, we determined the frequency of occurrence of fish-shaped RBC in different haematologic diseases. METHODS: We examined PB smears of patients with iron deficiency anaemia (IDA) (n=23), β-thalassaemia minor (BTM) (n=30), sickle cell disease (SCD) (n=7), autoimmune haemolytic anaemia (AIHA) (n=13), microangiopathic haemolytic anaemia (MAHA) (n=11), hereditary sphaerocytosis (HS) (n=4), hereditary elliptocytosis (HE) (n=3), vitamin B12 and folate deficiency (n=15), anaemia in liver disease (LD) (n=17), myelodysplastic syndrome (MDS) (n=15), acute myeloid leukaemia (AML) (n=29), chronic myeloid leukaemia (CML) (n=18), primary myelofibrosis (PMF) (n=12), chronic myelo-monocytic leukaemia (CMML) (n=15) and 21 healthy controls by light microscopy for the occurrence of fish-shaped erythrocytes...
July 21, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
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