Lung function abnormalities occur in children with sickle cell disease (SCD) and may be associated with elevated pulmonary blood volume. To investigate that association, we determined whether blood transfusion in SCD children acutely increased pulmonary capillary blood volume (PCBV) and increased respiratory system resistance (Rrs5). Measurements of Rrs5 and spirometry were made before and after blood transfusion in 18 children, median age 14.2 (6.6-18.5) years. Diffusing capacity for carbon monoxide and nitric oxide were assessed to calculate the PCBV. Post transfusion, the median Rrs5 had increased from 127.4 to 141.3% predicted (p<0.0001) and pulmonary capillary blood volume from 39.7 to 64.1 ml/m2 (p<0.0001); forced expiratory volume in one second (p=0.0056) and vital capacity (p=0.0008) decreased. The increase in Rrs5 correlated with the increase in PCBV (r=0.50, p=0.0493). Increased pulmonary capillary blood volume may at least partially explain the lung function abnormalities in SCD children.
The aim of the study was to identify oral baclofen dosing variability at steady state based on weight and Gross Motor Function Classification System level using a retrospective cross-sectional study design. The medical records of 500 pediatric aged patients (age 1–21 yrs) were reviewed to obtain 144 pediatric patients who met inclusion criteria. One-way analysis of variance tests revealed increasing mean doses in baclofen (in milligram per kilogram) with higher Gross Motor Function Classification System levels (P = 0.001). Post hoc Tukey analysis showed patients with higher ambulatory ability (Gross Motor Function Classification System I–II) received a lower total daily dosage than did patients with less ambulatory ability (Gross Motor Function Classification System III–V). A moderate correlation was observed with increasing oral baclofen dose as weight increased (r = 0.43, P < 0.0001). Because of the variability in dosing between Gross Motor Function Classification System levels, prescribing oral baclofen for pediatric patients with cerebral palsy may not follow the traditional model of weight-based dosing seen in other pediatric conditions.
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