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The number of identified IEIs is rapidly increasing, highlighting the non-negligible role of an interdisciplinary approach in clinical diagnosis. Kidney disorders are one of the important comorbidities in some of the affected patients and play a significant role in the diagnosis and course of disease. According to recent studies, 22 types of human IEI with renal manifestations have been identified so far, including immunodeficiency with congenital thrombocytopenia, thymic defects with additional congenital anomalies, complement deficiencies, type 1 interferonopathies, immunity related to non-hematopoietic tissues, congenital neutropenia\u2019s, common variable immunodeficiency disorder (CVID) phenotype and immuno-osseous dysplasia. Based on this classification, we herein review IEIs with renal features and explain the genetic defect, inheritance, and type of renal manifestations.<\/jats:p>","DOI":"10.1007\/s11255-023-03907-4","type":"journal-article","created":{"date-parts":[[2024,1,10]],"date-time":"2024-01-10T16:02:22Z","timestamp":1704902542000},"page":"1965-1972","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Inborn errors of immunity with kidney and urinary tract disorders: a review"],"prefix":"10.1007","volume":"56","author":[{"given":"Ahmad","family":"Shajari","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8832-1794","authenticated-orcid":false,"given":"Atefe","family":"Zare Ahmadabadi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammad Moein","family":"Ashrafi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9959-347X","authenticated-orcid":false,"given":"Tolue","family":"Mahdavi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8314-2412","authenticated-orcid":false,"given":"Mahbubeh","family":"Mirzaee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1048-6027","authenticated-orcid":false,"given":"Masoumeh","family":"Mohkam","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samin","family":"Sharafian","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-6296-0501","authenticated-orcid":false,"given":"Mehrdad","family":"Tamiji","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0732-9170","authenticated-orcid":false,"given":"Mahnaz","family":"Jamee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,1,10]]},"reference":[{"issue":"7","key":"3907_CR1","doi-asserted-by":"publisher","first-page":"1508","DOI":"10.1007\/s10875-022-01352-z","volume":"42","author":"A Bousfiha","year":"2022","unstructured":"Bousfiha A, Moundir A, Tangye SG, Picard C, Jeddane L, Al-Herz W et al (2022) The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. 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