Background: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED or APS-1) is a rare autosomal recessive disorder and diabetes (DM) is one of its more rare manifestations. Earlier, we have showed that reduction in fractional glucose removal rate (KG) , a known precursor of increased peripheral insulin resistance over time, was the only predictor candidate of abrupt, atypical APECED DM onset. We now explored if these changes in KG were age-dependent, and independent of DM status in all patients with APECED.
Materials and Methods: Bi-annually conducted intravenous glucose tolerance tests (ivGTT) and calculated KG values (0.693/T1/2Gluc*100) were reviewed for the Finnish APECED cohort (children and adults screened for DM in 1972-2000) . Pearson correlation coefficients (r) for KG and age at ivGTT were calculated with 2-sided p-values. A Fisher t to z transformation and a z-test were performed to investigate the difference between the groups (with DM diagnosis: DM+; without: DM-) .
Results: A total of 258 ivGTTs were performed on 13 DM+ (mean age at DM onset 27.8 ±11.7 years) and 46 DM- patients. KG values decreased over time in both groups (Figure) , with no statistical difference in the r-values between the groups (z=0.763, p=0.22) .
Conclusion: As markers of peripheral glucose uptake decline similarly over time in all patients with APECED, independent of DM status, we still lack accurate predictors of atypical DM onset in APECED.
E.R.Sandini: None. S.Laakso: None. O.Makitie: Advisory Panel; Kyowa Kirin Co., Ltd., Consultant; BridgeBio Pharma, Inc., Speaker's Bureau; Merck & Co., Inc. P.M.Paldanius: Consultant; Glyconics Ltd, UPM Biomedicals, Speaker's Bureau; Novartis AG, UPM Biomedicals.
Helsinki University Hospital; Foundation for Pediatric Research