Cortical impairment and reduced muscle mass in children and young adults with nephropathic cystinosis
Authors' conclusion
While bone density parameters were normal, bone geometry was altered, resulting in a thinner cortex with possible impact on bone strength.
Study context
- Population
- Children and young adults with genetically proven nephropathic cystinosis (29 males, 22 females)29 males · 22 females
- Participants
- 51 children
- Outcome
- Bone density and geometry
Methodological detailscompared with age- and gender-specific reference data
- Compared with age- and gender-specific reference data
Main finding
As reported in the abstract
Medullary cross-sectional area (CSA) z-score, not compromised
M = 0.12, SD = 1.56
Cortical density z-score, not compromised
M = −0.25, SD = 1.63
Cortical CSA z-score, reduced
M = −2.16, SD = 1.08
Strength-Strain Index (SSI) z-score, reduced
M = −2.07, SD = 1.08
Observational data: associations, not effects.
Further findings
Anthropometric z-scores vs reference data
- Height z-score, lower
- mean [M] = −1.75, standard deviation [SD] = 1.43
- Weight z-score, lower
- M = −1.67, SD = 1.29
- BMI z-score, lower
- M = −0.98, SD = 1.29
Muscle measures vs reference data
- Muscle CSA z-score, reduced
- M = −2.43, SD = 1.27
- Grip strength z-score, reduced
- M = −3.01, SD = 1.10
- Jump force lower than reference value
- 34%
Multiple regression: muscle mass and bone geometry
- Muscle mass associated with medullary CSA and SSI, but not with cortical CSA
Original publication
Cortical impairment and reduced muscle mass in children and young adults with nephropathic cystinosis. J Bone Miner Res. Published 12 June 2024. doi:10.
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