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DOI 10.1016/j.nmd.2024.05.007

Медицинская библиотека · Neuromuscular disorders : NMD · 2024

Disease-associated comorbidities, medication records and anthropometric measures in adults with Duchenne muscular dystrophy.

Schiava Marianela, Lofra Robert Muni, Bourke John P, James Meredith K, Díaz-Manera Jordi, Elseed Maha A, Michel-Sodhi Jassi, Moat Dionne, Mccallum Michelle, Mayhew Anna, Ghimenton Elisabetta, Díaz Carla Florencia Bolaño, Malinova Monika, Wong Karen, Richardson Mark, Tasca Giorgio, Grover Emma, Robinson Emma-Jayne, Tanner Stephanie, Eglon Gail, Behar Laura, Eagle Michelle, Turner Catherine, Verdú-Díaz José, Heslop Emma, Straub Volker, Bettolo Chiara Marini, Guglieri Michela

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Аннотация

We investigated the comorbidities, associated factors, and the relationship between anthropometric measures and respiratory function and functional abilities in adults with Duchenne muscular dystrophy (DMD). This was a single-centre cross-sectional study in genetically diagnosed adults with DMD (>16 years old). Univariate and multivariate analyses identified factors associated with dysphagia, constipation, Body Mass Index (BMI), and weight. Regression analysis explored associations between BMI, weight, and respiratory/motor abilities. We included 112 individuals (23.4 ± 5.2 years old), glucocorticoid-treated 66.1 %. The comorbidities frequency was 61.6 % scoliosis (61.0 % of them had spinal surgery), 36.6 % dysphagia, 36.6 % constipation, and 27.8 % urinary conditions. The use of glucocorticoids delayed the time to spinal surgery. The univariate analysis revealed associations between dysphagia and constipation with age, lack of glucocorticoid treatment, and lower respiratory and motor function. In the multivariate analysis, impaired cough ability remained as the factor consistently linked to both conditions. Constipation associated with lower BMI and weight. BMI and weight positively correlated with respiratory parameters, but they did not associate with functional abilities. Glucocorticoids reduce the frequency of comorbidities in adults with DMD. The ability to cough can help identifying dysphagia and constipation. Lower BMI and weight in individuals with DMD with compromised respiratory function may suggest a higher calories requirement.

Для цитирования: Schiava Marianela, Lofra Robert Muni, Bourke John P, James Meredith K, Díaz-Manera Jordi, Elseed Maha A, Michel-Sodhi Jassi, Moat Dionne, Mccallum Michelle, Mayhew Anna, Ghimenton Elisabetta, Díaz Carla Florencia Bolaño, Malinova Monika, Wong Karen, Richardson Mark, Tasca Giorgio, Grover Emma, Robinson Emma-Jayne, Tanner Stephanie, Eglon Gail, Behar Laura, Eagle Michelle, Turner Catherine, Verdú-Díaz José, Heslop Emma, Straub Volker, Bettolo Chiara Marini, Guglieri Michela. Disease-associated comorbidities, medication records and anthropometric measures in adults with Duchenne muscular dystrophy.. Neuromuscular disorders : NMD. 2024. DOI: 10.1016/j.nmd.2024.05.007.

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