Growth hormone response to standard provocative stimuli and combined tests in very young children with Prader-Willi syndrome

Girolamo Di Giorgio, Graziano Grugni, Danilo Fintini, Sarah Bocchini, Sabrina Spera, Marina Cuttini, Marco Cappa, Antonino Crinò

Research output: Contribution to journalArticlepeer-review

Abstract

Background: In Prader-Willi syndrome (PWS) a reduced growth hormone (GH) response to several stimulators has been documented in many studies, but none have focused on very young children. We evaluated the pattern of GH secretion in very young PWS patients. Patients and Methods: Twenty-seven genetically confirmed PWS children (10 females, aged 0.4-5 years, mean: 2.2 ± 1.4 years) were included. All subjects underwent standard provocative tests (clonidine, CLO; and arginine, ARG) and one combined test [growth hormone-releasing hormone (GHRH) plus pyridostigmine (13 patients) or GHRH plus arginine (14 patients)]. Insulin-like growth factor-1 (IGF-1) levels were also measured. Results: While standard tests (CLO and ARG) showed low GH peak in 85.2 and 70.4% of the patients, respectively, the combined test was found to be normal in 85.2%. IGF-1 was low in 66.7% of patients. Out of 27 patients, 3 (11%) showed a normal GH peak with both standard tests (group A), 6 (22%) to one of the standard tests (group B) and 18 (67%) presented a low response to both standard tests (group C). Four subjects showed low response to both the combined and standard tests and reduced IGF-1. Conclusion: Our data suggest that very young PWS children seem to have impaired hypothalamic GHRH secretion with a normal GH pituitary reserve.

Original languageEnglish
Pages (from-to)189-195
Number of pages7
JournalHormone Research in Paediatrics
Volume81
Issue number3
DOIs
Publication statusPublished - 2014

Keywords

  • Growth hormone deficiency
  • Hypothalamic disorders
  • Insulin like growth factor-1
  • Prader-Willi syndrome

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism
  • Pediatrics, Perinatology, and Child Health
  • Medicine(all)

Fingerprint

Dive into the research topics of 'Growth hormone response to standard provocative stimuli and combined tests in very young children with Prader-Willi syndrome'. Together they form a unique fingerprint.

Cite this