SHORT STATURE AND LOW IGF-1 AND IGFBP-3 DESPITE NORMAL GROWTH-HORMONESECRETION IN A 4 YEAR-OLD GIRL WITH PRIMARY EMPTY SELLA SYNDROME

A 4.1 year-old girl presented with short stature, cleft lip and additi onal upper incisor, Magnetic resonance imaging showed an empty sella d ue to a hypoplastic anterior pituitary, IGF-I and IGFBP-3 were at and below the 1(st) percentile, respectively. In contrast, normal spontane ous growth hormone (GH) secretion (5.5-h nocturnal sampling) was obser ved and pharmacological provocation raised GH to levels between 15.4 a nd 53 mu g/l. GH-binding protein levels were normal (210 pM). GH thera py led to an increase of growth velocity from 4.5 to 10.8 normalizatio n of IGF-I and The findings may imply an abnormal GH secretion pattern or a bioinactive GH in our patient. The data indicate that measuremen ts of IGF-1 and IGFBP-3 may be a more sensitive test for integrative G H activity than GH testing itself.