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Teenagers and young adults with nephropathic cystinosis display significant bone disease and cortical impairment

Pediatric Nephrology Jun 08, 2018

Bertholet-Thomas A, et al. - Given bone impairment appears to be a novel complication of nephropathic cystinosis despite cysteamine therapy, researchers assessed bone status among patients included in the French Crystobs study in order to gain clarity regarding its exact underlying pathophysiology. They compared bone status to age- and gender-matched healthy controls (1:2 basis) from the local reference cohorts. They reported that bone impairment (rather cortical than trabecular) represented a significant clinical problem in nephropathic cystinosis. During teenage or young adulthood, significant bone symptoms were shown by 70% of patients. This new complication having a potential dramatic impact on quality of life should be known by physicians.
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