Spontaneous breathing pattern as respiratory functional outcome in children with spinal muscular atrophy (SMA)

Antonella LoMauro, Andrea Aliverti, Chiara Mastella, Maria Teresa Arnoldi, Paolo Banfi, Giovanni Baranello

Research output: Contribution to journalArticle

Abstract

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 thoracoabdominal 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.

Original languageEnglish
Article numbere0165818
JournalPLoS One
Volume11
Issue number11
DOIs
Publication statusPublished - Nov 1 2016

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Spinal Muscular Atrophy
muscular atrophy
Spinal Muscular Atrophies of Childhood
breathing
Respiration
Muscle Weakness
Ventilation
Tachypnea
Muscle
Respiratory Muscles
Plethysmography
Albuterol
Supine Position
Spirometry
Respiratory Rate
Posture
natural history
Cough
muscles
Reference Values

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

Cite this

Spontaneous breathing pattern as respiratory functional outcome in children with spinal muscular atrophy (SMA). / LoMauro, Antonella; Aliverti, Andrea; Mastella, Chiara; Arnoldi, Maria Teresa; Banfi, Paolo; Baranello, Giovanni.

In: PLoS One, Vol. 11, No. 11, e0165818, 01.11.2016.

Research output: Contribution to journalArticle

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abstract = "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 thoracoabdominal 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.",
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