Thanks in part to volunteers at both the 2017 and 2019 CRN Family Conferences, this study was able to assess the grip strength of 76 adults and children living with Cystinosis.
“Results: We included 76 patients with a mean grip strength z-score of 2.1, which was lower than seen in patients with CKD without cystinosis. Male sex and delayed cysteamine initiation were independently associated with impaired grip strength. Among adults, a low level of physical activity was associated with lower grip strength z score, but no association was found in children. A third of the patients reported neuromuscular symptoms, with swallowing issues associated with lower grip strength. There was no significant correlation between eGFR and grip strength z-score.
Conclusion: Patients with cystinosis have impaired muscle strength compared with healthy control sub- jects and patients with CKD. This impairment is greater in male patients and in patients with late initiation of cysteamine therapy and is associated with lower physical activity. Further studies investigating the effect of different types of physical activities, optimizing cysteamine therapy, and other interventions are needed.”
Read the full publication, “Grip Strength in Adults and Children with Cystinosis” here.