28,432 results match your criteria Sickle Cell Nephropathy
Pediatr Dermatol 2018 Dec 9. Epub 2018 Dec 9.
Department of Dermatology and Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
A pseudoxanthoma elasticum (PXE)-like phenotype develops in a subset of patients with inherited hemoglobinopathies. Although PXE tissue changes are thought to develop in the absence of ABCC6 mutations in patients with beta-thalassemia, ABCC6 mutations have not been well evaluated among sickle cell disease patients with PXE-like disease. To our knowledge, we describe the first patient with sickle cell disease, PXE skin findings, and two confirmed pathogenic ABCC6 mutations. Read More
Am J Hematol 2018 Dec 8. Epub 2018 Dec 8.
Department of Pediatric Haematology, Evelina Children's Hospital, Guy's and St. Thomas NHS Trust, London, United Kingdom.
Commun Biol 2018 30;1:211. Epub 2018 Nov 30.
1Department of Infectious Diseases, Parasitology, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 324, 69120 Heidelberg, Germany.
Sickle cell trait, a common hereditary blood disorder, protects carriers from severe disease in infections with the human malaria parasite . Protection is associated with a reduced capacity of parasitized erythrocytes to cytoadhere to the microvascular endothelium and cause vaso-occlusive events. However, the underpinning cellular and biomechanical processes are only partly understood and the impact on endothelial cell activation is unclear. Read More
Mol Ther Methods Clin Dev 2018 Dec 1;11:167-179. Epub 2018 Nov 1.
Department of Life Sciences, University of Modena and Reggio Emilia, Modena, Italy.
Sickle cell disease (SCD) is caused by a mutation (E6V) in the hemoglobin (Hb) β-chain that induces polymerization of Hb tetramers, red blood cell deformation, ischemia, anemia, and multiple organ damage. Gene therapy is a potential alternative to human leukocyte antigen (HLA)-matched allogeneic hematopoietic stem cell transplantation, available to a minority of patients. We developed a lentiviral vector expressing a β-globin carrying three anti-sickling mutations (T87Q, G16D, and E22A) inhibiting axial and lateral contacts in the HbS polymer, under the control of the β-globin promoter and a reduced version of the β-globin locus-control region. Read More
Nat Biomed Eng 2018 23;2:453-463. Epub 2018 Apr 23.
Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, GA, USA.
Alterations in the mechanical properties of erythrocytes occurring in inflammatory and hematologic disorders such as sickle cell disease (SCD) and malaria often lead to increased endothelial permeability, haemolysis, and microvascular obstruction. However, the associations among these pathological phenomena remain unknown. Here, we report a perfusable, endothelialized microvasculature-on-a-chip featuring an interpenetrating-polymer-network hydrogel that recapitulates the stiffness of blood-vessel intima, basement membrane self-deposition and self-healing endothelial barrier function for longer than 1 month. Read More
Case Rep Genet 2018 7;2018:6898546. Epub 2018 Nov 7.
Consortium for Health and Military Performance, Department of Military and Emergency Medicine, Hébert School of Medicine, Uniformed Services University, 4301 Jones Bridge Rd., Bethesda, MD 20184, USA.
Individuals with Sickle Cell Trait (SCT), generally considered a benign carrier state of hemoglobin S (HbAS), are thought to be at risk for exertional rhabdomyolysis and hematuria, conditions that can also be caused by various other acquired and inherited factors. We report an SCT positive service member with an exertional rhabdomyolysis event, recurrent hematuria with transient proteinuria, and episodic burning pain in the lower extremities. Clinical and genetic studies revealed the multifactorial nature of his complex phenotype. Read More
Blood 2018 Dec 10. Epub 2018 Dec 10.
Center for Sickle Cell Disease, University of Tennessee Health Science Center, Memphis, TN, United States.
Genomics 2018 Dec 4. Epub 2018 Dec 4.
National Institute of Immunohematology (ICMR), 13th Floor, New Multi-storeyed Building, K.E.M. Hospital Campus, Parel, Mumbai 400012, India. Electronic address:
The master erythroid regulator KLF1,plays a pivotal role during erythroid lineage development by regulating the expression of many erythroid genes. Variations in the KLF1 gene are found to be associated with varied erythroid phenotypes. With the aim of determining the role of KLF1 gene variations in HbF induction and their genotype phenotype relationship, in this study, we screened 370 individuals with different hemoglobinopathy condition. Read More
Cochrane Database Syst Rev 2018 Dec 5;12:CD006975. Epub 2018 Dec 5.
Department of Paediatrics, University of Calabar Teaching Hospital, PMB 1115, Calabar, Cross River State, Nigeria, 540261.
Background: Salmonella infections are a common bacterial cause of invasive disease in people with sickle cell disease especially children, and are associated with high morbidity and mortality rates. Although available in some centres, people with sickle cell anaemia are not routinely immunized with salmonella vaccines. This is an update of a previously published Cochrane Review. Read More
PLoS One 2018 6;13(12):e0208916. Epub 2018 Dec 6.
Federal University of Sergipe, Aracaju, Brazil.
The aim of this study was to identify the levels of physical activity and sedentary behaviour of children and adolescents with sickle cell disease (SCA) compared to healthy individuals. A cross-sectional study with a quantitative approach was performed at a reference center for the treatment of patients with hemoglobinopathies in northeastern Brazil. Patients were recruited between October 2015 and January 2017. Read More
J Pediatr Hematol Oncol 2018 Dec 4. Epub 2018 Dec 4.
Departments of Otolaryngology-Head and Neck Surgery.
This study sought to examine if modern medical evaluations including newborn screening and early diagnosis along with better methods of disease control have improved rates of hearing loss in children with sickle cell disease (SCD). Audiometric and medical data for patients with SCD was obtained from the AudGen Database and analyzed for the presence of hearing loss, type of hearing loss, severity of hearing loss, and correlation with comorbid conditions. Children with sickle cell trait (SCT) were used as a comparison group. Read More
J Pediatr Hematol Oncol 2018 Dec 4. Epub 2018 Dec 4.
Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait.
Background: Thrombospondin-1 (TSP-1) and 25-hydroxyvitamin D (25-OHD) play significant roles in the pathogenesis of sickle cell anemia (SCA). TSP-1 enhances cellular adhesion/inflammation, hence contributing to vaso-occlusive crisis (VOC); vitamin D, in contrast, retards inflammation and may lower rate of pain episodes. We determined serum levels of TSP-1 and 25-OHD in Nigerian children with SCA and their matched hemoglobin AA controls; and assess the relationship between the 2 biomarkers. Read More
Saudi Med J 2018 Dec;39(12):1253-1258
Department of Biological Sciences, Beirut Arab University, Beirut, Lebanon. E-mail.
Objectives: To examine the association between beta-globin sequence variations and phenotypes of sickle-cell disease (SCD) complications among Palestinian refugees in Lebanon correlating them with chromatographic readings and co-inheritance with β-thalassemia traits. Methods: This cross-sectional study included 47 Palestinian refugees aged 4 to 54 living in different regions in Lebanon during the year 2015. Participant filled a well-designed questionnaire. Read More
Bone Marrow Transplant 2018 Dec 5. Epub 2018 Dec 5.
International Centre for Transplantation in Thalassaemia and Sickle Cell Anaemia, Mediterranean Institute of Haematology, Rome, Italy.
To candidate children as bone marrow donors raises two main concerns: donor safety and adequate marrow cell dose. Data in the field are limited and guidelines for child donor care management are lacking. In this context, we herein report the experience collected in our center by comparing very-young donors (defined as age ≤ 3 years) with young donors (defined as age > 3 years) who donated bone marrow (BM) for patients affected by beta-globin disorders. Read More
Blood Adv 2018 Dec;2(23):3462-3478
Laboratory of Complement Biology and.
The intraerythrocytic parasite is the number 1 cause of transfusion-transmitted infection and can induce serious, often life-threatening complications in immunocompromised individuals including transfusion-dependent patients with sickle cell disease (SCD). Despite the existence of strong long-lasting immunological protection against a second infection in mouse models, little is known about the cell types or the kinetics of protective adaptive immunity mounted following infection, especially in infection-prone SCD that are thought to have an impaired immune system. Here, we show, using a mouse infection model, that infected wild-type (WT) mice mount a very strong adaptive immune response, characterized by (1) coordinated induction of a robust germinal center (GC) reaction; (2) development of follicular helper T (T) cells that comprise ∼30% of splenic CD4 T cells at peak expansion by 10 days postinfection; and (3) high levels of effector T-cell cytokines, including interleukin 21 and interferon γ, with an increase in the secretion of antigen (Ag)-specific antibodies (Abs). Read More
Sensors (Basel) 2018 Dec 4;18(12). Epub 2018 Dec 4.
Centre of Advanced Electronic and Communication Engineering, Universiti Kebangsaan Malaysia, Bangi 43600, Malaysia.
A simple, compact sickle-shaped printed antenna with a slotted ground plane is designed and developed for broadband circularly polarized (CP) radiation. The sickle-shaped radiator with a tapered feed line and circular slotted square ground plane are utilized to realize the wideband CP radiation feature. With optimized dimensions of 0. Read More
Neurosci Lett 2018 Nov 30. Epub 2018 Nov 30.
Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, United States.
Pain is the leading cause for hospitalization in patients with sickle cell disease (SCD). While the characteristics of SCD pain can vary widely between patients and between phases of the disease (e.g. Read More
Transl Behav Med 2018 Dec 3. Epub 2018 Dec 3.
UCSF Center for Vulnerable Populations, Zuckerberg San Francisco General Hospital, University of California, San Francisco, CA, USA.
Sickle cell disease (SCD) is a severe hemoglobinopathy characterized by acute and chronic pain. Sufferers of the disease, most of whom are underrepresented minorities, are at increased risk for mental health disorders. The purpose of this study is to test the acceptability and implementation of a computerized cognitive behavioral therapy (cCBT) intervention, Beating the Blues, to improve depression, anxiety, and pain in patients with SCD. Read More
Methods Mol Biol 2019 ;1885:207-219
Department of Medical Genetics, St. Sophia's Children's Hospital, National and Kapodistrian University of Athens, Athens, Greece.
The inherited disorders of hemoglobin synthesis constitute the most common monogenic diseases worldwide. The clinical severity of β-thalassemia major and the sickle cell syndromes targets them as priority genetic diseases for prevention programs, which incorporates population screening to identify heterozygotes, with the option of prenatal diagnosis for carrier couples. Rapid genotype characterization is fundamental in the diagnostic laboratory, especially when offering prenatal diagnosis. Read More
J Trop Dis Public Health 2018 10;6(2):260. Epub 2018 Jul 10.
Department of Public Health Sciences, Loyola University Chicago Stritch School of Medicine, Maywood, IL, USA.
Sickle Cell Disease (SCD) is among the most common single-gene diseases in the world but evidence-based comprehensive health care has not been implemented where the highest prevalence of SCD occurs, in sub-Saharan Africa (SSA). It represents an urgent health burden, both in terms of mortality and morbidity with an estimated mortality of 8-16% in children under 5 years in SSA. Addressing the high mortality of SCD in SSA and for effective management of SCD, newborn screening (NBS) should be incorporated with prevention of infections (including pneumococcal septicaemia and malaria), parental education and support at all levels of healthcare provision to enable timely recognition. Read More
Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):493-506
Children's Hospital, Montefiore Medical Center, Bronx, NY; and.
Although the seminal event in sickle cell disease is the polymerization of abnormal hemoglobin, the downstream pathophysiology of vasoocclusion results from heterotypic interactions between the altered, adhesive sickle cell red blood cells, neutrophils, endothelium, and platelets. Ischemia reperfusion injury, hemolysis, and oxidant damage all contribute to heightened inflammation and activation of the hemostatic system. These various pathways are the focus of emerging treatments with potential to ameliorate disease manifestations. Read More
Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):482-492
Division of Hematology/Oncology and UPMC Heart, Lung and Blood Vascular Medicine Institute, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA.
Progress in the care of sickle cell disease (SCD) has been hampered by the extreme complexity of the SCD phenotype despite its monogenic inheritance. While epidemiological studies have identified clinical biomarkers of disease severity, with a few exceptions, these have not been routinely incorporated in clinical care algorithms. Furthermore, existing biomarkers have been poorly apt at providing objective parameters to diagnose sickle cell crisis, the hallmark, acute complication of SCD. Read More
Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):474-481
Division of Hematology, Department of Medicine, Johns Hopkins University, Baltimore, MD.
Sickle cell trait (SCT) is unique among the carrier states that are identified during newborn screening. Unlike other heterozygous states for rare recessive diseases, SCT is exceedingly prevalent throughout regions of the world, making sickle cell disease one of the most common monogenetic diseases worldwide. Because of this high frequency, reproductive counseling is of paramount importance. Read More
Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):418-425
Hematology, Oncology and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL; and Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL.
Development of exercise guidelines for individuals with sickle cell trait (SCT) and sickle cell anemia (SCA) is hampered by the need to weigh the benefits against risks of exercise in these populations. In SCT, concern for exercise collapse associated with sickle cell trait has resulted in controversial screening of student athletes for SCT. In SCA, there exists unsubstantiated concerns that high-intensity exercise may result in pain and other complications. Read More
Blood Adv 2018 Nov;2(Suppl 1):54-55
Hôpital Universitaire Justinien, Cap Haitien, Haiti.
Blood Adv 2018 Nov;2(Suppl 1):50-53
Cincinnati Children's Hospital Medical Center, Cincinnati OH.
Blood Adv 2018 Nov;2(Suppl 1):30-32
University of North Carolina Project-Malawi, Lilongwe, Malawi.
Blood Adv 2018 Nov;2(Suppl 1):26-29
Developmental Neurosciences, Section and Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health, London, United Kingdom.
Blood Adv 2018 Nov;2(Suppl 1):24-25
Paediatric Haematology, Evelina London Children's Hospital, Guy's and St Thomas' National Health Service Foundation Trust, London, United Kingdom.
Blood Adv 2018 Nov;2(Suppl 1):17-18
Center for Sickle Cell Disease, Howard University, Washington, DC.
Blood Adv 2018 Nov;2(Suppl 1):14-16
University of North Carolina Project-Malawi, Lilongwe, Malawi.
Blood Adv 2018 Nov;2(Suppl 1):4-7
Ministry of Health, Kampala, Uganda.
Blood Adv 2018 Nov;2(Suppl 1):1-3
Options for Children in Zambia, Foxborough, MA.
N Engl J Med 2018 Dec 1. Epub 2018 Dec 1.
From Centre Hospitalier Monkole, Kinshasa, Democratic Republic of Congo (L.T.); the Department of Medicine, University Health Network and Mt. Sinai Hospital, and the University of Toronto, Toronto (G.T.); the Kenya Medical Research Institute (KEMRI)-Wellcome Trust Research Program, Kilifi, Kenya (T.N.W.); the Department of Medicine, Imperial College London, London (T.N.W.); Hospital Pediátrico David Bernardino, Luanda, Angola (B.S.); Mbale Clinical Research Institute and Mbale Regional Referral and Teaching Hospital-Busitema University, Mbale, Uganda (P.O.-O.); the Division of Hematology, Department of Pediatrics, Cincinnati Children's Hospital (A.L., S.E.S., T.S.L., P.T.M., R.E.W.), University of Cincinnati College of Medicine (A.L., P.T.M., R.E.W.), and the Global Health Center, Cincinnati Children's Hospital Medical Center (S.E.S., P.T.M., R.E.W.), Cincinnati; and Cohen Children's Medical Center, New Hyde Park, and the Zucker School of Medicine at Hofstra/Northwell, Hempstead - both in New York (B.A.).
Background: Hydroxyurea is an effective treatment for sickle cell anemia, but few studies have been conducted in sub-Saharan Africa, where the burden is greatest. Coexisting conditions such as malnutrition and malaria may affect the feasibility, safety, and benefits of hydroxyurea in low-resource settings.
Methods: We enrolled children 1 to 10 years of age with sickle cell anemia in four sub-Saharan countries. Read More
Hemoglobin 2018 Dec 3:1-6. Epub 2018 Dec 3.
c Thalassaemia National Centre of Greece , Laiko General Hospital of Athens , Athens , Greece.
Hemoglobinopathies constitute the most frequent monogenic disorders worldwide and in Greece. In Greece, carrier frequency is estimated at about 8.0%, resulting in a heavy disease burden in the past. Read More
Blood Purif 2018 Dec 5:1-9. Epub 2018 Dec 5.
Division of Nephrology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts,
Background: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Read More
J Nutr 2018 Dec;148(12):1903-1910
Department of Nutrition, University of California, Davis, Davis, CA.
Background: The relation between subclinical hemoglobinopathies and concentrations of the iron-regulatory hormone hepcidin is not well characterized.
Objective: We investigated the relation of hepcidin concentration with hemoglobinopathies among young children in Kenya.
Methods: We quantified serum hepcidin and ferritin in 435 Kenyan children aged 14-20 mo in a subsample of the Water, Sanitation, and Handwashing (WASH) Benefits Trial. Read More
Rev Bras Enferm 2018 Nov-Dec;71(6):2974-2982
Universidade Estadual do Ceará. Fortaleza, Ceará, Brazil.
Objective: To know the main health guidance needs of family members of children with sickle cell disease.
Method: Qualitative research, developed in a pediatric reference hospital of Ceará State, between April and May 2017, through the participation of 12 family members of children with sickle cell disease. The data were collected through semi-structured interviewees and analyzed according to the Bardin's Thematic Categorical Analysis. Read More
Clinics (Sao Paulo) 2018 Dec 3;73:e721. Epub 2018 Dec 3.
Instituto da Crianca (ICr), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR.
The aim of this study was to provide an updated review of dental procedures undertaken at the dental unit of the Onco-hematology service of the Instituto da Criança at the Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (ICr/HC-FMUSP). We retrospectively reviewed 565 of 1902 medical and dental records of patients diagnosed with onco-hematological diseases who were seen in a 3-year study (January 2015 to December 2017). We assessed data regarding population characteristics, onco-hematological diagnosis and dental procedures performed. Read More
PLoS One 2018 5;13(12):e0206225. Epub 2018 Dec 5.
Division of Insect Sciences, The National Agriculture and Food Research Organization (NARO), Tsukuba, Japan.
Traumatic insemination (TI) is an extraordinary style of mating behavior wherein the female integument is pierced by the male extragenital structure to transfer the spermatozoa into the female's body through wounding. Flower bugs of the genus Orius belong to the family Anthocoridae (Heteroptera), which is referred to as the "TI family". Males possess sharp shaped extragenitalia, and females receive the extragenitalia using the copulatory tubes, which are specialized extragenital structures in Orius species. Read More
J Laparoendosc Adv Surg Tech A 2018 Dec 5. Epub 2018 Dec 5.
3 Department of Anaesthesia, King Fahd Armed Forces Hospital , Jeddah, Saudi Arabia .
Introduction: Few centers worldwide have advanced single-incision pediatric endosurgery (SIPES) splenectomy. The safety and feasibility of SIPES performed by trainees were not assessed before. SIPES splenectomy is a demanding technique that needs high level of skills. Read More
Nat Rev Drug Discov 2018 Dec 4. Epub 2018 Dec 4.
Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
For over 100 years, clinicians and scientists have been unravelling the consequences of the A to T substitution in the β-globin gene that produces haemoglobin S, which leads to the systemic manifestations of sickle cell disease (SCD), including vaso-occlusion, anaemia, haemolysis, organ injury and pain. However, despite growing understanding of the mechanisms of haemoglobin S polymerization and its effects on red blood cells, only two therapies for SCD - hydroxyurea and L-glutamine - are approved by the US Food and Drug Administration. Moreover, these treatment options do not fully address the manifestations of SCD, which arise from a complex network of interdependent pathophysiological processes. Read More
Microvasc Res 2018 Dec 1;122:111-116. Epub 2018 Dec 1.
UMR 5305 CNRS, Lyon, France; Université de Lyon 1, UMR 5305, Lyon, France.
Objective: Type 2 diabetes (T2D)-related vascular dysfunction and hemorheological abnormalities could possibly be amplified by sickle cell trait (SCT). These alterations could potentially increase the risk of vascular complications in individuals with combined T2D and SCT. Therefore, this study used a mouse model to determine whether vascular function and blood rheology were more severely altered in combined T2D and SCT than in T2D or SCT alone. Read More
South Med J 2018 Dec;111(12):742-745
From the Department of Obstetrics and Gynecology, University of Arkansas for Medical Sciences, Little Rock, the Department of Obstetrics and Gynecology of the University of Mississippi Medical Center, Jackson, and Department of Obstetrics and Gynecology, University of Kansas Medical Center, Kansas City.
Objective: To compare pregnancy outcomes in women with sickle cell disease from recent deliveries with a similar group delivered earlier.
Methods: During a 12-year period (2005-2016), data from pregnant women with hemoglobin SS or SC were collected from three university medical centers and compared with earlier studies (1979-2003) involving similar patients. The primary endpoints were maternal complications during pregnancy and newborn outcomes. Read More
Radiol Case Rep 2019 Feb 28;14(2):278-281. Epub 2018 Nov 28.
Department of Radiology, Royal London Hospital, Barts Health NHS Trust, Whitechapel Road, Whitechapel, E1 1BB, UK.
Segmental testicular infarction is a rare diagnosis and there are few documented cases in the literature. Those cases that have been reported are usually in the setting of epididymitis, hypercoaguable states, vasculitis, sickle cell disease, post orchidopexy or vasectomy, and idiopathic. We report a case of a patient who developed segmental testicular infarction that was managed conservatively, following nephrectomy for a ruptured kidney and the associated ultrasonographic appearances. Read More
Clin Imaging 2018 Nov 15;54:15-20. Epub 2018 Nov 15.
Department of Radiology, University of Florida, Gainesville, FL, United States of America.
Differentiation of bone infarct from osteomyelitis is one of the most challenging issues in the evaluation of acute bone pain in sickle cell patients. The imaging modalities that are currently being used for assessment of bone marrow in this population have several limitations. We present a case of an 18-year-old male with a history of sickle cell disease, who was transferred to our emergency department with progressively severe headache and jaw pain for one-week. Read More
Biol Blood Marrow Transplant 2018 Nov 27. Epub 2018 Nov 27.
Department of Medicine, Division of Hematology/Oncology, Vanderbilt University School of Medicine, Nashville, TN, USA. Electronic address:
Curative therapy for individuals with severe sickle cell disease (SCD) who lack an HLA-identical sibling donor has been frustratingly elusive. With the goal of improving engraftment while minimizing transplant related morbidity, a multi-institution learning collaborative was developed in 2013, in the context of a phase II clinical trial of a non-myeloablative, related HLA-haploidentical bone marrow transplant (haplo-BMT), with post-transplant cyclophosphamide. All eligible participants had HbSS, 89% (16/18) had an identifiable donor. Read More
J Pediatr Hematol Oncol 2018 Nov 28. Epub 2018 Nov 28.
Departments of Pediatrics.
Massive splenic infarction (MSI) is a rare complication of sickle cell disease, as the spleen generally atrophies within the first few years of life. We report a case of MSI in a 12-year-old boy with homozygous sickle cell anemia (Hb SS) whose chronic transfusion therapy resulted in hypersplenism. The occurrence of a complicated MSI in our patient should perhaps further encourage elective splenectomy in such patients, despite known potential perioperative complications and postsplenectomy risks of infection and thrombosis. Read More
J Pediatr Hematol Oncol 2018 Nov 28. Epub 2018 Nov 28.
Division of Behavioral Medicine.
Adolescents and young adults (AYA) with sickle cell disease (SCD) are at risk for poor health-related quality of life (HRQOL). Research suggests that vulnerability factors (eg, disease severity) and self-management resources (eg, disease self-efficacy) jointly impact health outcomes, including HRQOL; however, this has not been studied among AYA with SCD. This study examined the relationship between disease self-efficacy, HRQOL, and disease severity in AYA with SCD. Read More
Expert Opin Pharmacother 2018 Nov 30:1-14. Epub 2018 Nov 30.
a Hematology Center , University of Campinas - UNICAMP , Campinas , Brazil.
Introduction: Sickle cell disease (SCD) is an inherited disease with lifelong morbidity, whose complications include frequent acute painful vaso-occlusive episodes (VOEs) that often require hospitalization. The only pharmacotherapy currently in regular use for SCD management is hydroxyurea (hydroxycarbamide). Areas covered: We review recent advances in pharmacotherapy for SCD and summarize promising synthetic agents that are in late-stage development (phase 3) for SCD. Read More