Version 1
: Received: 5 September 2024 / Approved: 6 September 2024 / Online: 6 September 2024 (13:18:36 CEST)
How to cite:
Ginanneschi, F.; Bigliazzi, C.; Cimmino, F. A.; Casali, S.; Pelliccioni, P.; Emmanuello, E.; Bargagli, E.; De Stefano, N. Respiratory Trajectories and Correlation with Serum Biochemical Indices in Spinal and Bulbar Muscular Atrophy. Preprints2024, 2024090523. https://doi.org/10.20944/preprints202409.0523.v1
Ginanneschi, F.; Bigliazzi, C.; Cimmino, F. A.; Casali, S.; Pelliccioni, P.; Emmanuello, E.; Bargagli, E.; De Stefano, N. Respiratory Trajectories and Correlation with Serum Biochemical Indices in Spinal and Bulbar Muscular Atrophy. Preprints 2024, 2024090523. https://doi.org/10.20944/preprints202409.0523.v1
Ginanneschi, F.; Bigliazzi, C.; Cimmino, F. A.; Casali, S.; Pelliccioni, P.; Emmanuello, E.; Bargagli, E.; De Stefano, N. Respiratory Trajectories and Correlation with Serum Biochemical Indices in Spinal and Bulbar Muscular Atrophy. Preprints2024, 2024090523. https://doi.org/10.20944/preprints202409.0523.v1
APA Style
Ginanneschi, F., Bigliazzi, C., Cimmino, F. A., Casali, S., Pelliccioni, P., Emmanuello, E., Bargagli, E., & De Stefano, N. (2024). Respiratory Trajectories and Correlation with Serum Biochemical Indices in Spinal and Bulbar Muscular Atrophy. Preprints. https://doi.org/10.20944/preprints202409.0523.v1
Chicago/Turabian Style
Ginanneschi, F., Elena Bargagli and Nicola De Stefano. 2024 "Respiratory Trajectories and Correlation with Serum Biochemical Indices in Spinal and Bulbar Muscular Atrophy" Preprints. https://doi.org/10.20944/preprints202409.0523.v1
Abstract
Background/Objectives: The primary life-threatening complication in Spinal-bulbar muscular atrophy (SBMA) is ventilatory failure, which results from the weakening of the bulbar and respiratory muscles. However, systematic studies assessing respiratory function over the long-term have not been performed. The present study analyzes the longitudinal patterns of respiratory function tests over a follow-up of 11 years, aiming to identify any potential, sustained decline during this time. Furthermore, we explored possible correlations between respiratory function tests and clinical and biochemical serum indices.
Methods: We collected data from 9 genetically confirmed SBMA patients and conducted spirometric measurements twice a year for eleven years. The spirometric data included maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), and forced vital capacity (FVC)]. Additionally, we measured serum biochemical indices and SBMA functional rating scale (SBMAFRS) for clinical analysis.
Results: All three respiratory function tests showed a descending trend. A major decrease was observed for MEP (52%) and MIP (42%), while this was minor for FVC (25%). SBMAFRS score correlated with the three pulmonary function tests. MIP and MEP correlated with creatine phosphokinase and creatinine levels, and FVC correlated with serum level of transaminases.
Conclusions: This longitudinal study shows a progressive decline of spirometric data throughout life in patients with SBMA. Spirometric measures relative to maximal strength of the respiratory muscles (MIP and MEP) may have a better predictive value for pulmonary and muscular decline than FVC.
Keywords
blood analysis; Kennedy’s disease; spirometry
Subject
Medicine and Pharmacology, Neuroscience and Neurology
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
