ضعف تصفية مجرى الهواء في رنح فريدريك (ذروة تدفق السعال الزفيري < 270 لتر/دقيقة أو السعة الحيوية القسرية < 50% من المتوقع)

References

DOI: 10.1186/s13023-022-02568-3

In individuals with Friedreich ataxia and impaired airway clearance (PECF < 270 L/min or FVC < 50% predicted), we suggest assisted coughing (mechanical/manual) be implemented to assist in airway clearance and reduce the prevalence of chest infections.

We conditionally recommend non-invasive assisted ventilation for patients with Friedreich ataxia and documented restrictive lung disease meeting the following thresholds: FVC < 50% predicted; maximum inspiratory pressure < 60 cm H₂O; nocturnal hypercarbia (pCO₂ > 50 mm Hg for ≥ 2% of sleep time or a 10 mm Hg increase in pCO₂ compared to awake baseline pCO₂ for ≥ 2% of sleep time); nocturnal hypoxia (SpO₂ ≤ 88% for ≥ 2% of sleep time or 5 min continuously); or apnea–hypopnea index ≥ 5.