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

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dc.contributor.authorŞıklar, Zeynep
dc.contributor.authorGenens, Mikayir
dc.contributor.authorPoyrazoğlu, Şükran
dc.contributor.authorBaş, Firdevs
dc.contributor.authorDarendeliler, Feyza
dc.contributor.authorBundak, Rüveyde
dc.contributor.authorBerberoğlu, Merih
dc.date.accessioned2020-04-30T23:33:44Z
dc.date.available2020-04-30T23:33:44Z
dc.date.issued2016
dc.departmentDÜ, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümüen_US
dc.descriptionAbaci, Ayhan/0000-0002-1812-0321; ABALI, SAYGIN/0000-0001-6552-2801; yilmaz, gulay can/0000-0003-0525-1231; Buyukinan, Muammer/0000-0002-2937-823X; Turan, Serap/0000-0002-5172-5402; Elhan, Atilla Halil/0000-0003-3324-248X; GUVEN, AYLA/0000-0002-2026-1326; Ozgen, Ilker Tolga/0000-0001-6592-9652en_US
dc.descriptionWOS: 000385025700008en_US
dc.descriptionPubMed: 27125300en_US
dc.description.abstractObjective: 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. Results: A total of 124 NS patients from different centers were entered in the web-based 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.en_US
dc.description.sponsorshipTurkish Pediatric Endocrinology and Diabetes Society [042014]en_US
dc.description.sponsorshipThis work was supported by the Turkish Pediatric Endocrinology and Diabetes Society (Grant Number: 042014).en_US
dc.identifier.doi10.4274/jcrpe.3013en_US
dc.identifier.endpage312en_US
dc.identifier.issn1308-5727
dc.identifier.issn1308-5735
dc.identifier.issue3en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage305en_US
dc.identifier.urihttps://doi.org/10.4274/jcrpe.3013
dc.identifier.urihttps://hdl.handle.net/20.500.12684/5032
dc.identifier.volume8en_US
dc.identifier.wosWOS:000385025700008en_US
dc.identifier.wosqualityQ3en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakTR-Dizinen_US
dc.language.isoenen_US
dc.publisherGalenos Yayinciliken_US
dc.relation.ispartofJournal Of Clinical Research In Pediatric Endocrinologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectNoonan syndromeen_US
dc.subjectgrowth hormone treatmenten_US
dc.subjectgrowthen_US
dc.titleThe Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Studyen_US
dc.typeArticleen_US

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