Relationship between respiratory function and need for NIV in childhood SMA.

ABSTRACT:

Background

Spinal muscular atrophy (SMA) causes progressive respiratory muscle weakness but respiratory function (RF) in those using noninvasive ventilation (NIV) is not well described.

Objective

To describe RF in childhood SMA and assess differences between those using and not using NIV.

Methods

A cross-sectional study of childhood SMA assessed polysomnography (PSG), spirometry, forced oscillation technique (FOT), lung clearance index (LCI), sniff nasal inspiratory pressures, peak cough flow, maximal inspiratory and expiratory pressure, and NIV use and indication.

Results

Twenty-five children (median age [interquartile range], 8.96 [5.63] years; 10 F) with SMA 1 (n = 3), 2 (n = 15), and 3 (n = 7) were recruited. Spirometry and FOT testing was feasible in child

SUBMITTER: Kapur N 

PROVIDER: S-EPMC6852082 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature