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Mutations in the gene encoding adenylate kinase 2 (<jats:italic><jats:styled-content style=\"fixed-case\">AK<\/jats:styled-content>2<\/jats:italic>) were identified to cause this phenotype. In this review, we will demonstrate important clinical differences between reticular dysgenesis and other <jats:styled-content style=\"fixed-case\">SCID<\/jats:styled-content> entities and summarize recent concepts in the understanding of the pathophysiology of the disease and the management strategies for this difficult condition.<\/jats:p>","DOI":"10.1111\/bjh.15045","type":"journal-article","created":{"date-parts":[[2017,12,23]],"date-time":"2017-12-23T16:25:45Z","timestamp":1514046345000},"page":"644-653","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Recent advances in understanding the pathogenesis and management of reticular dysgenesis"],"prefix":"10.1111","volume":"180","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9165-1690","authenticated-orcid":false,"given":"Manfred","family":"Hoenig","sequence":"first","affiliation":[{"name":"Department of Paediatrics University Medical Centre Ulm Ulm 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