Limits...
Risk factors for osteoporosis, falls and fractures in hereditary myopathies and sporadic inclusion body myositis — A cross sectional survey ☆

View Article: PubMed Central - PubMed

ABSTRACT

Background: The risk of osteoporosis is known in myopathies requiring long-term steroid treatment and Pompe disease, but not in other hereditary myopathies or sporadic inclusion body myositis (sIBM).

Methods: Risk factors of osteoporosis, laboratory parameters of bone metabolism, frequency of falls and fractures, walking ability, and pain were surveyed using questionnaires in 89 patients with sIBM and genetically confirmed myopathies facioscapulohumeral muscular dystrophy (FSHD), myotonic dystrophy types 1 and 2 (DM1, DM2), limb girdle muscular dystrophies (LGMD2A, LGMD2B, LGMD2I), MATR3 myopathy, and oculopharyngeal muscular dystrophy (OPMD). Additionally laboratory parameters of bone metabolism were determined.

Results: The mean age at examination per disease group ranged from 32 years in LGMD2A to 70 years in sIBM. Myopathies with a higher degree of walking impairment had a higher risk of falls (sIBM, LGMD2A, LGMD2B). At the time of examination 3.4% had a history of osteoporosis. The 25-OH D3 level was decreased in 20% of patients (and in 55% of patients with LGMDs), 57% of them were ambulatory. The 25-OH D3 level was significantly lower in patients with myopathies than in other neurological disorders (p < 0.001). 2.7 falls per year per person occurred. Fractures were reported in 6.8% of patients within the last year. They involved frequently the tibia bone. The pain score didn't correlate with either the walking disability (WGMS) score or the 25-OH D3 level.

Conclusion: The risk for osteoporosis and reduced 25-OH D3 level seems to be increased in wheelchair-bound patients with myopathy but also in patients with DM1 and autosomal-recessive myopathies.

No MeSH data available.


Related in: MedlinePlus

A. Distribution of total number of fractures (n = 21) in all patients with myopathies.B. Serum 25-OH D3 level in all myopathies in relation to the months of the year.C. Serum 25-OH D3 level and WGMS in all myopathies.D. Frequency of fractures and decreased 25-OH D3 level (< 30 nmol/L) among the elderly (> 50 years, stratified for the age group 50–64 years and 65–74 years, respectively) men and women.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC5121322&req=5

f0005: A. Distribution of total number of fractures (n = 21) in all patients with myopathies.B. Serum 25-OH D3 level in all myopathies in relation to the months of the year.C. Serum 25-OH D3 level and WGMS in all myopathies.D. Frequency of fractures and decreased 25-OH D3 level (< 30 nmol/L) among the elderly (> 50 years, stratified for the age group 50–64 years and 65–74 years, respectively) men and women.

Mentions: In the total cohort, 13 patients (15%) reported ever experiencing a total of 21 fractures, among them were 8 patients ≥ 50 years (i.e. 8/45 patients ≥ 50 years, 18%). The rate of fractures in the total cohort within the year investigated by the survey was 6.8% (i.e. n = 6). In total 14 out of 21 fractures happened after the onset of myopathy. The distribution of fractures is given in Fig. 1A and involves fractures of the lower extremity in 43% and the upper extremity in 38%. Fractures occurred most frequently in patients with disorders associated with a severe walking impairment (n = 6), i.e. LGMD2A, LGMD2B, sIBM, but also myotonic dystrophies (n = 4). Wheelchair-bound patients with fractures had the following disorders: sIBM (n = 2), LGMD2A (n = 1), LGMD2B (n = 1). In the myotonic dystrophies all 4 patients who reported previous fractures were ambulatory (DM1 n = 2, DM2 n = 2) (Table 1). Six out of eight fractures located at a lower limb involved the tibia bone. Four of the tibial fractures occurred in wheelchair-bound patients.


Risk factors for osteoporosis, falls and fractures in hereditary myopathies and sporadic inclusion body myositis — A cross sectional survey ☆
A. Distribution of total number of fractures (n = 21) in all patients with myopathies.B. Serum 25-OH D3 level in all myopathies in relation to the months of the year.C. Serum 25-OH D3 level and WGMS in all myopathies.D. Frequency of fractures and decreased 25-OH D3 level (< 30 nmol/L) among the elderly (> 50 years, stratified for the age group 50–64 years and 65–74 years, respectively) men and women.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5121322&req=5

f0005: A. Distribution of total number of fractures (n = 21) in all patients with myopathies.B. Serum 25-OH D3 level in all myopathies in relation to the months of the year.C. Serum 25-OH D3 level and WGMS in all myopathies.D. Frequency of fractures and decreased 25-OH D3 level (< 30 nmol/L) among the elderly (> 50 years, stratified for the age group 50–64 years and 65–74 years, respectively) men and women.
Mentions: In the total cohort, 13 patients (15%) reported ever experiencing a total of 21 fractures, among them were 8 patients ≥ 50 years (i.e. 8/45 patients ≥ 50 years, 18%). The rate of fractures in the total cohort within the year investigated by the survey was 6.8% (i.e. n = 6). In total 14 out of 21 fractures happened after the onset of myopathy. The distribution of fractures is given in Fig. 1A and involves fractures of the lower extremity in 43% and the upper extremity in 38%. Fractures occurred most frequently in patients with disorders associated with a severe walking impairment (n = 6), i.e. LGMD2A, LGMD2B, sIBM, but also myotonic dystrophies (n = 4). Wheelchair-bound patients with fractures had the following disorders: sIBM (n = 2), LGMD2A (n = 1), LGMD2B (n = 1). In the myotonic dystrophies all 4 patients who reported previous fractures were ambulatory (DM1 n = 2, DM2 n = 2) (Table 1). Six out of eight fractures located at a lower limb involved the tibia bone. Four of the tibial fractures occurred in wheelchair-bound patients.

View Article: PubMed Central - PubMed

ABSTRACT

Background: The risk of osteoporosis is known in myopathies requiring long-term steroid treatment and Pompe disease, but not in other hereditary myopathies or sporadic inclusion body myositis (sIBM).

Methods: Risk factors of osteoporosis, laboratory parameters of bone metabolism, frequency of falls and fractures, walking ability, and pain were surveyed using questionnaires in 89 patients with sIBM and genetically confirmed myopathies facioscapulohumeral muscular dystrophy (FSHD), myotonic dystrophy types 1 and 2 (DM1, DM2), limb girdle muscular dystrophies (LGMD2A, LGMD2B, LGMD2I), MATR3 myopathy, and oculopharyngeal muscular dystrophy (OPMD). Additionally laboratory parameters of bone metabolism were determined.

Results: The mean age at examination per disease group ranged from 32 years in LGMD2A to 70 years in sIBM. Myopathies with a higher degree of walking impairment had a higher risk of falls (sIBM, LGMD2A, LGMD2B). At the time of examination 3.4% had a history of osteoporosis. The 25-OH D3 level was decreased in 20% of patients (and in 55% of patients with LGMDs), 57% of them were ambulatory. The 25-OH D3 level was significantly lower in patients with myopathies than in other neurological disorders (p&nbsp;&lt;&nbsp;0.001). 2.7 falls per year per person occurred. Fractures were reported in 6.8% of patients within the last year. They involved frequently the tibia bone. The pain score didn't correlate with either the walking disability (WGMS) score or the 25-OH D3 level.

Conclusion: The risk for osteoporosis and reduced 25-OH D3 level seems to be increased in wheelchair-bound patients with myopathy but also in patients with DM1 and autosomal-recessive myopathies.

No MeSH data available.


Related in: MedlinePlus