Albertine Donker

Chapter 8 286 Table 2A. Hepcidin values (nM) in IRIDA patients and IDA controls IRIDA patients IDA controls n Median Min-Max n Median Min-Max p Total group 21 7.5 1.3-25.4 39 0.3 0.3-0.8 0.000 Men 5 7.6 6.6-22.5 10 0.3 0.3-0.3 0.000 Premenopausal women 15 7.5 1.3-25.4 10 0.3 0.3-0.3 0.000 Postmenopausal women 1 2.4 na 19 0.3 0.3-0.8 na Adults 13 7.5 1.3-19.4 39 0.3 0.3-0.8 0.000 Children < 12 years 7 7.6 6.6-25.4 na na na na Bi-allelic TMPRSS6 defect 11 10.0 3.6-25.4 na na na na Mono-allelic TMPRSS6 defect 10 7.3 1.3-13.3 na na na na Table 2B. TSAT/hepcidin ratios (%/nM) in IRIDA patients and IDA controls IRIDA patients IDA controls n Median Min-Max n Median Min-Max p Total group 21 0.7 0.1-19.1 39 16.7 8.0-44.0 0.000 Men 5 0.5 0.1-1.7 10 20.0 8.0-44.0 0.002 Premenopausal women 15 0.8 0.3-9.1 10 16.0 8.0-28.0 0.000 Postmenopausal women 1 3.8 na 19 20.0 8.0-44.0 na Adults 13 1.0 0.3-9.1 39 16.7 8.0-44.0 0.000 Children < 12 years 7 0.5 0.3-1.7 na na na na Bi-allelic TMPRSS6 defect 11 0.5 0.1-1.0 na na na na Mono-allelic TMPRSS6 defect 10 1.4 0.3-9.1 na na na na For hepcidin levels below the detection limit of 0.5 nM were imputated a value of 0.25 nM. For reference values of hepcidin and TSAT/hepcidin ratio, see www.hepcidinanalys.com and Donker et al . 33,34 Since our data indicate that serum hepcidin relative to body iron significantly drops in both boys and girls after the age of 12 years, we intended to stratify for age <12 years and age 12-17 years. However, since our IRIDA case series contained only one case ≤12 years, comparison of the hepcidin and TSAT/hepcidin ratio between IRIDA cases <12 versus >12 years was not possible. Abbreviations: Min-Max denotes minimum-maximum