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Sickle cell disease

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https://www.readbyqxmd.com/read/29451227/statural-growth-and-prevalence-of-endocrinopathies-in-relation-to-liver-iron-content-lic-in-adult-patients-with-beta-thalassemia-major-btm-and-sickle-cell-disease-scd
#1
Mohamed A Yassin, Ashraf T Soliman, Vincenzo De Sanctis, Mohammad Aj Abdula, Lubna M Riaz, Firdous F Ghori, Anil Yousaf, Abdulqadir J Nashwan, Sandara Abusamaan, Abbas Moustafa, Samah Kohla, Dina S Soliman
Despite regular blood transfusion and iron chelation therapy, growth impairment and pubertal delay are commonly seen in children and adolescents with transfusion-dependent Beta thalassaemia major (BTM) and sickle cell disease (SCD). We evaluated growth parameters and endocrine disorders in relation to the liver iron concentration (LIC) assessed by the Ferriscan® method in a cohort of adults with SCD (n =40) and BTM (n = 52) receiving blood transfusions and iron chelation therapy since early childhood. Before transfusion, hemoglobin concentration had not been less than 9 g/dl in the past 12 years; subcutaneous daily desferrioxamine was administered for all of them since early childhood (2- 5 years of age)...
February 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29450676/comorbid-obstructive-sleep-apnea-and-increased-risk-for-sickle-cell-disease-morbidity
#2
Tal Katz, Jeffrey Schatz, Carla W Roberts
PURPOSE: Sickle cell disease (SCD) imparts an increased risk for obstructive sleep apnea (OSA) in childhood. Studies of pediatric SCD have identified an increased risk for pain and neurologic complications with comorbid OSA. We determined the rate of a broad range of SCD-related medical complications to better characterize the spectrum of SCD complications related to OSA. METHODS: Retrospective chart review at a single hematology clinic identified 641 youth with SCD who received consistent screenings for OSA as part of routine hematological health maintenance visits over an 11-year period...
February 15, 2018: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/29449005/association-between-oxidative-stress-genetic-factors-and-clinical-severity-in-children-with-sickle-cell-anemia
#3
Céline Renoux, Philippe Joly, Camille Faes, Pauline Mury, Buse Eglenen, Mine Turkay, Gokce Yavas, Ozlem Yalcin, Yves Bertrand, Nathalie Garnier, Daniela Cuzzubbo, Alexandra Gauthier, Marc Romana, Berenike Möckesch, Giovanna Cannas, Sophie Antoine-Jonville, Vincent Pialoux, Philippe Connes
OBJECTIVES: To investigate the associations between several sickle cell disease genetic modifiers (beta-globin haplotypes, alpha-thalassemia, and glucose-6-phosphate dehydrogenase deficiency) and the level of oxidative stress and to evaluate the association between oxidative stress and the rates of vaso-occlusive events. STUDY DESIGN: Steady-state oxidative and nitrosative stress markers, biological variables, genetic modulators, and vaso-occlusive crisis events requiring emergency admissions were measured during a 2-year period in 62 children with sickle cell anemia (58 SS and 4 Sβ 0 )...
February 13, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29447888/pathogenesis-of-chronic-rhinosinusitis-in-patients-affected-by-%C3%AE-thalassemia-major-and-sickle-cell-anaemia-post-allogenic-bone-marrow-transplant
#4
F Martino, R Di Mauro, K Paciaroni, J Gaziev, C Alfieri, L Greco, R Floris, S Di Girolamo, M Di Girolamo
OBJECTIVES: Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29446825/phytomedicines-medicines-derived-from-plants-for-sickle-cell-disease
#5
REVIEW
Oluseyi Oniyangi, Damian H Cohall
BACKGROUND: Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. Laboratory research and limited clinical trials have suggested positive effects of phytomedicines both in vivo and in vitro...
February 15, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29445298/presence-of-pain-on-three-or-more-days-of-the-week-is-associated-with-worse-patient-reported-outcomes-in-adults-with-sickle-cell-disease
#6
Nitya Bakshi, Diana Ross, Lakshmanan Krishnamurti
While acute episodic pain is the hallmark of sickle cell disease (SCD), transition to chronic pain is a major cause of morbidity and impaired quality of life. One of the core diagnostic criteria used by Analgesic, Anesthetic, and Addiction Clinical Trial Translations Innovations Opportunities and Networks-American Pain Society Pain Taxonomy (AAPT) to define chronic SCD pain is the presence of pain on a "majority of days" in the past 6 months in one or more locations. The frequency characteristic of "majority of days" is adapted from the criteria of 15 days or more per month, used to define chronic migraine, but there are inadequate data to support this cutoff in SCD...
2018: Journal of Pain Research
https://www.readbyqxmd.com/read/29444869/effective-screening-leads-to-better-outcomes-in-sickle-cell-disease
#7
EDITORIAL
Lisa M Shook, Russell E Ware
No abstract text is available yet for this article.
February 14, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29443408/vascular-access-for-red-blood-cell-exchange
#8
REVIEW
Zaher K Otrock, Suzanne R Thibodeaux, Ronald Jackups
Red blood cell exchange is the process of removing red blood cells from a patient and replacing them with donated blood using either automated or manual techniques. Red blood cell exchange is a well-recognized and effective therapy for many red blood cell-related diseases, especially sickle cell disease. However, decisions regarding the best methods for vascular access are not intuitive and must account for the patient's clinical condition, complication risks, and lifestyle, especially in the context of long-term vascular access...
February 2018: Transfusion
https://www.readbyqxmd.com/read/29438485/lower-than-expected-elevated-tricuspid-regurgitant-jet-velocity-in-adults-with-sickle-cell-disease-in-nigeria
#9
Baba Maiyaki Musa, Chisom N Odoh, Najibah A Galadanci, Hadiza Saidu, Muktar H Aliyu
Background: Cardiopulmonary disease is a major cause of morbidity and mortality in persons with sickle cell disease (SCD). Tricuspid regurgitant jet velocity (TRJV) and predicted forced expiratory volume in 1 s (FEV1%) predicted are independently associated with death in SCD. The goal of this study was to determine the prevalence of elevated TRJV and the association, if any, between TRJV and FEV1% predicted among persons with sickle cell anaemia (SCA) in Nigeria. Methods: Using a cross-sectional design, we enrolled 100 adult Nigerians (≥15 y) with SCA...
February 9, 2018: International Health
https://www.readbyqxmd.com/read/29438213/new-no-donor-ncx-1443-therapeutic-effects-on-pulmonary-hypertension-in-the-sad-mouse-model-of-sickle-cell-disease
#10
Shariq Abid, Kanny Kebe, Amal Houssaïni, Françoise Tomberli, Elisabeth Marcos, Emilie Bizard, Marielle Breau, Aurelien Parpaleix, Claire-Marie Tissot, Bernard Maitre, Larissa Lipskaia, Genevieve Derumeaux, Elena Bastia, Armand Mekontso-Dessap, Serge Adnot
Nitric oxide (NO) donors may be useful for treating pulmonary hypertension (PH) complicating sickle cell disease (SCD), as endogenous NO is inactivated by hemoglobin released by intravascular hemolysis. Here, we investigated the effects of the new NO donor NCX1443 on PH in transgenic SAD mice, which exhibit mild SCD without severe hemolytic anemia. In SAD and wild-type (WT) mice, the pulmonary pressure response to acute hypoxia was similar and was abolished by 100 mg/kg NCX1443. The level of PH was also similar in SAD and WT mice exposed to chronic hypoxia (9% O2) alone or with SU5416, and was similarly reduced by daily NCX1443 gavage...
February 8, 2018: Journal of Cardiovascular Pharmacology
https://www.readbyqxmd.com/read/29437860/antibiotic-prophylaxis-for-children-with-sickle-cell-anemia
#11
Sarah L Reeves, Alison C Tribble, Brian Madden, Gary L Freed, Kevin J Dombkowski
BACKGROUND: Children with sickle cell anemia (SCA) are at increased risk for invasive pneumococcal disease; antibiotic prophylaxis significantly reduces this risk. We calculated the proportion of children with SCA who received ≥300 days of antibiotic prophylaxis and identified predictors of such receipt. METHODS: Children aged 3 months to 5 years with SCA were identified by the presence of 3 or more Medicaid claims with a diagnosis of SCA within a calendar year (2005-2012) in Florida, Illinois, Louisiana, Michigan, South Carolina, and Texas...
February 5, 2018: Pediatrics
https://www.readbyqxmd.com/read/29436011/improving-disease-knowledge-in-6-to-10-year-olds-with-sickle-cell-disease-a-quasi-experimental-study
#12
N Morrison-Levy, J Knight-Madden, T Royal-Thomas, L King, M Asnani
BACKGROUND: Increasing knowledge and understanding of disease is known to improve outcomes in persons living with a chronic illness. In this paper, we aim to compare the disease knowledge of children with sickle cell disease (SCD), age 6-10 years, who received an intervention (an educational colouring book on SCD) geared towards improving disease knowledge, to those who did not received the colouring book. METHODS: A quasi-experimental study was conducted where disease knowledge was determined in 56 children who had received the colouring book and compared to 60 children who did not receive this intervention...
February 12, 2018: Child: Care, Health and Development
https://www.readbyqxmd.com/read/29432593/selective-difficulties-in-lexical-retrieval-and-nonverbal-executive-functioning-in-children-with-hbss-sickle-cell-disease
#13
Barbara Arfé, Maria Montanaro, Elena Mottura, Michele Scaltritti, Renzo Manara, Giuseppe Basso, Laura Sainati, Raffaella Colombatti
Language deficits in multilingual children with sickle cell disease (SCD) are poorly understood. We tested the hypothesis that selective language deficits in this population could relate to an impaired frontal lobe functioning often associated with high-risk homozygous HbS disease (HbSS). In all, 32 children from immigrant communities with HbSS SCD aged 6 to 12 years (mean age = 9.03, n = 9 with silent infarcts) and 35 demographically matched healthy controls (mean age = 9.14) were tested on their naming skills, phonological and semantic fluency, attention, and selected executive functions (response inhibition and planning skills)...
February 8, 2018: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/29432581/14q32-and-let-7-micrornas-regulate-transcriptional-networks-in-fetal-and-adult-human-erythroblasts
#14
Samuel Lessard, Mélissa Beaudoin, Stuart H Orkin, Daniel E Bauer, Guillaume Lettre
In humans, fetal erythropoiesis takes place in the liver whereas adult erythropoiesis occurs in the bone marrow. Fetal and adult erythroid cells are not only produced at different sites, but are also distinguished by their respective transcriptional program. In particular, whereas fetal erythroid cells express γ-globin chains to produce fetal hemoglobin (HbF), adult cells express β-globin chains to generate adult hemoglobin. Understanding the transcriptional regulation of the fetal-to-adult hemoglobin switch is clinically important as re-activation of HbF production in adult erythroid cells would represent a promising therapy for the hemoglobin disorders sickle cell disease and β-thalassemia...
February 8, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29431623/prevalence-and-risk-factors-for-venous-thromboembolism-in-children-with-sickle-cell-disease-an-administrative-database-study
#15
Riten Kumar, Joseph Stanek, Susan Creary, Amy Dunn, Sarah H O'Brien
A hypercoagulable state resulting in increased venous thromboembolism (VTE) has been described in adults with sickle cell disease (SCD), but similar data for children are lacking. The objective of this retrospective cohort study was to describe the rate of VTE and risk factors associated with VTE in children with SCD across tertiary-care children's hospitals in the United States between the years 2009 and 2015. We used the Pediatric Health Information System database to investigate all pediatric patients with SCD admitted to 1 of 48 participating institutions between 1 January 2009 and 30 September 2015...
February 13, 2018: Blood Advances
https://www.readbyqxmd.com/read/29431216/mast-cell-neural-interactions-contribute-to-pain-and-itch
#16
REVIEW
Kalpna Gupta, Ilkka T Harvima
Mast cells are best recognized for their role in allergy and anaphylaxis, but increasing evidence supports their role in neurogenic inflammation leading to pain and itch. Mast cells act as a "power house" by releasing algogenic and pruritogenic mediators, which initiate a reciprocal communication with specific nociceptors on sensory nerve fibers. Consequently, nerve fibers release inflammatory and vasoactive neuropeptides, which in turn activate mast cells in a feedback mechanism, thus promoting a vicious cycle of mast cell and nociceptor activation leading to neurogenic inflammation and pain/pruritus...
March 2018: Immunological Reviews
https://www.readbyqxmd.com/read/29428552/high-bone-mineral-density-in-sickle-cell-disease-prevalence-and-characteristics
#17
Gonzalo De Luna, Brigitte Ranque, Marie Courbebaisse, Jean-Antoine Ribeil, Djamal Khimoud, Sidonie Dupeux, Jonathan Silvera, Lucile Offredo, Jacques Pouchot, Jean-Benoît Arlet
BACKGROUND: Osteosclerosis (OSC) is a rarely studied complication of sickle cell disease (SCD). The objective of our study was to determine the prevalence and characteristics of high bone mineral density (BMD) and its radiological features in adult SCD patients. METHODS: This prospective observational study was conducted from May 2007 to May 2016 in consecutive patients with steady-state SCD at two university hospitals. The BMD of the lumbar spine (L1-L4) and right femoral neck was determined by dual energy X-ray absorptiometry...
February 8, 2018: Bone
https://www.readbyqxmd.com/read/29428339/sickle-cell-disease-an-overview-of-the-disease-and-its-systemic-effects
#18
REVIEW
Nadia Kawar, Sahar Alrayyes, Anne-Ashley Compton, Haider Aljewari, Danny Baghdan, Bin Yang, Reihaneh Goreishi, Sandra Mohama
No abstract text is available yet for this article.
February 7, 2018: Disease-a-month: DM
https://www.readbyqxmd.com/read/29419921/the-evolving-contribution-of-non-communicable-diseases-ncds-to-maternal-mortality-in-jamaica-1998-2015-a-population-based-study
#19
Affette M McCaw-Binns, Leroy V Campbell, Simone S Spence
OBJECTIVE: Describe trends in indirect cause-specific pregnancy-related mortality from 1998-2015. DESIGN: Secondary analysis of annual, national cross-sectional database of maternal and late maternal deaths, identified through active surveillance of deaths among women 10-50 years. SETTING: Jamaica, a middle-income Caribbean country. POPULATION: Maternal and late maternal deaths. METHODS: Descriptive trend analyses of demographic and cause-specific maternal and pregnancy-related mortality ratios undertaken comparing the periods 1998-2003, 2004-9 and 2010-15...
February 8, 2018: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/29419693/organ-damage-mitigation-with-the-baskent-sickle-cell-medical-care-development-program-bascare
#20
Hakan Ozdogu, Can Boga, Suheyl Asma, Ilknur Kozanoglu, Cigdem Gereklioglu, Mahmut Yeral, Nurhilal Turgut Buyukkurt, Soner Solmaz, Aslı Korur, Pelin Aytan, Erkan Maytalman, Mutlu Kasar
The Eastern Mediterranean is among the regions where sickle cell disease (SCD) is common. The morbidity and mortality of this disease can be postponed to adulthood through therapies implemented in childhood. The present study focuses on the organ damage-reducing effects of the Baskent Sickle Cell Medical Care Development Program (BASCARE), which was developed by a team who lives in this region and has approximately 25 years of experience. The deliverables of the program included the development of an electronic health recording system (PRANA) and electronic vaccination system; the use of low citrate infusion in routine prophylactic automatic erythrocyte exchange (ARCE) programs including pregnant women; the use of leukocyte-filtered and irradiated blood for transfusion; the use of magnetic resonance imaging methods (T2) for the management of transfusion-related hemosiderosis; and the implementation of an allogeneic hematopoietic stem cell transplantation protocol for adult patients...
February 2018: Medicine (Baltimore)
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