Growth hormone treatment in Aarskog syndrome: Analysis of the KIGS (Pharmacia International Growth Database) data

Darendeliler F. F., Larsson P., Neyzi O., Price A., Hagenas L., Sipila I., ...Daha Fazla

JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, cilt.16, sa.8, ss.1137-1142, 2003 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 16 Sayı: 8
  • Basım Tarihi: 2003
  • Dergi Adı: JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.1137-1142
  • İstanbul Üniversitesi Adresli: Evet

Özet

Aarskog syndrome is an X-linked disorder characterized by faciogenital dysplasia and short stature. The present study set out to determine the effect of growth hormone (GH) therapy in patients with Aarskog syndrome enrolled in KIGS - the Pharmacia International Growth Database.. Twenty-one patients (20 males) were evaluated. Median age at start of treatment was 8.3 years (10-90(th) percentiles, 5.1-14.1 years) and median height SDS was -2.8 (10-90(th) percentiles, -2.1 to -3.7). The median dose of GH was 0.22 mg/kg/week (10-90(th) percentiles, 0.15-0.30 mg/kg/week) given at a median frequency of six (4-7) times per week. Prepubertal patients were followed longitudinally for 1 year (n = 13) or 3 years (n = 7). After 1 year, the median height SDS had improved from -2.8 to -2.3 in 13 patients. After 3 years, height SDS had improved significantly (p <0.05) to -1.8 (10-90(th) percentiles, -2.1 to -1.1) in the seven patients. No adverse events were noted. Although final height data for these patients are still awaited, the present results support the use of GH to promote growth in children with Aarskog syndrome.