Lower airway obstruction is associated with increased morbidity in children with sickle cell disease.

Rationale: The association between pulmonary function and morbidity in children with sickle cell disease (SCD) has not been previously evaluated. Our objective was to study the relationship between abnormalities in pulmonary function and morbidity as represented by the rate of hospitalizations for pain or acute chest syndrome (ACS) in children with SCD.

Methods: Results of pulmonary function tests obtained for clinical indications in children ages 6-18 years were classified as lower airway obstruction (forced expiratory volume in 1 sec/forced volume capacity <95% confidence interval adjusted for age, gender, race, and height), restriction (total lung capacity <80% predicted adjusted for gender, age, race, and height), and normal lung function.

Methacholine challenge in children with sickle cell disease: a case series.

Lung disease is a major cause of morbidity in children with sickle cell disease (SCD). Asthma in children with SCD is associated with a twice greater rate of pain and acute chest syndrome (ACS) episodes when compared to children with SCD but without asthma. Provocation challenges with methacholine are used to diagnose asthma when spirometry is normal, bronchodilator reactivity is absent, or the clinical picture is ambiguous.

Major gene effect and additive familial pattern of inheritance of asthma exist among families of probands with sickle cell anemia and asthma.

In the United States, sickle cell anemia (SCA) affects approximately 1 in 400 African-American newborns. Acute episodes of pain and acute chest syndrome (ACS) are the two leading causes of hospitalization. A relationship between the diagnosis of asthma and the incidence of pain and ACS has been established.

Asthma is associated with increased mortality in individuals with sickle cell anemia.

An analysis of a prospective cohort of individuals with sickle cell anemia (SCA), enrolled from birth through adulthood, was conducted to determine if asthma is a risk factor for death in SCA. All-cause mortality was determined for participants after adjusting for known risk factors for death in SCA. The study included 1,963 individuals who were followed for 18,495 patient-years.

Daytime pulse oximeter measurements do not predict incidence of pain and acute chest syndrome episodes in sickle cell anemia.

A prospective, infant cohort study of children with sickle cell anemia was evaluated to determine the relationship between daytime pulse oximeter measurements and the incidence of pain and acute chest episodes (ACS). A total of 130 children were evaluated. The Pearson correlation between SpO2 and pain and ACS episode rates were 0.

Mailing of a sickle cell disease educational packet increases blood donors within an African American community.

Background: Low blood donor rates among African American persons are recognized; however, few strategies exist to increase these numbers.

Study Design And Methods: A 1-year, prospective, ecologic study performed before and after an educational intervention designed to test the hypothesis that increased education about the importance of blood donation for children with sickle cell disease (SCD) would result in an increase in total blood donors among African American persons.

Results: Approximately 5000 videos were mailed to 50 percent of the households in a zip code where 98 percent of the residents are African American.

Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia.

Pain and acute chest syndrome (ACS) episodes are 2 of the most common causes of hospitalization in children with sickle cell anemia (SCA). However, very few potentially modifiable risk factors for either condition have been identified. In this prospective infant cohort study, we tested the hypothesis that asthma is associated with an increased incidence rate of pain and ACS episodes.

Inadequate community knowledge about sickle cell disease among African-American women.

Background: Federal legislation was passed in 1972 initiating genetic screening and community education about sickle cell disease (SCD). Few assessments have examined the impact of these programs. The aim of this study is to assess existing knowledge about SCD among African-American women in St.

Asthma and acute chest in sickle-cell disease.

Our objective was to determine if physician-diagnosed asthma increases the risk of acute chest syndrome (ACS) in children with sickle-cell disease (SCD) hospitalized for pain. Our study design was a retrospective case-control study of all SCD patients, aged 2-21 years, hospitalized for pain during the interval 1999-2000. Medical records of first admissions during the interval were reviewed to determine the presence of ACS during the admission.