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Ovarian dysgenesis 11(ODG11)

MedGen UID:
1856331
•Concept ID:
C5935637
•
Disease or Syndrome
Synonyms: ODG11; OVARIAN DYSGENESIS 11
 
Gene (location): HROB (17q21.31)
 
Monarch Initiative: MONDO:0971176
OMIM®: 620897

Definition

Ovarian dysgenesis-11 (ODG11) is characterized by primary amenorrhea and delayed puberty, with absent ovaries on pelvic ultrasound (Tucker et al., 2022; Wu et al., 2023). For a discussion of genetic heterogeneity of ovarian dysgenesis, see ODG1 (233300). [from OMIM]

Professional guidelines

PubMed

Metformin treatment before and during IVF or ICSI in women with polycystic ovary syndrome.
Tso LO, Costello MF, Albuquerque LET, Andriolo RB, Macedo CR
Cochrane Database Syst Rev 2020 Dec 21;12(12):CD006105. doi: 10.1002/14651858.CD006105.pub4. PMID: 33347618Free PMC Article
Editorial: Congenital Adrenal Hyperplasia, Unresolved Issues and Implications on Clinical Management.
Livadas S, Stratakis CA, Macut D
Front Endocrinol (Lausanne) 2020;11:170. Epub 2020 Mar 31 doi: 10.3389/fendo.2020.00170. PMID: 32296393Free PMC Article
AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS, AMERICAN COLLEGE OF ENDOCRINOLOGY, AND ANDROGEN EXCESS AND PCOS SOCIETY DISEASE STATE CLINICAL REVIEW: GUIDE TO THE BEST PRACTICES IN THE EVALUATION AND TREATMENT OF POLYCYSTIC OVARY SYNDROME--PART 1.
Goodman NF, Cobin RH, Futterweit W, Glueck JS, Legro RS, Carmina E; American Association of Clinical Endocrinologists (AACE); American College of Endocrinology (ACE); Androgen Excess and PCOS Society (AES)
Endocr Pract 2015 Nov;21(11):1291-300. doi: 10.4158/EP15748.DSC. PMID: 26509855

Recent clinical studies

Etiology

Polycystic ovary syndrome.
Stener-Victorin E, Teede H, Norman RJ, Legro R, Goodarzi MO, Dokras A, Laven J, Hoeger K, Piltonen TT
Nat Rev Dis Primers 2024 Apr 18;10(1):27. doi: 10.1038/s41572-024-00511-3. PMID: 38637590
Turner Syndrome and Fertility.
Viuff M, Gravholt CH
Ann Endocrinol (Paris) 2022 Aug;83(4):244-249. Epub 2022 Jun 18 doi: 10.1016/j.ando.2022.06.001. PMID: 35728697
Metformin treatment before and during IVF or ICSI in women with polycystic ovary syndrome.
Tso LO, Costello MF, Albuquerque LET, Andriolo RB, Macedo CR
Cochrane Database Syst Rev 2020 Dec 21;12(12):CD006105. doi: 10.1002/14651858.CD006105.pub4. PMID: 33347618Free PMC Article
Turner syndrome and osteoporosis.
Augoulea A, Zachou G, Lambrinoudaki I
Maturitas 2019 Dec;130:41-49. Epub 2019 Sep 25 doi: 10.1016/j.maturitas.2019.09.010. PMID: 31706435
AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS, AMERICAN COLLEGE OF ENDOCRINOLOGY, AND ANDROGEN EXCESS AND PCOS SOCIETY DISEASE STATE CLINICAL REVIEW: GUIDE TO THE BEST PRACTICES IN THE EVALUATION AND TREATMENT OF POLYCYSTIC OVARY SYNDROME--PART 1.
Goodman NF, Cobin RH, Futterweit W, Glueck JS, Legro RS, Carmina E; American Association of Clinical Endocrinologists (AACE); American College of Endocrinology (ACE); Androgen Excess and PCOS Society (AES)
Endocr Pract 2015 Nov;21(11):1291-300. doi: 10.4158/EP15748.DSC. PMID: 26509855

Diagnosis

Polycystic ovary syndrome.
Stener-Victorin E, Teede H, Norman RJ, Legro R, Goodarzi MO, Dokras A, Laven J, Hoeger K, Piltonen TT
Nat Rev Dis Primers 2024 Apr 18;10(1):27. doi: 10.1038/s41572-024-00511-3. PMID: 38637590
Androgen excess: a hallmark of polycystic ovary syndrome.
Wang K, Li Y, Chen Y
Front Endocrinol (Lausanne) 2023;14:1273542. Epub 2023 Dec 13 doi: 10.3389/fendo.2023.1273542. PMID: 38152131Free PMC Article
Polycystic Ovarian Syndrome.
Shrivastava S, Conigliaro RL
Med Clin North Am 2023 Mar;107(2):227-234. Epub 2022 Dec 26 doi: 10.1016/j.mcna.2022.10.004. PMID: 36759093
A brief insight into the etiology, genetics, and immunology of polycystic ovarian syndrome (PCOS).
Siddiqui S, Mateen S, Ahmad R, Moin S
J Assist Reprod Genet 2022 Nov;39(11):2439-2473. Epub 2022 Oct 3 doi: 10.1007/s10815-022-02625-7. PMID: 36190593Free PMC Article
Polycystic ovary syndrome in adult women.
Ortiz-Flores AE, Luque-Ramírez M, Escobar-Morreale HF
Med Clin (Barc) 2019 Jun 7;152(11):450-457. Epub 2019 Jan 4 doi: 10.1016/j.medcli.2018.11.019. PMID: 30616916

Therapy

Artemisinins ameliorate polycystic ovarian syndrome by mediating LONP1-CYP11A1 interaction.
Liu Y, Jiang JJ, Du SY, Mu LS, Fan JJ, Hu JC, Ye Y, Ding M, Zhou WY, Yu QH, Xia YF, Xu HY, Shi YJ, Qian SW, Tang Y, Li W, Dang YJ, Dong X, Li XY, Xu CJ, Tang QQ
Science 2024 Jun 14;384(6701):eadk5382. doi: 10.1126/science.adk5382. PMID: 38870290
A brief insight into the etiology, genetics, and immunology of polycystic ovarian syndrome (PCOS).
Siddiqui S, Mateen S, Ahmad R, Moin S
J Assist Reprod Genet 2022 Nov;39(11):2439-2473. Epub 2022 Oct 3 doi: 10.1007/s10815-022-02625-7. PMID: 36190593Free PMC Article
Turner Syndrome and Fertility.
Viuff M, Gravholt CH
Ann Endocrinol (Paris) 2022 Aug;83(4):244-249. Epub 2022 Jun 18 doi: 10.1016/j.ando.2022.06.001. PMID: 35728697
Metformin treatment before and during IVF or ICSI in women with polycystic ovary syndrome.
Tso LO, Costello MF, Albuquerque LET, Andriolo RB, Macedo CR
Cochrane Database Syst Rev 2020 Dec 21;12(12):CD006105. doi: 10.1002/14651858.CD006105.pub4. PMID: 33347618Free PMC Article
AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS, AMERICAN COLLEGE OF ENDOCRINOLOGY, AND ANDROGEN EXCESS AND PCOS SOCIETY DISEASE STATE CLINICAL REVIEW: GUIDE TO THE BEST PRACTICES IN THE EVALUATION AND TREATMENT OF POLYCYSTIC OVARY SYNDROME--PART 1.
Goodman NF, Cobin RH, Futterweit W, Glueck JS, Legro RS, Carmina E; American Association of Clinical Endocrinologists (AACE); American College of Endocrinology (ACE); Androgen Excess and PCOS Society (AES)
Endocr Pract 2015 Nov;21(11):1291-300. doi: 10.4158/EP15748.DSC. PMID: 26509855

Prognosis

Markers of Fertility in Adolescents With Chronic Endocrinopathies at Transition From Paediatric to Adult Care.
Choukair D, Mittnacht J, Bettendorf M
Endocrinol Diabetes Metab 2024 Jul;7(4):e00493. doi: 10.1002/edm2.493. PMID: 38845445Free PMC Article
Growth During Pubertal Induction in Girls With Turner Syndrome: A Retrospective Cohort Analysis.
Nicholls J, Harris M, Hughes I, Huynh T, McMahon SK
J Clin Endocrinol Metab 2024 Oct 15;109(11):e1992-e2000. doi: 10.1210/clinem/dgae071. PMID: 38332670
Turner Syndrome and Fertility.
Viuff M, Gravholt CH
Ann Endocrinol (Paris) 2022 Aug;83(4):244-249. Epub 2022 Jun 18 doi: 10.1016/j.ando.2022.06.001. PMID: 35728697
Editorial: Congenital Adrenal Hyperplasia, Unresolved Issues and Implications on Clinical Management.
Livadas S, Stratakis CA, Macut D
Front Endocrinol (Lausanne) 2020;11:170. Epub 2020 Mar 31 doi: 10.3389/fendo.2020.00170. PMID: 32296393Free PMC Article
AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS, AMERICAN COLLEGE OF ENDOCRINOLOGY, AND ANDROGEN EXCESS AND PCOS SOCIETY DISEASE STATE CLINICAL REVIEW: GUIDE TO THE BEST PRACTICES IN THE EVALUATION AND TREATMENT OF POLYCYSTIC OVARY SYNDROME--PART 1.
Goodman NF, Cobin RH, Futterweit W, Glueck JS, Legro RS, Carmina E; American Association of Clinical Endocrinologists (AACE); American College of Endocrinology (ACE); Androgen Excess and PCOS Society (AES)
Endocr Pract 2015 Nov;21(11):1291-300. doi: 10.4158/EP15748.DSC. PMID: 26509855

Clinical prediction guides

Maternal diseases and congenital anomalies of the kidney and urinary tract in offspring: a cohort study.
Ma Q, Li YQ, Meng QT, Yang B, Zhang HT, Shi H, Liu CY, Xiang TC, Zhao N, Rao J
World J Pediatr 2024 Nov;20(11):1168-1178. Epub 2024 Jul 6 doi: 10.1007/s12519-024-00822-1. PMID: 38970731Free PMC Article
Markers of Fertility in Adolescents With Chronic Endocrinopathies at Transition From Paediatric to Adult Care.
Choukair D, Mittnacht J, Bettendorf M
Endocrinol Diabetes Metab 2024 Jul;7(4):e00493. doi: 10.1002/edm2.493. PMID: 38845445Free PMC Article
Ovarian reserve and fertility parameters in post-pubertal females with congenital adrenal hyperplasia: a case-control study.
Nawar M, Mohammad MS, Shabaan A, Elsedfy H
J Pediatr Endocrinol Metab 2024 Apr 25;37(4):336-340. Epub 2024 Mar 11 doi: 10.1515/jpem-2023-0462. PMID: 38459773
Ovotesticular cords and ovotesticular follicles: New histologic markers for human ovotesticular syndrome.
Baskin LS, Cao M, Li Y, Baker L, Cooper CS, Cunha GR
J Pediatr Urol 2024 Oct;20(5):799-809. Epub 2024 Jan 6 doi: 10.1016/j.jpurol.2023.12.016. PMID: 38218629
AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS, AMERICAN COLLEGE OF ENDOCRINOLOGY, AND ANDROGEN EXCESS AND PCOS SOCIETY DISEASE STATE CLINICAL REVIEW: GUIDE TO THE BEST PRACTICES IN THE EVALUATION AND TREATMENT OF POLYCYSTIC OVARY SYNDROME--PART 1.
Goodman NF, Cobin RH, Futterweit W, Glueck JS, Legro RS, Carmina E; American Association of Clinical Endocrinologists (AACE); American College of Endocrinology (ACE); Androgen Excess and PCOS Society (AES)
Endocr Pract 2015 Nov;21(11):1291-300. doi: 10.4158/EP15748.DSC. PMID: 26509855

Recent systematic reviews

Anatomical variations, treatment and outcomes of Herlyn-Werner-Wunderlich syndrome: a literature review of 1673 cases.
Liu Y, Li Z, Dou Y, Wang J, Li Y
Arch Gynecol Obstet 2023 Nov;308(5):1409-1417. Epub 2023 Feb 24 doi: 10.1007/s00404-022-06856-y. PMID: 36823415
Anti-Müllerian hormone levels in the diagnosis of adolescent polycystic ovarian syndrome: a systematic review and meta-analysis.
Tsukui Y, Kitahara Y, Hasegawa Y, Kobayashi M, Osuka S, Iwase A
Endocr J 2022 Aug 29;69(8):897-906. Epub 2022 Jun 8 doi: 10.1507/endocrj.EJ22-0081. PMID: 35675999
Metformin treatment before and during IVF or ICSI in women with polycystic ovary syndrome.
Tso LO, Costello MF, Albuquerque LET, Andriolo RB, Macedo CR
Cochrane Database Syst Rev 2020 Dec 21;12(12):CD006105. doi: 10.1002/14651858.CD006105.pub4. PMID: 33347618Free PMC Article
Ovarian Stimulation, Intrauterine Insemination, Multiple Pregnancy and Major Congenital Malformations: A Systematic Review and Meta- Analysis- The ART_Rev Study.
Chaabane S, Sheehy O, Monnier P, Fraser W, Bissonnette F, Trasler JM, Muanda FT, Boukhris T, Karam F, Santos F, Blais L, Bérard A
Curr Drug Saf 2016;11(3):222-61. doi: 10.2174/1574886311666160627094051. PMID: 27484228
Use of platinum derivatives during pregnancy.
Mir O, Berveiller P, Ropert S, Goffinet F, Goldwasser F
Cancer 2008 Dec 1;113(11):3069-74. doi: 10.1002/cncr.23935. PMID: 18985677

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