ABSTRACT
A 6 years male child was referred to our Endocrinology clinic with complaints of failure to thrive and he displayed the characteristic features of Russell Silver Syndrome which included short stature, relative macrocephaly, triangular facies and bilateral clinodactyly. He had a birth weight of 2.14 kg and an expected target height of 170 cm. He was subjected to a hormonal analysis which revealed a normal thyroid profile, but low serum markers of growth namely IGF-1=68 ng/ml (52-297 ng/ml) and basal growth hormone (GH) (1.5 µg/l). No defects were detected on MRI of the sella. Therefore a growth hormone stimulation test with Clonidine was performed which confirmed complete GH deficiency (at 0 min=0.16 µg/l, 60 min=0.27 µg/l, 120 min=4.73 µg/l). He was commenced on rhGH therapy at 8 years of age (height=102 cm, SDS=-4.53), due to financial restraints. Following initiation of GH therapy (1.5 IU/day) for 19 months, a height gain of 15 cm was obtained (Height=117 cm, SDS=-3.05). Bone age at 9 yr. was between 7-8 years.
ABSTRACT
Although the importance of the pituitary gland for growth was recognized in late 19(th) century, Growth hormone (GH) therapy was made available for severely GH-deficient children and adolescents only in late 1950s. Use of GH for other conditions was limited because of the limited supply of human pituitary-derived hormone. With unlimited availability of recombinant human GH (rhGH), the scenario of GH treatment has been changed enormously. Currently there is ever increasing list of indications of GH treatment in children, adolescents, and adults.
