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Menarcheal age of 64 INC and 302 CKD patients, all treated conservatively. Data is presented for the measuring periods 1998-2015 and ≥ 2016. Error bars represent 95% confidence intervals
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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...
Contexts in source publication
Context 1
... 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 > ...
Context 2
... fat storage is closely linked to the onset of menarche and maturation [29][30][31], the further decline of UFA to − 0.60 z-score in the second cluster can be considered one possible reason for the statistically non-significant delay in onset of menarche observed in our sample (Fig. 4). These findings are in accordance with theories by Frisch and McArthur, specifying a certain percentage of total body fat required for menarche, possibly undercut in girls with INC ...
