...Anna L. Slingerland; Madeline B. Karsten; Edward R. Smith; Amy E. Sobota; Alfred P. See Moyamoya syndrome increases the risk of stroke in sickle cell disease, but revascularization surgery can modify this risk. Collaborative management between hematology and neurosurgery offers effective strategies...

...Ana Cristina Silva-Pinto; Letícia Santos-Oliveira; Flávia Leite Souza Santos; Simone Kashima Haddad; Gil Cunha De Santis; Rodrigo do Tocantins Calado Sickle cell disease is characterized by vaso-occlusive phenomena and haemolytic anaemia. There is a significant concern that the overlap of COVID-19...

...Anwar Al-Awadhi; Adekunle Adekile; Rajaa Marouf Background: Thrombospondin 1 (TSP-1) is a multifunctional glycoprotein secreted by platelets. In sickle cell disease (SCD), TSP-1 promotes red cell adhesion to the endothelium by binding to von Willebrand factor (vWF) and inhibiting its degradation...

...Matthew S. Karafin; Pippa Simpson; Joshua J. Field Chronic pain affects 50% of adults with sickle cell disease (SCD). Although inflammation is thought to contribute to the pathogenesis of chronic pain, no studies have examined the differences in circulating cytokines between patients with SCD...

...Kate Travis; Anthony Wood; Peter Yeh; Sachin Allahabadi; Ling-Chen Chien; Sara Curtis; Allexa Hammond; Jaden Kohn; Christine Ogugbuaja; Matthew Rees; John Shumway; Vivien Sheehan Background/Aims: We surveyed sickle cell disease (SCD) patients who transitioned from pediatric care at Texas Children’s...

...Ameet Patel; Hants Williams; Maria R. Baer; Ann B. Zimrin; Jennie Y. Law Background: Venous thromboembolism (VTE) is a recognized complication of sickle cell disease (SCD), yet the optimal pharmacologic anticoagulant is unknown. Methods: A retrospective single-institution cohort study of patients...

...Oula Abdullah Najim; Mea’ad Kadhum Hassan Objectives: This prospective study was carried out to assess lactate dehydrogenase (LDH) as a biochemical marker during acute painful episode and steady state in children with sickle cell disease (SCD). Design and Methods: A prospective descriptive study...

... patients from 2 sickle cell disease referral centers during a total follow-up of 654 patient-years and total hydroxyurea exposure of 549 patient-years. Fifty-six adverse events occurred (incidence: 12%/patient-year), with leg ulcers being the most frequent. Adverse events could arise at any time and were...

.... Interleukins Sickle cell disease Stroke Genetic polymorphism Stroke is a common and serious complication of sickle cell anemia (SCA), affecting children as well as adults. With improved treatment and an increase in life expectancy in SCA, stroke in adults with SCA has received greater attention [ 1...

... to in the content or advertisements. Avascular necrosis Hb F Hb SD-Los Angeles Hemoglobin Sickle cell disease Vaso-occlusive crises Hemoglobin (Hb) D-Punjab or D-Los Angeles is a β-globin chain variant resulting from a Glu→Gln substitution at codon 121 with a GAA→CAA change at the DNA level...

...Renu Gupta; Rajaa Marouf; Adekunle Adekile Low bone mineral density (BMD) is common in sickle cell disease (SCD) patients of all ages due to erythropoietic stress and consequent bone marrow hyperplasia. Kuwaiti SCD patients have a mild clinical phenotype because of their high Hb F level. There has...

...Patrick B. Walter; Paul Harmatz; Elliott Vichinsky This review highlights recent advances in iron metabolism that are relevant to sickle cell disease (SCD). SCD is a common hemoglobinopathy that results in chronic inflammation. Improved understanding of how iron metabolism is controlled by proteins...

... in patients with various underlying anemias, including β-thalassemia, myelodysplastic syndromes, sickle cell disease, aplastic anemia, and other rare anemias. In addition to reducing key indicators of total body iron levels (serum ferritin, liver iron concentration, and toxic labile plasma iron), deferasirox...

...P.P. Landburg; E. Nur; N. Maria; D.P.M. Brandjes; B.J. Biemond; J.B. Schnog; A.J. Duits Inflammation and angiogenesis are of importance in the pathophysiology of sickle cell disease (SCD). Recently, the chemokine stromal-derived factor-1 (SDF-1) has been shown to be a key mediator of angiogenesis...

...W. Akin Isaacs Fifty individuals with sickle cell disease were given either steroids (testosterone for males and progesterone for females) at a dose of 10 mg or arachis oil intramuscularly. Sickling with metabi-sulphite was assessed at daily intervals for 5 days after the administration of the drug...

... and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. G-6-PD in Ghana Sickle cell disease Acta Haemat. 50: 105-111 (1973) Glucose-6-Phosphate Dehydrogenase Electrophoresis...

...A. Fertakis; G. Panitsas; B. Angelopoulos Serum haemopexin levels were determined by the radial immunodiffusion method in 96 patients with β -thalassaemia major (27 after splenectomy), in 36 with sickle cell disease and in 18 with sickle cell-thalassaemia disease (4 after splenectomy). Lowering...

... of this study. A sickle cell trait frequency of 0.123 evidenced by hemoglobin electrophoresis correlates closely with previous studies of hemoglobinopathies in Saudi Arabs. The presence of four subjects with sickle cell disease in the study group provides further evidence of the ‘benign’ nature of sickle cell...

...Anne Yardumian; Sally C. Davies Patients affected by sickle cell disease encounter varied and often severe clinical problems directly related to their condition and are at greater risk than the general population from particular infections, thrombosis and certain surgical pathologies. The current...

...Martin J. Pippard This paper reviews the factors governing the rate of iron loading and iron toxicity in the thalassaemia syndromes and sickle cell disease. It outlines the main determinants of iron mobilization by the iron-chelating drug, desferrioxamine, together with the effects of this drug...