Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Abnormal pulmonary function in adults with sickle cell anemia.

Elizabeth S Klings1, Diego F Wyszynski, Vikki G Nolan

  • 1Pulmonary Center, Department of Medicine, Boston Comprehensive Sickle Cell Center, Boston University School of Medicine and School of Public Health, Boston, MA 02118, USA. eklings@lung.bumc.bu.edu

American Journal of Respiratory and Critical Care Medicine
|March 25, 2006
PubMed
Summary

Pulmonary function tests reveal that 90% of adults with sickle cell anemia (Hb-SS) have abnormalities, primarily restrictive physiology and reduced diffusion capacity for carbon monoxide (DLCO). These findings may signal more severe sickle vasculopathy.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

A New Fetal Hemoglobin-Related Phenotype in Sickle Cell Anemia.

American journal of hematology·2026
Same author

Remdesivir Use During Pregnancy: Findings From the Prospective COVID-19 International Drug Pregnancy Registry and Narrative Literature Review.

Journal of pregnancy·2026
Same author

Lessons Learned From the COVID-19 Vaccines International Pregnancy Exposure Registry: Insights for Designing and Implementing Global Pregnancy Safety Studies.

Birth defects research·2026
Same author

Systemic lupus erythematosus and pregnancy: a comprehensive clinical review of maternal, fetal, and treatment considerations.

Advances in rheumatology (London, England)·2026
Same author

Exa-cel in Children with Transfusion-Dependent β-Thalassemia or Sickle Cell Disease.

The New England journal of medicine·2026
Same author

Modeling Genetic Diversity in Sickle Cell Disease Reveals Heterogeneous Responses to HbF-Inducing Therapies.

bioRxiv : the preprint server for biology·2026

Area of Science:

  • Pulmonary Medicine
  • Hematology
  • Cardiopulmonary Function

Background:

  • Pulmonary complications are a significant cause of morbidity in sickle cell anemia (Hb-SS).
  • Large-scale studies on pulmonary function tests (PFTs) in this population are limited.
  • Understanding pulmonary dysfunction is crucial for managing Hb-SS patients.

Purpose of the Study:

  • To analyze PFTs in a large cohort of adults with Hb-SS.
  • To determine the prevalence and patterns of pulmonary dysfunction.
  • To investigate the association between pulmonary dysfunction and other systemic complications.

Main Methods:

  • Analysis of spirometry, lung volumes, and DLCO in 310 adults with Hb-SS.
  • Comparison of PFT data with predicted values.

Related Experiment Videos

  • Classification of subjects into obstructive, restrictive, mixed, isolated low DLCO, or normal groups.
  • Multivariate linear regression to assess associations between PFT abnormalities and laboratory data.

    • Main Results:

    • Only 10% of patients had normal PFTs.
    • Common abnormalities included restrictive physiology (74%) and decreased DLCO (13%).
    • Reduced DLCO was associated with thrombocytosis, hepatic dysfunction, and trends toward renal dysfunction.

    Conclusions:

    • Pulmonary function is abnormal in 90% of adult Hb-SS patients.
    • Restrictive physiology and decreased DLCO are the most frequent PFT abnormalities.
    • Decreased DLCO may indicate more severe sickle vasculopathy with hepatic and renal impairment.