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46,XY disorder of sex development of endocrine origin

MedGen UID:
1842509
Concept ID:
C5679872
Disease or Syndrome
Synonym: 46,XY DSD of endocrine origin
 
Orphanet: ORPHA325351

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGV46,XY disorder of sex development of endocrine origin

Recent clinical studies

Etiology

Bouhouche A, Albaroudi N, El Alaoui MA, Askander O, Habbadi Z, El Hassani A, Iraqi H, El Fahime E, Belmekki M
Exp Eye Res 2021 Aug;209:108671. Epub 2021 Jun 13 doi: 10.1016/j.exer.2021.108671. PMID: 34133966
Lourenço D, Brauner R, Rybczynska M, Nihoul-Fékété C, McElreavey K, Bashamboo A
Proc Natl Acad Sci U S A 2011 Jan 25;108(4):1597-602. Epub 2011 Jan 10 doi: 10.1073/pnas.1010257108. PMID: 21220346Free PMC Article
Maciel-Guerra AT, de Mello MP, Coeli FB, Ribeiro ML, Miranda ML, Marques-de-Faria AP, Baptista MT, Moraes SG, Guerra-Júnior G
J Clin Endocrinol Metab 2008 Feb;93(2):339-43. Epub 2007 Dec 4 doi: 10.1210/jc.2007-1115. PMID: 18056774
Rosler A
Pediatr Endocrinol Rev 2006 Aug;3 Suppl 3:455-61. PMID: 17551466

Diagnosis

Cheng D, Lu CF, Gong F, Du J, Yuan S, Luo KL, Tan YQ, Lu GX, Lin G
Hum Reprod 2024 Apr 3;39(4):849-855. doi: 10.1093/humrep/deae026. PMID: 38420683
Makiyan Z
Organogenesis 2016 Oct;12(4):169-182. Epub 2016 Jul 8 doi: 10.1080/15476278.2016.1210749. PMID: 27391116Free PMC Article
Dutta D, Shivaprasad KS, Das RN, Ghosh S, Chatterjee U, Chowdhury S, Dasgupta R
Andrologia 2014 Mar;46(2):191-3. Epub 2012 Nov 19 doi: 10.1111/and.12048. PMID: 23163819
Stuchi-Perez EG, Lukas-Croisier C, De Castro M, Baptista MT, Ribeiro Scolfaro M, Marques-De-Faria AP, Hackel C, Maciel-Guerra AT, Guerra Júnior G
J Pediatr Endocrinol Metab 2000 Jun;13(6):605-12. doi: 10.1515/jpem.2000.13.6.605. PMID: 10905384
Krob G, Braun A, Kuhnle U
Eur J Pediatr 1994 Jan;153(1):2-10. doi: 10.1007/BF02000779. PMID: 8313919

Therapy

Juul A, Almstrup K, Andersson AM, Jensen TK, Jørgensen N, Main KM, Rajpert-De Meyts E, Toppari J, Skakkebæk NE
Nat Rev Endocrinol 2014 Sep;10(9):553-62. Epub 2014 Jun 17 doi: 10.1038/nrendo.2014.97. PMID: 24935122

Prognosis

Bouhouche A, Albaroudi N, El Alaoui MA, Askander O, Habbadi Z, El Hassani A, Iraqi H, El Fahime E, Belmekki M
Exp Eye Res 2021 Aug;209:108671. Epub 2021 Jun 13 doi: 10.1016/j.exer.2021.108671. PMID: 34133966
Winge SB, Dalgaard MD, Belling KG, Jensen JM, Nielsen JE, Aksglaede L, Schierup MH, Brunak S, Skakkebæk NE, Juul A, Rajpert-De Meyts E, Almstrup K
Cell Death Dis 2018 May 22;9(6):586. doi: 10.1038/s41419-018-0671-1. PMID: 29789566Free PMC Article
Lourenço D, Brauner R, Rybczynska M, Nihoul-Fékété C, McElreavey K, Bashamboo A
Proc Natl Acad Sci U S A 2011 Jan 25;108(4):1597-602. Epub 2011 Jan 10 doi: 10.1073/pnas.1010257108. PMID: 21220346Free PMC Article
Fernández-Cancio M, Nistal M, Gracia R, Molina MA, Tovar JA, Esteban C, Carrascosa A, Audí L
J Androl 2004 May-Jun;25(3):412-6. doi: 10.1002/j.1939-4640.2004.tb02808.x. PMID: 15064320

Clinical prediction guides

Livermore C, Simon M, Reeves R, Stévant I, Nef S, Pope M, Mallon AM, Wells S, Warr N, Greenfield A
Genetics 2020 Feb;214(2):467-477. Epub 2019 Dec 13 doi: 10.1534/genetics.119.302786. PMID: 31836612Free PMC Article
Winge SB, Dalgaard MD, Belling KG, Jensen JM, Nielsen JE, Aksglaede L, Schierup MH, Brunak S, Skakkebæk NE, Juul A, Rajpert-De Meyts E, Almstrup K
Cell Death Dis 2018 May 22;9(6):586. doi: 10.1038/s41419-018-0671-1. PMID: 29789566Free PMC Article
van Bever Y, Wolffenbuttel KP, Brüggenwirth HT, Blom E, de Klein A, Eussen BHJ, van der Windt F, Hannema SE, Dessens AB, Dorssers LCJ, Biermann K, Hersmus R, de Rijke YB, Looijenga LHJ
Sex Dev 2018;12(1-3):145-154. Epub 2017 Sep 20 doi: 10.1159/000479946. PMID: 28926831Free PMC Article
Juul A, Almstrup K, Andersson AM, Jensen TK, Jørgensen N, Main KM, Rajpert-De Meyts E, Toppari J, Skakkebæk NE
Nat Rev Endocrinol 2014 Sep;10(9):553-62. Epub 2014 Jun 17 doi: 10.1038/nrendo.2014.97. PMID: 24935122
Shammas C, Neocleous V, Toumba M, Costi C, Phedonos AA, Efstathiou E, Kyriakou A, Phylactou LA, Skordis N
Genet Test Mol Biomarkers 2012 Sep;16(9):1073-9. Epub 2012 Aug 2 doi: 10.1089/gtmb.2011.0381. PMID: 22857144

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