Turner sendromunda büyüme hormonu tedavi sonuçlarının değerlendirilmesi: Türkiye KIGS (Pfizer uluslararası büyüme veri tabanı) veri tabanı analiz sonuçları
Küçük Resim Yok
Tarih
2005
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Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Amaç: Türkiye'de Turner sendromu (TS) tanısı alan ve büyüme hormonu (BH) tedavisi başlanmış olan hastaların tedaviye ilişkin verilerini değerlendirmek. Yöntem: Türkiye'deki 11 merkezin KİGS (Pfizer Uluslararası Büyüme Veri Tabanı) veri tabanında 1989 yılından itibaren kayıtlı Turner sendromu vakalarının kayıtları alınarak Ocak 2004 tarihi itibarıyle 70 hastanın verileri değerlendirildi. Bu veri tabanından yararlanılarak TS vakalarının doğum boyu, doğum ağırlığı ve anne-baba boy değerleri, tedavi öncesi ve tedavi sırasında yaş, boy, vücut ağırlığı, hesaplanan vücut kitle indeksi, büyüme hızı ve hedef boy değerleri incelendi ve değerler standart deviasyon skor (SDS) olarak ifade edildi. TS hastalarına büyüme hormonu (Genotropin®) haftada medyan 6 kez olarak subkutan olarak başlanmıştı. Tüm değerler medyan (10/90. persantil) olarak verildi. Bulgular: Tedavi başlangıcında vakaların yaşı 12.5 (7.1/15.6) yıl, boy SDS -3.8 (-5.3/-2.6) büyüme hızı 4.1 (1.5/5.9) cm/yıl ve boy - hedef boy farkı (SDS)-2.9 (-4.3/-1.2)'du. BH 0.23 (0.16/0.32) mg/kg/hafta dozunda kullanılmıştı. Longitudinal olarak izlenen TS vakalarında büyüme hızı anlamlı olmayan bir artış göstererek 1. yılda 6.3 (4.3/8.5) cm/yıl, 2. yılda 5.9 (3.6/8.7) cm/yıl oldu. Boy SDS'si 1. yılda - 3.4 (- 4.6/-2.2), 2. yılda ise - 2.7 (- 4.2/-1.6)'ye yükseldi (p<0.05). Sonuç: Türkiye'de TS vakaları geç tanı almakta ve geç tedaviye başlanmaktadır. Kullanılan BH dozu önerilen doz aralığının altındadır. BH tedavisine yanıt literatürde bu dozlarla elde edilen büyüme yanıtlarına benzerlik göstermektedir. 136.1 BH tedavi yılı açısından yan etki görülme sıklığı enderdir.
Aim: To evaluate the response to growth hormone (GH) treatment in Turkish children with Turner syndrome (TS). Methods: The data of 70 TS patients registered from 11 centers in Turkey in the KIGS (Pfizer's International Growth Study) database were evaluated as of January 2004. The baseline measurements of the patients including birth weight and length and parental heights; height, weight, age, calculated body mass index, height velocity and mid parental height (MPH) at onset and during GH treatment were retrieved from the database. All measurements were expressed as standard deviation scores (SDS) and given as median (10th/90th centiles) values. GH (Genotropin®) was given to all patients at a median frequency of 6 times per week subcutaneously. Results: At onset of therapy age was 12.5 (7.1/15.6) years and height SDS -3.8 (-5.3/-2.6). Pretreatment height velocity was 4.1 (1.5/5.9) cm/year and height - MPH (SDS) was -2.9 (-4.3/-1.2). GH was given in a dose of 0.23 (0.16 / 0.32) mg/kg/week subcutaneously, 6 -7 times per week. Height velocity showed a nonsignificant increase to 6.3 (4.3 / 8.5) cm/year in the first year of therapy and 5.9 (3.6/8.7) cm/year in the 2nd year of therapy in the longitudinally followed TS patients. Height SDS showed an increase to - 3.4 (-4.6/-2.2) in the first year and to - 2.7 (-4.2/-1.6) in the 2nd year of therapy (p< 0.05). Conclusion: Age at diagnosis of TS and age of onset of GH therapy are both delayed in our children. The dose of GH used is lower than the dose range recommended currently. The growth response to GH treatment is similar to the reported values in the literature on similar doses. There were no major side effects during 136.1 years of GH therapy.
Aim: To evaluate the response to growth hormone (GH) treatment in Turkish children with Turner syndrome (TS). Methods: The data of 70 TS patients registered from 11 centers in Turkey in the KIGS (Pfizer's International Growth Study) database were evaluated as of January 2004. The baseline measurements of the patients including birth weight and length and parental heights; height, weight, age, calculated body mass index, height velocity and mid parental height (MPH) at onset and during GH treatment were retrieved from the database. All measurements were expressed as standard deviation scores (SDS) and given as median (10th/90th centiles) values. GH (Genotropin®) was given to all patients at a median frequency of 6 times per week subcutaneously. Results: At onset of therapy age was 12.5 (7.1/15.6) years and height SDS -3.8 (-5.3/-2.6). Pretreatment height velocity was 4.1 (1.5/5.9) cm/year and height - MPH (SDS) was -2.9 (-4.3/-1.2). GH was given in a dose of 0.23 (0.16 / 0.32) mg/kg/week subcutaneously, 6 -7 times per week. Height velocity showed a nonsignificant increase to 6.3 (4.3 / 8.5) cm/year in the first year of therapy and 5.9 (3.6/8.7) cm/year in the 2nd year of therapy in the longitudinally followed TS patients. Height SDS showed an increase to - 3.4 (-4.6/-2.2) in the first year and to - 2.7 (-4.2/-1.6) in the 2nd year of therapy (p< 0.05). Conclusion: Age at diagnosis of TS and age of onset of GH therapy are both delayed in our children. The dose of GH used is lower than the dose range recommended currently. The growth response to GH treatment is similar to the reported values in the literature on similar doses. There were no major side effects during 136.1 years of GH therapy.
Açıklama
Anahtar Kelimeler
Cerrahi, Pediatri
Kaynak
Çocuk Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
5
Sayı
1
