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OBJECTIVE Our study investigated the prevalence of retinopathy and maculopathy in sickle cell patients and tested the association between these two conditions. In addition, we tested whether hematological and hemorheological parameters, as well as genotype, were involved in the development of these two conditions. METHODS Seventy sickle cell adult patients were recruited: 37 with sickle cell anemia (SCA) and 33 with sickle cell hemoglobin C disease (SCC). All patients underwent retinal examination and macular ocular coherence tomography. Blood was sampled for the measurements of hematological and hemorheological parameters. RESULTS Twenty-six patients had maculopathy and 30 had retinopathy with no significant difference between SCA and SCC patients. No association between the presence of retinopathy and maculopathy was detected. RBC aggregation was higher and RBC deformability lower at 3 Pa in SCA patients. Blood viscosity and hematocrit were higher in SCC than in SCA patients. However, no association was found between biological parameters and the ocular complications studied. CONCLUSIONS Our study showed that retinopathy and maculopathy are common in sickle cell disease. Nevertheless, we found no association with hematological parameters, blood rheology or genotype.

34e Congrès de la Société de Biomécanique, TOULON, FRANCE, 31-/08/2009 - 02/09/2009; Le centre Médical Pédiatrique de la Maisonnée accueille des enfants présentant une pathologie neuromusculaire ventilés sur trachéotomie ou par les voies naturelles. La fonction respiratoire de ces enfants se détériore naturellement au fil des années et des programmes de développement du « souffle », associé ou non à des pressions thoraciques sont mis en place pour minimiser cette aggravation essentiellement restrictive. L'objectif de ce travail est d'évaluer ces programmes en quantifiant en particulier la mobilité thoracique qu'ils génèrent

12th International Conference on Home Mechanical Ventilation, BARCELONE, ESPAGNE, 27-/03/2009 - 28/03/2009; The CMP (Pediatric Medical Center) in la Maisonnée receives children with neuromuscular pathologies ventilated with invasive or non-invasive methods. The respiratory function of these children naturally deteriorates over time, and breathing development programs, that may or may not be associated with thoracic pressure, are put in place to minimize this mostly restrictive aggravation. The traditional use of IPPB lacks precise evaluation, especially of thoracic mobility. In collaboration with INRETS (the French National Institute for Transport and Safety Research), a measurement table, used in association with two digital video cameras, has been designed to record both pressure and thoracic movements. Seven children were recorded using two or three ventilation modes : - via spontaneous ventilation if the child is breathing unassisted - via mechanical ventilation - via IPPB. The targets were placed on the sternum, the abdomen, the xiphoid process and the ribs. All these children suffer from respiratory failure of neuromuscular origin. Their average age is seven (14 months to 16 years old). Initial results highlight a much higher thoracic compliance under IPPB in all cases. IPPB versus spontaneous ventilation : + 64,8% (the highest result is registred on costal targets +65,4%) IPPB versus barometric or volumetric ventilation : +54,1% An abdominal contention increase thoracic movement but decrease abdominal movement Conclusion This new tool enables the precise measurement of children's thoracic compliance, thereby making it easier to appreciate whether there is a need to implement hyperinsufflation programs that will limit the restriction of the respiratory function and thoracic stiffening in these children. Other measurements need to be carried out to confirm these initial results.