{"id":7317,"date":"2025-09-24T18:56:13","date_gmt":"2025-09-24T18:56:13","guid":{"rendered":"https:\/\/polikistikover.net\/yeni\/?page_id=7317"},"modified":"2025-12-22T01:54:20","modified_gmt":"2025-12-22T01:54:20","slug":"polikistik-over-sendromuna-en-cok-benzeyen-hastalik-adrenal-hiperplazi","status":"publish","type":"page","link":"https:\/\/polikistikover.net\/yeni\/polikistik-over-sendromuna-en-cok-benzeyen-hastalik-adrenal-hiperplazi\/","title":{"rendered":"Polikistik Over Sendromu\u2019na En \u00c7ok Benzeyen Hastal\u0131k: Adrenal Hiperplazi"},"content":{"rendered":"\n

Polikistik over sendromu ile b\u00f6brek \u00fcst\u00fc bezinin fazla \u00e7al\u0131\u015fmas\u0131 (Congenital Adrenal Hiperplasia) ancak kan testi veya genetik test ile ay\u0131rt edilebiliyor! Muayene ile ay\u0131rt edilmesi m\u00fcmk\u00fcn de\u011fildir! Tedavi y\u00f6ntemleri tamamen birbirinden farkl\u0131 olan bu iki hastal\u0131\u011f\u0131n herhangi bir tedaviye ba\u015flamadan \u00f6nce mutlaka ayr\u0131m\u0131n\u0131n yap\u0131lmas\u0131 gerekiyor. <\/mark><\/strong><\/p>\n\n\n\n

1-<\/mark><\/strong>Ergenlik d\u00f6neminden itibaren \u00fcreme \u00e7a\u011f\u0131ndaki kad\u0131nlarda erkeklik hormonlar\u0131n\u0131n miktar\u0131n\u0131n veya etkinli\u011finin art\u0131\u015f\u0131na ba\u011fl\u0131 olarak\u00a0t\u00fcylenme art\u0131\u015f\u0131,\u00a0sivilce,\u00a0erkek tipi sa\u00e7 d\u00f6k\u00fclmesi,\u00a0meme geli\u015fiminin durmas\u0131,\u00a0kas miktar\u0131n\u0131n art\u0131\u015f\u0131,\u00a0cinsel istek art\u0131\u015f\u0131,\u00a0klitoris b\u00fcy\u00fcmesi\u00a0ve\u00a0<\/em>ses kal\u0131nla\u015fmas\u0131 gibi belirtilerin polikistik over sendromu\u2019ndan sonra en \u00e7ok g\u00f6r\u00fclen nedeni do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezinin fazla \u00e7al\u0131\u015fmas\u0131d\u0131r (Congenital Adrenal Hiperplasia)\u00a0(1,2,3). B\u00f6brek \u00fcst\u00fc bezinde kortizol ve aldosteron hormonlar\u0131n\u0131n yap\u0131m\u0131n\u0131 sa\u011flayan 21- Hidroksilaz enziminin do\u011fu\u015ftan (congenital) az\u00a0\u00fcretilmesi ya da hi\u00e7 \u00fcretilmemesi b\u00f6brek \u00fcst\u00fc bezinde erkeklik hormonu \u00fcretiminde kullan\u0131lan 17-OH progesteron hormonu miktar\u0131n\u0131n artmas\u0131na neden olmaktad\u0131r. B\u00f6brek \u00fcst\u00fc bezinde fazla miktarda \u00fcretilen\u00a017-OH progesteron hormonu da erkeklik hormonlar\u0131na d\u00f6n\u00fc\u015fmektedir.<\/p>\n\n\n\n

2-<\/mark><\/strong>B\u00f6brek \u00fcst\u00fc bezinin fazla \u00e7al\u0131\u015fmas\u0131na neden olan 21- Hidroksilaz enziminin do\u011fu\u015ftan (congenital) az \u00fcretilmesine (Congenital Adrenal Hiperplasia) birden fazla genetik de\u011fi\u015fiklik (mutasyon) neden olmaktad\u0131r (4,5,6). Genetik de\u011fi\u015fikli\u011fe (mutasyon) g\u00f6re ortaya \u00e7\u0131kan belirtiler ve hastal\u0131\u011f\u0131n \u015fiddeti de\u011fi\u015fmektedir. Fiziksel ya da psikolojik stres durumunda salg\u0131lanan kortizol hormonu ve su ve tuz (sodyum) tutulumunu sa\u011flayan aldosteron hormonu \u00fcretiminin azalmas\u0131na veya hi\u00e7 \u00fcretilmemesine neden olan genetik de\u011fi\u015fikliklerin (mutasyon) neden oldu\u011fu do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezinin fazla \u00e7al\u0131\u015fmas\u0131 (Congenital Adrenal Hiperplasia) polikistik over sendromu\u2019ndan \u00e7ok farkl\u0131 olup yenido\u011fan k\u0131zlarda g\u00f6r\u00fclmektedir (7,8).<\/p>\n\n\n\n

3-<\/mark><\/strong>B\u00f6brek \u00fcst\u00fc bezinde \u00fcretilen erkeklik hormonlar\u0131n\u0131n etkinli\u011finin ve miktar\u0131n\u0131n erken d\u00f6nemde artmas\u0131na ba\u011fl\u0131 olarak k\u0131z \u00e7ocuklar\u0131nda koltuk alt\u0131 ve genital b\u00f6lge k\u0131llanmas\u0131 ile birlikte ter bezlerinde koku art\u0131\u015f\u0131n\u0131n 8 ya\u015f\u0131ndan \u00f6nce olmas\u0131na (Premature Pubarche) en \u00e7ok do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezinin fazla \u00e7al\u0131\u015fmas\u0131 (Congenital Adrenal Hiperplasia) neden olmaktad\u0131r (9,10). <\/p>\n\n\n\n

Do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezi fazla \u00e7al\u0131\u015fan kad\u0131nlarda \u00e7ocukluktan itibaren ergenlik d\u00f6neminde klitoris b\u00fcy\u00fcmesi (Klitoromegali) s\u0131kl\u0131kla g\u00f6r\u00fclen di\u011fer bir belirtidir (11).<\/mark><\/strong><\/p>\n\n\n\n

Klitoris b\u00fcy\u00fcmesi erkeklik hormonlar\u0131n\u0131n miktar\u0131n\u0131n ve etkinli\u011finin art\u0131\u015f\u0131n\u0131 g\u00f6steren t\u00fcylenme art\u0131\u015f\u0131, sivilce, erkek tipi sa\u00e7 d\u00f6k\u00fclmesi, meme geli\u015fiminin durmas\u0131, kas miktar\u0131n\u0131n art\u0131\u015f\u0131, cinsel istek art\u0131\u015f\u0131 ve ses kal\u0131nla\u015fmas\u0131 gibi ba\u015fka bir belirti olmadan da tek ba\u015f\u0131na g\u00f6r\u00fclebilmektedir (11).<\/p>\n\n\n\n

4-<\/mark><\/strong>Ergenlik d\u00f6neminden itibaren \u00fcreme \u00e7a\u011f\u0131ndaki do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezi fazla \u00e7al\u0131\u015fan kad\u0131nlarda genellikle t\u00fcylenme art\u0131\u015f\u0131, sivilce ve erkek tipi sa\u00e7 d\u00f6k\u00fclmesi g\u00f6r\u00fclmektedir (5,11,12). Polikistik over sendromu\u2019nda oldu\u011fu gibi do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezi fazla \u00e7al\u0131\u015fan kad\u0131nlar\u0131n bir k\u0131sm\u0131nda da yumurta geli\u015fimi ve yumurtlaman\u0131n olmamas\u0131na veya gecikmeli olmas\u0131na ba\u011fl\u0131 olarak adet kanamas\u0131 gecikmekte veya olmamaktad\u0131r (11,13). Do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezi fazla \u00e7al\u0131\u015fan ve \u00e7ocuk isteyen kad\u0131nlar\u0131n bir k\u0131sm\u0131nda yumurta geli\u015fimi ve yumurtlama sorunu nedeniyle gebelik olu\u015fumunda zorluk ya\u015fanmaktad\u0131r (5,11,14). Polikistik over sendromu\u2019nda oldu\u011fu gibi do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezi fazla \u00e7al\u0131\u015fan kad\u0131nlar\u0131n bir k\u0131sm\u0131n\u0131n yumurtal\u0131klar\u0131nda polikistik over g\u00f6r\u00fcn\u00fcm\u00fc vard\u0131r (12). <\/p>\n\n\n\n

Bulgu ve belirtilerin benzerli\u011fi nedeniyle polikistik over sendromu oldu\u011fu d\u00fc\u015f\u00fcn\u00fclen her kad\u0131n\u0131n do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezlerinin fazla \u00e7al\u0131\u015f\u0131p \u00e7al\u0131\u015fmad\u0131\u011f\u0131 17-OH progesteron hormonu miktar\u0131 \u00f6l\u00e7\u00fcm\u00fc ve\/veya ACTH stim\u00fclasyon testi ve\/veya genetik inceleme yap\u0131larak belirlenmelidir.<\/mark><\/strong><\/p>\n\n\n\n

5-<\/mark><\/strong>Genetik de\u011fi\u015fiklikler (mutasyon) nedeniyle do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezi fazla \u00e7al\u0131\u015fan kad\u0131nlar\u0131n k\u00fc\u00e7\u00fck bir k\u0131sm\u0131nda ise hi\u00e7 bir belirti g\u00f6r\u00fclmemekte olup ancak bu kad\u0131nlar\u0131n k\u0131zlar\u0131nda hastal\u0131k tespit edildikten sonra yap\u0131lan genetik incelemede do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezlerinin fazla \u00e7al\u0131\u015ft\u0131\u011f\u0131 ortaya \u00e7\u0131kmaktad\u0131r (15).<\/p>\n\n\n\n

 Do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezi fazla \u00e7al\u0131\u015fan kad\u0131nlarda b\u00f6brek \u00fcst\u00fc bezi kitlelerine (adenoma) daha \u00e7ok rastlanmaktad\u0131r. Bu nedenle herhangi bir nedenle b\u00f6brek \u00fcst\u00fc bezinde kitle (incidentaloma) saptanan kad\u0131nlarda do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezinin fazla \u00e7al\u0131\u015f\u0131p \u00e7al\u0131\u015fmad\u0131\u011f\u0131 belirlenmelidir (16).<\/mark><\/strong><\/p>\n\n\n

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Do\u011fu\u015ftan b\u00f6brek \u00fcst\u00fc bezinin fazla \u00e7al\u0131\u015fmas\u0131na (Congenital adrenal hyperplasia) genetik de\u011fi\u015fiklik (mutasyon) neden olmaktad\u0131r.<\/em><\/p>\n\n\n\n

Kaynaklar<\/strong><\/p>\n\n\n\n

1-Virilisme cliniquement tardif avec excretion de pregnanetriol et insufisance de la production du cortisol. Decourt MJ, Jayle MF, Baulieu E. Ann Endocrinol 1957;18:416.<\/p>\n\n\n\n

2-High frequency of nonclassical steroid 21-hydroxylase deficiency. Speiser PW, Dupont B, Rubinstein P, Piazza A, Kastelan A, New MI. Am J Hum Genet. 1985 Jul;37(4):650-67.<\/p>\n\n\n\n

3-Androgen excess in women: experience with over 1000 consecutive patients. Azziz R, Sanchez LA, Knochenhauer ES, Moran C, Lazenby J, Stephens KC, Taylor K, Boots LR. J Clin Endocrinol Metab. 2004 Feb;89(2):453-62.<\/p>\n\n\n\n

4-A multicenter study of women with nonclassical congenital adrenal hyperplasia: relationship between genotype and phenotype. Speiser PW, Knochenhauer ES, Dewailly D, Fruzzetti F, Marcondes JA, Azziz R. Mol Genet Metab. 2000 Nov;71(3):527-34.<\/p>\n\n\n\n

5-Clinical and molecular characterization of a cohort of 161 unrelated women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and 330 family members. Bidet M, Bellann\u00e9-Chantelot C, Galand-Portier MB, Tardy V, Billaud L, Laborde K, Coussieu C, Morel Y, Vaury C, Golmard JL,Claustre A, Mornet E, Chakhtoura Z, Mowszowicz I, Bachelot A, Touraine P, Kuttenn F. J Clin Endocrinol Metab. 2009May;94(5):1570-8.<\/p>\n\n\n\n

6-In vitro functional studies of rare CYP21A2 mutations and establishment of an activity gradient for nonclassic mutations improve phenotype predictions in congenital adrenal hyperplasia. Clin Endocrinol (Oxf). 2015 Jan;82(1):37-44. Barbaro M, Soardi FC, \u00d6stberg LJ, Persson B, de Mello MP, Wedell A, Lajic S.<\/p>\n\n\n\n

7-Adrenal insufficiency. Charmandari E, Nicolaides NC, Chrousos GP.Lancet. 2014 Jun 21;383(9935):2152-67.<\/p>\n\n\n\n

8-Congenital adrenal hyperplasia due to 21 hydroxylase deficiency: from birth to adulthood. White PC , Bachega TA. Semin Reprod Med. 2012 Oct;30(5):400-9.<\/p>\n\n\n\n

9-Follow-up study of adolescent girls with a history of premature pubarche. Miller D, Emans SJ, Kohane I. J Adolesc Health. 1996 Apr;18(4):301-5.<\/p>\n\n\n\n

10-Precocious pubarche: distinguishing late-onset congenital adrenal hyperplasia from premature adrenarche. Armengaud JB, Charkaluk ML, Trivin C, Tardy V, Br\u00e9art G, Brauner R, Chalumeau M. J Clin Endocrinol Metab. 2009 Aug;94(8):2835-40.<\/p>\n\n\n\n

11-21-Hydroxylase-deficient nonclassic adrenal hyperplasia is a progressive disorder: a multicenter study. Moran C, Azziz R, Carmina E, Dewailly D, Fruzzetti F, Iba\u00f1ez L, Knochenhauer ES, Marcondes JA, Mendonca BB, Pignatelli D,Pugeat M, Rohmer V, Speiser PW, Witchel SF. Am J Obstet Gynecol. 2000 Dec;183(6):1468-74.<\/p>\n\n\n\n

12-The phenotype of hirsute women: a comparison of polycystic ovary syndrome and 21-hydroxylase-deficient nonclassic adrenal hyperplasia. Pall M, Azziz R, Beires J, Pignatelli D. Fertil Steril. 2010 Jul;94(2):684-9.<\/p>\n\n\n\n

13-The spectrum of clinical, hormonal and molecular findings in 280 individuals with nonclassical congenital adrenal hyperplasia caused by mutations of the CYP21A2 gene. Livadas S, Dracopoulou M, Dastamani A, Sertedaki A, Maniati-Christidi M, Magiakou AM, Kanaka-Gantenbein C, Chrousos GP,Dacou-Voutetakis C. Clin Endocrinol (Oxf). 2015 Apr;82(4):543-9.<\/p>\n\n\n\n

14-Fertility in patients with congenital adrenal hyperplasia. Reichman DE , White PC, New MI, Rosenwaks Z. Fertil Steril. 2014 Feb;101(2):301-9.<\/p>\n\n\n\n

15-Phenotypic profiling of parents with cryptic nonclassic congenital adrenal hyperplasia: findings in 145 unrelated families. Nandagopal R, Sinaii N, Avila NA, Van Ryzin C, Chen W, Finkielstain GP, Mehta SP, McDonnell NB, Merke DP. Eur J Endocrinol. 2011 Jun;164(6):977-84.<\/p>\n\n\n\n

16-Congenital adrenal hyperplasia due to 21- hydroxylase deficiency presenting as adrenal incidentaloma: A systematic review and meta- analysis. Falhammar H, Torpy DJ. Endocr Pract. 2016 Jun;22(6):736-52.<\/p>\n","protected":false},"excerpt":{"rendered":"

Polikistik over sendromu ile b\u00f6brek \u00fcst\u00fc bezinin fazla \u00e7al\u0131\u015fmas\u0131 (Congenital Adrenal Hiperplasia) ancak kan testi veya genetik test ile ay\u0131rt edilebiliyor! Muayene ile ay\u0131rt edilmesi m\u00fcmk\u00fcn de\u011fildir! Tedavi y\u00f6ntemleri tamamen birbirinden farkl\u0131 olan bu iki hastal\u0131\u011f\u0131n herhangi bir tedaviye ba\u015flamadan \u00f6nce mutlaka ayr\u0131m\u0131n\u0131n yap\u0131lmas\u0131 gerekiyor. 1-Ergenlik d\u00f6neminden itibaren \u00fcreme \u00e7a\u011f\u0131ndaki kad\u0131nlarda erkeklik hormonlar\u0131n\u0131n miktar\u0131n\u0131n veya […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-7317","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/polikistikover.net\/yeni\/wp-json\/wp\/v2\/pages\/7317","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/polikistikover.net\/yeni\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/polikistikover.net\/yeni\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/polikistikover.net\/yeni\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/polikistikover.net\/yeni\/wp-json\/wp\/v2\/comments?post=7317"}],"version-history":[{"count":21,"href":"https:\/\/polikistikover.net\/yeni\/wp-json\/wp\/v2\/pages\/7317\/revisions"}],"predecessor-version":[{"id":10399,"href":"https:\/\/polikistikover.net\/yeni\/wp-json\/wp\/v2\/pages\/7317\/revisions\/10399"}],"wp:attachment":[{"href":"https:\/\/polikistikover.net\/yeni\/wp-json\/wp\/v2\/media?parent=7317"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}