Definitions of the phenotypic manifestations of sickle cell disease

SK Ballas, S Lieff, LJ Benjamin… - American journal of …, 2010 - Wiley Online Library
Sickle cell disease (SCD) is a pleiotropic genetic disorder of hemoglobin that has profound
multiorgan effects. The low prevalence of SCD (∼ 100,000/US) has limited progress in …

American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease

RI Liem, S Lanzkron, T D. Coates, L DeCastro… - Blood …, 2019 - ashpublications.org
Background: Prevention and management of end-organ disease represent major
challenges facing providers of children and adults with sickle cell disease (SCD) …

Understanding the natural progression in% FEV1 decline in patients with cystic fibrosis: a longitudinal study

D Taylor-Robinson, M Whitehead, F Diderichsen… - Thorax, 2012 - thorax.bmj.com
Background Forced expiratory volume in 1 s as a percentage of predicted (% FEV1) is a key
outcome in cystic fibrosis (CF) and other lung diseases. As people with CF survive for longer …

Transition and sickle cell disease

MR DeBaun, J Telfair - Pediatrics, 2012 - publications.aap.org
Sickle cell disease (SCD), the most common genetic disease screened for in the newborn
period, occurs in∼ 1 in 2400 newborns in the general population and 1 in 400 individuals of …

[HTML][HTML] Pulmonary, gonadal, and central nervous system status after bone marrow transplantation for sickle cell disease

MC Walters, K Hardy, S Edwards… - Biology of Blood and …, 2010 - Elsevier
We conducted a prospective, multicenter investigation of human-leukocyte antigen (HLA)
identical sibling bone marrow transplantation (BMT) in children with severe sickle cell …

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 …

[HTML][HTML] Chronic organ injuries in children with sickle cell disease

S Allali, M Taylor, J Brice, M de Montalembert - haematologica, 2021 - ncbi.nlm.nih.gov
Median life expectancy of patients with sickle cell disease has increased to up to 55 years
but there are still frequent cases of premature death, mostly in patients with pre-existing …

Asthma management: reinventing the wheel in sickle cell disease

CR Morris - American journal of hematology, 2009 - Wiley Online Library
Asthma is a common comorbidity in sickle cell disease (SCD) with a reported prevalence of
30–70%. The high frequency of asthma in this population cannot be attributed to genetic …

Acute chest syndrome, airway inflammation and lung function in sickle cell disease

A De, S Williams, Y Yao, Z Jin, GM Brittenham… - PloS one, 2023 - journals.plos.org
Background Acute chest syndrome (ACS) is an acute complication in SCD but its effects on
lung function are not well understood. Inflammation is a key component of SCD …

The impact of recurrent acute chest syndrome on the lung function of young adults with sickle cell disease

JM Knight-Madden, TS Forrester, NA Lewis… - Lung, 2010 - Springer
The aim of this study was to assess the impact of recurrent acute chest syndrome (ACS)
episodes on the lung function of young adults with sickle cell disease (SCD). Our …