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Table 3 Correlators of sleep related clinical outcome based on multivariate analysis

From: Sleep disordered breathing in mucopolysaccharidosis I: a multivariate analysis of patient, therapeutic and metabolic correlators modifying long term clinical outcome

Outcome Modifier Patient group Exp (β) Lower 95% CI Upper 95% CI p-value
SDB Present Phenotype All (n = 61)     ns
Treatment     ns
Age @ start of treatment     ns
Follow-up duration     ns
DS:CS ratio @ 1 year −5.94 −12.39 −1.61 0.0017
Mean ODI 4% Phenotype All (n = 61)     ns
Treatment     ns
Age @ start of treatment     ns
Follow-up duration     ns
DS:CS ratio @ 1 year 6.35 1.77 10.95 0.008
Therapeutic Intervention Phenotype All (n = 61)     ns
Treatment     ns
Age @ start of treatment −0.21 −0.45 −0.03 0.012
Follow-up duration     ns
DS:CS ratio @ 1 year     ns
SDB Present Donor Type HSCT Treated Hurler (n = 41)     ns
IDUA level @ 1 year 0.08 0.02 0.16 0.004
Age @ start of treatment     ns
Follow-up duration     ns
DS:CS ratio @ 1 year     ns
ODI 4% at time of antibody status DS:CS Ratio ERT Treated Attenuated (n = 17)     ns
Inhibitory Antibodies −8.27 −11.19 −5.36 0.001
Age @ start of treatment     ns
Follow-up duration     ns
  1. p-values for modifiers based on multivariate analysis using stepwise fit (minimum AICc) followed by standard least squares for continuous outcomes and logistic fit for binomial outcomes. CI indicates confidence interval; ERT, enzyme replacement therapy; HSCT, haemopoietic stem cell transplant; IDUA, alpha-L-iduronidase enzyme level; SDB, Sleep disordered breathing; ODI 4%, oxygen desaturation index 4%. Inhibitory antibodies defined as IgG titre > 4000 and uptake inhibition >30%.