Association of Muscle Strength and Walking Performance in Adult Patients With Pompe Disease.
Marein M Favejee, Jan C van der Meijden, Michelle E Kruijshaar and 3 others
PMID 30265367WHAT IT FOUND
Hip and knee strength, age, sex, BMI, and ventilator use predict walking ability in adults with Pompe disease.
A nomogram using these factors matched observed walking status in 66% of cases, helping clinicians identify patients at risk of overload or underload.
Key findings
01Walking performance declined as muscle strength decreased, particularly for hip flexors and abductors, which showed significant differences between all consecutive walking categories.
02A multivariate model including hip abductor and knee extensor strength, age, sex, BMI, and ventilator use accurately predicted walking performance in 66% of patients.
03Discrepancies between predicted and observed walking ability may indicate overload or underload, suggesting a need to adjust supportive measures such as walking aids or strength training.
STILL TO COME
How it was doneWhat they foundWhat it means for PTsWhat it means for OTs
Read the rest of this summary
You get three full summaries a month, free, and we do not ask for a card. Search, the TL;DRs and your library stay unlimited either way.
What it does not show
The study is cross-sectional, so the model predicts current walking status but cannot forecast future decline or prognosis. The model was not externally validated, meaning its accuracy in other groups of patients with Pompe disease is unknown. Muscle strength was measured by only three testers, and interrater reliability was not assessed, which could affect the consistency of the strength data. The model predicted walking performance accurately in only 66% of cases, leaving a significant proportion of patients where other unmeasured factors (such as trunk muscle weakness) influence walking. Trunk muscles and plantar flexors were not included in the model, despite their known importance in gait and potential involvement in Pompe disease.
The easy way to misread this
Do not use this model to predict how a patient's walking will change over time or to determine their long-term prognosis. The data is cross-sectional, so the nomogram only estimates the probability of a patient being in a specific walking category at the time of assessment.