title: Short stature and low IGF-1 and IGFBP-3 despite normal growth hormone secretion in a 4 year-old girl with primary empty sella syndrome.
abstract: A 4.1 year-old girl presented with short stature, cleft lip and additional upper incisor. Magnetic resonance imaging showed an empty sella due to a hypoplastic anterior pituitary. IGF-I and IGFBP-3 were at and below the 1st percentile, respectively. In contrast, normal spontaneous growth hormone (GH) secretion (5.5-h nocturnal sampling) was observed and pharmacological provocation raised (GH to levels between 15.4 and 53 micrograms/l. GH-binding protein levels were normal (210 pM). GH therapy led to an increase of growth velocity from 4.5 to 10.8 cm/year and a normalization of IGF-I and IGFBP-3 levels. The findings may imply an abnormal GH secretion pattern or a bioinactive GH in our patient. The data indicate that measurements of IGF-1 and IGFBP-3 may be a more sensitive test for integrative GH activity than GH testing itself.
authors.affiliation: Department of Pediatrics, University of Giessen, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Dötsch
authors.forename: W F
authors.initials: WF
authors.lastname: Blum
authors.forename: W
authors.initials: W
authors.lastname: Rascher
authors.forename: J
authors.initials: J
authors.lastname: Kreuder
authors.forename: W
authors.initials: W
authors.lastname: Kiess
mesh_terms: D001827:Body Height; D002352:Carrier Proteins / Q000097:blood; D002675:Child, Preschool; D002971:Cleft Lip / Q000150:complications; D004652:Empty Sella Syndrome / Q000150:complications / Q000503:physiopathology*; D005260:Female; D019382:Human Growth Hormone / Q000378:metabolism* / Q000627:therapeutic use; D006801:Humans; D018972:Insulin-Like Growth Factor Binding Protein 3 / Q000097:blood*; D007334:Insulin-Like Growth Factor I / Q000378:metabolism*; D008279:Magnetic Resonance Imaging; D014096:Tooth, Supernumerary / Q000150:complications
chemical_list: D002352:Carrier Proteins; D018972:Insulin-Like Growth Factor Binding Protein 3; D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I; C051815:somatotropin-binding protein