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Sickle cell disease

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https://www.readbyqxmd.com/read/28447889/bone-marrow-as-a-hematopoietic-stem-cell-source-for-gene-therapy-in-sickle-cell-disease-scd-evidence-from-rhesus-and-scd-patients
#1
Naoya Uchida, Atsushi Fujita, Matthew M Hsieh, Aylin Bonifacino, Allen E Krouse, Mark E Metzger, Robert E Donahue, John F Tisdale
Steady state bone marrow (BM) is the preferred hematopoietic stem cell (HSC) source for gene therapy in sickle cell disease (SCD) due to the recognized risk of vaso-occlusive crisis during granulocyte colony-stimulating factor mobilization. We previously established clinically relevant HSC gene transfer in the rhesus model following transplantation of mobilized peripheral blood (PB) CD34+ cells transduced with lentiviral vectors. In this study, we examined steady state bone marrow (BM) in the rhesus competitive repopulation model and demonstrate similar gene marking in vitro and in vivo, as compared to mobilized PB CD34+ cells...
April 27, 2017: Human Gene Therapy. Clinical Development
https://www.readbyqxmd.com/read/28446442/paracentral-acute-middle-maculopathy-precursor-to-macular-thinning-in-sickle-cell-retinopathy
#2
Syed Amal Hussnain, Patrick A Coady, Kathleen M Stoessel
We present a case of paracentral acute middle maculopathy (PAMM) in a patient with sickle cell disease (SCD). Though gradual capillary loss may contribute to pathophysiology of sickle cell retinopathy as well, our case suggests that PAMM may be the precursor lesion to macular thinning commonly observed in patients with SCD. In addition, fluorescein angiography may be unable to detect these acute vascular events occurring at the deep capillary plexus.
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28444215/behavioral-and-pharmacological-adherence-in-pediatric-sickle-cell-disease-parent-child-agreement-and-family-factors-associated-with-adherence
#3
Page H Klitzman, Julia K Carmody, Mary H Belkin, David M Janicke
This study aimed to evaluate agreement between children and parents on a measure of behavioral and pharmacological adherence in children with sickle cell disease (SCD), and the associations among family factors (i.e., problem-solving skills, routines, communication) and adherence behaviors. In all, 85 children (aged 8-18 years) with SCD and their parents completed questionnaires assessing individual and family factors. Overall parent-child agreement on an adherence measure was poor, particularly for boys and older children...
April 21, 2017: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/28443234/plastic-bronchitis-an-unusual-complication-of-acute-chest-syndrome-in-adult
#4
Sarah Feray, Pierre Mora, Maxens Decavele, Tài Pham, El Mahdi Hafiani, Muriel Fartoukh
Plastic bronchitis is used to designate endobronchial plugs of rubber-like consistency that form into bronchial trees. It has been described in several diseases like asthma, cystic fibrosis, pulmonary infection, cyanotic congenital heart disease and in few young children with homozygous sickle cell disease. We report the first sickle cell adult case of plastic bronchitis during acute chest syndrome. He developed severe acute respiratory distress syndrome. This unusual presentation related to obstruction by voluminous casts may alert physicians to focus more on the bronchi in sickle cell patients...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28441316/hemolysis-following-intraoperative-cell-salvage-replacement-in-a-scoliosis-patient-with-sickle-cell-trait-a-case-report
#5
Daniel You, Alejandro Peiro Garcia, Fabio Ferri-de-Barros, David Parsons
STUDY DESIGN: Case Report. OBJECTIVE: To describe a novel presentation of acute renal failure associated with hemolysis following intraoperative cell salvage (ICS) in a neuromuscular scoliosis patient with sickle cell trait (SCT). SUMMARY OF BACKGROUND DATA: Hemolysis associated acute renal failure following ICS in patients with SCT has not been previously reported. Sickle cell disease is regarded as a relative contraindication for ICS due to the risk of red blood cell sickling in the hypoxic cell saver reservoir...
April 24, 2017: Spine
https://www.readbyqxmd.com/read/28440126/transcranial-doppler-screening-for-stroke-risk-in-children-with-sickle-cell-disease-a-systematic-review
#6
Sara Mazzucco, Marina Diomedi, Amrana Qureshi, Laura Sainati, Soundrie T Padayachee
Background Sickle cell disease (SCD) is one of the most common causes of stroke in children worldwide. Based on the results of the Stroke Prevention Trial in Sickle Cell Anemia (STOP), annual transcranial Doppler ultrasound (TCD) screening for affected children is standard practice. However, the need for TCD surveillance programs could override the accuracy of the screening, affecting the correct stratification of stroke risk and subsequent clinical management of the target population. Aims To shed light on this issue, a systematic review of the literature on TCD screening for children and adolescents with SCD was carried out (CRD42016050549), according to a list of clinically relevant questions, with a particular focus on screening practices in European countries...
January 1, 2017: International Journal of Stroke: Official Journal of the International Stroke Society
https://www.readbyqxmd.com/read/28439419/chronic-complications-and-quality-of-life-of-patients-living-with-sickle-cell-disease-and-receiving-care-in-three-hospitals-in-cameroon-a-cross-sectional-study
#7
Anne M Andong, Eveline D T Ngouadjeu, Cavin E Bekolo, Vincent S Verla, Daniel Nebongo, Yannick Mboue-Djieka, Simeon-Pierre Choukem
BACKGROUND: Sickle Cell Disease (SCD) is associated with chronic multisystem complications that significantly influence the quality of life (QOL) of patients early in their life. Although sub-Saharan Africa bears 75% of the global burden of SCD, there is a paucity of data on these complications and their effects on the QOL. We aimed to record these chronic complications, to estimate the QOL, and to identify the corresponding risk factors in patients with SCD receiving care in three hospitals in Cameroon...
2017: BMC Hematology
https://www.readbyqxmd.com/read/28438159/prevalence-of-plasmodium-falciparum-and-non-p-falciparum-infections-in-a-highland-district-in-ghana-and-the-influence-of-hiv-and-sickle-cell-disease
#8
Ewurama D A Owusu, Charles A Brown, Martin P Grobusch, Petra Mens
BACKGROUND: In the past two decades, there has been a reported decline in malaria in Ghana and the rest of the world; yet it remains the number one cause of mortality and morbidity. Human immuno-deficiency virus (HIV) and sickle cell disease (SCD) share a common geographical space with malaria in sub-Saharan Africa and an interaction between these three conditions has been suggested. This study determined the Plasmodium falciparum and non-P. falciparum status of symptomatic and non-symptomatic residents of Mpraeso in the highlands of Kwahu-South district of Ghana based on evidence of current national decline...
April 24, 2017: Malaria Journal
https://www.readbyqxmd.com/read/28437457/tissue-factor-dependent-coagulation-activation-by-heme-a-thromboelastometry-study
#9
Gleice Regina de Souza, Bidossessi Wilfried Hounkpe, Maiara Marx Luz Fiusa, Marina Pereira Colella, Joyce M Annichino-Bizzacchi, Fabiola Traina, Fernando Ferreira Costa, Erich Vinicius De Paula
Heme has been characterized as potent trigger of inflammation. In hemostasis, although heme has been shown to both induce and inhibit different compartments of hemostasis, its net effect on the hemostatic balance, and the biological relevance of these effects remain to be determined. Herein we evaluated the effect of heme on hemostasis using a global assay able to generate clinically relevant data in several other complex hemostatic diseases. Citrated whole blood samples from healthy participants were stimulated by heme or vehicle and incubated for 4h at 37°C...
2017: PloS One
https://www.readbyqxmd.com/read/28436975/haploidentical-stem-cell-transplantation-with-cd3-cd19-depleted-peripheral-stem-cells-for-patients-with-advanced-stage-sickle-cell-disease-and-no-alternative-donor-results-of-a-pilot-study
#10
J Foell, B Pfirstinger, K Rehe, D Wolff, E Holler, S Corbacioglu
No abstract text is available yet for this article.
April 24, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28436274/depletion-of-hdl3-high-density-lipoprotein-and-altered-functionality-of-hdl2-in-blood-from-sickle-cell-patients
#11
Eric Soupene, Sandra K Larkin, Frans A Kuypers
In sickle cell disease (SCD), alterations of cholesterol metabolism is in part related to abnormal levels and activity of plasma proteins such as lecithin cholesterol acyltransferase (LCAT), and apolipoprotein A-I (ApoA-I). In addition, the size distribution of ApoA-I high density lipoproteins (HDL) differs from normal blood. The ratio of the amount of HDL2 particle relative to the smaller higher density pre-β HDL (HDL3) particle was shifted toward HDL2. This lipoprotein imbalance is exacerbated during acute vaso-occlusive episodes (VOE) as the relative levels of HDL3 decrease...
January 1, 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28435481/a-patient-centered-emergency-department-management-strategy-for-sickle-cell-disease-super-utilizers
#12
Grant G Simpson, Hallie R Hahn, Alex A Powel, Robert R Leverence, Linda A Morris, Lara G Thompson, Marc S Zumberg, Deepa J Borde, Joseph A Tyndall, Jonathan J Shuster, Donald M Yealy, Brandon R Allen
INTRODUCTION: A subpopulation of sickle-cell disease patients, termed super-utilizers, presents frequently to emergency departments (EDs) for vaso-occlusive events and may consume disproportionate resources without broader health benefit. To address the healthcare needs of this vulnerable patient population, we piloted a multidisciplinary intervention seeking to create and use individualized patient care plans that alter utilization through coordinated care. Our goals were to assess feasibility primarily, and to assess resource use secondarily...
April 2017: Western Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28435037/therapeutic-strategies-in-sickle-cell-anemia-the-past-present-and-future
#13
REVIEW
Queenie Fernandes
Sickle Cell Anemia (SCA) was one of the first hemoglobinopathies to be discovered. It is distinguished by the mutation-induced expression of a sickle cell variant of hemoglobin (HbS) that triggers erythrocytes to take a characteristic sickled conformation. The complex physiopathology of the disease and its associated clinical complications has initiated multi-disciplinary research within its field. This review attempts to lay emphasis on the evolution, current standpoint and future scope of therapeutic strategies in SCA...
April 20, 2017: Life Sciences
https://www.readbyqxmd.com/read/28432164/use-of-gabapentin-in-the-treatment-of-chronic-pain-in-an-adolescent-with-sickle-cell-disease
#14
Cátia R Correia, Ana Teresa Soares, Laura Azurara, Maria João Palaré
Vaso-occlusive crises are the most common manifestation of sickle cell disease (SCD) and the main cause of hospital admission in these patients. There is emerging evidence that vaso-occlusive pain has both nociceptive and neuropathic components. However, the treatment of SCD-related pain with neuropathic drugs has not yet been systematically studied, particularly in children. We describe a 14-year-old girl with SCD and multiple hospital admissions for pain management for severe acute vaso-occlusive pain episodes...
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28426137/hydroxyurea-hydroxycarbamide-for-sickle-cell-disease
#15
REVIEW
Sarah J Nevitt, Ashley P Jones, Jo Howard
BACKGROUND: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea (hydroxycarbamide), an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising fetal haemoglobin. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the effects of hydroxyurea therapy in people with SCD (all genotypes), of any age, regardless of setting...
April 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28425750/modeling-alloantibody-formation-to-high-incidence-red-blood-cell-antigens-in-immune-responders-using-genotypic-data
#16
Patricia A R Brunker, Keerthana Ravindran, R S Shirey
Alloimmunization to red blood cell antigens is unpredictable and poorly understood. Patients who are negative for high-incidence antigens (HIAs) are at risk for developing the corresponding antibodies. Molecular methods can easily predict the lack of an antigen and thus, the risk of an individual to become immunized. We examined the prevalence and risk factors for HIA alloimmunization in patients at risk based on genotyping results. Genotyping using a molecular method (HEA BeadChip™, Immucor, Warren, NJ) was performed on all patient specimens referred for molecular testing over 45 months; serologic and clinical data were analyzed...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28425749/hemolytic-transfusion-reaction-attributable-to-anti-dia
#17
Arthur J Joyce, Kelli M Quantock, Ray Banh, Yew W Liew
In situations when a patient's antibody detection test is negative, many institutions have moved from an indirect antiglobulin test (IAT) crossmatch to an electronic crossmatch system. Here we report a case of an acute hemolytic transfusion reaction attributable to anti-Dia in a patient with a negative antibody detection test. A 22-year-old female patient with a diagnosis of β thalassemia and sickle cell anemia commenced a routine exchange transfusion of 5 units of red blood cells (RBCs) in the apheresis unit as part of her regular treatment...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28424766/the-need-for-hematology-nurse-education-in-low-and-middle-income-countries-a-community-case-study-in-tanzania
#18
Julie M Buser
Hematology-related diseases, such as anemia, malaria, sickle cell disease (SCD), and blood cancers, have differing rates of survival between high-income and low- and middle-income countries (LMICs). Nurses in LMICs have an unmet need for specialty training and education to address hematology and hemato-oncology disorders. A gap in the literature exists about hematology nurse education and clinical service demands in LMICs. This community case study documents a collaborative hematology and basic hemato-oncology education program to sustainably strengthen nurse capacity at a national referral hospital and university in Tanzania...
2017: Frontiers in Public Health
https://www.readbyqxmd.com/read/28423290/sickle-cell-disease
#19
Frédéric B Piel, Martin H Steinberg, David C Rees
No abstract text is available yet for this article.
April 20, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28422403/blood-chimerism-in-dizygotic-monochorionic-twins-during-five-years-observation
#20
Morten Hanefeld Dziegiel, Morten Høgh Hansen, Sofie Haedersdal, Angela Natalie Barrett, Klaus Rieneck, Katharina Maria Main, Anne Todsen Hansen, Frederik Banch Clausen
Dizygotic monochorionic twin pregnancies can result in blood chimerism due to in utero twin-to-twin exchange of stem cells. In this case, we examined the proportion of allogeneic red blood cells (RBCs) by flow cytometry and the proportion of allogeneic nucleated cells by digital PCR at seven months and again at five years. We found an increase in the proportion of allogeneic cells from 63% to 89% in one twin, and a similar increase in autologous cells in the other twin from 57% to 84%. A paradigm for stem cell therapy could be modelled on this case: induction of tolerance and chimerism by antenatal transfusion of donor stem cells...
April 19, 2017: American Journal of Transplantation
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