Treatment of respiratory system (not just lung!) abnormalities in Mucopolysaccharidosis I.
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Adolescent↗
Biomedical subjects
Julian L Allen
Publications and source records attributed to Julian L Allen.
Hypoxaemia in sickle cell disease: biomarker modulation and relevance to pathophysiology.
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 controls. Cellular activation was assessed by measurement of soluble vascular cell adhesion molecule 1, P-selectin, L-selectin, and leukotriene B4. Erythrocyte-endothelial adhesion and routine haematological variables were assessed. Oxygen saturation (SaO2) was measured by pulse oximetry while children were awake and asleep. Children with a mean sleeping SaO2 of < or =93% were identified as hypoxaemic. Children were divided into four groups: controls (ten children), HbSC (nine, all normoxic), HbSS normoxic (13), and HbSS hypoxaemic (15). FINDINGS: Among haematological variables, sleeping SaO2 correlated only with packed-cell volume (r=0.7; p<0.0001). Inverse relations were noted between sleeping SaO2 and adhesion (-0.45; p<0.01), and markers of white-cell (-0.51; p<0.01), platelet (-0.61; p<0.001), and endothelial activation (-0.46; p<0.01). In the HbSS group who had sleeping hypoxaemia, waking SaO2 measurements showed continuing hypoxaemia, with similar correlation between SaO2 and cell activation markers. INTERPRETATION: Our adhesion-related findings suggest a potential mechanism for the increased occurrence of clinical vaso-occlusive crises in individuals with sickle cell disease who have oxyhaemoglobin desaturation. Release of cellular mediators in hypoxaemia, and the relation between anaemia and oxyhaemoglobin desaturation, suggest that risk factors for stroke, including anaemia, might have a role in CNS-vasculopathy through hypoxia-mediated pathways. Further more, hypoxaemia in the older child also occurs during the day; such mild untreated hypoxia could lead to an increased risk of vaso-occlusive episodes.
Adolescent↗
Infant pulmonary function testing.
Pulmonary function tests in infants have been used for many years, but they have been largely relegated to specialized centers and research purposes. More recently, development of commercial equipment and newer techniques has allowed for more broad use and application of these tests. This review describes different techniques that have been used to study infants and toddlers, and their clinical applications.
Child, Preschool↗
Noninvasive measurement of the tension-time index in children with neuromuscular disease.
Respiratory muscle weakness is common in children with neuromuscular disease (NMD). We hypothesized that weakness puts them at risk for respiratory muscle fatigue, a harbinger of chronic respiratory failure. We therefore measured a noninvasive index of respiratory muscle fatigue, the tension-time index of the respiratory muscles (TT(mus)), in 11 children with NMD and 13 control subjects. Spirometric flow rates and maximal inspiratory pressure were significantly lower in the NMD group than in controls (43 +/- 23 vs. 99 +/- 21 cmH2O, P < 0.001). The mean TT(mus) was significantly higher in the NMD group than in controls (0.205 +/- 0.117 vs. 0.054 +/- 0.021, P < 0.001). The increase in TT(mus) was primarily due to an increase in the ratio of average mean inspiratory pressure to maximal inspiratory pressure, indicating decreased respiratory muscle strength reserve. We found a significant correlation between TT(mus) and the residual volume-to-total lung capacity ratio (r = 0.504, P = 0.03) and a negative correlation between TT(mus) and forced expiratory volume in 1 s (r = -0.704, P < 0.001). In conclusion, children with NMD are prone to respiratory muscle fatigue. TT(mus) may be useful in assessing tolerance during weaning from mechanical ventilation, identifying impending respiratory failure, and aiding in the decision to institute therapies.
Adolescent↗
Respiratory inductance plethysmography in healthy 3- to 5-year-old children.
STUDY OBJECTIVES: Because of the challenges of using standard measurements such as spirometry to measure respiratory function in 3- to 5-year-old children, there may be a role for respiratory inductive plethysmography (RIP), which is noninvasive and requires minimal subject cooperation. In this study, we described normative values of thoracoabdominal motion and timing mechanics in 3- to 5-year-old children, and hypothesized positional dependence of these measurements in this age group. DESIGN: We measured relative thoracoabdominal motion during tidal breathing using the phase angle (Phi), the labored breathing index, and the phase relation during the total breath and timing mechanics with the ratio of time to peak tidal expiratory flow to expiratory time (TPTEF/TE). SETTING: Preschools within the greater Philadelphia area and the Pulmonary Office of The Children's Hospital of Philadelphia. PATIENTS OR PARTICIPANTS: Fifty healthy children between 3 years and 5 years of age. INTERVENTIONS: RIP. MEASUREMENTS AND RESULTS: All measures varied with position. Thoracoabdominal motion was nearly synchronous in the sitting position and most asynchronous in the supine position (Phi, 15.7 +/- 4.0 degrees vs 56.1 +/- 4.3 degrees, respectively; p < 0.001). This also led to an increase in the TPTEF/TE from the sitting to the supine positions (30.3 +/- 1.4% vs 37.0 +/- 1.6%, respectively; p < 0.001). Measurements of thoracoabdominal motion and timing mechanics did not change with age, weight, height, or gender. CONCLUSIONS: We conclude that the positional dependence of these measurements is due to the alteration in respiratory mechanics between the sitting, standing, and supine positions. We further conclude that if RIP is to be a useful longitudinal measure of respiratory function in this age range, comparison measurements should be made in the same position.
Abdominal Wall↗