TY - JOUR T1 - The effectiveness of AMBU-bag to recruit ribcage volume in spinal muscle atrophy JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2016.PA1612 VL - 48 IS - suppl 60 SP - PA1612 AU - Antonella Lo Mauro AU - Chiara Mastella AU - Giovanni Baranello AU - Andrea Aliverti Y1 - 2016/09/01 UR - http://erj.ersjournals.com/content/48/suppl_60/PA1612.abstract N2 - Spinal Muscle Atrophy(SMA) is an autosomal recessive disease characterized by degeneration of motor neurons of spinal cord resulting in locomotor and respiratory muscles wasting.The diaphragm is preserved whereas intercostal muscles are progressively impaired leading tobbell-shaped chest,poor airway clearance,reduced cough efficacy,alveolar hypoventilation and micro-atelectasis.The respiratory care management of SMA children includes, among others, daily sessions of AMBU bag to recruit the lung, stabilize gas exchange, improve cough and airways clearance efficiency and reverse microatelectasis.Since there's no published evidence on the effects of AMBU on the respiratory function in SMA children, we used opto-electronic plethysmography to measure the operational thoraco-abdominal volumes on 9 SMA children (4.9±3.3 years;4 affected by the most severe type I and 5 by the moderate type II) during quiet breathing before and after ten AMBU inflations(figure).While end-expiratory (EE) chest wall and abdominal volumes did not change significantly (the former increases by 8.9±51.9ml,the latter decreases by 11.0±34.3ml),EE ribcage volumes inflates by 20.1±23.7ml(p=0.034).AMBU bag is therefore useful in increasing EE ribcage volume in SMA.Because the inflation was localized in the ribcage,it can be speculated that was a consequence of lung recruitment and for this reason AMBU should be recommended in the daily management of SMA children. ER -