Long-term assessment of urinary glycosaminoglycan-related biomarkers in patients with MPS-IH after HCT according to age or time of follow-up. (A-D) Urine glycosaminoglycan-related biomarkers. I0S0 and I0S6 are nonreducing ends. uHS represents total heparin sulfate calculated from the addition of the internal disaccharides, D0A0 + D0S0. Total uGAG is the total glycosaminoglycan determined in the urine. Urine samples were normalized to creatinine. Shown are the means and standard deviation; P values are derived from a Student’s t test. (E-H) Urinary biomarkers over time. Only patients with myeloid chimerism >85% were included in analyses. The P values derived from ordinary least squares linear regression analyses are shown in each panel, and dotted lines show 95% confidence intervals. The black lines and R² are derived from a curve fitting a logarithmic function (log [Y]) across the entire cohort. The yellow boxes show the normal range for age. (E) uHS normal range: age 1 to 10 years: 0.048 to 0.624 mg/mmol Cr; >10 years: 0.007 to 0.422 mg/mmol Cr. (F) The normal ranges are: age 1 to 2 years: 5.4 to 30.8 mg/mmol Cr; 3 to 6 years: 5.2 to 16.7 mg/mmol Cr; 7 to 13 years: 2.4 to 10.2 mg/mmol Cr; ≥14 years: 0.0 to 7.1 mg/mmol Cr. (G) The normal ranges are: age 1 to 10 years: ≤0.004 mg/mmol Cr; >10 years: ≤0.002 mg/mmol Cr. (H) The normal ranges are: age 1 to 10 years: ≤0.02 mg/mmol Cr; >10 years: ≤0.01 mg/mmol/Cr. (I) shows the multivariate correlation matrix and corresponding scatterplots for MPS biomarkers; the color scale shows the strength of the correlation coefficient. (J) shows the P values associated with the multivariate correlation table. (K) shows the linear regression between leukocyte IDUA and uHS. R² = 0.1818. P = .0237.