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Peak Cough Flow in Children with Neuromuscular Disorders

  • RESPIRATORY MUSCLE PHYSIOLOGY
  • Published:
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Abstract

Purpose

Patients with neuromuscular disease (NMD) experience weakened cough due to progressive respiratory muscle weakness. Peak cough flow (PCF) measurements derived from adult populations are used to recommend initiation of assisted cough therapies. The objective of this study was to characterize PCF values among pediatric patients with NMD.

Methods

Retrospective chart review was performed for patients seen in the multidisciplinary pediatric muscular dystrophy clinic from 2010 to 2016. Clinical and demographic variables included age, gender, ambulation status, and PCF measurements.

Results

366 patients with an established diagnosis of NMD (median age 11.8 years) were included in this study. 102 (27.8%) out of the 366 patients were affected by Duchenne muscular dystrophy (DMD), 42 (11.5%) by congenital muscular dystrophy (CMD), 42 (11.5%) by Charcot Marie Tooth disease (CMT) and 24 (6.5%) by Becker’s muscular dystrophy (BMD). The mean PCF values in DMD (255.8 L/min) and CMD (249.1 L/min) were lower than CMT (321.5 L/min) with p-values of 0.007 and 0.02, respectively. The mean PCF of BMD (333.3 L/min) was higher than that of DMD and CMD but the difference was not statistically significant. PCFs were not statistically different between ambulatory and non-ambulatory status (263.0 L/min versus 290.8 L/min, p = 0.12). Children under 10 years of age had lower PCF relative to older subjects (179.5 L/min versus 300.9 L/min, p < 0.0001).

Conclusion

Baseline PCF values in young children are below the adult-specific values suggested for starting assisted cough techniques. Further longitudinal trials are required to derive pediatric-specific reference values for PCF in patients with NMD.

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References

  1. Deenen JC, Horlings CG, Verschuuren JJ, Verbeek AL, van Engelen BG (2015) The epidemiology of neuromuscular disorders: a comprehensive overview of literature. J Neuromuscul Dis 2(1):73–85

    Article  Google Scholar 

  2. Dowling JJ, Gonorazky HD, Cohn RD, Campbell C (2018) Treating pediatric neuromuscular disorders: the future is now. Am J Med Genet A 176(4):804–841

    Article  Google Scholar 

  3. Perrin C, Unterborn JN, Ambrosio CD, Hill NS (2004) Pulmonary complications of chronic neuromuscular diseases. Muscle Nerve 29(1):5–27

    Article  Google Scholar 

  4. Panitch HB (2017) Respiratory implications of pediatric neuromuscular disease. Respir Care 62(6):826–848

    Article  Google Scholar 

  5. Tzeng AC, Bach JR (2000) Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest 118(5):1390–1396

    Article  CAS  Google Scholar 

  6. Leiner GC, Abramowitz S, Small MJ, Stenby VB (1966) Cough peak flow rate. Am J Med Sci 251(2):211–214

    Article  CAS  Google Scholar 

  7. Smina M, Salam A, Khamiees M, Gada P, Amoateng-Adjepong Y, Manthous Constantine A (2003) Cough peak flow and extubation outcomes. Chest 124(1):262–268

    Article  Google Scholar 

  8. Gregg I, Nunn AJ (1973) Peak expiratory flow in normal subjects. Br Med J 3(5874):282–284

    Article  CAS  Google Scholar 

  9. Leiner GC, Abramowitz S, Small MJ, Stenby VB, Lewis WA (1963) Expiratory peak flow rate, standard value for normal subjects use as a clinical test of ventilator function. Am Rev Respir Dis 88:644–651

    CAS  PubMed  Google Scholar 

  10. Birnkrant DJ, Bushby K, Bann CM, Alman BA, Apkon SD, Blackwell A, Case LE, Cripe L, Hadjiyannakis S, Olson AK, Sheehan DW, Bolen J, Weber DR, Ward LM, DMD Care Considerations Working Group (2018) Diagnosis and management of Duchenne muscular dystrophy. Part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol 17(4):347–361

    Article  Google Scholar 

  11. Bach JR, Ishikawa Y, Kim H (1997) Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 112(4):1024–1028

    Article  CAS  Google Scholar 

  12. Voulgaris A, Antoniadou M, Agrafiotis M, Steiropoulos P (2019) Respiratory involvement in patients with neuromuscular diseases: a narrative review. Pulm Med 2019:2734054

    Article  Google Scholar 

  13. Tzani P, Chiesa S, Aiello M, Scarascia A, Catellani C, Elia D, Marangio E, Chetta A (2014) The value of cough peak flow in the assessment of cough efficacy in neuromuscular patients a cross sectional study. Eur J Phys Rehabil Med 50(4):427–432

    CAS  PubMed  Google Scholar 

  14. Beardsmore CS, Wimpress SP, Thomson AH, Patel HR, Goodenough P, Simpson H (1987) Maximum voluntary cough: an indication of airway function. Bull Eur Physiopathol Respir 23(5):465–472

    CAS  PubMed  Google Scholar 

  15. Beardsmore CS, Park A, Wimpress SP, Thomson AH, Simpson H (1989) Cough flow-volume relationships in normal and asthmatic children. Pediatr Pulmonol 6(4):223–231

    Article  CAS  Google Scholar 

  16. Bianchi C, Baiardi P (2008) Cough peak flows: standard values in children and adolescents. Am J Phys Med Rehabil 87(6):461–467

    Article  Google Scholar 

  17. Leith D (1985) The development of cough. Am Rev Respir Dis 131(5):S39–S42

    CAS  PubMed  Google Scholar 

  18. Henricson EK, Abresch RT, Cnaan A et al (2013) The Cooperative International Neuromuscular Research Group Duchenne Natural History Study: glucocorticoid treatment preserves clinically meaningful functional milestones and reduces rate of disease progression as measured by manual muscle testing and other commonly used clinical trial outcome measures. Muscle Nerve 48:55–67

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Pulmonology and Allergy, Department of Pediatrics, University of Maryland, Maryland, 737 W Lombard St, Ste 314, Baltimore, MD, 21201, USA

    Nidhi Kotwal

  2. Division of Pulmonary and Sleep Medicine, Children’s National Hospital, 111 Michigan Ave, NW, Washington, DC, 20010, USA

    Nidhi Kotwal & Geovanny F. Perez

  3. Department of Pediatrics, MedStar Georgetown University Hospital, 3800 Reservoir Rd, NW, Washington, DC, 20007, USA

    Prateek J. Shukla

  4. Department of Pediatrics, Oishei Children’s Hospital, Jacobs School of Medicine and Biomedical Sciences, University of Buffalo, New York, NY, 14203, USA

    Geovanny F. Perez

Authors
  1. Nidhi Kotwal
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  2. Prateek J. Shukla
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  3. Geovanny F. Perez
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Correspondence to Nidhi Kotwal.

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Kotwal, N., Shukla, P.J. & Perez, G.F. Peak Cough Flow in Children with Neuromuscular Disorders. Lung 198, 371–375 (2020). https://doi.org/10.1007/s00408-020-00340-7

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  • DOI: https://doi.org/10.1007/s00408-020-00340-7

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