title: Impairment of growth hormone responsiveness to growth hormone releasing hormone and pyridostigmine in patients affected by Prader-Labhardt-Willi syndrome.
abstract: In order to evaluate the impairment of GH response in patients affected by Prader-Labhardt-Willi (PLW) syndrome, in 18 patients we studied GH response to clonidine and to GHRH + pyridostigmine, a cholinergic drug which enhances GHRH induced GH responsiveness in obese patients. After clonidine GH response was abnormal in 14/18 subjects (mean GH peak: 4.1 +/- 1.3 micrograms/l; area under curve: 208.1 +/- 74.2 micrograms/l.h) while all but 5 patients showed an inadequate GH response to GHRH + pyridostigmine (mean GH peak: 13.4 +/- 2.5 micrograms/l; area under curve: 903.4 +/- 171.0 micrograms/l.h). However, in the three patients with low adiposity index, GH response to GHRH + pyridostigmine was significantly higher than that observed in fatter subjects. In addition, GH response to GHRH + pyridostigmine was negatively correlated to age and adiposity index. In conclusion, our data are consistent with the hypothesis of the existence of a complex derangement of GH neuroendocrine regulation in these subjects.
authors.affiliation: Clinica Pediatrica III, Istituto Scientifico H San Raffaele, Università degli Studi di Milano, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Beccaria
authors.forename: F
authors.initials: F
authors.lastname: Benzi
authors.forename: A
authors.initials: A
authors.lastname: Sanzari
authors.forename: L
authors.initials: L
authors.lastname: Bosio
authors.forename: P
authors.initials: P
authors.lastname: Brambilla
authors.forename: G
authors.initials: G
authors.lastname: Chiumello
mesh_terms: D000293:Adolescent; D000316:Adrenergic alpha-Agonists / Q000494:pharmacology* / Q000627:therapeutic use; D000328:Adult; D002648:Child; D002800:Cholinesterase Inhibitors / Q000494:pharmacology* / Q000627:therapeutic use; D003000:Clonidine / Q000494:pharmacology* / Q000627:therapeutic use; D005260:Female; D019382:Human Growth Hormone / Q000097:blood* / Q000187:drug effects / Q000378:metabolism; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008297:Male; D011218:Prader-Willi Syndrome / Q000097:blood* / Q000188:drug therapy / Q000503:physiopathology; D011729:Pyridostigmine Bromide / Q000494:pharmacology* / Q000627:therapeutic use; D017337:Sermorelin / Q000494:pharmacology* / Q000627:therapeutic use
chemical_list: D000316:Adrenergic alpha-Agonists; D002800:Cholinesterase Inhibitors; D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I; D017337:Sermorelin; D011729:Pyridostigmine Bromide; D003000:Clonidine