Next Article in Journal
A Possible Role of Remdesivir and Plasma Therapy in the Selective Sweep and Emergence of New SARS-CoV-2 Variants
Next Article in Special Issue
Attention Deficits and ADHD Symptoms in Adults with Fabry Disease—A Pilot Investigation
Previous Article in Journal
Compressed Sensing Real-Time Cine Reduces CMR Arrhythmia-Related Artifacts
Previous Article in Special Issue
Management of Corneal Clouding in Patients with Mucopolysaccharidosis
Review

Airway Abnormalities in Adult Mucopolysaccharidosis and Development of Salford Mucopolysaccharidosis Airway Score

1
Ear Nose and Throat Department, Salford Royal NHS Foundation Trust, Manchester M6 8HD, UK
2
Adult Inherited Metabolic Department, Salford Royal NHS Foundation Trust, Manchester M6 8HD, UK
3
Anaesthetics Department, Salford Royal NHS Foundation Trust, Manchester M6 8HD, UK
4
Intensive Care & Respiratory Medicine, Manchester University NHS Foundation Trust, Wythenshawe Hospital, Manchester M23 9LT, UK
*
Author to whom correspondence should be addressed.
Academic Editor: Sukhwinder Singh Sohal
J. Clin. Med. 2021, 10(15), 3275; https://doi.org/10.3390/jcm10153275
Received: 30 June 2021 / Revised: 15 July 2021 / Accepted: 21 July 2021 / Published: 24 July 2021
(1) Background: Mucopolysaccharidoses (MPS) are a heterogeneous group of lysosomal storage disorders caused by the absence of enzymes required for degradation of glycosaminoglycans (GAGs). GAGs deposition in tissues leads to progressive airway narrowing and/or tortuosity. Increased longevity of patients has posed newer problems, especially the airway. This study aims to characterise various airway abnormalities in adult MPS from a regional centre and proposes a method to quantify the severity of the airway disease. (2) Methods: Retrospective analysis by case notes review, clinical examination, endoscopy, cross-sectional imaging, 3-dimensional reconstruction, and physiological investigations were used to assess the airway abnormalities. Quantitative assessment of the airway severity was performed a validated questionnaire of 15 parameters to derive Salford Mucopolysaccharidosis Airway Score (SMAS). (3) Results: Thirty-one adult MPS patients (21M/ 9F; median 26.7 years; range 19–42 years) were reviewed. There were 9 MPS I, 12 MPS II, 2 MPS III, 5 MPS IV, 2 MPS VI, and 1 MPS VII. Airway abnormalities in each MPS type are described. Patients scoring more than 35 on SMAS had some form of airway intervention. The area under curve of 0.9 was noted at a score of 25, so SMAS more than 25 may predict a difficult airway and potential to have complications. Pearson’s correlation between SMAS and height, weight, BMI were poor (p < 0.05). (4) Conclusions: Airway abnormalities in adult MPS are varied and complex. Assessment of the airway should be holistic and include multiple parameters. An objective multidimensional score such as SMAS may help to predict and manage difficult airways warranting further investigation and validation. View Full-Text
Keywords: mucopolysaccharidoses; airway; obstruction; management mucopolysaccharidoses; airway; obstruction; management
Show Figures

Figure 1

MDPI and ACS Style

Gadepalli, C.; Stepien, K.M.; Sharma, R.; Jovanovic, A.; Tol, G.; Bentley, A. Airway Abnormalities in Adult Mucopolysaccharidosis and Development of Salford Mucopolysaccharidosis Airway Score. J. Clin. Med. 2021, 10, 3275. https://doi.org/10.3390/jcm10153275

AMA Style

Gadepalli C, Stepien KM, Sharma R, Jovanovic A, Tol G, Bentley A. Airway Abnormalities in Adult Mucopolysaccharidosis and Development of Salford Mucopolysaccharidosis Airway Score. Journal of Clinical Medicine. 2021; 10(15):3275. https://doi.org/10.3390/jcm10153275

Chicago/Turabian Style

Gadepalli, Chaitanya, Karolina M. Stepien, Reena Sharma, Ana Jovanovic, Govind Tol, and Andrew Bentley. 2021. "Airway Abnormalities in Adult Mucopolysaccharidosis and Development of Salford Mucopolysaccharidosis Airway Score" Journal of Clinical Medicine 10, no. 15: 3275. https://doi.org/10.3390/jcm10153275

Find Other Styles
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Back to TopTop