Literature DB >> 9766254

Growth hormone secretion in Prader-Willi syndrome.

S Grosso1, M Cioni, S Buoni, L Peruzzi, L Pucci, R Berardi.   

Abstract

Prader-Willi Syndrome (PWS) is a multisystem defect characterized by obesity, hypogenitalism and short stature for genetic background. Low GH serum levels have been found in patients with PWS and were related to a hypothalamic-pituitary dysfunction. We studied spontaneous nocturnal GH secretion and GH-response to provocative tests in five patients affected by PWS. We observed in three of them (Group A) abnormally low GH and IGF-1 serum levels. In the other two patients (Group B) GH secretion and IGF-1 serum levels were normal. In all patients no thyroid dysfunction was observed. These data might suggest the presence of two different subgroups of patients affected by PWS, from an endocrinological point of view. An abnormally low GH secretion would be evident only in a subgroup of patients, which appears to be normal in the remaining patients. This casistic is small in number, but if our data will be confirmed by more extensive studies it may be possible to identify a specific population of PWS patients who could benefit from recombinant GH-therapy.

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Year:  1998        PMID: 9766254     DOI: 10.1007/BF03347319

Source DB:  PubMed          Journal:  J Endocrinol Invest        ISSN: 0391-4097            Impact factor:   4.256


  24 in total

1.  The frequency of uniparental disomy in Prader-Willi syndrome. Implications for molecular diagnosis.

Authors:  M J Mascari; W Gottlieb; P K Rogan; M G Butler; D A Waller; J A Armour; A J Jeffreys; R L Ladda; R D Nicholls
Journal:  N Engl J Med       Date:  1992-06-11       Impact factor: 91.245

2.  Linear growth response to exogenous growth hormone in Prader-Willi syndrome.

Authors:  P D Lee; D M Wilson; L Rountree; R L Hintz; R G Rosenfeld
Journal:  Am J Med Genet       Date:  1987-12

3.  Immunoreactive insulin and growth hormone responses in patients with Prader-Willi syndrome.

Authors:  A Parra; C Cervantes; R B Schultz
Journal:  J Pediatr       Date:  1973-10       Impact factor: 4.406

4.  Growth hormone secretion and effects of growth hormone therapy on growth velocity and weight gain in children with Prader-Willi syndrome.

Authors:  M Angulo; M Castro-Magana; B Mazur; J A Canas; P M Vitollo; M Sarrantonio
Journal:  J Pediatr Endocrinol Metab       Date:  1996 May-Jun       Impact factor: 1.634

5.  Growth hormone secretion in the Prader-Labhart-Willi syndrome.

Authors:  J Hasen; R H Wu
Journal:  Fertil Steril       Date:  1982-10       Impact factor: 7.329

6.  Growth hormone (GH) response to combined pyridostigmine and GH-releasing hormone administration in patients with Prader-Labhard-Willi syndrome.

Authors:  M Cappa; A Grossi; P Borrelli; E Ghigo; J Bellone; S Benedetti; D Carta; S Loche
Journal:  Horm Res       Date:  1993

7.  [Prader-Labhart-Willi syndrome].

Authors:  W H Fesseler; J R Bierich
Journal:  Monatsschr Kinderheilkd       Date:  1983-12       Impact factor: 0.323

8.  [Endocrine study in the Prader-Willi syndrome. Apropos of 5 cases].

Authors:  L Calisti; N Giannessi; G Cesaretti; G Saggese
Journal:  Minerva Pediatr       Date:  1991-09       Impact factor: 1.312

9.  Pyridostigmine counteracts the blunted growth hormone response to growth hormone-releasing hormone of obese children.

Authors:  S Loche; C Pintor; M Cappa; E Ghigo; R Puggioni; V Locatelli; E E Müller
Journal:  Acta Endocrinol (Copenh)       Date:  1989-05

10.  Prader-Willi syndrome: consensus diagnostic criteria.

Authors:  V A Holm; S B Cassidy; M G Butler; J M Hanchett; L R Greenswag; B Y Whitman; F Greenberg
Journal:  Pediatrics       Date:  1993-02       Impact factor: 7.124

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  4 in total

Review 1.  Growth hormone therapy in the Prader-Willi syndrome.

Authors:  W F Paterson; M D C Donaldson
Journal:  Arch Dis Child       Date:  2003-04       Impact factor: 3.791

2.  GH/IGF-I axis in Prader-Willi syndrome: evaluation of IGF-I levels and of the somatotroph responsiveness to various provocative stimuli. Genetic Obesity Study Group of Italian Society of Pediatric Endocrinology and Diabetology.

Authors:  A Corrias; J Bellone; L Beccaria; L Bosio; G Trifirò; C Livieri; L Ragusa; A Salvatoni; M Andreo; P Ciampalini; G Tonini; A Crinò
Journal:  J Endocrinol Invest       Date:  2000-02       Impact factor: 4.256

3.  Central precocious puberty and abnormal chromosomal patterns.

Authors:  S Grosso; C Anichini; R Berardi; P Balestri; L Pucci; G Morgese
Journal:  Endocr Pathol       Date:  2000       Impact factor: 3.943

Review 4.  Prader Willi Syndrome: Genetics, Metabolomics, Hormonal Function, and New Approaches to Therapy.

Authors:  Krystal A Irizarry; Mark Miller; Michael Freemark; Andrea M Haqq
Journal:  Adv Pediatr       Date:  2016-08
  4 in total

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