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. 2018 Nov 1;9:2411. doi: 10.3389/fimmu.2018.02411

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Copyright © 2018 Gambineri, Ciullini Mannurita, Hagin, Vignoli, Anover-Sombke, DeBoer, Segundo, Allenspach, Favre, Ochs and Torgerson.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Kaplan-Meier survival curve for overall survival of patients. (A) The overall survival in the whole cohort of patient is evaluated (n = 163); (B) IPEX (n = 85) vs. IPEX-like (n = 78) patients are compared; (C) IPEX patients stratified for type of mutation: frameshift (FS) n = 6, in frame deletion (IFD) n = 11, missense (MS) n = 43, splicing mutations (SP) n = 14 and mutations in polyadenylation site (POLA) n = 11 are compared; (D) IPEX-like without mutations (NA= not available) n = 57 and IPEX-like with mutation (Mut) n = 21 are compared; (E) IPEX patient stratified whether (n = 39) or not (n = 46) they underwent HSCT and (F) IPEX-like patient stratified whether (n = 10) or not (n = 68) they underwent HSCT.