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Sickle cell diseases

Laura Alonso, Marta González-Vicent, Cristina Belendez, Isabel Badell, Ana Sastre, Antonia Rodríguez-Villa, Mar Bermúdez-Cortés, Raquel Hladun, Cristina Díaz de Heredia
BACKGROUND AND OBJECTIVES: A recently occurring increase of the prevalence of haemoglobinopathies, β-thalassaemia major (TM) and sickle cell disease (SCD) over the last two decades in our country has generated new needs in terms of medical resources for both prevention and treatment of these patients. Allogeneic haematopoietic stem cell transplant (allo-HSCT) is a curative treatment available for patients who have severe haemoglobinopathies. The main objective of this study was to evaluate the results of allo-HSCT in paediatric patients with TM or SCD performed in paediatric hematopoietic transplant units within the Spanish Group of Bone Marrow Transplantation in Children (GETMON)...
July 9, 2018: Medicina Clínica
Joseph R Hageman
No abstract text is available yet for this article.
July 1, 2018: Pediatric Annals
Sherif M Badawy, Amanda B Payne, Mark J Rodeghier, Robert I Liem
OBJECTIVES: To determine factors associated with exercise capacity in adults with sickle cell disease (SCD) and its relationship to hospitalizations and mortality. METHODS: A total of 223 participants in the Cooperative Study of Sickle Cell Disease (CSSCD) (64% female, 70% hemoglobin SS/Sβ0 thalassemia, mean age 43.3 ± 7.5 years) underwent maximal exercise testing using a treadmill protocol with a mean duration of 11.6 ± 5.2 minutes. RESULTS: Female sex (β = -3...
July 12, 2018: European Journal of Haematology
Nuno Galamba, Silvio Pipolo
Protein aggregation is associated with various diseases, including Alzheimer and Parkinson as well as sickle cell disease (SCD). From a molecular point of view protein aggregation depend on a complex balance of electrostatic and hydrophobic interactions mediated by water. An impressive manifestation of the importance of this balance concerns the human hemoglobin (HbA) mutant, HbS (sickle cell Hb), where a single substitution at the 6th position of HbA β-chains, from glutamic acid to valine, causes the polymerization of deoxygenated HbS (deoxy-HbS), responsible for SCD...
July 11, 2018: Journal of Physical Chemistry. B
Carolyn Berger, Jessica Valenzuela, Joanna Tsikis, Cynthia Fletcher
BACKGROUND: Existing research demonstrates that youth with chronic illness often experience challenges including poor academic performance, attendance, and social success. However, past research demonstrates a lack of support for teachers to best educate students with chronic illness. In this study, we aim to describe the needs of local educators when working with students with chronic illness. METHODS: A questionnaire was completed by 383 middle or high school professionals...
August 2018: Journal of School Health
Kwesi Nyan Amissah-Arthur, Evelyn Mensah
Sickle cell retinopathy is a potentially blinding condition that affects young people in the working age group. This review looks at the past, present and future management of sickle cell retinopathy within an African context. After Sickle cell disease was first reported in 1910, some reports from Africa were pivotal in describing the retinal changes associated with the disease. It soon became obvious that there was a varied clinical picture and clinical course. Several landmark studies were carried out in Jamaica to help elucidate the complexities of the disease and outline appropriate clinical management...
July 10, 2018: Eye
O S Olatunya, D M Albuquerque, A D Adekile, F F Costa
Background: Sickle leg ulcer (SLU) is a chronic and debilitating complication of sickle cell disease (SCD) associated with huge physical and psychosocial discomfort. The occurrence of SLU has remained steady despite successful preventive strategies and advances in SCD care. Although multifactorial factors have been implicated in SLU, these are not fully understood, and data on how these relate to young Nigerian SCD patients are scanty. Aims: This study aims to evaluate the sociodemographic, clinical, and laboratory markers of SLU in a young Nigerian SCD cohort...
July 2018: Nigerian Journal of Clinical Practice
Alice Vianello, Elisa Vencato, Maurizio Cantini, Giovanni Zanconato, Erminia Manfrin, Alberto Zamo, Francesco Zorzi, Filippo Mazzi, Nicola Martinelli, Elena Cavaliere, Francesca Monari, Donatella Venturelli, Francesca Ferrara, Oliviero Olivieri, Lucia De Franceschi
BACKGROUND: The desire for pregnancy in sickle cell disease (SCD) women has become a true challenge for hematologists, requiring a multidisciplinary approach. Erythrocytapheresis (ECP) is an important therapeutic tool in SCD, but only limited data on starting time and the effects of ECP during pregnancy are available. STUDY DESIGN AND METHODS: This is a double-center retrospective cross-sectional study on a total of 46 single pregnancies in SCD women from January 2008 to June 2017...
July 8, 2018: Transfusion
Shruti Chaturvedi, Djamila Labib Ghafuri, Natalie Jordan, Adetola Kassim, Mark Rodeghier, Michael R DeBaun
Chronic end-organ complications result in morbidity and mortality in adults with sickle cell disease (SCD). In a prospective cohort of 150 adults with SCD who received standard care screening for pulmonary function abnormalities, cardiac disease, and renal assessment from January 2003 to January 2016, we tested the hypothesis that clustering of end-organ disease is common and multiple organ impairment predicts mortality. Any end-organ disease occurred in 59.3% of individuals, and 24.0% developed multiple organ (>1) end-organ disease...
July 7, 2018: American Journal of Hematology
Cherif Ben Hamda, Raphael Sangeda, Liberata Mwita, Ayton Meintjes, Siana Nkya, Sumir Panji, Nicola Mulder, Lamia Guizani-Tabbane, Alia Benkahla, Julie Makani, Kais Ghedira
A chronic inflammatory state to a large extent explains sickle cell disease (SCD) pathophysiology. Nonetheless, the principal dysregulated factors affecting this major pathway and their mechanisms of action still have to be fully identified and elucidated. Integrating gene expression and genome-wide association study (GWAS) data analysis represents a novel approach to refining the identification of key mediators and functions in complex diseases. Here, we performed gene expression meta-analysis of five independent publicly available microarray datasets related to homozygous SS patients with SCD to identify a consensus SCD transcriptomic profile...
2018: PloS One
Chka Fernando, S Mendis, A P Upasena, Y J Costa, H S Williams, D Moratuwagama
Introduction: Splenic syndrome is a rare presentation of sickle cell disease. It is important to rule out this possibility when an ethnically vulnerable patient presents with an acute abdominal symptoms in a background of precipitating events. Case Report: A 26-year-old man who developed a severe abdominal pain at high altitude, found to have a tender splenomegaly. However, further inquiry revealed he is from an area where sickle cell disease is prevalent. Screening for sickle cell disease was positive...
June 2018: Journal of Patient Experience
Robert Michael Cronin, Tilicia L Mayo-Gamble, Sarah-Jo Stimpson, Sherif M Badawy, Lori E Crosby, Jeannie Byrd, Emmanuel J Volanakis, Adetola A Kassim, Jean L Raphael, Velma M Murry, Michael R DeBaun
Background: Evidence-based guidelines for sickle cell disease (SCD) health maintenance and management have been developed for primary health care providers, but not for individuals with SCD. To improve the quality of care delivered to individuals with SCD and their caregivers, the main purposes of this study were to: (1) understand the desire for patient-centered guidelines among the SCD community; and (2) adapt guideline material to be patient-centered using community-engagement strategies involving health care providers, community -based organizations, and individuals with the disease...
2018: BMC Hematology
Fekri Samarah, Mahmoud A Srour, Dirgham Yaseen, Kamal Dumaidi
Background: Transfusion of red blood cells (RBC) is an essential therapeutic tool in sickle cell disease (SCD). Repeated RBC transfusions can cause alloimmunization which causes difficulty in cross-matching and finding compatible blood for transfusions. This study aimed to investigate the frequency of RBC alloimmunization and related risk factors among Palestinian SCD patients. Materials and Methods: A multicenter cross-sectional study on 116 previously transfused SCD patients from three centers in West Bank, Palestine...
2018: Advances in Hematology
Shehab M Abd El-Kader, Fadwa M Al-Shreef
Background & objective: Sickle cell anemia (SCA) is well recognized as a chronic inflammatory disease. Despite progress in therapy, SCA remains a cause of significant morbidity and mortality. The relationship between exercise and immune function has been of great interest to the scientific community and the lay public. The aim of this study was to measure the impact of aerobic exercise training on the immunologic parameters and inflammatory cytokines of patients with sickle cell anemia (SCA) in asymptomatic steady state...
March 2018: African Health Sciences
Thao Nguyen-Khoa, Louis Mine, Bichr Allaf, Jean-Antoine Ribeil, Christelle Remus, Aurélie Stanislas, Valérie Gauthereau, Sarah Enouz, Jason S Kim, Xiaoxi Yang, Eliane Gluckman, Jean-Louis Beaudeux, Arnold Munnich, Robert Girot, Marina Cavazzana
Sickle SCAN™ is a rapid, qualitative, point-of-care lateral flow immunoassay for the identification of AS, AC, SS/Sβ0 thal, SC and CC/Cβ0 thal phenotype. We evaluated this test under the conditions encountered in the French newborn screening (NBS) program for sickle cell disease: a total of 104 dried blood spots (DBSs) were tested with an HPLC reference method and then with the Sickle SCAN™ device. Sickle SCAN™ identified the hemoglobin (Hb) phenotype correctly on 96% of cases. In the four non-concordant cases, the antibody anti-HbS cross-reacted with HbE (n=2), HbD (n=1) or HbX (n=1)...
July 5, 2018: Annales de Biologie Clinique
Prashant Warang, Takujiro Homma, Riddhi Pandya, Anuja Sawant, Nikhil Shinde, Debjeet Pandey, Junichi Fujii, Manisha Madkaikar, Malay B Mukherjee
The ubiquitin-proteasome system (UPS) is an important intracellular proteolytic pathway responsible for the degradation of proteins and oxidative damage; hence it plays a central role in maintaining homeostasis of red blood cells (RBCs). The present study investigated the levels of polyubiquitination, the function of proteasomes and effect of hydroxycarbamide (HC) therapy in RBCs from sickle cell disease (SCD) patients. Polyubiquitinated proteins were found to be elevated in untreated SCD (UT-SCD) patients compared to those in HC-treated SCD patients (HC-SCD) and controls...
July 5, 2018: British Journal of Haematology
Lívia Maria Nunes Campelo, Nayara Fernandes Oliveira, Juliana Macêdo Magalhães, Alcineide Mendes de Sousa Julião, Fernanda Cláudia Miranda Amorim, Magda Coeli Vitorino Sales Coelho
OBJECTIVE: To describe how nurses identify the pain in children with sickle cell disease (SCD) and to list the strategies used by them in the evaluation and control of pain. METHOD: This is a qualitative, descriptive and exploratory research, performed through semi-structured interviews with 13 nurses. The interviews were transcribed and after this process the qualitative data were organized according to thematic analysis. RESULTS: This study showed that the nurses can identify pain in children with SCD from the signals they emit, such as: constant crying, restlessness, facial expressions and verbal reports...
2018: Revista Brasileira de Enfermagem
Bo-Kyoung Park, Hyo-Sup Kim, Seongkoo Kim, Jae-Wook Lee, Young Shil Park, Pil-Sang Jang, Nack-Gyun Chung, Dae-Chul Jeong, Bin Cho
Background: Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative therapy for β-thalassemia major (TM) and sickle cell disease (SCD) in children. Graft-versus-host disease (GVHD) and treatment-related mortality (TRM) remain significant challenges to improving survival after HSCT. Here, we analyzed the outcome of TM and SCD patients, who received allogeneic HSCT with myeloablative conditioning at our institution. Methods: Twenty-two patients (15 TM, 7 SCD), with a median age of 9 years (range, 1...
June 2018: Blood Research
Fadare Joseph Olusesan, Olatunya Oladele Simeon, Ogundare Ezra Olatunde, Oluwayemi Isaac Oludare, Agaja Oyinkansola Tolulope
Background: Sickle cell disease (SCD) is a genetic haematological disorder that affects millions of people around the world especially people of African heritage. The treatment of the symptoms of SCD includes the use of analgesics, antibiotics, and anti-malarial drugs. Studying the pattern of drug prescription is a veritable tool for establishing the current practice and how it conforms to existing guidelines. Objectives: The main objective of this study was to assess the pattern of drug prescription in children with sickle cell disease (SCD) attending the paediatric outpatients' clinic of a tertiary care centre in Ado-Ekiti, South-West Nigeria...
December 2017: Malawi Medical Journal: the Journal of Medical Association of Malawi
April D Adams, Kendra Schaa, Rachael T Overcash
OBJECTIVE: It has been shown that hemoglobinopathies increase the risk of pregnancy complications and placental dysfunction. This could alter the placental analytes examined during prenatal aneuploidy screening. Our objective was to determine whether there is a difference in maternal serum screening results for women with hemoglobin S variants (AS, SS, SC, S/beta thalassemia) compared with women with normal hemoglobin (AA). STUDY DESIGN: This is a retrospective cohort study in African-American women receiving aneuploidy screening at MedStar Washington Hospital Center from 2008 to 2015...
July 2, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
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