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https://www.readbyqxmd.com/read/28637291/pediatric-to-adult-care-transition-perspectives-of-young-adults-with-sickle-cell-disease
#1
Jerlym S Porter, Kimberly M Wesley, Mimi S Zhao, Rebecca J Rupff, Jane S Hankins
The aim of this study was to explore perspectives of transition and transition readiness of young adult patients (YAs) with sickle cell disease (SCD) who have transitioned to adult health care. In all, 19 YAs with SCD (ages 18-30 years) participated in one of three focus groups and completed a brief questionnaire about transition topics. Transcripts were coded and emergent themes were examined using the social-ecological model of adolescent and young adult readiness for transition (SMART). Themes were consistent with most SMART components...
June 15, 2017: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/28635598/sickle-cell-disease-tipping-the-balance-of-genomic-research-to-catalyse-discoveries-in-africa
#2
Julie Makani, Solomon F Ofori-Acquah, Furahini Tluway, Nicola Mulder, Ambroise Wonkam
No abstract text is available yet for this article.
June 17, 2017: Lancet
https://www.readbyqxmd.com/read/28632024/performance-validity-testing-in-a-clinical-sample-of-adults-with-sickle-cell-disease
#3
Katherine E Dorociak, Evan T Schulze, Lauren E Piper, Robert E Molokie, Julie K Janecek
OBJECTIVE: Neuropsychologists utilize performance validity tests (PVTs) as objective means for drawing inferences about performance validity. The Test of Memory Malingering (TOMM) is a well-validated, stand-alone PVT and the Reliable Digit Span (RDS) and Reliable Digit Span-Revised (RDS-R) from the Digit Span subtest of the WAIS-IV are commonly employed, embedded PVTs. While research has demonstrated the utility of these PVTs with various clinical samples, no research has investigated their use in adults with sickle cell disease (SCD), a condition associated with multiple neurological, physical, and psychiatric symptoms...
June 15, 2017: Clinical Neuropsychologist
https://www.readbyqxmd.com/read/28629283/prevalence-and-correlates-of-major-depression-among-nigerian-adults-with-sickle-cell-disease
#4
Saheed O Raji, Ambrose O Lawani, Bawo O James
Objective To ascertain the current and lifetime prevalence of depression among adults with sickle cell disease and identify relevant socio-demographic and clinical correlates. Method A cross-sectional study of 205 stable adult out-patient attendees at a treatment center in southern Nigeria between April and September, 2014. A socio-demographic questionnaire, the depression module of Mini International Neuropsychiatric Interview, and the eight-item Morisky Medication Adherence Scale were administered. Categorical and continuous variables associated with a diagnosis of depression were tested using chi-squared and t-tests respectively...
July 2016: International Journal of Psychiatry in Medicine
https://www.readbyqxmd.com/read/28627939/off-label-prescribing-for-children-with-chronic-diseases-in-nigeria-findings-and-implications
#5
Kazeem Adeola Oshikoya, Ibrahim Adekunle Oreagba, Brian Godman, Joseph Fadare, Samuel Orubu, Amos Massele, Idowu Odunayo Senbanjo
BACKGROUND AND AIMS: Prescribing medicines in an off-label manner for children with chronic conditions is sparsely documented, even more so among developing countries. This needs addressing. The objective was to investigate the extent of this prescribing among children with epilepsy, asthma, and sickle cell anaemia in Nigeria. METHODS: Prescriptions for children ≤16 years documented in their case files that attended paediatric clinics in Lagos, Nigeria, for these three conditions between January and October 2015, were reviewed retrospectively to extract data on the medicines prescribed...
June 19, 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/28626540/acute-crises-and-complications-of-sickle-cell-anemia-among-patients-attending-a-pediatric-tertiary-unit-in-kinshasa-democratic-republic-of-congo
#6
Michel Ntetani Aloni, Bertin Tshimanga Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu
In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years' retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28624257/moderate-exercise-training-decreases-inflammation-in-transgenic-sickle-cell-mice
#7
Emmanuelle Charrin, John J Dubé, Philippe Connes, Vincent Pialoux, Samit Ghosh, Camille Faes, Solomon F Ofori-Acquah, Cyril Martin
Chronic systemic inflammation is a pathophysiological feature of sickle cell disease (SCD). Considering that regular exercise exerts multiple beneficial health effects including anti-inflammatory actions, we investigated whether a treadmill training program could minimize the inflammatory state in transgenic sickle cell (SS) mice. To test this hypothesis, SS mice were subjected to a treadmill training protocol of 1h/day, 5days a week for 8weeks. Exercise training increased the percent of venous oxyhemoglobin and sharply decreased the percent of carboxyhemoglobin suggesting that exercise training may limit the proportion of erythrocytes that were deoxygenated in the venous circulation...
June 13, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28624233/-a-new-therapeutic-era-in-sickle-cell-disease
#8
J-B Arlet
No abstract text is available yet for this article.
June 14, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28618753/development-of-a-new-real-time-pcr-screening-kit-for-hbs-and-common-beta-thalassemia-mutations-observed-in-turkey
#9
Derya Kan Karaer, Mehmet Ali Ergün, Hatice Ilgın Ruhi, Jale Öztürk, Halil Kara, Deniz Reisoğlu Çakmak, Talihanur Aydoğmuş, Emriye Ferda Perçin
BACKGROUND/AIM: IVSI-110 (G>A), IVSI-6 (T>C), IVSII-1 (G>A), IVSII-745 (C>G), IVSI-1 (G>A), and HbS are mutations covering 76% of all the β-globin mutations in the Turkish population. In this study, our aim is to develop a reliable, fast, real-time kit for these mutations using the TaqMan probe method. MATERIALS AND METHODS: This study included 100 individuals with beta-thalassemia or sickle cell anemia who had unknown mutations, and 21 controls with known mutations...
June 12, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28617057/preliminary-evaluation-of-a-point-of-care-testing-device-sicklescan%C3%A2-in-screening-for-sickle-cell-disease
#10
Maxwell M Nwegbu, Hezekiah A Isa, Biyaya B Nwankwo, Chinedu C Okeke, Uduak J Edet-Offong, Norah O Akinola, Adekunle D Adekile, John C Aneke, Emmanuel C Okocha, Thomas Ulasi, Usman Abjah, Ngozi I Ugwu, Angela A Okolo, Anazoeze Madu, Ifeoma Emodi, Ahmed Girei, Taiwo M Balogun, Ijeoma N Diaku-Akinwumi, Chinatu Ohiaeri, Biobele J Brown, John A Olaniyi, Abdulaziz Hassan, Sani Awwalu, Dorothy A Okoh, Obiageli E Nnodu
Sickle cell disease affects about 150,000 births annually in Nigeria. Early diagnosis is hampered by factors such as centralized and urban localization of laboratories, high cost of diagnostic equipment and inadequate skilled manpower to operate them. The need for a low-cost, portable, easy-to-use diagnostic test for sickle cell disease is critical, especially in resource-poor countries. In this study, we evaluated the performance characteristics of a novel point-of-care testing device (SickleSCAN™), and its acceptability and feasibility, as a possible screening tool for sickle cell disease...
June 15, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28616936/encephaloduroateriosynangiosis-edas-in-the-management-of-moyamoya-syndrome-in-children-with-sickle-cell-disease
#11
Alexander Alamri, Pennylouise Hever, Jebet Cheserem, Catia Gradil, Sanj Bassi, Christos M Tolias
BACKGROUND: Encephalo-duro-arterio-synangiosis (EDAS) in Moyamoya syndrome (MMS) treatment has been well described in the literature, however in MMS caused by sickle cell anaemia (SCA), EDAS use remains controversial with poor long-term follow-up. We present a case-series of SCA patients who have undergone EDAS for SCA-related MMS and describe their post-operative course as well as provide a literature review of the role of EDAS in the treatment of sickle cell anaemia. METHODS: A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015...
June 15, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/28615064/anti-toxoplasma-gondii-antibodies-in-patients-with-beta-hemoglobinopathies-the-first-report-in-the-americas
#12
Marina Neves Ferreira, Claudia Regina Bonini-Domingos, Isabeth Fonseca Estevão, Clarice Lopes de Castro Lobo, Gisele Cristina Souza Carrocini, Aparecida Perpétuo Silveira-Carvalho, Octávio Ricci, Luiz Carlos de Mattos, Cinara Cássia Brandão de Mattos
BACKGROUND: In Brazil, there have been no previous studies of Toxoplasma gondii infection in sickle cell anemia patients and carriers of severe forms of beta-thalassemia. This study evaluated T. gondii infection in patients with beta-hemoglobinopathies. METHODS: A total of 158 samples, 77 (48.7%) men and 81 (51.3%) women, were evaluated. Three groups were formed: G1 (85 patients with sickle cell disease); G2 (11 patients with homozygous beta-thalassemia; G3 (62 patients with heterozygous beta-thalassemia)...
June 14, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28614479/knowledge-of-and-attitudes-toward-heel-prick-screening-for-sickle-cell-disease-in-saint-lucia
#13
Sonia Alexander, Sharon Belmar-George, Alisha Eugene, Vanessa Elias
Objectives: In the Caribbean country of Saint Lucia, umbilical-cord-blood screening for sickle cell disease (SCD) was the testing method that health care workers (HCWs) on the maternity wards of the hospitals preferred until the new heel prick (HP) testing method was introduced in the country in 2014. This SCD study sought to assess HCWs' knowledge of and attitude toward HP screening and also determine new mothers' favorability toward HP screening. Methods: A total of 70 HCWs and 132 new mothers answered survey questionnaires in three hospitals...
June 8, 2017: Revista Panamericana de Salud Pública, Pan American Journal of Public Health
https://www.readbyqxmd.com/read/28612714/-gene-therapy-for-sickle-cell-disease
#14
Jean-Antoine Ribeil, Stéphane Blanche, Marina Cavazzana
No abstract text is available yet for this article.
May 2017: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/28612449/changes-in-urine-albumin-to-creatinine-ratio-with-the-initiation-of-hydroxyurea-therapy-among-children-and-adolescents-with-sickle-cell-disease
#15
Sarah Tehseen, Clinton H Joiner, Peter A Lane, Marianne E Yee
BACKGROUND: Renal damage is a progressive complication of sickle cell disease (SCD) that begins in childhood and may progress to renal failure and early mortality in 12% of adults with hemoglobin SS (HbSS) SCD. Early sickle nephropathy is characterized by hyperfiltration and microalbuminuria; therefore, urine albumin to creatinine ratio (ACR) is an effective screening tool for its detection. PROCEDURE: This study investigated the effect of hydroxyurea (HU) therapy on urine ACR levels among children with SCD...
June 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28611233/splenic-uptake-on-bone-scan
#16
Harleen Kaur, Mitchel Muhleman, Helena R Balon
We present a patient with splenic uptake on bone scan that was due to sickle cell disease. We also discuss other etiologies for this finding.
June 13, 2017: Journal of Nuclear Medicine Technology
https://www.readbyqxmd.com/read/28610635/cellular-function-reinstitution-of-offspring-red-blood-cells-cloned-from-the-sickle-cell-disease-patient-blood-post-crispr-genome-editing
#17
Jianguo Wen, Wenjing Tao, Suyang Hao, Youli Zu
BACKGROUND: Sickle cell disease (SCD) is a disorder of red blood cells (RBCs) expressing abnormal hemoglobin-S (HbS) due to genetic inheritance of homologous HbS gene. However, people with the sickle cell trait (SCT) carry a single allele of HbS and do not usually suffer from SCD symptoms, thus providing a rationale to treat SCD. METHODS: To validate gene therapy potential, hematopoietic stem cells were isolated from the SCD patient blood and treated with CRISPR/Cas9 approach...
June 13, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28609750/association-of-plasma-cd40l-with-acute-chest-syndrome-in-sickle-cell-anemia
#18
Vanessa Tonin Garrido, Laura Sonzogni, Siana Nkya Mtatiro, Fernando F Costa, Nicola Conran, Swee Lay Thein
Platelet activation and platelet-derived cytokines contribute to the vascular inflammation and increased thrombotic activity known to occur in patients with sickle cell anemia (SCA). CD40 ligand (CD40L), a platelet-associated pro-inflammatory molecule that promotes endothelial cell activation, is elevated in the circulation of SCA patients. We sought to evaluate the association of CD40L and inflammation with sickle-related clinical complications and laboratory variables in SCA patients. Soluble CD40L, thrombospondin (TSP)-1 and tumor necrosis factor (TNF)-α were determined in the platelet-poor plasma of healthy individuals and steady-state SCA patients by ELISA...
June 10, 2017: Cytokine
https://www.readbyqxmd.com/read/28606098/determining-the-longitudinal-validity-and-meaningful-differences-in-hrql-of-the-pedsql%C3%A2-sickle-cell-disease-module
#19
Julie A Panepinto, J Paul Scott, Oluwakemi Badaki-Makun, Deepika S Darbari, Corrie E Chumpitazi, Gladstone E Airewele, Angela M Ellison, Kim Smith-Whitley, Prashant Mahajan, Sharada A Sarnaik, T Charles Casper, Larry J Cook, Julie Leonard, Monica L Hulbert, Elizabeth C Powell, Robert I Liem, Robert Hickey, Lakshmanan Krishnamurti, Cheryl A Hillery, David C Brousseau
BACKGROUND: Detecting change in health status over time and ascertaining meaningful changes are critical elements when using health-related quality of life (HRQL) instruments to measure patient-centered outcomes. The PedsQL™ Sickle Cell Disease module, a disease specific HRQL instrument, has previously been shown to be valid and reliable. Our objectives were to determine the longitudinal validity of the PedsQL™ Sickle Cell Disease module and the change in HRQL that is meaningful to patients...
June 12, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28602890/posterior-reversible-encephalopathy-syndrome-after-hematopoietic-cell-transplantation-in-children-with-hemoglobinopathies
#20
Javid Gaziev, Simone Marziali, Katia Paciaroni, Antonella Isgrò, Francesca Di Giuliano, Giorgia Rossi, Marco Marziali, Gioia De Angelis, Cecilia Alfieri, Michela Ribersani, Marco Andreani, Maria Giuseppina Palmieri, Fabio Placidi, Andrea Romigi, Francesca Izzi, Roberto Floris, Nicola Biagio Mercuri
Posterior reversible encephalopathy syndrome (PRES) is a serious adverse event associated with calcineurin inhibitors (CI) used for graft-versus-host disease (GVHD) prophylaxis. We compared the incidence of PRES in children with thalassemia (n=222, 1.4-17.8 years old) versus sickle cell disease (SCD; n=59, 2-17 years old) who underwent hematopoietic cell transplantation (HCT) from HLA-matched siblings or alternative donors and analyzed the risk factors for PRES. Overall, 31 children developed CI-related PRES (11%), including 30 patients with seizures and 1 patient without seizures...
June 5, 2017: Biology of Blood and Marrow Transplantation
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