Data in support of the longitudinal characterization of pulmonary function in children with Mucopolysaccharidoses IVA
| Autor: | Iain A. Bruce, Gabrielle Thompson, Simon Jones, Stuart Wilkinson, Johnny J. Kenth |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Spirometry
Pediatrics medicine.medical_specialty Morquio syndrome MPS Respiratory changes lcsh:Computer applications to medicine. Medical informatics Pulmonary function testing 03 medical and health sciences FEV1/FVC ratio 0302 clinical medicine Lysosomal storage disease medicine Mucopolysaccharidosis IVA Sleep-disordered breathing lcsh:Science (General) 030304 developmental biology Cause of death 0303 health sciences Multidisciplinary medicine.diagnostic_test business.industry Enzyme replacement therapy Medicine and Dentistry medicine.disease Respiratory failure lcsh:R858-859.7 sense organs business 030217 neurology & neurosurgery lcsh:Q1-390 |
| Zdroj: | Data in Brief, Vol 27, Iss, Pp-(2019) Data in Brief |
| ISSN: | 2352-3409 |
| Popis: | Mucopolysaccharidoses type IVA (Morquio disease) is a rare, autosomal recessive lysosomal storage disease that causes both obstructive and restrictive airway pathology, with respiratory failure being the primary cause of death. This article provides original data on the longitudinal characterization of pulmonary function changes in children with Mucopolysaccharidoses (MPS) IVA by presenting the data and nuanced trends of changes from sequential spirometry and oximetry. The sample size included 16 subjects, 13 had undergone enzyme replacement therapy (ERT), three had not undergone ERT treatment. A total of 180 individual plots are presented for spirometry variables (FEV1, FEV1 [%Pred] FVC, FVC [%Pred] and FEV1/FVC), 6MWT and oximetry variables (median %Spo2, ODI 3%, mean nadir 3%, ODI 4%, mean nadir 4% and min dip SpO2 [%]); over a nine-year period at a single quaternary paediatric metabolic centre. This data has been made public and has utility to clinicians and researchers due to the following: [1,2] by providing the first comprehensive report of detailed changes in pulmonary function in children with MPS IVA, with and without ERT; [1–3] as well as changes in pulmonary function following the institution of non-invasive ventilation (NIV) and adenotonsillectomy. The data presented is related to the research article by Kenth et al. “The Characterization of Pulmonary Function in Patients with Mucopolysaccharidoses IVA: A Longitudinal Analysis”. Keywords: Morquio syndrome, Mucopolysaccharidosis IVA, MPS, Respiratory changes, Enzyme replacement therapy, Sleep-disordered breathing |
| Databáze: | OpenAIRE |
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