Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study

Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population.Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014.Results: The most common karyotype was 45,X (50.7%), followed by 45,X/46,XX (10.8%), 46,X,i(Xq) (10.1%) and 45,X/46,X,i(Xq) (9.5%). Mean age at diagnosis was 10.2±4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45,X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosis) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto's thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%.Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespan

Anahtar Kelime:

Konular: Endokrinoloji ve Metabolizma Pediatri

Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık

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APA	YEŞİLKAYA E, Bereket A, DARENDELİLER F, BAŞ F, Poyrazoglu S, KÜÇÜKEMRE AYDIN B, darcan s, DÜNDAR B, buyukinan m, KARA C, SARI E, ADAL S, AKINCI A, Atabek M, DEMİREL F, ÇELİK N, ÖZKAN B, ÖZHAN B, Orbak Z, Ersoy B, doğan m, ATAŞ A, Demircioglu S, GÖKŞEN ŞİMŞEK R, TARIM Ö, yuksel b, ERCAN O, HATUN Ş, Şimşek E, ÖKTEN A, ABACI A, DÖNERAY H, Ozbek M, Keskin M, ÖNAL H, akyurek n, BULAN K, TEPE D, EMEKSİZ H, DEMİR K, Özalp Kızılay D, TOPALOGLU A, Eren E, Ozen S, ABALI S, AKIN L, SELVER EKLİOĞLU B, KABA S, ANIK A, BAŞ S, ÜNÜVAR T, Sağlam H, bolu s, ÖZGEN İ, DOĞAN D, ÇAKIR E, ŞEN Y, ANDIRAN N, ÇİZMECİOĞLU F, EVLİYAOĞLU S, Karagüzel G, PİRGON M, Çatlı G, CAN H, gürbüz f, binay c, baş v, FİDANCI M, POLAT A, GÜL D, AÇIKEL C, Demirbilek H, CİNAZ P, BONDY C (2015). Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. , 27 - 36.
Chicago	YEŞİLKAYA Ediz,Bereket Abdullah,DARENDELİLER FATMA FEYZA,BAŞ Firdevs,Poyrazoglu Sukran,KÜÇÜKEMRE AYDIN Banu,darcan sukran,DÜNDAR Bumin Nuri,buyukinan muammer,KARA Cengiz,SARI Erkan,ADAL Servet Erdal,AKINCI Ayşehan,Atabek Mehmet Emre,DEMİREL Fatma,ÇELİK Nurullah,ÖZKAN Behzat,ÖZHAN Bayram,Orbak Zerrin,Ersoy Betül,doğan murat,ATAŞ Ali,Demircioglu Serap,GÖKŞEN ŞİMŞEK RUHSAR DAMLA,TARIM Ömer Faruk,yuksel bilgin,ERCAN OYA,HATUN Şükrü,Şimşek Enver,ÖKTEN Ayşenur,ABACI Ayhan,DÖNERAY Hakan,Ozbek Mehmet Nuri,Keskin Mehmet,ÖNAL Hasan,akyurek nesibe,BULAN Kezban,TEPE Derya,EMEKSİZ Hamdi Cihan,DEMİR Korcan,Özalp Kızılay Deniz,TOPALOGLU ALI,Eren Erdal,Ozen Samim,ABALI SAYGIN,AKIN Leyla,SELVER EKLİOĞLU BERAY,KABA Sultan,ANIK AHMET,BAŞ Serpil,ÜNÜVAR TOLGA,Sağlam Halil,bolu semih,ÖZGEN İlker Tolga,DOĞAN Durmuş,ÇAKIR Esra Deniz,ŞEN Yaşar,ANDIRAN Nesibe,ÇİZMECİOĞLU FİLİZ MİNE,EVLİYAOĞLU SAADET OLCAY,Karagüzel Gülay,PİRGON Mustafa Özgür,Çatlı Gönül,CAN Hatice Dilek,gürbüz fatih,binay cigdem,baş veysel,FİDANCI Muzaffer Kürşat,POLAT Adem,GÜL Davut,AÇIKEL Cengizhan,Demirbilek Huseyin,CİNAZ Peyami,BONDY Carolyn Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. (2015): 27 - 36.
MLA	YEŞİLKAYA Ediz,Bereket Abdullah,DARENDELİLER FATMA FEYZA,BAŞ Firdevs,Poyrazoglu Sukran,KÜÇÜKEMRE AYDIN Banu,darcan sukran,DÜNDAR Bumin Nuri,buyukinan muammer,KARA Cengiz,SARI Erkan,ADAL Servet Erdal,AKINCI Ayşehan,Atabek Mehmet Emre,DEMİREL Fatma,ÇELİK Nurullah,ÖZKAN Behzat,ÖZHAN Bayram,Orbak Zerrin,Ersoy Betül,doğan murat,ATAŞ Ali,Demircioglu Serap,GÖKŞEN ŞİMŞEK RUHSAR DAMLA,TARIM Ömer Faruk,yuksel bilgin,ERCAN OYA,HATUN Şükrü,Şimşek Enver,ÖKTEN Ayşenur,ABACI Ayhan,DÖNERAY Hakan,Ozbek Mehmet Nuri,Keskin Mehmet,ÖNAL Hasan,akyurek nesibe,BULAN Kezban,TEPE Derya,EMEKSİZ Hamdi Cihan,DEMİR Korcan,Özalp Kızılay Deniz,TOPALOGLU ALI,Eren Erdal,Ozen Samim,ABALI SAYGIN,AKIN Leyla,SELVER EKLİOĞLU BERAY,KABA Sultan,ANIK AHMET,BAŞ Serpil,ÜNÜVAR TOLGA,Sağlam Halil,bolu semih,ÖZGEN İlker Tolga,DOĞAN Durmuş,ÇAKIR Esra Deniz,ŞEN Yaşar,ANDIRAN Nesibe,ÇİZMECİOĞLU FİLİZ MİNE,EVLİYAOĞLU SAADET OLCAY,Karagüzel Gülay,PİRGON Mustafa Özgür,Çatlı Gönül,CAN Hatice Dilek,gürbüz fatih,binay cigdem,baş veysel,FİDANCI Muzaffer Kürşat,POLAT Adem,GÜL Davut,AÇIKEL Cengizhan,Demirbilek Huseyin,CİNAZ Peyami,BONDY Carolyn Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. , 2015, ss.27 - 36.
AMA	YEŞİLKAYA E,Bereket A,DARENDELİLER F,BAŞ F,Poyrazoglu S,KÜÇÜKEMRE AYDIN B,darcan s,DÜNDAR B,buyukinan m,KARA C,SARI E,ADAL S,AKINCI A,Atabek M,DEMİREL F,ÇELİK N,ÖZKAN B,ÖZHAN B,Orbak Z,Ersoy B,doğan m,ATAŞ A,Demircioglu S,GÖKŞEN ŞİMŞEK R,TARIM Ö,yuksel b,ERCAN O,HATUN Ş,Şimşek E,ÖKTEN A,ABACI A,DÖNERAY H,Ozbek M,Keskin M,ÖNAL H,akyurek n,BULAN K,TEPE D,EMEKSİZ H,DEMİR K,Özalp Kızılay D,TOPALOGLU A,Eren E,Ozen S,ABALI S,AKIN L,SELVER EKLİOĞLU B,KABA S,ANIK A,BAŞ S,ÜNÜVAR T,Sağlam H,bolu s,ÖZGEN İ,DOĞAN D,ÇAKIR E,ŞEN Y,ANDIRAN N,ÇİZMECİOĞLU F,EVLİYAOĞLU S,Karagüzel G,PİRGON M,Çatlı G,CAN H,gürbüz f,binay c,baş v,FİDANCI M,POLAT A,GÜL D,AÇIKEL C,Demirbilek H,CİNAZ P,BONDY C Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. . 2015; 27 - 36.
Vancouver	YEŞİLKAYA E,Bereket A,DARENDELİLER F,BAŞ F,Poyrazoglu S,KÜÇÜKEMRE AYDIN B,darcan s,DÜNDAR B,buyukinan m,KARA C,SARI E,ADAL S,AKINCI A,Atabek M,DEMİREL F,ÇELİK N,ÖZKAN B,ÖZHAN B,Orbak Z,Ersoy B,doğan m,ATAŞ A,Demircioglu S,GÖKŞEN ŞİMŞEK R,TARIM Ö,yuksel b,ERCAN O,HATUN Ş,Şimşek E,ÖKTEN A,ABACI A,DÖNERAY H,Ozbek M,Keskin M,ÖNAL H,akyurek n,BULAN K,TEPE D,EMEKSİZ H,DEMİR K,Özalp Kızılay D,TOPALOGLU A,Eren E,Ozen S,ABALI S,AKIN L,SELVER EKLİOĞLU B,KABA S,ANIK A,BAŞ S,ÜNÜVAR T,Sağlam H,bolu s,ÖZGEN İ,DOĞAN D,ÇAKIR E,ŞEN Y,ANDIRAN N,ÇİZMECİOĞLU F,EVLİYAOĞLU S,Karagüzel G,PİRGON M,Çatlı G,CAN H,gürbüz f,binay c,baş v,FİDANCI M,POLAT A,GÜL D,AÇIKEL C,Demirbilek H,CİNAZ P,BONDY C Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. . 2015; 27 - 36.
IEEE	YEŞİLKAYA E,Bereket A,DARENDELİLER F,BAŞ F,Poyrazoglu S,KÜÇÜKEMRE AYDIN B,darcan s,DÜNDAR B,buyukinan m,KARA C,SARI E,ADAL S,AKINCI A,Atabek M,DEMİREL F,ÇELİK N,ÖZKAN B,ÖZHAN B,Orbak Z,Ersoy B,doğan m,ATAŞ A,Demircioglu S,GÖKŞEN ŞİMŞEK R,TARIM Ö,yuksel b,ERCAN O,HATUN Ş,Şimşek E,ÖKTEN A,ABACI A,DÖNERAY H,Ozbek M,Keskin M,ÖNAL H,akyurek n,BULAN K,TEPE D,EMEKSİZ H,DEMİR K,Özalp Kızılay D,TOPALOGLU A,Eren E,Ozen S,ABALI S,AKIN L,SELVER EKLİOĞLU B,KABA S,ANIK A,BAŞ S,ÜNÜVAR T,Sağlam H,bolu s,ÖZGEN İ,DOĞAN D,ÇAKIR E,ŞEN Y,ANDIRAN N,ÇİZMECİOĞLU F,EVLİYAOĞLU S,Karagüzel G,PİRGON M,Çatlı G,CAN H,gürbüz f,binay c,baş v,FİDANCI M,POLAT A,GÜL D,AÇIKEL C,Demirbilek H,CİNAZ P,BONDY C "Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study." , ss.27 - 36, 2015.
ISNAD	YEŞİLKAYA, Ediz vd. "Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study". (2015), 27-36.

APA	YEŞİLKAYA E, Bereket A, DARENDELİLER F, BAŞ F, Poyrazoglu S, KÜÇÜKEMRE AYDIN B, darcan s, DÜNDAR B, buyukinan m, KARA C, SARI E, ADAL S, AKINCI A, Atabek M, DEMİREL F, ÇELİK N, ÖZKAN B, ÖZHAN B, Orbak Z, Ersoy B, doğan m, ATAŞ A, Demircioglu S, GÖKŞEN ŞİMŞEK R, TARIM Ö, yuksel b, ERCAN O, HATUN Ş, Şimşek E, ÖKTEN A, ABACI A, DÖNERAY H, Ozbek M, Keskin M, ÖNAL H, akyurek n, BULAN K, TEPE D, EMEKSİZ H, DEMİR K, Özalp Kızılay D, TOPALOGLU A, Eren E, Ozen S, ABALI S, AKIN L, SELVER EKLİOĞLU B, KABA S, ANIK A, BAŞ S, ÜNÜVAR T, Sağlam H, bolu s, ÖZGEN İ, DOĞAN D, ÇAKIR E, ŞEN Y, ANDIRAN N, ÇİZMECİOĞLU F, EVLİYAOĞLU S, Karagüzel G, PİRGON M, Çatlı G, CAN H, gürbüz f, binay c, baş v, FİDANCI M, POLAT A, GÜL D, AÇIKEL C, Demirbilek H, CİNAZ P, BONDY C (2015). Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology, 7(1), 27 - 36.
Chicago	YEŞİLKAYA Ediz,Bereket Abdullah,DARENDELİLER FATMA FEYZA,BAŞ Firdevs,Poyrazoglu Sukran,KÜÇÜKEMRE AYDIN Banu,darcan sukran,DÜNDAR Bumin Nuri,buyukinan muammer,KARA Cengiz,SARI Erkan,ADAL Servet Erdal,AKINCI Ayşehan,Atabek Mehmet Emre,DEMİREL Fatma,ÇELİK Nurullah,ÖZKAN Behzat,ÖZHAN Bayram,Orbak Zerrin,Ersoy Betül,doğan murat,ATAŞ Ali,Demircioglu Serap,GÖKŞEN ŞİMŞEK RUHSAR DAMLA,TARIM Ömer Faruk,yuksel bilgin,ERCAN OYA,HATUN Şükrü,Şimşek Enver,ÖKTEN Ayşenur,ABACI Ayhan,DÖNERAY Hakan,Ozbek Mehmet Nuri,Keskin Mehmet,ÖNAL Hasan,akyurek nesibe,BULAN Kezban,TEPE Derya,EMEKSİZ Hamdi Cihan,DEMİR Korcan,Özalp Kızılay Deniz,TOPALOGLU ALI,Eren Erdal,Ozen Samim,ABALI SAYGIN,AKIN Leyla,SELVER EKLİOĞLU BERAY,KABA Sultan,ANIK AHMET,BAŞ Serpil,ÜNÜVAR TOLGA,Sağlam Halil,bolu semih,ÖZGEN İlker Tolga,DOĞAN Durmuş,ÇAKIR Esra Deniz,ŞEN Yaşar,ANDIRAN Nesibe,ÇİZMECİOĞLU FİLİZ MİNE,EVLİYAOĞLU SAADET OLCAY,Karagüzel Gülay,PİRGON Mustafa Özgür,Çatlı Gönül,CAN Hatice Dilek,gürbüz fatih,binay cigdem,baş veysel,FİDANCI Muzaffer Kürşat,POLAT Adem,GÜL Davut,AÇIKEL Cengizhan,Demirbilek Huseyin,CİNAZ Peyami,BONDY Carolyn Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology 7, no.1 (2015): 27 - 36.
MLA	YEŞİLKAYA Ediz,Bereket Abdullah,DARENDELİLER FATMA FEYZA,BAŞ Firdevs,Poyrazoglu Sukran,KÜÇÜKEMRE AYDIN Banu,darcan sukran,DÜNDAR Bumin Nuri,buyukinan muammer,KARA Cengiz,SARI Erkan,ADAL Servet Erdal,AKINCI Ayşehan,Atabek Mehmet Emre,DEMİREL Fatma,ÇELİK Nurullah,ÖZKAN Behzat,ÖZHAN Bayram,Orbak Zerrin,Ersoy Betül,doğan murat,ATAŞ Ali,Demircioglu Serap,GÖKŞEN ŞİMŞEK RUHSAR DAMLA,TARIM Ömer Faruk,yuksel bilgin,ERCAN OYA,HATUN Şükrü,Şimşek Enver,ÖKTEN Ayşenur,ABACI Ayhan,DÖNERAY Hakan,Ozbek Mehmet Nuri,Keskin Mehmet,ÖNAL Hasan,akyurek nesibe,BULAN Kezban,TEPE Derya,EMEKSİZ Hamdi Cihan,DEMİR Korcan,Özalp Kızılay Deniz,TOPALOGLU ALI,Eren Erdal,Ozen Samim,ABALI SAYGIN,AKIN Leyla,SELVER EKLİOĞLU BERAY,KABA Sultan,ANIK AHMET,BAŞ Serpil,ÜNÜVAR TOLGA,Sağlam Halil,bolu semih,ÖZGEN İlker Tolga,DOĞAN Durmuş,ÇAKIR Esra Deniz,ŞEN Yaşar,ANDIRAN Nesibe,ÇİZMECİOĞLU FİLİZ MİNE,EVLİYAOĞLU SAADET OLCAY,Karagüzel Gülay,PİRGON Mustafa Özgür,Çatlı Gönül,CAN Hatice Dilek,gürbüz fatih,binay cigdem,baş veysel,FİDANCI Muzaffer Kürşat,POLAT Adem,GÜL Davut,AÇIKEL Cengizhan,Demirbilek Huseyin,CİNAZ Peyami,BONDY Carolyn Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology, vol.7, no.1, 2015, ss.27 - 36.
AMA	YEŞİLKAYA E,Bereket A,DARENDELİLER F,BAŞ F,Poyrazoglu S,KÜÇÜKEMRE AYDIN B,darcan s,DÜNDAR B,buyukinan m,KARA C,SARI E,ADAL S,AKINCI A,Atabek M,DEMİREL F,ÇELİK N,ÖZKAN B,ÖZHAN B,Orbak Z,Ersoy B,doğan m,ATAŞ A,Demircioglu S,GÖKŞEN ŞİMŞEK R,TARIM Ö,yuksel b,ERCAN O,HATUN Ş,Şimşek E,ÖKTEN A,ABACI A,DÖNERAY H,Ozbek M,Keskin M,ÖNAL H,akyurek n,BULAN K,TEPE D,EMEKSİZ H,DEMİR K,Özalp Kızılay D,TOPALOGLU A,Eren E,Ozen S,ABALI S,AKIN L,SELVER EKLİOĞLU B,KABA S,ANIK A,BAŞ S,ÜNÜVAR T,Sağlam H,bolu s,ÖZGEN İ,DOĞAN D,ÇAKIR E,ŞEN Y,ANDIRAN N,ÇİZMECİOĞLU F,EVLİYAOĞLU S,Karagüzel G,PİRGON M,Çatlı G,CAN H,gürbüz f,binay c,baş v,FİDANCI M,POLAT A,GÜL D,AÇIKEL C,Demirbilek H,CİNAZ P,BONDY C Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology. 2015; 7(1): 27 - 36.
Vancouver	YEŞİLKAYA E,Bereket A,DARENDELİLER F,BAŞ F,Poyrazoglu S,KÜÇÜKEMRE AYDIN B,darcan s,DÜNDAR B,buyukinan m,KARA C,SARI E,ADAL S,AKINCI A,Atabek M,DEMİREL F,ÇELİK N,ÖZKAN B,ÖZHAN B,Orbak Z,Ersoy B,doğan m,ATAŞ A,Demircioglu S,GÖKŞEN ŞİMŞEK R,TARIM Ö,yuksel b,ERCAN O,HATUN Ş,Şimşek E,ÖKTEN A,ABACI A,DÖNERAY H,Ozbek M,Keskin M,ÖNAL H,akyurek n,BULAN K,TEPE D,EMEKSİZ H,DEMİR K,Özalp Kızılay D,TOPALOGLU A,Eren E,Ozen S,ABALI S,AKIN L,SELVER EKLİOĞLU B,KABA S,ANIK A,BAŞ S,ÜNÜVAR T,Sağlam H,bolu s,ÖZGEN İ,DOĞAN D,ÇAKIR E,ŞEN Y,ANDIRAN N,ÇİZMECİOĞLU F,EVLİYAOĞLU S,Karagüzel G,PİRGON M,Çatlı G,CAN H,gürbüz f,binay c,baş v,FİDANCI M,POLAT A,GÜL D,AÇIKEL C,Demirbilek H,CİNAZ P,BONDY C Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology. 2015; 7(1): 27 - 36.
IEEE	YEŞİLKAYA E,Bereket A,DARENDELİLER F,BAŞ F,Poyrazoglu S,KÜÇÜKEMRE AYDIN B,darcan s,DÜNDAR B,buyukinan m,KARA C,SARI E,ADAL S,AKINCI A,Atabek M,DEMİREL F,ÇELİK N,ÖZKAN B,ÖZHAN B,Orbak Z,Ersoy B,doğan m,ATAŞ A,Demircioglu S,GÖKŞEN ŞİMŞEK R,TARIM Ö,yuksel b,ERCAN O,HATUN Ş,Şimşek E,ÖKTEN A,ABACI A,DÖNERAY H,Ozbek M,Keskin M,ÖNAL H,akyurek n,BULAN K,TEPE D,EMEKSİZ H,DEMİR K,Özalp Kızılay D,TOPALOGLU A,Eren E,Ozen S,ABALI S,AKIN L,SELVER EKLİOĞLU B,KABA S,ANIK A,BAŞ S,ÜNÜVAR T,Sağlam H,bolu s,ÖZGEN İ,DOĞAN D,ÇAKIR E,ŞEN Y,ANDIRAN N,ÇİZMECİOĞLU F,EVLİYAOĞLU S,Karagüzel G,PİRGON M,Çatlı G,CAN H,gürbüz f,binay c,baş v,FİDANCI M,POLAT A,GÜL D,AÇIKEL C,Demirbilek H,CİNAZ P,BONDY C "Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study." Journal of Clinical Research in Pediatric Endocrinology, 7, ss.27 - 36, 2015.
ISNAD	YEŞİLKAYA, Ediz vd. "Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study". Journal of Clinical Research in Pediatric Endocrinology 7/1 (2015), 27-36.