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46,XX disorder of gonadal development

MedGen UID:
1842323
Concept ID:
C5680950
Disease or Syndrome
Orphanet: ORPHA325055

Term Hierarchy

Professional guidelines

PubMed

Yavas Abalı Z, Guran T
Front Endocrinol (Lausanne) 2024;15:1354759. Epub 2024 May 15 doi: 10.3389/fendo.2024.1354759. PMID: 38812815Free PMC Article
Ferrari MTM, Silva ESDN, Nishi MY, Batista RL, Mendonca BB, Domenice S
Front Endocrinol (Lausanne) 2024;15:1385901. Epub 2024 Apr 24 doi: 10.3389/fendo.2024.1385901. PMID: 38721146Free PMC Article
Syryn H, Van De Vijver K, Cools M
Horm Res Paediatr 2023;96(2):180-189. Epub 2021 Sep 1 doi: 10.1159/000519323. PMID: 34469891

Recent clinical studies

Etiology

Fiot E, Alauze B, Donadille B, Samara-Boustani D, Houang M, De Filippo G, Bachelot A, Delcour C, Beyler C, Bois E, Bourrat E, Bui Quoc E, Bourcigaux N, Chaussain C, Cohen A, Cohen-Solal M, Da Costa S, Dossier C, Ederhy S, Elmaleh M, Iserin L, Lengliné H, Poujol-Robert A, Roulot D, Viala J, Albarel F, Bismuth E, Bernard V, Bouvattier C, Brac A, Bretones P, Chabbert-Buffet N, Chanson P, Coutant R, de Warren M, Demaret B, Duranteau L, Eustache F, Gautheret L, Gelwane G, Gourbesville C, Grynberg M, Gueniche K, Jorgensen C, Kerlan V, Lebrun C, Lefevre C, Lorenzini F, Manouvrier S, Pienkowski C, Reynaud R, Reznik Y, Siffroi JP, Tabet AC, Tauber M, Vautier V, Tauveron I, Wambre S, Zenaty D, Netchine I, Polak M, Touraine P, Carel JC, Christin-Maitre S, Léger J
Orphanet J Rare Dis 2022 Jul 12;17(Suppl 1):261. doi: 10.1186/s13023-022-02423-5. PMID: 35821070Free PMC Article
Eggers S, Sadedin S, van den Bergen JA, Robevska G, Ohnesorg T, Hewitt J, Lambeth L, Bouty A, Knarston IM, Tan TY, Cameron F, Werther G, Hutson J, O'Connell M, Grover SR, Heloury Y, Zacharin M, Bergman P, Kimber C, Brown J, Webb N, Hunter MF, Srinivasan S, Titmuss A, Verge CF, Mowat D, Smith G, Smith J, Ewans L, Shalhoub C, Crock P, Cowell C, Leong GM, Ono M, Lafferty AR, Huynh T, Visser U, Choong CS, McKenzie F, Pachter N, Thompson EM, Couper J, Baxendale A, Gecz J, Wheeler BJ, Jefferies C, MacKenzie K, Hofman P, Carter P, King RI, Krausz C, van Ravenswaaij-Arts CM, Looijenga L, Drop S, Riedl S, Cools M, Dawson A, Juniarto AZ, Khadilkar V, Khadilkar A, Bhatia V, Dũng VC, Atta I, Raza J, Thi Diem Chi N, Hao TK, Harley V, Koopman P, Warne G, Faradz S, Oshlack A, Ayers KL, Sinclair AH
Genome Biol 2016 Nov 29;17(1):243. doi: 10.1186/s13059-016-1105-y. PMID: 27899157Free PMC Article
Rodriguez-Buritica D
Curr Opin Pediatr 2015 Dec;27(6):675-84. doi: 10.1097/MOP.0000000000000275. PMID: 26335769
King TF, Conway GS
Curr Opin Endocrinol Diabetes Obes 2014 Dec;21(6):504-10. doi: 10.1097/MED.0000000000000113. PMID: 25314337
Zenteno-Ruiz JC, Kofman-Alfaro S, Méndez JP
Arch Med Res 2001 Nov-Dec;32(6):559-66. doi: 10.1016/s0188-4409(01)00322-8. PMID: 11750731

Diagnosis

Reyes AP, León NY, Frost ER, Harley VR
Nat Rev Urol 2023 Jul;20(7):434-451. Epub 2023 Apr 5 doi: 10.1038/s41585-023-00754-x. PMID: 37020056
McElreavey K, Bashamboo A
Horm Res Paediatr 2023;96(2):144-168. Epub 2021 Dec 28 doi: 10.1159/000521381. PMID: 34963118
Fiot E, Alauze B, Donadille B, Samara-Boustani D, Houang M, De Filippo G, Bachelot A, Delcour C, Beyler C, Bois E, Bourrat E, Bui Quoc E, Bourcigaux N, Chaussain C, Cohen A, Cohen-Solal M, Da Costa S, Dossier C, Ederhy S, Elmaleh M, Iserin L, Lengliné H, Poujol-Robert A, Roulot D, Viala J, Albarel F, Bismuth E, Bernard V, Bouvattier C, Brac A, Bretones P, Chabbert-Buffet N, Chanson P, Coutant R, de Warren M, Demaret B, Duranteau L, Eustache F, Gautheret L, Gelwane G, Gourbesville C, Grynberg M, Gueniche K, Jorgensen C, Kerlan V, Lebrun C, Lefevre C, Lorenzini F, Manouvrier S, Pienkowski C, Reynaud R, Reznik Y, Siffroi JP, Tabet AC, Tauber M, Vautier V, Tauveron I, Wambre S, Zenaty D, Netchine I, Polak M, Touraine P, Carel JC, Christin-Maitre S, Léger J
Orphanet J Rare Dis 2022 Jul 12;17(Suppl 1):261. doi: 10.1186/s13023-022-02423-5. PMID: 35821070Free PMC Article
Eggers S, Sadedin S, van den Bergen JA, Robevska G, Ohnesorg T, Hewitt J, Lambeth L, Bouty A, Knarston IM, Tan TY, Cameron F, Werther G, Hutson J, O'Connell M, Grover SR, Heloury Y, Zacharin M, Bergman P, Kimber C, Brown J, Webb N, Hunter MF, Srinivasan S, Titmuss A, Verge CF, Mowat D, Smith G, Smith J, Ewans L, Shalhoub C, Crock P, Cowell C, Leong GM, Ono M, Lafferty AR, Huynh T, Visser U, Choong CS, McKenzie F, Pachter N, Thompson EM, Couper J, Baxendale A, Gecz J, Wheeler BJ, Jefferies C, MacKenzie K, Hofman P, Carter P, King RI, Krausz C, van Ravenswaaij-Arts CM, Looijenga L, Drop S, Riedl S, Cools M, Dawson A, Juniarto AZ, Khadilkar V, Khadilkar A, Bhatia V, Dũng VC, Atta I, Raza J, Thi Diem Chi N, Hao TK, Harley V, Koopman P, Warne G, Faradz S, Oshlack A, Ayers KL, Sinclair AH
Genome Biol 2016 Nov 29;17(1):243. doi: 10.1186/s13059-016-1105-y. PMID: 27899157Free PMC Article
King TF, Conway GS
Curr Opin Endocrinol Diabetes Obes 2014 Dec;21(6):504-10. doi: 10.1097/MED.0000000000000113. PMID: 25314337

Therapy

García-Acero M, Moreno-Niño O, Suárez-Obando F, Molina M, Manotas MC, Prieto JC, Forero C, Céspedes C, Pérez J, Fernandez N, Rojas A
Mol Med Rep 2020 Jan;21(1):97-106. Epub 2019 Nov 12 doi: 10.3892/mmr.2019.10819. PMID: 31746433Free PMC Article
AlSiraj Y, Chen X, Thatcher SE, Temel RE, Cai L, Blalock E, Katz W, Ali HM, Petriello M, Deng P, Morris AJ, Wang X, Lusis AJ, Arnold AP, Reue K, Thompson K, Tso P, Cassis LA
Nat Commun 2019 Jun 14;10(1):2631. doi: 10.1038/s41467-019-10462-z. PMID: 31201301Free PMC Article
King TF, Conway GS
Curr Opin Endocrinol Diabetes Obes 2014 Dec;21(6):504-10. doi: 10.1097/MED.0000000000000113. PMID: 25314337
Hiort O
Ann Endocrinol (Paris) 2014 May;75(2):64-6. Epub 2014 Apr 17 doi: 10.1016/j.ando.2014.03.008. PMID: 24746403
Parada-Bustamante A, Ríos R, Ebensperger M, Lardone MC, Piottante A, Castro A
Fertil Steril 2010 Nov;94(6):2330.e13-6. Epub 2010 May 7 doi: 10.1016/j.fertnstert.2010.03.066. PMID: 20451191

Prognosis

Hagen CP, Fischer MB, Mola G, Mikkelsen TB, Cleemann LH, Gravholt CH, Viuff MH, Juul A, Pedersen AT, Main KM
Front Endocrinol (Lausanne) 2023;14:1173600. Epub 2023 Jun 29 doi: 10.3389/fendo.2023.1173600. PMID: 37455919Free PMC Article
Miyado M, Fukami M, Ogata T
Sex Dev 2022;16(2-3):126-137. Epub 2021 Oct 25 doi: 10.1159/000519298. PMID: 34695834
Wong YS, Tam YH, Pang KKY, Yau HC
J Pediatr Surg 2018 Dec;53(12):2498-2501. Epub 2018 Sep 1 doi: 10.1016/j.jpedsurg.2018.08.010. PMID: 30224237
King TF, Conway GS
Curr Opin Endocrinol Diabetes Obes 2014 Dec;21(6):504-10. doi: 10.1097/MED.0000000000000113. PMID: 25314337
Hiort O
Ann Endocrinol (Paris) 2014 May;75(2):64-6. Epub 2014 Apr 17 doi: 10.1016/j.ando.2014.03.008. PMID: 24746403

Clinical prediction guides

Hiort M, Rohayem J, Knaf R, Laurentino S, Berglund A, Gravholt CH, Gromoll J, Wistuba J
Sex Dev 2023;17(1):32-42. Epub 2023 Feb 6 doi: 10.1159/000528955. PMID: 36746123
Fiot E, Alauze B, Donadille B, Samara-Boustani D, Houang M, De Filippo G, Bachelot A, Delcour C, Beyler C, Bois E, Bourrat E, Bui Quoc E, Bourcigaux N, Chaussain C, Cohen A, Cohen-Solal M, Da Costa S, Dossier C, Ederhy S, Elmaleh M, Iserin L, Lengliné H, Poujol-Robert A, Roulot D, Viala J, Albarel F, Bismuth E, Bernard V, Bouvattier C, Brac A, Bretones P, Chabbert-Buffet N, Chanson P, Coutant R, de Warren M, Demaret B, Duranteau L, Eustache F, Gautheret L, Gelwane G, Gourbesville C, Grynberg M, Gueniche K, Jorgensen C, Kerlan V, Lebrun C, Lefevre C, Lorenzini F, Manouvrier S, Pienkowski C, Reynaud R, Reznik Y, Siffroi JP, Tabet AC, Tauber M, Vautier V, Tauveron I, Wambre S, Zenaty D, Netchine I, Polak M, Touraine P, Carel JC, Christin-Maitre S, Léger J
Orphanet J Rare Dis 2022 Jul 12;17(Suppl 1):261. doi: 10.1186/s13023-022-02423-5. PMID: 35821070Free PMC Article
Miyado M, Fukami M, Ogata T
Sex Dev 2022;16(2-3):126-137. Epub 2021 Oct 25 doi: 10.1159/000519298. PMID: 34695834
Eggers S, Sadedin S, van den Bergen JA, Robevska G, Ohnesorg T, Hewitt J, Lambeth L, Bouty A, Knarston IM, Tan TY, Cameron F, Werther G, Hutson J, O'Connell M, Grover SR, Heloury Y, Zacharin M, Bergman P, Kimber C, Brown J, Webb N, Hunter MF, Srinivasan S, Titmuss A, Verge CF, Mowat D, Smith G, Smith J, Ewans L, Shalhoub C, Crock P, Cowell C, Leong GM, Ono M, Lafferty AR, Huynh T, Visser U, Choong CS, McKenzie F, Pachter N, Thompson EM, Couper J, Baxendale A, Gecz J, Wheeler BJ, Jefferies C, MacKenzie K, Hofman P, Carter P, King RI, Krausz C, van Ravenswaaij-Arts CM, Looijenga L, Drop S, Riedl S, Cools M, Dawson A, Juniarto AZ, Khadilkar V, Khadilkar A, Bhatia V, Dũng VC, Atta I, Raza J, Thi Diem Chi N, Hao TK, Harley V, Koopman P, Warne G, Faradz S, Oshlack A, Ayers KL, Sinclair AH
Genome Biol 2016 Nov 29;17(1):243. doi: 10.1186/s13059-016-1105-y. PMID: 27899157Free PMC Article
Zenteno-Ruiz JC, Kofman-Alfaro S, Méndez JP
Arch Med Res 2001 Nov-Dec;32(6):559-66. doi: 10.1016/s0188-4409(01)00322-8. PMID: 11750731

Recent systematic reviews

Phadte A, Dhole C, Hegishte S, Sarathi V, Lila A, Gada JV, Memon SS, Arya S, Karlekar M, Patil V, Varthakavi PK, Shah N, Bhagwat NM, Bandgar T
Clin Endocrinol (Oxf) 2024 May;100(5):431-440. Epub 2024 Feb 18 doi: 10.1111/cen.15032. PMID: 38368602
Barros BA, Guaragna MS, Fabbri-Scallet H, Palandi de Mello M, Guerra-Júnior G, Maciel-Guerra AT
Sex Dev 2022;16(4):242-251. Epub 2023 Jan 19 doi: 10.1159/000526036. PMID: 36657429

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