Rib Cage Deformities Alter Respiratory Muscle Action and Chest Wall Function in Patients with Severe Osteogenesis Imperfecta

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder characterized by bone fragility, multiple fractures and significant chest wall deformities. Cardiopulmonary insufficiency is the leading cause of death in these patients.Seven patients with severe OI type III, 15 with moderate OI type IV and 26 healthy subjects were studied. In addition to standard spirometry, rib cage geometry, breathing pattern and regional chest wall volume changes at rest in seated and supine position were assessed by opto-electronic plethysmography to investigate if structural modifications of the rib cage in OI have consequences on ventilatory pattern. One-way or two-way analysis of variance was performed to compare the results between the three groups and the two postures. compared to predicted values, on condition that updated reference equations are considered. In both positions, ventilation was lower in OI patients than control because of lower tidal volume (p<0.01). In contrast to OI type IV patients, whose chest wall geometry and function was normal, OI type III patients were characterized by reduced (p<0.01) angle at the sternum (pectus carinatum), paradoxical inspiratory inward motion of the pulmonary rib cage, significant thoraco-abdominal asynchronies and rib cage distortions in supine position (p<0.001).In conclusion, the restrictive respiratory pattern of Osteogenesis Imperfecta is closely related to the severity of the disease and to the sternal deformities. Pectus carinatum characterizes OI type III patients and alters respiratory muscles coordination, leading to chest wall and rib cage distortions and an inefficient ventilator pattern. OI type IV is characterized by lower alterations in the respiratory function. These findings suggest that functional assessment and treatment of OI should be differentiated in these two forms of the disease

Dietary exposure estimates of twenty-one trace elements from a Total Diet Study carried out in Pavia, northern Italy

The significant role of trace elements in human health is well documented. Trace elements are those compounds that need to be present in the human diet to maintain normal physiological functions. However, some microelements may become harmful at high levels of exposure, or, on the other hand, may give rise to malnutrition, when their exposure is too low. The aim of the present study was to provide a reliable estimate of the dietary exposure of twenty-one trace elements in a Northern Italian area. For this purpose, trace element analyses were undertaken on total diet samples collected from a university cafeteria in Pavia, Northern Italy. The average daily exposure for the adult people was calculated on the basis of food consumption frequency, portion size and trace element levels in foodstuffs. The mean exposure values satisfy the Italian RDA for all the essential trace elements, except for Fe exposure in females, and are well below the Provisional Tolerable Daily Intake for all the toxic compounds, showing that the probability of dietary exposure to health risks is overall small. As far as Fe exposure is concerned, a potential risk of anaemia in the female adult population should be considered, then studies aimed at evaluating the Fe nutritional status of adult Italian women should be addressed. In conclusion, while not excluding the possibility that the daily exposure determined in the present study may not be representative of the population as a whole, this study provides a good estimate of the Italian adult consumer exposure to twenty-one trace elements

Kinematic analysis of upper limb during walking in diplegic children with Cerebral Palsy

Movements of the lower limbs during walking have been widely investigated in literature, while quantification of arm movement during gait is scanty. The aim of the present study was to assess quantitatively the upper limb motion during gait in children with Cerebral Palsy (CP). Sixteen children with diplegic CP were evaluated using a full-body marker set, which allows assessing both the lower and upper limb kinematics. Our results demonstrated that movement of the arms was characterized by an abducted shoulder and a more flexed elbow position at the initial contact of the gait cycle with a quite physiological range of motion if compared to controls. These data showed that gait of children with diplegic CP is generally characterized by abnormal upper limb position which could be considered a strategy to keep balance and posture control during walking

Could an Immersive Virtual Reality Training Improve Navigation Skills in Children with Cerebral Palsy? A Pilot Controlled Study

Children with cerebral palsy (CP) suffer deficits in their motor, sensory, and cognitive abilities, as well as in their visuospatial competences. In the last years, several authors have tried to correlate the visuospatial abilities with the navigational ones. Given their importance in everyday functions, navigation skills have been deeply studied using increasingly cutting-edge techniques such as virtual reality (VR). However, to our knowledge, there are no studies focused on training using immersive VR (IVR) in children with movement disorders. For this reason, we proposed an IVR training to 35 young participants with CP and conceived to improve their navigation skills in a &ldquo;simil-real&rdquo; environment while playing on a dynamic platform. A subgroup performed a part of the training which was specifically dedicated to the use of the allocentric strategy (i.e., looking for landmarks) to navigate the virtual environment. We then compared the children&rsquo;s navigation and spatial skills pre- and post-intervention. All the children improved their visual&ndash;spatial abilities; particularly, if the IVR activities specifically trained their ability to look for landmarks and use them to navigate. The results of this work highlight the potential of an IVR training program to increase the navigation abilities of patients with CPs

Immersive Virtual Reality to Improve Walking Abilities in Cerebral Palsy: A Pilot Study

Immersive virtual reality (IVR) offers new possibilities to perform treatments in an ecological and interactive environment with multimodal online feedbacks. Sixteen school-aged children (mean age 11 ± 2.4 years) with Bilateral CP—diplegia, attending mainstream schools were recruited for a pilot study in a pre–post treatment experimental design. The intervention was focused on walking competences and endurance and performed by the Gait Real-time Analysis Interactive Lab (GRAIL), an innovative treadmill platform based on IVR. The participants underwent eighteen therapy sessions in 4 weeks. Functional evaluations, instrumental measures including GAIT analysis and parental questionnaire were utilized to assess the treatment effects. Walking pattern (stride length left and right side, respectively p = 0.001 and 0.003; walking speed p = 0.001), endurance (6MWT, p = 0.026), gross motor abilities (GMFM-88, p = 0.041) and most kinematic and kinetic parameters significantly improved after the intervention. The changes were mainly predicted by age and cognitive abilities. The effect could have been due to the possibility of IVR to foster integration of motor/perceptual competences beyond the training of the walking ability, giving a chance of improvement also to older and already treated children

Efficacy of Robotic-Assisted Gait Training Compared with Intensive Task-Oriented Physiotherapy for Children with Cerebral Palsy

Abstract -The purpose of this study was to evaluate if adding paediatric robotic-assisted gait training (RAGT) to taskoriented physiotherapy (TOP) in children with Cerebral Palsy (CP) could improve gross motor abilities and gait compared to intensive TOP. Nineteen ambulatory children with Bilateral Spastic CP were assigned to two 10-week training groups. 9 children had 20 sessions of RAGT and 20 sessions of TOP, the other 10 children had 40 sessions only of TOP. The Gross Motor Function Measure, 6-Minute Walk Test and 3D Gait Analysis were assessed prior to, at the end of, and 3 months after the end of the treatment. After the training and during the follow up, both groups improved their Gross Motor Function Measure scores, maintained their gait pattern and had unchanged 6-Minute Walk Test results. No between group differences were found in any outcome measures. In conclusion, compared to intensive TOP alone, the addition of RAGT to TOP was demonstrated to be equally effective at improving gross motor abilities and maintaining gait pattern over time. The robotic rehabilitation allows care-providers to administer a standardized, controlled, dosed therapy, and DSSHDUV WR LQFUHDVH SDWLHQW ¶V PRWLYDWLR

Are patients with hereditary spastic paraplegia different from patients with spastic diplegia during walking? Gait evaluation using 3D gait analysis

Patients with hereditary spastic paraplegia (HSP) often resemble patients with mild spastic diplegia (SD), although their motor limitations differ. The aim of this study was to analyse quantitatively the gait of HSP and SD subjects in order to define the gait pattern in HSP and the differences between the two conditions. Fifteen subjects with HSP, 40 patients with SD and 20 healthy subjects underwent gait analysis (GA). The spatio-temporal and kinematic parameters at the proximal joints were found to be similar in HSP and SD, whereas the most significant differences were found at the knee and ankle joints. Both groups displayed a tendency for knee hyperextension in the midstance phase, but the duration of this hyperextension was longer in the HSP patients. This study shows that GA complements traditional clinical evaluations, making it possible to distinguish, clearly, between motor ability in HSP and in SD patients; the duration of the knee hyperextension during midstance was found to discriminate between the two gait pattern