title: The Growth Characteristics of Patients with Noonan Syndrome: Results of Three Years of Growth Hormone Treatment: A Nationwide Multicenter Study.
abstract: 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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Ankara University Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, Turkey, Phone: +90 312 595 66 35 E-mail: zeynepsklr@gmail.com.
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Şıklar
authors.forename: Mikayir
authors.initials: M
authors.lastname: Genens
authors.forename: Şükran
authors.initials: Ş
authors.lastname: Poyrazoğlu
authors.forename: Firdevs
authors.initials: F
authors.lastname: Baş
authors.forename: Feyza
authors.initials: F
authors.lastname: Darendeliler
authors.forename: Rüveyde
authors.initials: R
authors.lastname: Bundak
authors.forename: Zehra
authors.initials: Z
authors.lastname: Aycan
authors.forename: Şenay
authors.initials: Ş
authors.lastname: Savaş Erdeve
authors.forename: Semra
authors.initials: S
authors.lastname: Çetinkaya
authors.forename: Ayla
authors.initials: A
authors.lastname: Güven
authors.forename: Saygın
authors.initials: S
authors.lastname: Abalı
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Atay
authors.forename: Serap
authors.initials: S
authors.lastname: Turan
authors.forename: Cengiz
authors.initials: C
authors.lastname: Kara
authors.forename: Gülay
authors.initials: G
authors.lastname: Can Yılmaz
authors.forename: Nesibe
authors.initials: N
authors.lastname: Akyürek
authors.forename: Ayhan
authors.initials: A
authors.lastname: Abacı
authors.forename: Gamze
authors.initials: G
authors.lastname: Çelmeli
authors.forename: Erkan
authors.initials: E
authors.lastname: Sarı
authors.forename: Semih
authors.initials: S
authors.lastname: Bolu
authors.forename: Hüseyin Anıl
authors.initials: HA
authors.lastname: Korkmaz
authors.forename: Enver
authors.initials: E
authors.lastname: Şimşek
authors.forename: Gönül
authors.initials: G
authors.lastname: Çatlı
authors.forename: Muammer
authors.initials: M
authors.lastname: Büyükinan
authors.forename: Atilla
authors.initials: A
authors.lastname: Çayır
authors.forename: Olcay
authors.initials: O
authors.lastname: Evliyaoğlu
authors.forename: Pınar
authors.initials: P
authors.lastname: İşgüven
authors.forename: Tolga
authors.initials: T
authors.lastname: Özgen
authors.forename: Nihal
authors.initials: N
authors.lastname: Hatipoğlu
authors.forename: Atilla Halil
authors.initials: AH
authors.lastname: Elhan
authors.forename: Merih
authors.initials: M
authors.lastname: Berberoğlu
mesh_terms: D000293:Adolescent; D000704:Analysis of Variance; D001827:Body Height / Q000187:drug effects* / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006130:Growth Disorders / Q000188:drug therapy* / Q000503:physiopathology; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009634:Noonan Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D016896:Treatment Outcome; D014421:Turkey
chemical_list: D019382:Human Growth Hormone