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1.
PLoS One ; 11(11): e0165818, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27820869

RESUMO

INTRODUCTION: SMA is characterised by progressive motor and respiratory muscle weakness. We aimed to verify if in SMA children 1)each form is characterized by specific ventilatory and thoraco-abdominal pattern(VTAp) during quiet breathing(QB); 2)VTAp is affected by salbutamol therapy, currently suggested as standard treatment, or by the natural history(NH) of SMA; 3)the severity of global motor impairment linearly correlates with VTAp. MATERIALS AND METHODS: VTAp was analysed on 32 SMA type I (SMA1,the most severe form), 51 type II (SMA2,the moderate), 8 type III (SMA3,the mildest) and 20 healthy (HC) using opto-electronic plethysmography. Spirometry, cough and motor function were measured in a subgroup of patients. RESULTS: In SMA1, a normal ventilation is obtained in supine position by rapid and shallow breathing with paradoxical ribcage motion. In SMA2, ventilation is within a normal range in seated position due to an increased respiratory rate(p<0.05) with reduced tidal volume(p<0.05) secondary to a poor contribution of pulmonary ribcage(%ΔVRC,P, p<0.001). Salbutamol therapy had no effect on VTAp during QB(p>0.05) while tachypnea occurred in type I NH. A linear correlation(p<0.001) was found between motor function scales and VTAp. CONCLUSION: A negative or reduced %ΔVRC,P, indicative of ribcage muscle weakness, is a distinctive feature of SMA1 and SMA2 since infancy. Its quantitative assessment represents a non-invasive, non-volitional index that can be obtained in all children, even uncollaborative, and provides useful information on the action of ribcage muscles that are known to be affected by the disease.Low values of motor function scales indicate impairment of motor but also of respiratory function.


Assuntos
Atrofia Muscular Espinal/fisiopatologia , Mecânica Respiratória/fisiologia , Músculos Respiratórios/fisiopatologia , Criança , Pré-Escolar , Tosse/fisiopatologia , Estudos Transversais , Feminino , Humanos , Lactente , Pulmão/fisiopatologia , Masculino , Pletismografia/métodos , Estudos Prospectivos , Respiração , Espirometria/métodos , Decúbito Dorsal/fisiologia , Parede Torácica/fisiopatologia , Volume de Ventilação Pulmonar/fisiologia
2.
Minerva Pediatr ; 62(2): 147-51, 2010 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-20440234

RESUMO

AIM: The aim of this study was to determine the role of parents after extubation of their children affected by Spinal Muscular Atrophy Type 1 (SMA1) in the Pediatric Intensive Care Unit. Currently, children affected by SMA1 are often treated with non-invasive mechanical ventilation and mechanical support of cough. During the first two or three years of life they frequently present severe respiratory failure requiring intubation. Extubation may be at severe risk of failure even because of inadequate care. METHODS: Parents of SMA1 children were offered an early education on the most critical aspects and a training in non-invasive respiratory support after diagnosis. They were asked and allowed to stay as longer as possible with their child after extubation. Quality and quantity of care given by parents during the first 24 hours after extubation were recorded. RESULTS: All parents participated to the success of the weaning procedure: they gave continuous care and all children could be extubated. CONCLUSION: The presence of parents after extubation of SMA1 patients is important for the success of the procedure; otherwise, the presence of a skilled nurse is needed, with a nurse-patient ratio of 1:1.


Assuntos
Pais , Atrofias Musculares Espinais da Infância/terapia , Desmame do Respirador , Feminino , Humanos , Lactente , Unidades de Terapia Intensiva , Masculino
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