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Mean SDS values of anterior-posterior chest/height ratio (APC/height ratio; filled circle), anterior-posterior chest/ transversal ratio (APC/transversal ratio; filled square), sitting height index (star), body mass index (empty diamond), and upper arm fat area (empty
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Background
Infantile nephropathic cystinosis (INC) is a rare lysosomal storage disorder, mostly and often firstly affecting the kidneys, together with impaired disharmonious growth and rickets, eventually resulting in progressive chronic kidney disease (CKD). With the introduction of cysteamine therapy, most pediatric patients reach adulthood with...
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Context 1
... thoracic configurations, which are presented in Fig. 3, both APC/height ratio (2.03 vs. 1.87 z-score) and APC/transverse ratio (1.01 vs. 1.57 z-score) remained the two most markedly elevated parameters in children with INC over the complete observation period; indicating severe trunk deformation. On the other hand, ...
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... upper arm fat area (UFA) (− 0.48 to − 0.60 z-score) and BMI (0.05 to − 0.37 z-score) declined, though only the decline in BMI reached levels of statistical significance. Contrarily, in their peers with CKD, BMI (− 0.16 to 0.03 z-score) as well as UFA (− 0.39 to 0.08 z-score) significantly increased over the course of the two measurement clusters (Fig. 3). Accordingly, age at menarche in girls with INC increased from a mean age of 13.47 ± 2.20 to 13.81 ± 1.13 years while conversely, in the CKD group, menarcheal age declined from a mean age of 13.00 ± 1.38 to 12.03 ± 0.93 years after 2015 (Fig. 4). Both tendencies did not reach levels of statistical significance (p > ...
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... ∆2.47 z-score), while in children with CKD, these values markedly declined (∆1.19 to ∆0.88 z-score), indicating a synchronization and harmonization of body proportions and composition in this group. The findings of persisting elevated delta values in patients with INC hint at a sustained striking disharmony of thoracic shape and body composition (Fig. ...
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... in INC patients. In their peers with CKD, the only observed significant association between APC/height ratio and biochemical parameters was observed with bicarbonate which showed negative associations with APC/height ratio in the second measurement cluster. Corresponding with our findings of persisting reduced body fat stores in children with INC (Fig. 3), none of the observed clinical parameters showed any significant association in INC patients with UFA in both measurement clusters. In CKD patients, higher hemoglobin levels as well as lower sodium levels were associated with increased UFA z-scores in the second measurement ...
