Category: Other - growth disease
Turner syndrome (TS) is a chromosomal disorder of females involving many physical abnormalities; it includes short stature and slow growth rate. Adult height is 20 cm shorter than in general population. Short stature has been treated with anabolic steroids, low doses of estrogen, human growth hormone (GH), or a combination therapy. GH could induce a substantial increase in the rate of increase in height. Final height results depend on age at diagnosis, duration of therapy and doses of GH. Our aim has been to evaluate growth and safety during the first five years of GH treatment in patients with TS.
Methods : Ten prepubertal girls with TS have been included; mean age was 11.54 ys. They have been treated with a mean dose of GH=0.037mg/kg/d and followed with at least five years of therapy.
The mean height SDS score increased from -3.61 at baseline to -1.02 at 5 ys. The average height gain after 5 years was 19.55cm. The mean weight SDS score increased from -1.28 at baseline to -0.00 at 5 ys. Bone age was delayed at diagnosis (calculated by Greulich & Pyle Atlas) by a mean value of 1.17 ys and after five years the bone age was consistent with the chronological age.
Safety profile: There were no cases of diabetes mellitus, impaired glucose tolerance or malignancies; four patients had transient increase in fasting glucose (>100< 126mg/dl); three patients developed hypothyroidism and have been treated with L-thyroxin.
In TS, GH and IGF levels are normal during childhood and growth impairment is clinically evident before the period when the activity of GH-IGF axis is decreased. GH therapy is capable to accelerate growth and to increase adult height. Delayed diagnosis of TS has a negative impact on growth outcomes.
In our study GH treatment was associated with highly significant changes in growth. Height velocity was maximum (8.53 cm/yr) in the first year of GH treatment; the improvements in growth decreases with time. GH therapy had a favorable safety profile. In our study the diagnosis was delayed in some cases, which has had a negative impact on the gain of growth.
University of Medicine and Pharmacy
Iasi, Iasi, Romania
Mrs. Corina Găleşanu is clinical professor, Department of Endocrinology at University of Medicine and Pharmacy Iaşi. Her research interests: Osteoporosis, Menopause, Neuroendocrine Tumours, Growth Disorders. She is a regular speaker at national and international meetings on Menopause, Osteoporosis, Height and Weight Growth Disorders, Obesity. She was investigator in eighteen international Clinical Research Studies. From 2008, Mrs. Corina Găleşanu is full Clinician Faculty ISCD for Bone Densitometry Course. She is on the Editorial board of Acta Endocrinologica; Gineco.ro - A Journal of Obstetrics and Gynecology; The Romanian Journal of Endocrinology and Metabolism. She is Fellow of the American College of Endocrinology (FACE).
Romanian Academy of Medical Sciences, Radiology, Iasi, Romania
Mr. MR.Galesanu is Professor of Radiology/radiotherapy. Currently working in private radiology-diagnosis.He is member of the Academy of Medical Sciences in Romania.Holds the Romanian Academy Award for the Book