Sickle cell disease: clinical presentation and management of a global health challenge

ME Houwing, PJ De Pagter, EJ Van Beers, BJ Biemond… - Blood reviews, 2019 - Elsevier
Sickle cell disease is an autosomal recessive, multisystem disorder, characterised by
chronic haemolytic anaemia, painful episodes of vaso-occlusion, progressive organ failure …

[HTML][HTML] A systematic literature review of frequency of vaso-occlusive crises in sickle cell disease

AU Zaidi, AK Glaros, S Lee, T Wang… - Orphanet journal of rare …, 2021 - Springer
Background and purpose Sickle cell disease (SCD) is a collection of rare inherited blood
disorders affecting approximately 100,000 people in the US and 20–25 million people …

[HTML][HTML] AAPT diagnostic criteria for chronic sickle cell disease pain

C Dampier, TM Palermo, DS Darbari, K Hassell… - The Journal of …, 2017 - Elsevier
Pain in sickle cell disease (SCD) is associated with increased morbidity, mortality, and high
health care costs. Although episodic acute pain is the hallmark of this disorder, there is an …

Sickle cell disease: monitoring, current treatment, and therapeutics under development

C Hoppe, L Neumayr - Hematology/Oncology Clinics, 2019 - hemonc.theclinics.com
Sickle cell disease (SCD) is a complex, clinically heterogeneous disorder affecting
approximately 100,000 individuals in the United States and millions worldwide. The disease …

Identifying clinical and research priorities in sickle cell lung disease. An Official American Thoracic Society Workshop Report

AP Ruhl, SC Sadreameli, JL Allen… - Annals of the …, 2019 - atsjournals.org
Background: Pulmonary complications of sickle cell disease (SCD) are diverse and
encompass acute and chronic disease. The understanding of the natural history of …

Acute chest syndrome of sickle cell disease: genetics, risk factors, prognosis, and management

ES Klings, MH Steinberg - Expert Review of Hematology, 2022 - Taylor & Francis
Introduction Sickle cell disease, one of the world's most prevalent Mendelian disorders, is a
chronic hemolytic anemia punctuated by acute vasoocclusive events. Both hemolysis and …

The intersection between asthma and acute chest syndrome in children with sickle-cell anaemia

MR DeBaun, RC Strunk - The Lancet, 2016 - thelancet.com
Acute chest syndrome is a frequent cause of acute lung disease in children with sickle-cell
disease. Asthma is common in children with sickle-cell disease and is associated with …

[HTML][HTML] Vaso-occlusion in sickle cell disease: is autonomic dysregulation of the microvasculature the trigger?

S Veluswamy, P Shah, CC Denton… - Journal of clinical …, 2019 - mdpi.com
Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by polymerization
of hemoglobin S upon deoxygenation that results in the formation of rigid sickled-shaped red …

Current standards of care and long term outcomes for thalassemia and sickle cell disease

S Chonat, CT Quinn - Gene and cell therapies for beta-globinopathies, 2017 - Springer
Thalassemia and sickle cell disease (SCD) are disorders of hemoglobin that affect millions
of people worldwide. The carrier states for these diseases arose as common, balanced …

A systematic review of the literature for severity predictors in children with sickle cell anemia

ER Meier, RM Fasano, PR Levett - Blood Cells, Molecules, and Diseases, 2017 - Elsevier
All patients with HbSS (SCA) share the same genetic mutation but the clinical phenotype is
variable and difficult to predict early in life. A reliable severity predictor would be invaluable …