Rib cage mobility in pectus excavatum

Pectus excavatum is generally regarded as a cosmetic deformity; however, some children with pectus excavatum complain of chest pain and exercise limitation. Physiologic studies sometimes show mild restrictive changes and suggest an increased oxygen cost of breathing. Limitation of rib cage mobility related to the deformity may explain these findings. If rib cage mobility is limited, the ability of the actively inspiring rib cage to lower abdominal pressure would be decreased. If this were so, increased swings in abdominal pressure would be seen during the respiratory cycle, especially at times of stress such as during exercise. To test the hypothesis that pectus excavatum is associated with decreased rib cage mobility, we studied 11 patients with pectus excavatum and 11 control subjects. Four control subjects were also studied with rib cage mobility restricted by chest wall strapping sufficient to decrease vital capacity by 5, 10, and 40%. Gastric pressure was measured using balloon catheters and was used as an index of abdominal pressure. Flow at the mouth was recorded and integrated to give volume. Measurements were made at rest, immediately after exercise, and during graded voluntary inspiration to total lung capacity. Gastric pressure was related to tidal volume, and pressure-volume loops were constructed. There were no differences in abdominal pressure swings during respiration between the patients with pectus excavatum and the control subjects. Both groups showed moderate increase in gastric pressure during inspiration at rest and smaller increases or even decreases in abdominal pressure at end inspiration after exercise and at total lung capacity. Control subjects with chest-wall strapping showed marked rises in end-inspiratory abdominal pressure at rest, after exercise, and at total lung capacity. These rises were proportional to the degree of rib cage restriction. Examination of pressure-volume loops also showed no differences between control subjects and the patients with pectus excavatum. However, marked differences were seen in the control subjects with chest wall strapping. We conclude that limitation of rib cage mobility is associated with increased abdominal pressure during inspiration; however, this would not appear to be responsible for the symptoms complained of or the reductions in vital capacity sometimes associated with mild to moderate degrees of pectus excavatum

Rib cage mobility in pectus excavatum

Pectus excavatum is generally regarded as a cosmetic deformity; however, some children with pectus excavatum complain of chest pain and exercise limitation. Physiologic studies sometimes show mild restrictive changes and suggest an increased oxygen cost of breathing. Limitation of rib cage mobility related to the deformity may explain these findings. If rib cage mobility is limited, the ability of the actively inspiring rib cage to lower abdominal pressure would be decreased. If this were so, increased swings in abdominal pressure would be seen during the respiratory cycle, especially at times of stress such as during exercise. To test the hypothesis that pectus excavatum is associated with decreased rib cage mobility, we studied 11 patients with pectus excavatum and 11 control subjects. Four control subjects were also studied with rib cage mobility restricted by chest wall strapping sufficient to decrease vital capacity by 5, 10, and 40%. Gastric pressure was measured using balloon catheters and was used as an index of abdominal pressure. Flow at the mouth was recorded and integrated to give volume. Measurements were made at rest, immediately after exercise, and during graded voluntary inspiration to total lung capacity. Gastric pressure was related to tidal volume, and pressure-volume loops were constructed. There were no differences in abdominal pressure swings during respiration between the patients with pectus excavatum and the control subjects. Both groups showed moderate increase in gastric pressure during inspiration at rest and smaller increases or even decreases in abdominal pressure at end inspiration after exercise and at total lung capacity. Control subjects with chest-wall strapping showed marked rises in end-inspiratory abdominal pressure at rest, after exercise, and at total lung capacity. These rises were proportional to the degree of rib cage restriction. Examination of pressure-volume loops also showed no differences between control subjects and the patients with pectus excavatum. However, marked differences were seen in the control subjects with chest wall strapping. We conclude that limitation of rib cage mobility is associated with increased abdominal pressure during inspiration; however, this would not appear to be responsible for the symptoms complained of or the reductions in vital capacity sometimes associated with mild to moderate degrees of pectus excavatum

Mobile lidar measurements to investigate the boundary layer and flow regimes over Reunion Island

International audienceTwo mobile lidar instruments of new generation were installed on the Reunion Island in the frame of the ECLAIR (Expérience sur la Couche Limite Atmosphérique à l'Ile de la Réunion) field experiment held from 26 November to 5 December 2008. The first one was installed on a pick-up to investigate the structure and temporal evolution of the planetary boundary layer around and inside the island. The second one was located on the site of the University of Reunion Island. This study is a new approach preliminary to the development of an atmospheric observatory, OPAR, located at Piton Maïdo, a high summit (2200m) near the west coast. The field campaign aimed at understanding to what extent, and when, the future observatory will be under the influence of the boundary layer, clouds, and the upward transport of low-level air masses. Results of lidar observation periods will be presented and discussed in relation to high-resolution model outputs. For instance, the lidar instruments evidence the presence of complex vertical structures of the low troposphere above Reunion Island. Such structures remain even inside the island both in the corries and on the mountains. Moreover, some hydraulic character of the wind trade flow around the island could be highlighted from lidar measurements. Indeed, the Venturi acceleration on each side of the obstacle is accompanied by subsidence and a significant shallowing of the boundary layer along the flow

Mobile lidar measurements to investigate the boundary layer and flow regimes over Reunion Island

International audienceTwo mobile lidar instruments of new generation were installed on the Reunion Island in the frame of the ECLAIR (Expérience sur la Couche Limite Atmosphérique à l'Ile de la Réunion) field experiment held from 26 November to 5 December 2008. The first one was installed on a pick-up to investigate the structure and temporal evolution of the planetary boundary layer around and inside the island. The second one was located on the site of the University of Reunion Island. This study is a new approach preliminary to the development of an atmospheric observatory, OPAR, located at Piton Maïdo, a high summit (2200m) near the west coast. The field campaign aimed at understanding to what extent, and when, the future observatory will be under the influence of the boundary layer, clouds, and the upward transport of low-level air masses. Results of lidar observation periods will be presented and discussed in relation to high-resolution model outputs. For instance, the lidar instruments evidence the presence of complex vertical structures of the low troposphere above Reunion Island. Such structures remain even inside the island both in the corries and on the mountains. Moreover, some hydraulic character of the wind trade flow around the island could be highlighted from lidar measurements. Indeed, the Venturi acceleration on each side of the obstacle is accompanied by subsidence and a significant shallowing of the boundary layer along the flow

Mobile lidar measurements to investigate the boundary layer and flow regimes over Reunion Island

International audienceTwo mobile lidar instruments of new generation were installed on the Reunion Island in the frame of the ECLAIR (Expérience sur la Couche Limite Atmosphérique à l'Ile de la Réunion) field experiment held from 26 November to 5 December 2008. The first one was installed on a pick-up to investigate the structure and temporal evolution of the planetary boundary layer around and inside the island. The second one was located on the site of the University of Reunion Island. This study is a new approach preliminary to the development of an atmospheric observatory, OPAR, located at Piton Maïdo, a high summit (2200m) near the west coast. The field campaign aimed at understanding to what extent, and when, the future observatory will be under the influence of the boundary layer, clouds, and the upward transport of low-level air masses. Results of lidar observation periods will be presented and discussed in relation to high-resolution model outputs. For instance, the lidar instruments evidence the presence of complex vertical structures of the low troposphere above Reunion Island. Such structures remain even inside the island both in the corries and on the mountains. Moreover, some hydraulic character of the wind trade flow around the island could be highlighted from lidar measurements. Indeed, the Venturi acceleration on each side of the obstacle is accompanied by subsidence and a significant shallowing of the boundary layer along the flow

Mobile lidar measurements to investigate the boundary layer and flow regimes over Reunion Island

International audienceTwo mobile lidar instruments of new generation were installed on the Reunion Island in the frame of the ECLAIR (Expérience sur la Couche Limite Atmosphérique à l'Ile de la Réunion) field experiment held from 26 November to 5 December 2008. The first one was installed on a pick-up to investigate the structure and temporal evolution of the planetary boundary layer around and inside the island. The second one was located on the site of the University of Reunion Island. This study is a new approach preliminary to the development of an atmospheric observatory, OPAR, located at Piton Maïdo, a high summit (2200m) near the west coast. The field campaign aimed at understanding to what extent, and when, the future observatory will be under the influence of the boundary layer, clouds, and the upward transport of low-level air masses. Results of lidar observation periods will be presented and discussed in relation to high-resolution model outputs. For instance, the lidar instruments evidence the presence of complex vertical structures of the low troposphere above Reunion Island. Such structures remain even inside the island both in the corries and on the mountains. Moreover, some hydraulic character of the wind trade flow around the island could be highlighted from lidar measurements. Indeed, the Venturi acceleration on each side of the obstacle is accompanied by subsidence and a significant shallowing of the boundary layer along the flow

Monitoring of therapy for mucopolysaccharidosis Type I Using dysmorphometric facial phenotypic signatures

There is a pattern of progressive facial dysmorphology in mucopolysaccharidosis type I (MPS I). Advances in 3D facial imaging have facilitated the development of tools, including dysmorphometrics, to objectively and precisely detect these facial phenotypes. Therefore, we investigated the application of dysmorphometrics as a noninvasive therapy-monitoring tool, by longitudinally scoring facial dysmorphology in a child with MPS I receiving enzyme replacement therapy (ERT) and bone marrow transplantation (BMT). Both dysmorphometric measures showed a decreasing trend, and the greatest differences were found in the severity of facial discordance (Z-RMSE), displaying scores >3 SD higher than the mean at their peak, in comparison to Z-RSD scores that mostly fell within the normative range (maximum; 1.5 SD from the mean). In addition to the general trend of reduced facial dysmorphology with treatment, initial fluctuations were also evident that may have related to transient subcutaneous facial fluctuations, in the context of conditioning for bone marrow transplant. These findings support the potential of our approach as a sensitive, noninvasive, and rapid means of assessing treatment response or failure in clinical trials, and for established therapies, and would be applicable for other inherited disorders of metabolism