keyword
MENU ▼
Read by QxMD icon Read
search

Sickle cell

keyword
https://www.readbyqxmd.com/read/28930976/detection-of-residual-donor-erythroid-progenitor-cells-after-hematopoietic-stem-cell-transplantation-for-patients-with-hemoglobinopathies
#1
Roman Crazzolara, Gabriele Kropshofer, Michael Steurer, Sieghart Sopper, Wolfgang Schwinger
The presence of incomplete chimerism is noted in a large proportion of patients following bone marrow transplant for thalassemia major or sickle cell disease. This observation has tremendous implications, as subsequent therapeutic immunomodulation strategies can improve clinical outcome. Conventionally, polymerase chain reaction-based analysis of short tandem repeats is used to identify chimerism in donor-derived blood cells. However, this method is restricted to nucleated cells and cannot distinguish between dissociated single-cell lineages...
September 6, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28930919/2017-military-supplement-mini-review-perfluorocarbons-oxygen-transport-and-microcirculation-in-low-flow-states-in-vivo-and-in-vitro-studies
#2
Ivo P Torres Filho
The in vivo study of microvascular oxygen transport requires accurate and challenging measurements of several mass transfer parameters. Although recommended, blood flow and oxygenation are typically not measured in many studies where treatments for ischemia are tested. Therefore, the aim of this communication is to briefly review cardinal aspects of oxygen transport, and the effects of perfluorocarbon treatment on blood flow and oxygenation based mostly on studies performed in our laboratory. Since physiologically relevant events in oxygen transport take place at the microvascular level, we implemented the phosphorescence quenching technique coupled with non-invasive intravital videomicroscopy for quantitative evaluation of these events in vivo...
September 19, 2017: Shock
https://www.readbyqxmd.com/read/28930183/substituting-sodium-hydrosulfite-with-sodium-metabisulfite-improves-long-term-stability-of-a-distributable-paper-based-test-kit-for-point-of-care-screening-for-sickle-cell-anemia
#3
Kian Torabian, Dalia Lezzar, Nathaniel Z Piety, Alex George, Sergey S Shevkoplyas
Sickle cell anemia (SCA) is a genetic blood disorder that is particularly lethal in early childhood. Universal newborn screening programs and subsequent early treatment are known to drastically reduce under-five SCA mortality. However, in resource-limited settings, cost and infrastructure constraints limit the effectiveness of laboratory-based SCA screening programs. To address this limitation our laboratory previously developed a low-cost, equipment-free, point-of-care, paper-based SCA test. Here, we improved the stability and performance of the test by replacing sodium hydrosulfite (HS), a key reducing agent in the hemoglobin solubility buffer which is not stable in aqueous solutions, with sodium metabisulfite (MS)...
September 20, 2017: Biosensors
https://www.readbyqxmd.com/read/28929945/innovative-pcr-without-dna-extraction-for-african-sickle-cell-disease-diagnosis
#4
L Detemmerman, S Olivier, V Bours, F Boemer
OBJECTIVES: Hemoglobin (Hb) disorders consist of thalassemia and Hb structural variants, of which the major forms are associated with severe anemia and/or vascular occlusion. Current diagnostic techniques are highly accurate and mostly based on isoelectric focusing, high-performance liquid chromatography or mass spectrometry, which often require advanced laboratory equipment. In sub-Saharan Africa, the Hb disorders are mainly associated to the pathological variants hemoglobin S (HbS) and HbC...
September 20, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28928925/the-fortuitous-repair-of-a-common-bile-duct-injury-following-placement-of-a-percutaneous-transhepatic-cholangiogram-catheter
#5
Branum G Griswold, Jared A White
Common bile duct injuries are associated with a high rate of morbidity and mortality and are discussed frequently in the literature. These injuries may be difficult to diagnose intraoperatively and are often challenging to repair, necessitating referral to hepatobiliary surgery specialists at academic institutions. This case report highlights the management of a completely disrupted common bile duct identified post-operatively using a percutaneous transhepatic cholangiography (PTC) catheter to bridge the gap between the proximal and distal ductal injury prior to operative repair...
September 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28928607/speckle-tracking-evaluation-of-right-ventricular-functions-in-children-with-sickle-cell-disease
#6
Osama Abd Rab Elrasol Tolba, Mohamed Ramadan El-Shanshory, Mohamed Abd Elaziz El-Gamasy, Walid Ahmed El-Shehaby
BACKGROUND: Cardiac dysfunction is a risk factor for death in patients with sickle cell disease (SCD). AIM OF THE WORK: Aim of the work is to evaluate the right ventricular systolic and diastolic functions by tissue Doppler and speckling tracking imaging in children with SCD. SUBJECTS AND METHODS: Thirty children with SCD and thirty controls were subjected to clinical, laboratory evaluations, and echocardiographic study using GE Vivid 7 (GE Medical System, Horten, Norway with a 3...
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28927555/comparative-study-of-clinical-presentation-and-hematological-indices-in-hospitalized-sickle-cell-patients-infected-with-severe-plasmodium-falciparum-malaria
#7
Prasanta Purohit, Pradeep Kumar Mohanty, Siris Patel, Padmalaya Das, Jogeswar Panigrahi, Kishalaya Das
BACKGROUND: Sickle-cell-gene has a high frequency in malaria endemic regions. In India, though the prevalence of both sickle-cell-gene and malaria are high, no study has been carried out. This study aims to find out the possible differences in hematological and clinical parameters in severe falciparum malaria with respect to sickle cell genotypes. METHODS: Five hundred fourteen adults with severe falciparum malaria hospitalized at Department of Medicine, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, between August, 2010 to December, 2014 were included and categorized on the basis of sickle cell genotypes...
September 15, 2017: Journal of Infection and Public Health
https://www.readbyqxmd.com/read/28926088/treatments-for-priapism-in-boys-and-men-with-sickle-cell-disease
#8
REVIEW
Francis I Chinegwundoh, Sherie Smith, Kofi A Anie
BACKGROUND: Sickle cell disease comprises a group of genetic haemoglobin disorders. The predominant symptom associated with sickle cell disease is pain resulting from the occlusion of small blood vessels by abnormally 'sickle-shaped' red blood cells. There are other complications, including chronic organ damage and prolonged painful erection of the penis, known as priapism. Severity of sickle cell disease is variable, and treatment is usually symptomatic. Priapism affects up to half of all men with sickle cell disease, however, there is no consistency in treatment...
September 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28925802/higher-prevalence-of-spontaneous-cerebral-vasculopathy-and-cerebral-infarcts-in-a-mouse-model-of-sickle-cell-disease
#9
Hyacinth I Hyacinth, Courtney L Sugihara, Thomas L Spencer, David R Archer, Andy Y Shih
Stroke is a dramatic complication of sickle cell disease (SCD), which is associated with cerebral vasculopathies including moya moya, intravascular thrombi, cerebral hyperemia, and increased vessel tortuosity. The spontaneous occurrence of these pathologies in the sickle cell mouse model has not been described. Here, we studied Townes humanized sickle cell and age-matched control mice that were 13 months old. We used in vivo two-photon microscopy to assess blood flow dynamics, vascular topology, and evidence of cerebral vasculopathy...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28924974/incidence-and-predictive-score-for-delayed-hemolytic-transfusion-reaction-in-adult-patients-with-sickle-cell-disease
#10
David Narbey, Anoosha Habibi, Philippe Chadebech, Armand Mekontso-Dessap, Mehdi Khellaf, Jean-Daniel Lelièvre, Bertrand Godeau, Marc Michel, Frédéric Galactéros, Rachid Djoudi, Pablo Bartolucci, France Pirenne
Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication of transfusion in sickle cell disease (SCD). The frequency of DHTR is underestimated because its symptoms mimic those of vaso-occlusive crisis and antibodies (Abs) are often not detectable. No predictive factors for identifying patients likely to develop DHTR have yet been defined. We conducted a prospective single-center observational study over 30 months in adult sickle cell patients. We included 694 transfusion episodes (TEs) in 311 patients, divided into occasional TEs (OTEs: 360) and chronic transfusion program (CTEs: 334)...
September 19, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28922657/methods-employed-in-cytofluorometric-assessment-of-eryptosis-the-suicidal-erythrocyte-death
#11
Mohamed Jemaà, Myriam Fezai, Rosi Bissinger, Florian Lang
Suicidal erythrocyte death or eryptosis contributes to or even accounts for anemia in a wide variety of clinical conditions, such as iron deficiency, dehydration, hyperphosphatemia, vitamin D excess, chronic kidney disease (CKD), hemolytic-uremic syndrome, diabetes, hepatic failure, malignancy, arteriitis, sepsis, fever, malaria, sickle-cell disease, beta-thalassemia, Hb-C and G6PD-deficiency, Wilsons disease, as well as advanced age. Moreover, eryptosis is triggered by a myriad of xenobiotics and endogenous substances including cytotoxic drugs and uremic toxins...
September 1, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28919773/sickle-cell-retinopathy-improving-care-with-a-multidisciplinary-approach
#12
REVIEW
Farid Menaa, Barkat Ali Khan, Bushra Uzair, Abder Menaa
Sickle cell retinopathy (SCR) is the most representative ophthalmologic complication of sickle cell disease (SCD), a hemoglobinopathy affecting both adults and children. SCR presents a wide spectrum of manifestations and may even lead to irreversible vision loss if not properly diagnosed and treated at the earliest. Over the past decade, multidisciplinary research developments have focused upon systemic, genetic, and ocular risk factors of SCR, enabling the clinician to better diagnose and manage these patients...
2017: Journal of Multidisciplinary Healthcare
https://www.readbyqxmd.com/read/28919009/distinct-patterns-of-response-to-transfusion-therapy-for-different-chronic-complications-of-sickle-cell-disease-a-useful-insight
#13
Dimitris A Tsitsikas, Funmilayo Orebayo, Alexandra Agapidou, Roger J Amos
Two main sub-phenotypes have been described in sickle cell disease: one with higher baseline haemoglobin and a higher rate of painful crises and one with lower baseline haemoglobin, increased markers of haemolysis and a higher incidence of pulmonary hypertension, priapism and leg ulcers. We compared the patterns of response to regular automated red cell exchange transfusion over a five-year period of 21 patients with recurrent painful crises from the first group and 3 patients with pulmonary hypertension and 5 with recurrent severe stuttering priapism form the second and found them to be distinctly different...
August 18, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28913922/a-clinically-meaningful-fetal-hemoglobin-threshold-for-children-with-sickle-cell-anemia-during-hydroxyurea-therapy
#14
Jeremie H Estepp, Matthew P Smeltzer, Guolian Kang, Chen Li, Winfred C Wang, Christina Abrams, Banu Aygun, Russell E Ware, Kerri Nottage, Jane S Hankins
Hydroxyurea has proven clinical benefits and is recommended to be offered to all children with sickle cell anemia (SCA), but the optimal dosing regimen remains controversial. Induction of red blood cell fetal hemoglobin (HbF) by hydroxyurea appears to be dose-dependent. However, it is unknown whether maximizing HbF% improves clinical outcomes. HUSTLE (NCT00305175) is a prospective observational study with a primary goal of describing the long-term clinical effects of hydroxyurea escalated to maximal tolerated dose (MTD) in children with SCA...
September 14, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28912951/the-frequency-and-severity-of-epistaxis-in-children-with-sickle-cell-anaemia-in-eastern-uganda-a-case-control-study
#15
Amina Nardo-Marino, Thomas N Williams, Peter Olupot-Olupot
BACKGROUND: There are a paucity of data on epistaxis as it pertains to sickle cell anaemia. Some case studies suggest epistaxis to be a significant complication in patients with sickle cell anaemia in sub-Saharan Africa; however, no robust studies have sought to establish the epidemiology or pathophysiology of this phenomenon. METHODS: We conducted a case-control study with the aim of investigating the importance of epistaxis among children presenting with sickle cell anaemia at the Mbale Regional Referral Hospital in eastern Uganda...
2017: BMC Hematology
https://www.readbyqxmd.com/read/28912927/the-sixth-vital-sign-body-mass-index-in-patients-with-sickle-cell-disease
#16
Samir K Ballas
No abstract text is available yet for this article.
October 2017: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/28912365/d-dimer-in-african-americans-whole-genome-sequence-analysis-and-relationship-to-cardiovascular-disease-risk-in-the-jackson-heart-study
#17
Laura M Raffield, Neil A Zakai, Qing Duan, Cecelia Laurie, Joshua D Smith, Marguerite R Irvin, Margaret F Doyle, Rakhi P Naik, Ci Song, Ani W Manichaikul, Yongmei Liu, Peter Durda, Jerome I Rotter, Nancy S Jenny, Stephen S Rich, James G Wilson, Andrew D Johnson, Adolfo Correa, Yun Li, Deborah A Nickerson, Kenneth Rice, Ethan M Lange, Mary Cushman, Leslie A Lange, Alex P Reiner
OBJECTIVE: Plasma levels of the fibrinogen degradation product D-dimer are higher among African Americans (AAs) compared with those of European ancestry and higher among women compared with men. Among AAs, little is known of the genetic architecture of D-dimer or the relationship of D-dimer to incident cardiovascular disease. APPROACH AND RESULTS: We measured baseline D-dimer in 4163 AAs aged 21 to 93 years from the prospective JHS (Jackson Heart Study) cohort and assessed association with incident cardiovascular disease events...
September 14, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28912175/in-vitro-evidence-of-complement-activation-in-patients-with-sickle-cell-disease
#18
Eleni Gavriilaki, Maria Mainou, Ioanna Christodoulou, Eudoxia-Evaggelia Koravou, Aggeliki Paleta, Tasoula Touloumenidou, Apostolia Papalexandri, Anastasia Athanasiadou, Chrysa Apostolou, Philippos Klonizakis, Achilles Anagnostopoulos, Efthymia Vlachaki
No abstract text is available yet for this article.
September 14, 2017: Haematologica
https://www.readbyqxmd.com/read/28911832/association-of-silent-infarcts-in-sickle-cell-anemia-with-decreased-annexin-a5-resistance
#19
Kerry A Morrone, Lydia H Pecker, Jacob Rand, Jennifer Davila, Suzette Oyeku, Jane A Little, Xue Xiaonan, Deepa Manwani
BACKGROUND: Sickle cell anemia (SCA) is characterized by abnormally shaped, adhesive RBCs that interact with white blood cells and the endothelium, leading to chronic hemolysis, vasculopathy and a prothrombotic state. About 10% of subjects with a thrombotic event in the general population will have an associated antiphospholipid (aPL) antibody. One proposed mechanism for the thrombophilic nature of aPL antibodies is the disruption of the potent anticoagulant annexin A5 or Annexin A5 resistance (A5R)...
September 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28910308/pulmonary-hypertension-among-5-to-18-year-old-children-with-sickle-cell-anaemia-in-nigeria
#20
Ogochukwu J Sokunbi, Ekanem N Ekure, Edamisan O Temiye, Roosevelt Anyanwu, Christy A N Okoromah
BACKGROUND: Pulmonary hypertension (PHT) is a significant cause of mortality in patients with sickle cell disease (SCD). Few studies on PHT in SCD have been carried out in children. This study aimed to estimate the prevalence of PHT in children with sickle cell anaemia (SCA) and determine its clinical and laboratory correlates. METHODS: In this cross sectional study, evaluation involved obtaining bio-data, history and physical examination findings in 175 SCA subjects with haemoglobin genotype SS aged 5 to 18 years and 175 age and sex matched controls with haemoglobin genotype AA...
2017: PloS One
keyword
keyword
3743
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"