High prevalence of pituitary magnetic resonance abnormalities and gene mutations in a cohort of Brazilian children with growth hormone deficiency and response to treatment

J Pediatr Endocrinol Metab. 2008 Jul;21(7):673-80. doi: 10.1515/jpem.2008.21.7.673.

Abstract

Data were retrospectively collected from 69 Brazilian patients (45 boys) with growth hormone deficiency (GHD) who received exogenous growth hormone (GH) for a median duration of 4 years (range 1-13 years). Forty-two patients had multiple pituitary hormone deficiencies and 27 had isolated GHD. Peak GH was < 7 ng/ml (IRMA) or < 3.2 ng/ml (IFMA) after two stimulation tests. Therapy was started at median age of 10.0 years (range 2.2-21.6 years), bone age of 5.8 years (0.5-13.5 years) and height standard deviation score -4.4 (range -9.3 to -1.6). MRI revealed pituitary abnormalities in 87% of patients. Homozygous mutations in PROP-1, GHRH-R, GH-1 or HESX-1 genes were found in 12 patients. Mean height velocities were 3.3 pretreatment and 10.3, 7.8, 7.4 and 6.4 cm/yr, respectively, during 1-4 years of treatment with GH. In conclusion, the high prevalence (96%) of genetic and/or pituitary abnormalities probably reflects the stringent diagnostic criteria used, and GH replacement resulted in significant catch-up growth.

MeSH terms

  • Adolescent
  • Adult
  • Body Height
  • Brazil
  • Child
  • Child, Preschool
  • Cohort Studies
  • DNA / genetics
  • Female
  • Growth Disorders / drug therapy*
  • Growth Disorders / genetics*
  • Growth Disorders / pathology
  • Human Growth Hormone / genetics*
  • Human Growth Hormone / therapeutic use*
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Pituitary Gland / pathology*
  • Puberty
  • Recombinant Proteins / therapeutic use
  • Retrospective Studies
  • Sexual Maturation

Substances

  • Recombinant Proteins
  • Human Growth Hormone
  • DNA