{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T17:08:49Z","timestamp":1790874529824,"version":"4.1.0"},"reference-count":6,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:p>Disorders of sexual development (DSDs) are heterogenous group of disorders characterized by discrepancy in chromosomal, gonadal, and phenotypic sex. DSD is a Medico-Social Emergency. 5 \u03b1 reductase type 2 deficiency is one of the rare causes of 46 XY DSD. The degree of under virilization varies from clinically female to a near male phenotype. M\u00fcllerian structures were absent. At puberty, there might be features of virilization. Gender incongruence or dysphoria occurs frequently during puberty. An important marker for diagnosis is an elevated testosterone to dihydrotestosterone ratio in the basal state or following HCG stimulation. All 46 XY DSD require an appropriate molecular diagnosis prior to gender assignment and gender reassignment surgery. Gender reassignment surgery and\/or Gonadectomy should be performed only after 18 years if there are compelling indications like suspicion of malignancy or presence of gender dysphoria or gender incongruence.<\/jats:p>","DOI":"10.4103\/ijem.ijem_179_21","type":"journal-article","created":{"date-parts":[[2022,1,12]],"date-time":"2022-01-12T09:08:15Z","timestamp":1641978495000},"page":"392-394","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":3,"title":["\u201cCongruency the ART of Being Real\u201d - 46XY DSD Due to 5 \u03b1 Reductase Deficiency - Challenges in Decision Making"],"prefix":"10.4103","volume":"25","author":[{"given":"Sridhar","family":"Subbiah","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Government Rajaji Hospital and Madurai Medical College, Madurai, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raghavendran","family":"Priyanka","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Government Rajaji Hospital and Madurai Medical College, Madurai, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sangumani","family":"Jayaraman","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Government Rajaji Hospital and Madurai Medical College, Madurai, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2022,1,12]]},"reference":[{"key":"-20240828","doi-asserted-by":"crossref","first-page":"e488","DOI":"10.1542\/peds.2006-0738","article-title":"International Consensus Conference on Intersex Organized by the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. Consensus statement on management of intersex disorders. International Consensus Conference on Intersex","volume":"118","author":"Lee","year":"2006","journal-title":"Pediatrics"},{"key":"-20240828","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1159\/000509812","article-title":"Spectrum of pathogenic variants in SRD5A2 in Indian children with 46, XY disorders of sex development and clinically suspected steroid 5\u03b1-reductase 2 deficiency","volume":"13","author":"Kumar","year":"2019","journal-title":"Sex Dev"},{"key":"-20240828","doi-asserted-by":"crossref","first-page":"593","DOI":"10.4103\/ijem.IJEM_553_19","article-title":"Dynamic testing for evaluation of adrenal and gonadal function in pediatric and adult endocrinology. An overview","volume":"23","author":"Goyal","year":"2019","journal-title":"Indian J Endocr Metab"},{"key":"-20240828","doi-asserted-by":"crossref","first-page":"1132","DOI":"10.1111\/andr.12108","article-title":"Phenotype, genotype and gender identity in a large cohort of patients from India with 5\u03b1-reductase 2 deficiency","volume":"3","author":"Shabir","year":"2015","journal-title":"Andrology"},{"key":"-20240828","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1210\/jc.2010-1024","article-title":"Phenotypical, biological, and molecular heterogeneity of 5\u03b1-reductase deficiency: An extensive international experience of 55 patients","volume":"96","author":"Maimoun","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"key":"-20240828","journal-title":"Guidelines for regulation of treatment and surgeries on individuals with a different of sex development (DSD) and standard treatment guidelines on medical\/surgical management of DSD. G.O (Ms) No. 63, Government of Tamilnadu, Health and Family welfare (EAP \u2013II) department, dated 18.2.2020"}],"container-title":["Indian Journal of Endocrinology and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/ijem.ijem_179_21","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T14:54:04Z","timestamp":1787756044000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/ijem.ijem_179_21"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9]]},"references-count":6,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2021]]}},"URL":"https:\/\/doi.org\/10.4103\/ijem.ijem_179_21","relation":{},"ISSN":["2230-9500","2230-8210"],"issn-type":[{"value":"2230-9500","type":"print"},{"value":"2230-8210","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,9]]},"assertion":[{"value":"2021-04-29","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-10-24","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}