Response to growth hormone therapy in Turner syndrome

Year 2013, Volume: 48 Issue: 4, 294 - 298, 01.12.2013

İlker Tolga Özgen Erdal Adal Tolga Ünüvar Hasan Önal Aliye Sevil Sarıkaya Leyla Akın

Abstract

Aim: Short stature a common feature of Turner syndrome may be treated effectively by recombinant human growth hormone rhGH In this study we aimed to evaluate response to rhGH in the first three years of therapy Material and Method: Medical records of 46 girls with Turner syndrome treated with rhGH were evaluated retrospectively Karyotypes age of admission to the hospital age of the beginning of rhGH therapy the height of the mother and father were recorded Data including height and weight growth velocity dose of rhGH therapy bone age and predicted height of subjects at the beginning after first second and thirds years of rhGH therapy were recorded Difference between target height Z score and height Z score is defined as delta Z score Results: Age of admission to hospital age of beginning of rhGH therapy target height were 10 2 plusmn;3 8 years 11 54 plusmn;3 03 years and 157 0 plusmn;6 0 cm respectively Height Z score at the beginning at the end of first second and thirds year of therapy were 3 76 plusmn;1 00 3 37 plusmn; 1 01 2 99 plusmn;0 97 2 82 plusmn;1 01 respectively and growth velocity were 3 24 plusmn;1 05 6 53 plusmn;1 52 5 77 plusmn;1 53 5 19 plusmn;1 25 cm per year respectively Bivariate correlation analyzes have revealed that at the first year of therapy growth velocity was correlated negatively with chronologic and bone age of beginning to GH therapy delta Z score and father rsquo;s height and positively with rhGH dose Conclusions: Major factors to affect response to rhGH therapy were age rhGH dose and delta Z score As the major predictor of the growth velocity is age at the beginning of the therapy these girls must be treated with rhGH as soon as possible after diagnosis Turk Arch Ped 2013; 48: 294 8

Keywords

Growth hormone, treatment, Turner syndrome

Turner sendromlu hastalarda büyüme hormonu tedavisine yanıt

Abstract

Amaç: Turner sendromlu hastalarda boy kısalığı büyüme hormonu ile tedavi edilebilmektedir Bu çalışmada Turner sendromlu Türk çocuklarında üç yıllık büyüme hormonu tedavisine yanıtı ve tedavi sırasında büyümeye etki edebilecek etmenlerin değerlendirilmesi amaçlanmıştır Gereç ve Yöntem: Büyüme hormonu tedavisi almış 46 Turner sendromlu hastanın tıbbi kayıtları geriye dönük olarak incelendi Karyotip hastaneye başvuru yaşı büyüme hormonu tedavisi başlama yaşı anne ve baba boyları ayrıca tedavinin başlangıcında birinci ikinci ve üçüncü yıllarında çocuğun boy ağırlık yıllık büyüme hızı büyüme hormonu dozu kemik yaşı ve öngörülen boy değerleri kaydedildi Hedef boy Z skoru ve var olan boy Z skoru arasındaki fark delta Z skor olarak ifade edildi Bulgular: Hastaneye başvuru yaşı 10 2 plusmn;3 8 yıl tedavi başlangıç yaşı 11 54 plusmn;3 03 yıl ve hedef boy 157 0 plusmn;6 0 cm idi Tedavinin başlangıcında birinci ikinci ve üçüncü yılında boy Z skoru sırasıyla 3 76 plusmn;1 00 3 37 plusmn;1 01 2 99 plusmn;0 97 2 82 plusmn;1 01 olarak saptandı yıllık büyüme hızı ise yine sırasıyla 3 24 plusmn;1 05 6 53 plusmn;1 52 5 77 plusmn;1 53 5 19 plusmn;1 25 cm idi Birinci yıl büyüme hızı ile tedavi başlangıcındaki kronolojik ve kemik yaşı delta Z skor ve baba boyu ile negatif ilişki büyüme hormonu dozu ile arasında ise pozitif ilişki saptandı Çoklu regresyon analizlerinde birinci yıl büyüme hızına etki eden en önemli etmenin tedavi başlangıç yaşı ve büyüme hormonu dozu olduğu görüldü Çıkarımlar: Birinci yıl büyüme hızını etkileyen etmenler; tedavi başlangıç yaşı büyüme hormonu dozu ve delta Z skor olarak saptandı Bu çalışma tanı sonrası çok kısa bir sürede tedavi başlanmasının önemli olduğunu göstermektedir Türk Ped Arfl 2013; 48: 294 8

Keywords

Büyüme hormonu, tedavi, Turner sendromu

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