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The diagnosis of pediatric CVID is challenging due to the immaturity of the immune response and sustained actively developing antibody affinity to antigens and immunological memory that may overlap with the inborn error of immunity. Significant progress has been recently done in the field of immunogenetics, yet a paucity of experimental and clinical studies on different systemic manifestations and immunological features of CVID in children may contribute to a delayed diagnosis and therapy. In this review, we aimed at defining the variable epidemiological, etiological, and clinical aspects of pediatric CVID with special emphasis on predominating infectious and non-infectious phenotypes in affected children.<\/jats:p><jats:p><jats:italic>Conclusion<\/jats:italic>: While pediatric CVID is a multifaceted and notorious disease, increasing the pediatricians\u2019 awareness of this disease entity and preventing the diagnostic and therapeutic delay are needed, thereby improving the prognosis and survival of pediatric CVID patients.\n<jats:table-wrap><jats:table><jats:tbody>\n                    <jats:tr>\n                      <jats:td colspan=\"2\"><jats:bold>What is Known:<\/jats:bold><jats:italic>\u2022 CVID is an umbrella diagnosis characterized by complex pathophysiology with an antibody deficiency as a common denominator<\/jats:italic>.<jats:italic>\u2022 It is a multifaceted disease characterized by marked genetic, immunological, and clinical heterogeneity.<\/jats:italic>.<\/jats:td>\n                    <\/jats:tr>\n                    <jats:tr>\n                      <jats:td colspan=\"2\"><jats:bold>What is New:<\/jats:bold><jats:italic>\u2022 The diagnosis of pediatric CVID is challenging due to the immaturity of innate and adaptive immune response<\/jats:italic>.<jats:italic>\u2022 Increasing the pediatricians\u2019 awareness of CVID for the early disease recognition, timely therapeutic intervention, and improving the prognosis is needed<\/jats:italic>.<\/jats:td>\n                    <\/jats:tr>\n                  <\/jats:tbody><\/jats:table><\/jats:table-wrap><\/jats:p>","DOI":"10.1007\/s00431-021-04287-6","type":"journal-article","created":{"date-parts":[[2021,12,23]],"date-time":"2021-12-23T01:04:11Z","timestamp":1640221451000},"page":"1371-1383","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["The pediatric common variable immunodeficiency \u2014 from genetics to therapy: a review"],"prefix":"10.1007","volume":"181","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7244-1882","authenticated-orcid":false,"given":"Aleksandra","family":"Szczawinska-Poplonyk","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eyal","family":"Schwartzmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ewelina","family":"Bukowska-Olech","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michal","family":"Biernat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stanislaw","family":"Gattner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomasz","family":"Korobacz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filip","family":"Nowicki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monika","family":"Wiczuk-Wiczewska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,12,23]]},"reference":[{"key":"4287_CR1","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1186\/s13052-016-0279-y","volume":"42","author":"A Szczawinska-Poplonyk","year":"2016","unstructured":"Szczawinska-Poplonyk A, Tapolska-Jozwiak K, Samara H (2016) The B cell compartment in antibody-deficient infants and young children \u2013 developing common variable immunodeficiency or transient immune maturation? 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