The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study

Yıl: 2016 Cilt: 8 Sayı: 3 Sayfa Aralığı: 305 - 312 Metin Dili: İngilizce İndeks Tarihi: 29-07-2022

The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study

Öz:
Objective: Noonan syndrome (NS) is a multisystem disorder, and short stature is its most striking manifestation. Optimal growth hormone (GH) treatment for NS is still controversial. In this study, using a nationwide registration system, we aimed to evaluate the growth characteristics and the clinical features of NS patients in Turkey and their growth response to GH treatment. Methods: Children and adolescents with a diagnosis of NS were included inthe study. Laboratory assessment including standard GH stimulation test results were evaluated. Height increment of patients with or without GH treatment were analyzed after three years of therapy.This study aimed to descript the characteristics of Noonan syndrome at a national level.Information about the clinical characteristics, especially in growth parameters of NS patients at national level is scarce. In this study, using a nationwide registration system, we aimed to evaluate the growth characteristics, clinical features, and response to GH treatment of NS patients in Turkey.MethodsIn this study, we retrospectively analysed the data of 124 children and adolescents with NS who were being followed in 20 centers in Turkey. Study approval was given by the Ankara University Ethics Committee. A nation-wide Results: A total of 124 NS patients from different centers were entered in the webbased system. Short stature and typical face appearance were the most frequently encountered diagnostic features of our patients. Of the 84 patients who were followed long-term, 47 hadreceived recombinant human GH (rhGH). In this group of 47 patients, height standard deviation score (HSDS) increased from -3.62±1.14 to -2.85±0.96 after three years of therapy, indicating significant differences from the patients who did not receive GH treatment. PTPN11 gene was analyzed in 61 patients, and 64% of these patients were found to have a mutation. HSDS at admission was similar in patients with or without PTPN11 gene mutation.Conclusion: A diagnosis of NS should be kept in mind in all patients with short stature showing systemic clinical findings. GH therapy is effective for improvement of short stature especially in the first two years of treatment. Further studies are needed for optimisation of GH therapy and evaluation of final height data in NS patients.
Anahtar Kelime:

Konular: Endokrinoloji ve Metabolizma Pediatri
Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
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APA siklar z, GENENS M, Poyrazoglu S, BAŞ F, DARENDELİLER F, BUNDAK R, aycan z, SAVAŞ ERDEVE Ş, Çetinkaya S, GÜVEN A, ABALI S, Atay Z, Demircioglu S, KARA C, Can Yilmaz G, akyurek n, Abaci A, Celmeli G, SARI E, bolu s, ANIL KORKMAZ H, Şimşek E, Çatlı G, buyukinan m, Cayir A, EVLİYAOĞLU S, İŞGÜVEN P, ÖZGEN T, Hatipoglu N, ELHAN A, BERBEROĞLU M (2016). The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. , 305 - 312.
Chicago siklar zeynep,GENENS Mikayir,Poyrazoglu Sukran,BAŞ Firdevs,DARENDELİLER FATMA FEYZA,BUNDAK Rüveyde,aycan zehra,SAVAŞ ERDEVE ŞENAY,Çetinkaya Semra,GÜVEN Ayla,ABALI SAYGIN,Atay Zeynep,Demircioglu Serap,KARA Cengiz,Can Yilmaz Gulay,akyurek nesibe,Abaci Ayhan,Celmeli Gamze,SARI Erkan,bolu semih,ANIL KORKMAZ Hüseyin,Şimşek Enver,Çatlı Gönül,buyukinan muammer,Cayir Atilla,EVLİYAOĞLU SAADET OLCAY,İŞGÜVEN Pınar,ÖZGEN Tolga,Hatipoglu Nihal,ELHAN Atilla Halil,BERBEROĞLU Merih The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. (2016): 305 - 312.
MLA siklar zeynep,GENENS Mikayir,Poyrazoglu Sukran,BAŞ Firdevs,DARENDELİLER FATMA FEYZA,BUNDAK Rüveyde,aycan zehra,SAVAŞ ERDEVE ŞENAY,Çetinkaya Semra,GÜVEN Ayla,ABALI SAYGIN,Atay Zeynep,Demircioglu Serap,KARA Cengiz,Can Yilmaz Gulay,akyurek nesibe,Abaci Ayhan,Celmeli Gamze,SARI Erkan,bolu semih,ANIL KORKMAZ Hüseyin,Şimşek Enver,Çatlı Gönül,buyukinan muammer,Cayir Atilla,EVLİYAOĞLU SAADET OLCAY,İŞGÜVEN Pınar,ÖZGEN Tolga,Hatipoglu Nihal,ELHAN Atilla Halil,BERBEROĞLU Merih The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. , 2016, ss.305 - 312.
AMA siklar z,GENENS M,Poyrazoglu S,BAŞ F,DARENDELİLER F,BUNDAK R,aycan z,SAVAŞ ERDEVE Ş,Çetinkaya S,GÜVEN A,ABALI S,Atay Z,Demircioglu S,KARA C,Can Yilmaz G,akyurek n,Abaci A,Celmeli G,SARI E,bolu s,ANIL KORKMAZ H,Şimşek E,Çatlı G,buyukinan m,Cayir A,EVLİYAOĞLU S,İŞGÜVEN P,ÖZGEN T,Hatipoglu N,ELHAN A,BERBEROĞLU M The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. . 2016; 305 - 312.
Vancouver siklar z,GENENS M,Poyrazoglu S,BAŞ F,DARENDELİLER F,BUNDAK R,aycan z,SAVAŞ ERDEVE Ş,Çetinkaya S,GÜVEN A,ABALI S,Atay Z,Demircioglu S,KARA C,Can Yilmaz G,akyurek n,Abaci A,Celmeli G,SARI E,bolu s,ANIL KORKMAZ H,Şimşek E,Çatlı G,buyukinan m,Cayir A,EVLİYAOĞLU S,İŞGÜVEN P,ÖZGEN T,Hatipoglu N,ELHAN A,BERBEROĞLU M The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. . 2016; 305 - 312.
IEEE siklar z,GENENS M,Poyrazoglu S,BAŞ F,DARENDELİLER F,BUNDAK R,aycan z,SAVAŞ ERDEVE Ş,Çetinkaya S,GÜVEN A,ABALI S,Atay Z,Demircioglu S,KARA C,Can Yilmaz G,akyurek n,Abaci A,Celmeli G,SARI E,bolu s,ANIL KORKMAZ H,Şimşek E,Çatlı G,buyukinan m,Cayir A,EVLİYAOĞLU S,İŞGÜVEN P,ÖZGEN T,Hatipoglu N,ELHAN A,BERBEROĞLU M "The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study." , ss.305 - 312, 2016.
ISNAD siklar, zeynep vd. "The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study". (2016), 305-312.
APA siklar z, GENENS M, Poyrazoglu S, BAŞ F, DARENDELİLER F, BUNDAK R, aycan z, SAVAŞ ERDEVE Ş, Çetinkaya S, GÜVEN A, ABALI S, Atay Z, Demircioglu S, KARA C, Can Yilmaz G, akyurek n, Abaci A, Celmeli G, SARI E, bolu s, ANIL KORKMAZ H, Şimşek E, Çatlı G, buyukinan m, Cayir A, EVLİYAOĞLU S, İŞGÜVEN P, ÖZGEN T, Hatipoglu N, ELHAN A, BERBEROĞLU M (2016). The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology, 8(3), 305 - 312.
Chicago siklar zeynep,GENENS Mikayir,Poyrazoglu Sukran,BAŞ Firdevs,DARENDELİLER FATMA FEYZA,BUNDAK Rüveyde,aycan zehra,SAVAŞ ERDEVE ŞENAY,Çetinkaya Semra,GÜVEN Ayla,ABALI SAYGIN,Atay Zeynep,Demircioglu Serap,KARA Cengiz,Can Yilmaz Gulay,akyurek nesibe,Abaci Ayhan,Celmeli Gamze,SARI Erkan,bolu semih,ANIL KORKMAZ Hüseyin,Şimşek Enver,Çatlı Gönül,buyukinan muammer,Cayir Atilla,EVLİYAOĞLU SAADET OLCAY,İŞGÜVEN Pınar,ÖZGEN Tolga,Hatipoglu Nihal,ELHAN Atilla Halil,BERBEROĞLU Merih The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology 8, no.3 (2016): 305 - 312.
MLA siklar zeynep,GENENS Mikayir,Poyrazoglu Sukran,BAŞ Firdevs,DARENDELİLER FATMA FEYZA,BUNDAK Rüveyde,aycan zehra,SAVAŞ ERDEVE ŞENAY,Çetinkaya Semra,GÜVEN Ayla,ABALI SAYGIN,Atay Zeynep,Demircioglu Serap,KARA Cengiz,Can Yilmaz Gulay,akyurek nesibe,Abaci Ayhan,Celmeli Gamze,SARI Erkan,bolu semih,ANIL KORKMAZ Hüseyin,Şimşek Enver,Çatlı Gönül,buyukinan muammer,Cayir Atilla,EVLİYAOĞLU SAADET OLCAY,İŞGÜVEN Pınar,ÖZGEN Tolga,Hatipoglu Nihal,ELHAN Atilla Halil,BERBEROĞLU Merih The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology, vol.8, no.3, 2016, ss.305 - 312.
AMA siklar z,GENENS M,Poyrazoglu S,BAŞ F,DARENDELİLER F,BUNDAK R,aycan z,SAVAŞ ERDEVE Ş,Çetinkaya S,GÜVEN A,ABALI S,Atay Z,Demircioglu S,KARA C,Can Yilmaz G,akyurek n,Abaci A,Celmeli G,SARI E,bolu s,ANIL KORKMAZ H,Şimşek E,Çatlı G,buyukinan m,Cayir A,EVLİYAOĞLU S,İŞGÜVEN P,ÖZGEN T,Hatipoglu N,ELHAN A,BERBEROĞLU M The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology. 2016; 8(3): 305 - 312.
Vancouver siklar z,GENENS M,Poyrazoglu S,BAŞ F,DARENDELİLER F,BUNDAK R,aycan z,SAVAŞ ERDEVE Ş,Çetinkaya S,GÜVEN A,ABALI S,Atay Z,Demircioglu S,KARA C,Can Yilmaz G,akyurek n,Abaci A,Celmeli G,SARI E,bolu s,ANIL KORKMAZ H,Şimşek E,Çatlı G,buyukinan m,Cayir A,EVLİYAOĞLU S,İŞGÜVEN P,ÖZGEN T,Hatipoglu N,ELHAN A,BERBEROĞLU M The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study. Journal of Clinical Research in Pediatric Endocrinology. 2016; 8(3): 305 - 312.
IEEE siklar z,GENENS M,Poyrazoglu S,BAŞ F,DARENDELİLER F,BUNDAK R,aycan z,SAVAŞ ERDEVE Ş,Çetinkaya S,GÜVEN A,ABALI S,Atay Z,Demircioglu S,KARA C,Can Yilmaz G,akyurek n,Abaci A,Celmeli G,SARI E,bolu s,ANIL KORKMAZ H,Şimşek E,Çatlı G,buyukinan m,Cayir A,EVLİYAOĞLU S,İŞGÜVEN P,ÖZGEN T,Hatipoglu N,ELHAN A,BERBEROĞLU M "The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study." Journal of Clinical Research in Pediatric Endocrinology, 8, ss.305 - 312, 2016.
ISNAD siklar, zeynep vd. "The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study". Journal of Clinical Research in Pediatric Endocrinology 8/3 (2016), 305-312.