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Journals / Türkiye Çocuk Hastalıkları Dergisi / 2009 / Cilt: 3 - Sayı: 2

Mayer-rokitansky-kuster-hauser and polycystic ovary syndrome assocciation

Mayer-rokitansky-kuster-hauser ve polikistik over sendromu birlikteliği

Ayça KUYBULU
Baha ORAL
Bumin DÜNDAR
Pages
35–39
Publication Date
2022-11-05
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Here we presented a 13 years old female patient who was admitted to our outpatient clinic because of short stature, amenorrhea and hirsutism and diagnosed as MRKH syndrome and polycystic ovary syndrome.

📌 3 Key Takeaways

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Objective & Hypothesis:Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome is a disorder characterized by utero-vaginal agenesis which result from interrupted embryonic development of the mullerian ducts.
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Key Findings & Data:The incidence in girls is approximately 1/4500.
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Conclusion & Impact:Here we presented a 13 years old female patient who was admitted to our outpatient clinic because of short stature, amenorrhea and hirsutism and diagnosed as MRKH syndrome and polycystic ovary syndrome.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome is a disorder characterized by utero-vaginal agenesis which result from interrupted embryonic development of the mullerian ducts. The incidence in girls is approximately 1/4500. It is the second most common cause of primary amenorhea.Ovarian anomalies may be seen especially in atypical form of the disease, but the association of polycystic ovary syndrome and MRKH syndrome has been reported extremely rare in the literature. Here we presented a 13 years old female patient who was admitted to our outpatient clinic because of short stature, amenorrhea and hirsutism and diagnosed as MRKH syndrome and polycystic ovary syndrome.

Özet

Mayer-Rokitansky-Kuster-Hauser (MRKH) Sendromu, müller kanallarının gelişimsel bozukluğu sonrası ortaya çıkan utero-vajinal atrezi ile karakterize bir hastalıktır. Kızlarda görülme sıklığı yaklaşık olarak 1/4500 canlı doğumdur. Sekonder amenorenin en sık rastlanan ikinci nedenidir.Özellikle atipik MRKH sendromunda over anomalileri saptanabilir, fakat polikistik over sendromu ile MRKH sendromu birlikteliği literatürde oldukça nadir olarak bildirilmiştir. Burada kliniğimize kısa boy, amenore ve hirsutizm yakınmaları ile başvurup MRKH sendromu ve polikistik over sendromu tanısı alan 13 yaşında kız olgu sunulmuştur.

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Cite This Article

KUYBULU, A., ORAL, B., DÜNDAR, B. (2022). Mayer-rokitansky-kuster-hauser and polycystic ovary syndrome assocciation. Türkiye Çocuk Hastalıkları Dergisi, Cilt: 3 - Sayı: 2, 35–39. https://ajindex.com/articles/627702

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References (13)

  1. Oppelt P, Renner SP, Kellermann A, Brucker S, Hauser GA, Ludwig KS, Strissel PL, Strick R, Wallwiener D, Beckmann MW. Clinical aspects of Mayer–Rokitansky–Kuester–Hauser syndrome: recommendations for clinical diagnosis and staging. Human Reproduction 2006; 21: 792–797
  2. Guerrier D, Mouchel T, Pasquier L, Pellerin I. The Mayer-RokitanskyKüster-Hauser syndrome (congenital absence of uterus and vagina) – phenotypic manifestations and genetic approaches. J Negat Results Biomed 2006;27:5-11
  3. Plevraki E, Kita M, Goulis DG, Hatnisevastou-Loukidou H, Lambropoulos AF, avramides A. Bilateral ovarian agenesis and the presence of the testis-specific protein 1-Y-linked gene: two new features of Mayer-Rokitansky-Kuster-Hauser syndrome. Fertil Steril 2004;81:689-692
  4. Takeuchi K, Oomori S, Oda N, Maeda K, Kaji Y, Maro T. Coexistence of Mayer-Rokitansky-Kustner-Hauser syndrome and yolk sac tumor of the ovary in a prepubertal girl. Acta Obstet Gynecol Scand 2006;85:245-247
  5. Griffin JE, Edwards C, Madden JD, Harrod MJ and Wilson JD. Congenital absence of the vagina. The Mayer-Rokitansky-Kuster-Hauser syndrome. Ann Intern Med 1976; 85,224–236
  6. Schmid-Tannwald I and Hauser GA. [Atypical forms of the Mayer– Rokitansky–Kuester syndrome (author’s transl)]. Geburtshilfe Frauenheilkd 1977; 37:386–392
  7. Duncan PA, Shapiro LR, Stangel JJ, Klein RM, Addonizio JC. The MURCS association: Müllerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. J Pediatr 1979; 95:399–402
  8. Pellicano M, Penner I, Connola D, Cascone D, Sorrentino V, Gargano V, Masucci A, Stella N, Nappi C. Voluminous ectopic polycystic ovaries in Mayer-Rokitansky-Kuster-Hauser syndrome. Minerva Ginecol. 2005;57:655-658
  9. Ugur M, Karakaya S, Zorlu G, Arslan S, Gülerman C, Kükner S, Gökmen O. Polycystic ovaries in association with mullerian anomalies. Eur J Obstet Gynecol Reprod Biol 1995; 62:57-59
  10. Appelman Z, Hazan Y, Hagay Z. High prevalence of müllerian anomalies diagnosed by ultrasound in women with polycystic ovaries. J Reprod Med 2003; 48:362-364
  11. Biason-Lauber A, Konrad, D, Navratil F, Schoenle EJ. A WNT4 mutation associated with Mullerian-duct regression and virilization in a 46,XX woman. New Eng J Med 2004;351:792-798
  12. Resendes BL, Sohn SH, Stelling JR, Tineo R, Davis AJ, Gray MR, Reindollar RH. Role for anti-Mullerian hormone in congenital absence of the uterus and vagina. Am J Med Genet 2001; 98:129–136
  13. Oppelt P, Strissel PL, Kellermann A, Seeber S, Hum