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BACKGROUND Pediatric health-related quality of life (HRQOL) questionnaires have been validated in children with sickle cell disease (SCD), but small sample sizes in these studies have limited clinical comparisons. We used the baseline clinical data from the Collaborative Data (C-Data) Project of the Comprehensive Sickle Cell Centers (CSCC) Clinical Trial(More)
A cohort of patients with sickle cell disease, consisting of children, adolescents, and adults, who reported experiencing three or more episodes of vaso-occlusive pain the preceding year, were enrolled in a prospective two-period treatment protocol. Following a 4-month conventional treatment baseline phase, a supplemental cognitive-behavioral pain(More)
BACKGROUND This study aimed to clarify associations between pain, psychological adjustment, and family functioning with health-related quality of life (HRQOL) in a sample of adolescents with sickle cell disease (SCD) utilizing teen- and parent-report. METHODS Forty-two adolescents (between the ages of 12 and 18) with SCD and their primary caregivers(More)
BACKGROUND Nocturnal oxyhaemoglobin desaturation might have a role in CNS complications related to sickle cell disease, and rates of painful crises. We attempted to examine the biological relations, and describe the haematological risk factors for oxyhaemoglobin desaturation. METHODS The study population included children with sickle cell disease and(More)
Pulse oximetry is a noninvasive method of measuring oxyhemoglobin saturation. The validity of pulse oximetry in sickle cell disease (SCD) has been questioned. We evaluated pulse oximetry, arterial blood gas analysis, and co-oximetry in patients with SCD, and we assessed the effect of dyshemoglobin and altered blood-oxygen affinity on their accuracy. Sixteen(More)
Sickle cell disease (SCD) is a pleiotropic genetic disorder of hemoglobin that has profound multiorgan effects. The low prevalence of SCD ( approximately 100,000/US) has limited progress in clinical, basic, and translational research. Lack of a large, readily accessible population for clinical studies has contributed to the absence of standard definitions(More)
CONTEXT Inhaled nitric oxide has shown evidence of efficacy in mouse models of sickle cell disease (SCD), case series of patients with acute chest syndrome, and 2 small placebo-controlled trials for treatment of vaso-occlusive pain crisis (VOC). OBJECTIVE To determine whether inhaled nitric oxide gas reduces the duration of painful crisis in patients with(More)
Progressive restrictive defect with increasing age, obstructive lung disease, and bronchodilator responsiveness have been reported in sickle cell disease (SCD). Because airway hyperreactivity (AHR) can be underestimated when assessed by bronchodilator responsiveness in patients with normal baseline lung function, the aim of this study was to investigate the(More)
Analgesic trials pose unique scientific, ethical, and practical challenges in pediatrics. Participants in a scientific workshop sponsored by the US Food and Drug Administration developed consensus on aspects of pediatric analgesic clinical trial design. The standard parallel-placebo analgesic trial design commonly used for adults has ethical and practical(More)
Several lines of evidence suggest that sickle cell disease (SCD) is associated with a chronic inflammatory state. In this study of 70 children with SCD at steady state evaluated by a broad panel of biomarkers representing previously examined mechanisms of pathogenicity in SCD, high sensitivity C-reactive protein (hs-CRP), a marker of low-grade, systemic(More)