Reduced fitness and abnormal cardiopulmonary responses to maximal exercise testing in children and young adults with sickle cell anemia.

Physiologic contributors to reduced exercise capacity in individuals with sickle cell anemia (SCA) are not well understood. The objective of this study was to characterize the cardiopulmonary response to maximal cardiopulmonary exercise testing (CPET) and determine factors associated with reduced exercise capacity among children and young adults with SCA. A cross-sectional cohort of 60 children and young adults (mean 15.

Access to patient-centered medical homes in children with sickle cell disease.

To determine the proportion of children with sickle cell disease (SCD) followed in a subspecialty clinic with access to a primary care provider (PCP) exhibiting practice-level qualities of a patient-centered medical home (PCMH). We surveyed 200 parents/guardians of children with SCD using a 44-item tool addressing PCP access, caregiver attitudes toward PCPs, barriers to healthcare utilization, perceived disease severity, and satisfaction with care received in the PCP versus SCD clinic settings. Individual PCMH criteria measured were a personal provider relationship and medical care characterized as accessible, comprehensive and coordinated.

The impact of thalassemia on Southeast Asian and Asian Indian families in the United States: a qualitative study.

Objective: To describe the challenges, including sociocultural and socioeconomic barriers, faced by an urban immigrant population in the United States affected by thalassemia major.

Design: Ethnographic, semi-structured, 1-on-1 interviews using an interview guide developed for this study. Digital recordings were transcribed and data analyzed using constant comparative method.

Reproducibility of tricuspid regurgitant jet velocity measurements in children and young adults with sickle cell disease undergoing screening for pulmonary hypertension.

The reproducibility of tricuspid regurgitant jet velocity (TRJV) measurements by Doppler echocardiography has not been subjected to systematic evaluation among individuals with sickle cell disease (SCD) undergoing screening for pulmonary hypertension. We examined sources of disagreement associated with peak TRJV in children and young adults with SCD. Peak TRJV was independently measured and interpreted a week apart by separate sonographers and readers, respectively, in 30 subjects (mean age, 15.

Parental attitudes toward research participation in pediatric sickle cell disease.

Background: Socio-cultural attitudes and perceptions are commonly cited barriers to the recruitment of African-Americans for medical research, yet no studies have examined the factors influencing research participation among individuals with sickle cell disease (SCD) or caregivers of children with SCD.

Procedure: We distributed a 32-item, self-administered survey to parents or legal guardians of children with SCD over a 6-month period. We used Pearson's chi-square to determine factors associated with a favorable attitude toward research participation and logistic regression to determine independent associations.