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

J Pediatr Endocrinol Metab. 1996 May-Jun;9(3):415-8. doi: 10.1515/jpem.1996.9.3.415.

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.

Publication types

  • Case Reports

MeSH terms

  • Body Height*
  • Carrier Proteins / blood
  • Child, Preschool
  • Cleft Lip / complications
  • Empty Sella Syndrome / complications
  • Empty Sella Syndrome / physiopathology*
  • Female
  • Human Growth Hormone / metabolism*
  • Human Growth Hormone / therapeutic use
  • Humans
  • Insulin-Like Growth Factor Binding Protein 3 / blood*
  • Insulin-Like Growth Factor I / metabolism*
  • Magnetic Resonance Imaging
  • Tooth, Supernumerary / complications

Substances

  • Carrier Proteins
  • Insulin-Like Growth Factor Binding Protein 3
  • Human Growth Hormone
  • Insulin-Like Growth Factor I
  • somatotropin-binding protein