Recent evidence indicates healthy outcomes of weight and height can be achieved with growth hormone therapy and dietary restriction and should be the current target for all individuals with PWS.
Patients with PWS present with a decreased physical performance and impaired cardiorespiratory (maximal oxygen consumption, heart rate recovery after exercise) and hormonal (growth hormone release) responses to exercise.
The present study demonstrated favorable evolution of hip radiographic parameters in the PWS population treated with growth hormone early in development.
This study aimed to investigate the GH/insulin-like growth factor (IGF-I) axis and the prevalence of GHD in previously GH-treated young adults with PWS.
Increased sensitivity may accelerate spinal growth and contribute to scoliosis, particularly in GH-deficient and treated populations such as Prader-Willi syndrome (PWS).
Twenty-seven young adults with PWS were stratified for gender and BMI and then randomly and blindly assigned to receive GH (0.67 mg/m<sup>2</sup> /day) or placebo for 1 year, after which they crossed over to the alternative treatment for another year.
To investigate the effects of GH vs placebo on metabolic health, in young adults with PWS who were GH-treated for many years during childhood and had attained adult height (AH).
We also reported the onset of diabetes mellitus (DM), scoliosis, and respiratory problems in patients with Prader-Willi syndrome who were treated with GH.
There were no differences in rates of aspiration for gender, genetic subtype, or GH use.A high incidence of aspiration was identified indicating swallow dysfunction may frequently be present in infants with PWS.
This study emphasizes the importance of evaluating bone mineralization in individuals with PWS and the beneficial effects of prolonged treatment with growth hormone.
Paediatric GH is currently licensed in six different conditions: growth hormone deficiency (GHD), Turner syndrome (TS), small for gestational age (SGA), Prader-Willi-syndrome (PWS), chronic renal insufficiency (CRI), and short stature due to SHOX deficiency; all of these have been ratified by the most recent (2010) NICE review.
Curves for height/length, weight and body mass index were constructed and compared between Prader-Willi syndrome patients treated with and without growth hormone to determine how growth hormone treatment affected body composition.
However, patients with PWS carrying a d3 allele had significantly greater height standard deviation scores (SDS) (P = 0.025) and higher insulin-like growth factor I (IGF-I) level (P = 0.041), although the age at the start of GH therapy, weight, BMI, and body fat were not different.
Thirty children with PWS (median age 7.1 yr, 18 males, 12 females) that were being treated with GH and 32 obese children not receiving GH treatment (median age 9.1 yr, 15 males, 17 females) for comparison were enrolled.