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Factors associated with adverse outcome among children with sickle cell disease admitted to the pediatric intensive care unit: an observational cohort
BackgroundSickle cell disease (SCD) is one of the most frequent inherited diseases in the world. Over the last decades, in high-income countries, an...
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How does sickle cell disease affect the peri-operative outcome in patients undergoing total knee arthroplasty? A large-scale, National Inpatient Sample-based study
IntroductionIn view of the vaso-occlusive pathophysiology affecting osseous micro-circulation, sickle cell disease (SCD) is well known to present...
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Preoperative Transfusion in Sickle Cell Disease Children Undergoing Adenotonsillectomy
ObjectiveTo perform a systematic review and meta-analysis comparing two pre-operative transfusion regimens (conservative versus aggressive) in...
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Evaluation of treatment patterns, healthcare resource utilization and cost of illness for sickle cell disease in Ghana: a private medical insurance claims database study
BackgroundSickle cell disease (SCD) is a major public health concern in sub-Saharan Africa, accounting for nearly 75% of the global disease burden....
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Pain management in adult patients with sickle cell disease in the emergency department: how does current practice compare with existing standards of care?
PurposeSickle cell disease (SCD) is an inherited blood disorder with a natural course punctuated by acute complications including painful...
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Incidence and risk factors for osteonecrosis of the femoral head in five hundred and ten sickle cell disease paediatric patients
PurposeOsteonecrosis of the femoral head (ONFH) is a degenerative and progressive disorder that mainly affects people with sickle cell disease (SCD)....
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Expert consensus on the management of infusion-related reactions (IRRs) in patients with sickle cell disease (SCD) receiving crizanlizumab: a RAND/UCLA modified Delphi panel
Crizanlizumab, a monoclonal antibody against P-selectin, has been shown to reduce vaso-occlusive crises (VOCs) compared to placebo in patients ≥ 16...
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Transcranial doppler as screening method for sickling crises in children with sickle cell anemia: a latin America cohort study
BackgroundSickle cell anemia (SCA) is the leading cause of childhood stroke. We aimed to evaluate whether altered cerebral flow velocities, as...
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Numb chin syndrome in sickle cell disease: a case series of Jamaican patients
Numb chin syndrome is an uncommon presentation that has been reported as secondary to metastatic disease, trauma, and infections of the maxilla,...
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Substance use disorder of equimolar oxygen-nitrous oxide mixture in French sickle-cell patients: results of the PHEDRE study
BackgroundIn many countries, nitrous oxide is used in a gas mixture (EMONO) for short-term analgesia. Cases of addiction, with significant misuse,...
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L-glutamine for sickle cell disease: more than reducing redox
Oxidative stress is a major contributor to the pathophysiology of sickle cell disease (SCD) including hemolysis and vaso-occlusive crisis (VOC)....
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Population Pharmacokinetic Analysis of Rivipansel in Healthy Subjects and Subjects with Sickle Cell Disease
BackgroundSickle cell disease is an inherited blood disorder with reduced blood-carrying capacity. It is associated with a tendency to form...
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The ratio of ATP11C/PLSCR1 mRNA transcripts has clinical significance in sickle cell anemia
One of the physiologic mechanisms responsible to maintain asymmetric phospholipid distribution (in particular phosphatidylserine, PS) in human...
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Sickle Cell Disease Treatment with Arginine Therapy (STArT): study protocol for a phase 3 randomized controlled trial
BackgroundDespite substantial illness burden and healthcare utilization conferred by pain from vaso-occlusive episodes (VOE) in children with sickle...
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Subclinical myocardial injury during vaso-occlusive crisis in pediatric sickle cell disease
Acute painful crisis is the most frequent complication of sickle cell disease (SCD) in children. Recurrent vaso-occlusion may be associated with...
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Madrid Newborn Sickle Cell Disease Cohort: clinical outcomes, stroke prevention and survival
In May 2003, Madrid established the universal newborn screening (NBS) for sickle cell disease (SCD). However, there are no studies resembling the...
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Association of KLOTHO polymorphisms with clinical complications of sickle cell anemia
The clinical and phenotypic heterogeneity of patients with sickle cell anemia (SCA) is influenced by environmental and genetic factors. Several...
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The clinical and radiological effectiveness of autologous bone marrow derived osteoblasts (ABMDO) in the management of avascular necrosis of femoral head (ANFH) in sickle cell disease (SCD)
PurposeAvascular necrosis of the femoral head is a common issue faced by orthopaedic surgeons that ranges between 10 and 18%, but in patients with...
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Spontaneous splenic rupture, mesenteric ischemia and spinal infarction after aortic repair for acute type A dissection in a patient with sickle cell trait
Sickle cell trait (SCT), a benign hematological condition affecting approximately 300 million individuals globally, is associated with an increased...
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Sleep disordered breathing and its relation to stroke and pulmonary hypertension in children with sickle cell disease: a single-center cross-sectional study
Sleep disordered breathing (SDB) is a common underdiagnosed sequela of sickle cell disease (SCD) that has been linked to the frequency of...
