Pulmonary Complications of Sickle Cell Disease

Firth, Paul G.

doi:10.1056/nejmc082638

Cited by 3 publications

(1 citation statement)

References 48 publications

(102 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Sickle cell disease (SCD) is a common autosomal recessive hemoglobinopathy, which can lead to a dramatic reduction in quality of life and average life expectancy. As one of the most common monogenetic disorders globally, it affects nearly 1 in 600 African Americans, up to 4% of children in sub-Saharan Africa being born with this disorder ( 1 ). The number of individuals affected in Germany is around 3,000 and rising, approximately 200 children being born with this hemoglobinopathy each year ( 2 , 3 ).…”

Section: Introductionmentioning

confidence: 99%

Evaluation of lung function in a German single center cohort of young patients with sickle cell disease using EIT and standard techniques

et al. 2023

View full text Add to dashboard Cite

Background and objectiveSickle cell disease (SCD) is a very common autosomal recessive hemoglobinopathy leading to multiple pulmonary complications that are closely associated with mortality. The pathophysiology of chronic pulmonary involvement is not yet fully understood and no specific therapies are available.MethodsThe aim of this cross-sectional study was to characterize the lung function of children and young adolescents with SCD in a German single-center cohort and to extend conventional lung function testing by the use of a new imaging method. We performed spirometry and body plethysmography in 35 children and young adults with hemoglobin SS, SC, S/β-thalassemia as well as 50 controls. These data were compared with clinical characteristics and typical laboratory parameters of hemolysis and disease activity in SCD. To identify lung inhomogeneities, for example due to atelectasis, hyperinflation, air trapping or vascular occlusions, we used the promising new method of electrical impedance tomography (EIT) and calculated global inhomogeneity indices.ResultsLung function of patients with SCD was significantly reduced compared to that of healthy controls. When the result was found to be pathological, the most commonly observed type of breathing disorder was classified as restrictive. Laboratory parameters showed typical features of SCD including decreased levels of hemoglobin and hematocrit and elevated levels of leucocytes, platelets, lactate dehydrogenase and total bilirubin. However, there was no correlation between blood values and reduced lung function. Electrical impedance tomography (EIT) revealed no abnormalities in SCD patients compared to healthy controls. In particular, we were unable to demonstrate any regional inhomogeneities in lung ventilation.ConclusionIn our study, SCD patients showed impaired lung function, with a relevant percentage of patients suffering from restrictive breathing disorder. Signs of obstruction could not be detected. Electrical impedance tomography (EIT) measurements revealed no unevenness that would suggest air entrapment, blockage of blood vessels, excessive inflation, obstruction, or other forms of lung disease. Additionally, the reduction in lung function observed in SCD patients was not related to the disease severity or laboratory test results.

show abstract

Section: Introductionmentioning

confidence: 99%