Pain in Children and Adolescents with Spinal Muscular Atrophy: A Longitudinal Study from a Patient Registry
Spinal muscular atrophy (SMA) is a devastating genetic neurodegenerative disease caused by the insufficient production of Survival Motor Neuron (SMN) protein. It presents different phenotypes with frequent contractures and dislocations, scoliosis, and pain. This study aims to report the prevalence a...
Saved in:
| Main Authors: | , , , , , , , , |
|---|---|
| Format: | Book |
| Published: |
MDPI AG,
2023-11-01T00:00:00Z.
|
| Subjects: | |
| Online Access: | Connect to this object online. |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
MARC
| LEADER | 00000 am a22000003u 4500 | ||
|---|---|---|---|
| 001 | doaj_1fa68c40e96a4d09b7aa2772b026b2e6 | ||
| 042 | |a dc | ||
| 100 | 1 | 0 | |a Inmaculada Pitarch-Castellano |e author |
| 700 | 1 | 0 | |a David Hervás |e author |
| 700 | 1 | 0 | |a Maria Grazia Cattinari |e author |
| 700 | 1 | 0 | |a Eugenia Ibáñez Albert |e author |
| 700 | 1 | 0 | |a Mercedes López Lobato |e author |
| 700 | 1 | 0 | |a Nancy Carolina Ñungo Garzón |e author |
| 700 | 1 | 0 | |a Juan Rojas |e author |
| 700 | 1 | 0 | |a Cristina Puig-Ram |e author |
| 700 | 1 | 0 | |a Marcos Madruga-Garrido |e author |
| 245 | 0 | 0 | |a Pain in Children and Adolescents with Spinal Muscular Atrophy: A Longitudinal Study from a Patient Registry |
| 260 | |b MDPI AG, |c 2023-11-01T00:00:00Z. | ||
| 500 | |a 10.3390/children10121880 | ||
| 500 | |a 2227-9067 | ||
| 520 | |a Spinal muscular atrophy (SMA) is a devastating genetic neurodegenerative disease caused by the insufficient production of Survival Motor Neuron (SMN) protein. It presents different phenotypes with frequent contractures and dislocations, scoliosis, and pain. This study aims to report the prevalence and description of pain and how it affects daily life by analyzing a new ad hoc questionnaire. An observational study of patients under 18 years of age with SMA was conducted at two referral centers in Spain. Data were analyzed using a descriptive analysis and a Bayesian ordinal regression model to assess the association with clinical and demographic variables. Fifty-one individuals were included in this study, 27% of whom reported pain with a median duration of 5.2 years and a mean Visual Analogic Scale (VAS) score of 5. Notably, 77% were receiving disease-modifying treatment, with more than 50% receiving analgesic treatment. The Bayesian model showed that functional status, lower limb contractures, and number of visits have a high probability (>90%) of influencing pain. Thus, the prevalence of pain in the SMA population under 18 years is substantial, and its presence could be associated with lower limb contractures, better functional status, and higher RULM (Revised Upper Limb Module) scores. | ||
| 546 | |a EN | ||
| 690 | |a pain | ||
| 690 | |a spinal muscular atrophy | ||
| 690 | |a neuromuscular disease | ||
| 690 | |a children | ||
| 690 | |a adolescent natural history | ||
| 690 | |a patient registry | ||
| 690 | |a Pediatrics | ||
| 690 | |a RJ1-570 | ||
| 655 | 7 | |a article |2 local | |
| 786 | 0 | |n Children, Vol 10, Iss 12, p 1880 (2023) | |
| 787 | 0 | |n https://www.mdpi.com/2227-9067/10/12/1880 | |
| 787 | 0 | |n https://doaj.org/toc/2227-9067 | |
| 856 | 4 | 1 | |u https://doaj.org/article/1fa68c40e96a4d09b7aa2772b026b2e6 |z Connect to this object online. |