Figure 1

Insights into Wolfram syndrome. (a) Natural history of WS. The proportion of patients (density) for each clinical feature at onset age, measured as a nonparametric probability density distribution, is plotted. For descriptive statistics, see Supplementary Table S3 online. (b) Probability of development of WS clinical features by age group. The probability of developing each of the clinical features over age, calculated using a life-table analysis, is displayed. For extended information, see Supplementary Table S4 online. (c) Kaplan–Meier analysis of WS. A Kaplan–Meier approach has been used for analyzing the probability of survival for patients with WS. For extended information, see Supplementary Table S4 online. (d) Order of clinical feature onset in WS. The order in which each clinical feature appears is shown as the percentage of patients with that clinical feature. DM (n = 385); OA (n = 322); HD (n = 189); DI (n = 148); ND (n = 67); UD (n = 72). DE, deceased; DI, diabetes insipidus; DM, diabetes mellitus; HD, hearing defects; ND, neurological, psychiatric, and developmental defects; OA, optic atrophy; UD, urological or renal defects; WS, Wolfram syndrome.