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Furthermore, it may allow cross-sectional and cohort comparisons and support treatment decisions such as an evaluation for transplantation. On the basis of a literature review, we provide a descriptive comparison of ten selected scores and measures frequently used in IEI and divide these into three categories: (1)\n                    <jats:italic>diagnostic tools<\/jats:italic>\n                    (for Hyper-IgE syndrome, hemophagocytic lymphohistiocytosis, and Wiskott-Aldrich syndrome), (2)\n                    <jats:italic>morbidity and disease activity measures<\/jats:italic>\n                    (for common variable immune deficiency [CVID], profound combined immune deficiency, CTLA-4 haploinsufficiency, immune deficiency and dysregulation activity [IDDA], IPEX organ impairment, and the autoinflammatory disease activity index), and (3)\n                    <jats:italic>treatment stratification<\/jats:italic>\n                    scores (shown for hypogammaglobulinemia). The depth of preclinical and statistical validations varies among the presented tools, and disease-inherent and user-dependent factors complicate their broader application. To support a comparable, standardized evaluation for prospective monitoring of diseases with immune dysregulation, we propose the IDDA2.1 score (comprising 22 parameters on a 2\u20135-step scale) as a simple yet comprehensive and powerful tool. Originally developed for use in a retrospective study in LRBA deficiency, this new version may be applied to all IEI with immune dysregulation. Reviewing published aggregate cohort data from hundreds of patients, the IDDA\n                    <jats:italic>kaleidoscope function<\/jats:italic>\n                    is presented for 18 exemplary IEI as an instructive phenotype\u2013pattern visualization tool, and an unsupervised, hierarchically clustered heatmap mathematically confirms similarities and differences in their phenotype expression profiles.\n                  <\/jats:p>","DOI":"10.1007\/s10875-021-01177-2","type":"journal-article","created":{"date-parts":[[2021,11,19]],"date-time":"2021-11-19T08:02:41Z","timestamp":1637308961000},"page":"484-498","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["The Immune Deficiency and Dysregulation Activity (IDDA2.1 \u2018Kaleidoscope\u2019) Score and Other Clinical Measures in Inborn Errors of Immunity"],"prefix":"10.1007","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0981-8661","authenticated-orcid":false,"given":"Markus G.","family":"Seidel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victoria K.","family":"Tesch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linlin","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9644-2003","authenticated-orcid":false,"given":"Fabian","family":"Hauck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Lena","family":"Horn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1021-0899","authenticated-orcid":false,"given":"Maria Anna","family":"Smolle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0764-9497","authenticated-orcid":false,"given":"Franz","family":"Quehenberger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Benesch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,11,19]]},"reference":[{"issue":"5","key":"1177_CR1","doi-asserted-by":"publisher","first-page":"1452","DOI":"10.1016\/j.jaci.2019.12.896","volume":"145","author":"VK Tesch","year":"2020","unstructured":"Tesch VK, Abolhassani H, Shadur B, Zobel J, Mareika Y, Sharapova S, et al. 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