REVIEW
Growth Hormone Deficiency in the Transition Period
Çocukluktan Erişkinliğe Geçiş Döneminde Büyüme Hormonu Eksikliği
Received Date : 15 Mar 2019
Accepted Date : 19 Jun 2019
Doğuş VURALLI
Hacettepe University Faculty of Medicine, Division of Pediatric Endocrinology, Ankara, Turkey
Doi: 10.25179/tjem.2019-65793 - Makale Dili: EN
Turk J Endocrinol Metab 2019;23:181-186
ABSTRACT
The importance of continuing growth hormone therapy in
the transition and adulthood periods must be studied in
adolescents with growth hormone deficiency with childhood
onset. Continuation of growth hormone therapy during the
transition period in patients with permanent growth hormone
deficiency is recommended as the therapy has positive
effects on adult body composition and regional body fat
distribution as well as its promotion of an increase in bone
mineral content. The chance of having permanent growth
hormone deficiency is higher in patients with a mass lesion
involving the pituitary area, multiple pituitary hormone deficiency,
isolated growth hormone deficiency associated with
an identified mutation (i.e., growth hormone 1 (GH1) gene,
growth hormone releasing hormone receptor (GHRHR)
gene, sex determining region Y(SRY)-box 3 (SOX3) gene),
midline defects, and congenital structural hypothalamo-pituitary
disorders, such as an ectopic posterior pituitary;
thus, such patients do not require retesting. The patients
with idiopathic isolated growth hormone deficiency are recommended
to undergo retesting. The most appropriate
time for a retest is the age when the child achieves 98-99%
of the adult height. The insulin tolerance test is preferred
during the transition period and a threshold level of <5
ng/mL is recommended as the diagnostic criterion for complete
growth hormone deficiency. Growth hormone doses
higher than those used in adults (e.g., 70 mcg/kg/day) should
be used until linear growth has been achieved. Decreased
quality of life is another criterion for the initiation of
therapy. This review addresses the definition of growth hormone
deficiency and indications and usage of growth hormone
therapy during the transition period.
Keywords: Growth hormone deficiency; transition period; retesting
ÖZET
Çocukluk döneminde büyüme hormonu eksikliği (BHE) başlayan
ve büyüme hormonu tedavisi kullanan ergenlerin, geçiş
ve erişkin dönemde büyüme hormonu tedavisine devam etmesinin
önemi araştırılmalıdır. Büyüme hormonu tedavisinin
erişkin vücut kompozisyonu ve bölgesel yağ dağılımı üzerine
pozitif etkisi ve kemik mineral yoğunluğunda artış sağlaması
nedeniyle kalıcı büyüme hormonu eksikliği olan olgularda
geçiş döneminde büyüme hormonu tedavisine devam edilmesi
önerilmektedir. Hipofizer bölgeyi ilgilendiren kitle lezyonu,
çoğul hipofizer hormon eksiklikleri, tanımlanmış bir
mutasyonla ilişkilendirilen izole büyüme hormonu eksikliği
(örn. büyüme hormonu 1 (BH-1) geni, büyüme hormonu salgılatıcı
hormon reseptör (BHRHR) geni, Y kromozomu üzerindeki
cinsiyet farklılaşmasını kontrol eden bölge (SRY)-box
3 (SOX3) geni), orta hat kusurları ve ektopik posterior hipofiz
gibi konjenital yapısal hipotalamo-hipofizer bozuklukları
olan olgularda kalıcı büyüme hormonu eksikliği olasılığı çok
yüksek olduğundan, tekrar testlerinin yapılmasına gerek yoktur.
İdiyopatik izole büyüme hormonu eksikliği olanlara tekrar
testlerinin yapılması önerilmektedir. Test tekrarı için en
uygun zaman çocuğun boyunun erişkin boyun %98-99’una
ulaştığı yaştır. Geçiş döneminde insülin tolerans testi tercih
edilmektedir ve eşik değerin <5 ng/mL olması tam büyüme
hormonu eksikliği eksikliği için tanı kriteri olarak önerilmektedir.
Lineer büyüme tamamlanıncaya kadar erişkinlerde kullanılan
dozlardan daha yüksek dozlarda (örneğin; 70
mcg/kg/gün) büyüme hormonu kullanılması gerekmektedir.
Yaşam kalitesinde azalma, tedaviye başlamak için kullanılan
başka bir kriterdir. Bu çalışmada, büyüme hormonu eksikliğinin
tanımı ve büyüme hormonu tedavisinin geçiş döneminde
kullanılma endikasyonları üzerinde durulması amaçlanmıştır.
Anahtar Kelimeler: Büyüme hormonu eksikliği; geçiş dönemi; retest (tekrar testi)
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