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https://www.readbyqxmd.com/read/29027199/characterization-of-a-mouse-model-of-sickle-cell-trait-parallels-to-human-trait-and-a-novel-finding-of-cutaneous-sensitization
#1
Katherine J Zappia, Yihe Guo, Dawn Retherford, Nancy J Wandersee, Cheryl L Stucky, Cheryl A Hillery
Sickle cell trait (SCT) has classically been categorized as a benign condition except in rare cases or upon exposure to severe physical conditions. However, several lines of evidence indicate that individuals with SCT are not always asymptomatic, and additional physiological changes and risks may remain unexplored. Here, we utilized mice harbouring one copy of normal human β globin and one copy of sickle human β globin as a model of SCT to assess haematological, histopathological and somatosensory outcomes...
October 12, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29026475/human-parvovirus-b19-in-children-with-sickle-cell-disease-poking-the-spleen
#2
Ashraf Abdellah Saad, Ismail Beshlawi, Abdul Hakim Al-Rawas, Mathew Zachariah, Hanan Fawzy Nazir, Yasser Wali
Parvovirus is a known culprit of transient red cell aplasia (TRCA) in children with sickle cell disease (SCD). Few reports have previously described the association between the virus and acute splenic sequestration crisis (ASSC) in the same patient. Here, we are shedding light on such a potentially serious combination by reporting two cases of siblings with SCD complicated with concurrent ASSC and TRCA and presenting a review of the relevant literature.
September 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/29026401/the-sensitivity-of-na-k-atpase-as-an-indicator-of-blood-diseases
#3
Abulnaja Kkalid Omar, Kherd Ali Ahmed, Nawal Mohammed Helmi, Kumosani Taha Abdullah, Mohamad H Qarii, Huwait Etimad Hasan, Albukhari Ashwag, Alaama Mohammed Nabil, Al-Ghamdi Maryam Abdu, Moselhy Said Salama
BACKGROUND: Blood-related hereditary diseases are widespread in Eastern and SouthWestern regions of Saudi Arabia until recently. In this study, we used Na(+), K(+)ATPase as an enzymatic indicator for the diagnosis of the diseases. MATERIALS AND METHODS: Individuals with different blood diseases (iron deficiency (n=13), anemia (n=14), thalassemia (n=16) and sickle cell anemia (n=12) were studied for Na(+), K(+)-ATPase activity in the plasma membrane of red blood cell and compared with those of the healthy ones (n=20) of the same age and gender living in Jeddah, Saudi Arabia...
March 2017: African Health Sciences
https://www.readbyqxmd.com/read/29026400/hydroxyurea-therapy-in-adult-nigerian-sickle-cell-disease-a-monocentric-survey-on-pattern-of-use-clinical-effects-and-patient-s-compliance
#4
Ademola Samson Adewoyin, Omokiniovo Sunday Oghuvwu, Omolade Augustina Awodu
BACKGROUND: The clinical prospects of hydroxyurea therapy in the management of sickle cell disease (SCD) require evaluation in the Nigerian setting to develop indigenous guidelines. This survey examines the pattern of hydroxyurea therapy, its clinico-haematologic benefits and safety profile in Nigerian SCD subjects. METHODS: A cross sectional pilot survey was carried out among 60 adult SCD subjects over 3 months. Data on clinical phenotypes, relevant haematological parameters and details of hydroxyurea therapy were obtained using a structured questionnaire through an interview process and case file review...
March 2017: African Health Sciences
https://www.readbyqxmd.com/read/29024896/identification-of-a-soluble-guanylate-cyclase-in-rbcs-preserved-activity-in-patients-with-coronary-artery-disease
#5
Miriam M Cortese-Krott, Evanthia Mergia, Christian M Kramer, Wiebke Lückstädt, Jiangning Yang, Georg Wolff, Christina Panknin, Thilo Bracht, Barbara Sitek, John Pernow, Johannes-Peter Stasch, Martin Feelisch, Doris Koesling, Malte Kelm
Endothelial dysfunction is associated with decreased NO bioavailability and impaired activation of the NO receptor soluble guanylate cyclase (sGC) in the vasculature and in platelets. Red blood cells (RBCs) are known to produce NO under hypoxic and normoxic conditions; however evidence of expression and/or activity of sGC and downstream signaling pathway including phopshodiesterase (PDE)-5 and protein kinase G (PKG) in RBCs is still controversial. In the present study, we aimed to investigate whether RBCs carry a functional sGC signaling pathway and to address whether this pathway is compromised in coronary artery disease (CAD)...
September 8, 2017: Redox Biology
https://www.readbyqxmd.com/read/29024210/a-case-of-anaplastic-lymphoma-kinase-positive-renal-cell-carcinoma-coincident-with-hodgkin-lymphoma
#6
Yuzo Oyama, Haruto Nishida, Takahiro Kusaba, Hiroko Kadowaki, Motoki Arakane, Tsutomu Daa, Dai Watanabe, Yasuyuki Akita, Fuminori Sato, Hiromitsu Mimata, Shigeo Yokoyama
We report a case of ALK-positive renal cell carcinoma coincident with Hodgkin lymphoma. The patient was a 19 year-old-girl without sickle cell trait. The right renal tumor was discovered concomitantly with Hodgkin lymphoma (HL). After chemotherapy for HL, right nephrectomy was performed. Microscopically, the tumor showed a solid and focally pseudo-papillary growth pattern studded with tubular structures. Most tumor cells were small bland eosinophilic cells, but rhabdoid cells, vacuolated cells, pleomorphic multinucleated giant cells were also admixed...
October 12, 2017: Pathology International
https://www.readbyqxmd.com/read/29023896/predicting-changes-in-hemoglobin-s-after-simple-transfusion-using-complete-blood-counts
#7
Gagan Mathur, Patrick Ten Eyck, C Michael Knudson
BACKGROUND: Hemoglobin S percentages are used in the management of patients who have sickle cell disease. However, hemoglobin S measurements often are not routinely or rapidly performed. Rapid and accurate methods to estimate hemoglobin S levels after simple transfusion may improve the care of patients with sickle cell disease. STUDY DESIGN AND METHODS: A comprehensive review of the electronic medical record identified 24 stable patients with sickle cell disease who received simple red blood cell transfusions and had hemoglobin S measurements before and after the transfusion that were less than 72 hours apart...
October 11, 2017: Transfusion
https://www.readbyqxmd.com/read/29021902/significantly-elevated-foetal-haemoglobin-levels-in-individuals-with-glucose-6-phosphate-dehydrogenase-disease-and-or-sickle-cell-trait-a-cross-sectional-study-in-cape-coast-ghana
#8
Patrick Adu, Essel K M Bashirudeen, Florence Haruna, Edward Morkporkpor Adela, Richard K D Ephraim
BACKGROUND: Previously published data have demonstrated that sickle red blood cells produce twice as much reactive oxygen species (ROS) suggesting that co-inheritance of sickle cell disease (SCD) and glucose 6-phosphate dehydrogenase (G6PD) enzymopathy could lead to more severe anaemia during sickling crises. Elevated foetal haemoglobin (Hb F) levels have been shown to have positive modulatory effects on sickling crises and disease outcomes. This study sought to assess how inheritance of G6PD enzymopathy affects the level of Hb F and haemoglobin concentration in adults in steady state...
2017: BMC Hematology
https://www.readbyqxmd.com/read/29020197/sickle-cell-trait-and-heat-injury-among-u-s-army-soldiers
#9
D Alan Nelson, Patricia A Deuster, Francis G O'Connor, Lianne M Kurina
There is concern that sickle cell trait increases risk of exertional collapse, a primary cause of which is heat injury. However, no population-based studies among active individuals have addressed this, representing a critical evidence gap. We conducted a retrospective cohort study of sickle cell trait-tested African American soldiers on active-duty in the U.S. Army anytime between January 2011 and December 2014. Using Cox proportional hazards models and adjusting for demographic and medical factors, we observed no significant associations between sickle cell trait and either mild heat injury (hazard ratio (HR), 1...
August 17, 2017: American Journal of Epidemiology
https://www.readbyqxmd.com/read/29019038/utilisation-and-safety-of-deferasirox-results-from-an-observational-cohort-study-in-england
#10
Vicki Osborne, Miranda Davies, Deborah Layton, Saad A W Shakir
INTRODUCTION: Deferasirox (EXJADE(®), Novartis, UK) is an oral iron-chelating agent primarily used to reduce chronic iron overload in patients receiving blood transfusions for various chronic anaemias and some non-transfusion dependant anaemias. OBJECTIVE: The aim of this study was to examine the utilisation and safety of deferasirox used in general practice in England. METHOD: A single exposure observational cohort study design was used...
October 10, 2017: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
https://www.readbyqxmd.com/read/29018763/ocular-manifestations-of-sickle-cell-disease-and-genetic-susceptibility-for-refractive-errors
#11
Palak Shukla, Henu Verma, Santosh Patel, P K Patra, L V K S Bhaskar
PURPOSE: Sickle cell disease (SCD) is the most common and serious form of an inherited blood disorder that lead to higher risk of early mortality. SCD patients are at high risk for developing multiorgan acute and chronic complications linked with significant morbidity and mortality. Some of the ophthalmological complications of SCD include retinal changes, refractive errors, vitreous hemorrhage, and abnormalities of the cornea. MATERIALS AND METHODS: The present study includes 96 SCD patients...
April 2017: Taiwan Journal of Ophthalmology
https://www.readbyqxmd.com/read/29018296/pharmaceutical-approval-update
#12
Michele B Kaufman
L-glutamine oral powder (Endari) for reducing the acute complications of sickle cell disease; edaravone (Radicava) for the treatment of amyotrophic lateral sclerosis; and midostaurin (Rydapt) for the treatment of acute myeloid leukemia in combination with chemotherapy.
October 2017: P & T: a Peer-reviewed Journal for Formulary Management
https://www.readbyqxmd.com/read/28994899/prophylactic-antibiotics-for-preventing-pneumococcal-infection-in-children-with-sickle-cell-disease
#13
REVIEW
Angela E Rankine-Mullings, Shirley Owusu-Ofori
BACKGROUND: Persons with sickle cell disease (SCD) are particularly susceptible to infection. Infants and very young children are especially vulnerable. The 'Co-operative Study of Sickle Cell Disease' observed an incidence rate for pneumococcal septicaemia of 10 per 100 person years in children under the age of three years. Vaccines, including customary pneumococcal vaccines, may be of limited use in this age group. Therefore, prophylactic penicillin regimens may be advisable for this population...
October 10, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28993503/myh9-e1841k-mutation-augments-proteinuria-and-podocyte-injury-and-migration
#14
Sylvia Cechova, Fan Dong, Fang Chan, Michael J Kelley, Phillip Ruiz, Thu H Le
Intronic variants of the MYH9 gene that encodes the nonmuscle myosin heavy chain IIA are associated with diabetic nephropathy in European Americans and with sickle cell disease-associated nephropathy. However, the causal functional variants of MYH9 have remained elusive. Rare missense mutations in MYH9 cause macrothrombocytopenia and are occasionally associated with development of nephropathy. The E1841K mutation is among the common MYH9 missense mutations and has been associated with nephropathy in some carriers...
October 9, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28993082/noncemented-total-hip-arthroplasty-in-sickle-cell-disease-long-term-results
#15
Imran Ilyas, Husam A Alrumaih, Samar Rabbani
BACKGROUND: Avascular necrosis of the femoral head is a well-known sequela of sickle-cell disease (SCD) requiring a surgical intervention that comes with technical challenges. METHODS: Uncemented hip arthroplasty for avascular necrosis of the femoral head due to SCD was carried out for 101 patients (133 hips) between 2000 and 2012. The duration of follow-up ranged from 5 to 17 years (mean, 14.59 years). All patients received a noncemented femoral stem and a noncemented acetabular shell...
September 14, 2017: Journal of Arthroplasty
https://www.readbyqxmd.com/read/28992220/blood-pressure-and-arterial-stiffness-in-kenyan-adolescents-with-the-sickle-cell-trait
#16
Anthony O Etyang, Christopher K Wandabwa, Sailoki Kapesa, Esther Muthumbi, Emily Odipo, Marylene Wamukoya, Nicholas Ngomi, Tilahun Haregu, Catherine Kyobutungi, Thomas N Williams, Johnstone Makale, Alex Macharia, J Kennedy Cruickshank, Liam Smeeth, J Anthony G Scott
The potential association between sickle cell trait (SCT) and increased arterial stiffness/blood pressure (BP) has not been evaluated in detail despite its association with stroke, sudden death and renal disease. We performed 24-hour ambulatory BP monitoring and arterial stiffness measurements in adolescents raised in a malaria free environment in Kenya.Between December 2015 and June 2016, 938 randomly selected adolescents that had been continuous residents of Nairobi from birth were invited to participate in the study...
June 12, 2017: American Journal of Epidemiology
https://www.readbyqxmd.com/read/28988427/hydroxyurea-therapy-in-uk-children-with-sickle-cell-anaemia-a-single-centre-experience
#17
Kate Phillips, Laura Healy, Louise Smith, Russell Keenan
INTRODUCTION: Despite the demonstrated efficacy of hydroxyurea therapy, children with sickle cell anaemia in the UK are preferentially managed with supportive care or transfusion. Hydroxyurea is reserved for children with severe disease phenotype. This is in contrast to North America and other countries where hydroxyurea is widely used for children of all clinical phenotypes. The conservative UK practice may in part be due to concerns about toxicity, in particular marrow suppression with high doses, and growth in children...
October 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28988213/management-of-pneumatosis-intestinalis-in-children-over-the-age-of-6-months-a-conservative-approach
#18
Leel Nellihela, Mohamed Mutalib, David Thompson, Kammermeier Jochen, Manasvi Upadhyaya
BACKGROUND: Pneumatosis intestinalis (PI) is an uncommon and poorly understood condition. Although it can be an incidental finding in asymptomatic individuals, it can also be secondary to life-threatening bowel ischaemia and sepsis. In premature infants, it is a pathognomonic sign of necrotising enterocolitis. There is no consensus regarding management and long-term outcome of children with PI. AIM: Review of our experience of PI in children beyond the early infantile period...
October 7, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28984727/sickle-cell-disease-and-the-eye
#19
Brian K Do, Damien C Rodger
PURPOSE OF REVIEW: To review recent literature pertaining to sickle cell retinopathy (SCR) and, in particular, sickle cell maculopathy. RECENT FINDINGS: Several recent studies suggest that macular perfusion abnormalities seen in patients with sickle cell disease of various genotypes may affect both the superficial and deep capillary plexi, with a predilection for the deep capillary plexus. Further, these changes may be associated with areas of macular thinning, as well as with peripheral retinal ischemia, even in individuals without visual symptoms, contrary to what has previously been described in both diabetic retinopathy and retinal vein occlusion...
November 2017: Current Opinion in Ophthalmology
https://www.readbyqxmd.com/read/28983899/understanding-patient-preferences-for-femoral-central-venous-catheterization-among-patients-with-sickle-cell-disease
#20
Peter Ju Park, Stephen Scott Cole, Zachary Louis Bercu, Jonathan Martin, Janice Newsome
No abstract text is available yet for this article.
October 3, 2017: Journal of Vascular Access
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