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Comparative Analysis of Human Growth Hormone in Serum Using SPRi, Nano-SPRi and ELISA Assays
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Diagnosis and treatment of GH deficiency in Prader-Willi syndrome

Graziano Grugni1, Paolo Marzullo2

  • 1Division of Auxology, I.R.C.C.S. Istituto Auxologico Italiano, Ospedale S. Giuseppe, Verbania, 28921, Italy.

Best Practice & Research. Clinical Endocrinology & Metabolism
|December 16, 2016
PubMed

Insights

Prader-Willi syndrome (PWS) is linked to growth issues due to growth hormone deficiency (GHD). Growth hormone therapy (GHT) improves growth, body composition, and neurocognition in PWS patients.

Area of Science:

  • Endocrinology
  • Genetics

Background:

  • Prader-Willi syndrome (PWS) is a genetic disorder caused by the under-expression of the paternal 15q11-13 chromosomal region.
  • Growth failure and growth hormone deficiency (GHD) are common in both children and adults with PWS.

Purpose of the Study:

  • To review the benefits and safety of growth hormone therapy (GHT) in individuals with Prader-Willi syndrome.
  • To assess the impact of GHT on growth, body composition, metabolic health, neurocognition, and quality of life in PWS patients.

Main Methods:

  • Review of published data on growth hormone therapy in Prader-Willi syndrome patients.
  • Analysis of biochemical criteria for GHD in PWS adults.
  • Evaluation of the effects of GHT on various physiological and functional parameters.

Main Results:

  • GHD is biochemically confirmed in a significant percentage of PWS adults (8-38%).
  • Early GHT in PWS children improves statural growth, body composition, metabolic homeostasis, and neurocognitive function.
  • GHT in PWS adults enhances lean body mass, reduces fat mass, improves exercise capacity, and boosts quality of life.

Conclusions:

  • Growth hormone therapy is beneficial for both pediatric and adult patients with Prader-Willi syndrome.
  • While generally safe, prolonged GH replacement in PWS requires careful risk monitoring.

Keywords:
GH deficiencyGH therapyPrader–Willi syndromeshort stature

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