ESPE Abstracts

Context: Some of the features of subjects with Prader-Willi syndrome (PWS) resemble those seen in subjects with growth hormone deficiency (GHD). Children with PWS are treated with long-term growth hormone (GH), which has substantially changed their phenotype. Currently, young adults with PWS have to stop GH treatment after attainment of adult height when they do not have adult GHD. Limited information is available about the prevalence of adult GHD in patients with PWS.<p c...

Background: Prader-Willi syndrome (PWS) is known for hyperphagia with impaired satiety and a specific behavioral phenotype with stubbornness, manipulative and controlling behavior and obsessive-compulsive features. PWS is associated with hypothalamic and oxytocinergic dysfunction. In humans without PWS, intranasal oxytocin administration had positive effects on social behavior and weight balance.Objective and hypotheses: To evaluate the effects of intran...

Context: Children with Prader-Willi syndrome (PWS) generally have mild to moderate cognitive impairment with an IQ between 60 and 70. Growth hormone (GH) treatment is a registered treatment for children with PWS and has been associated with cognitive benefits, attributed to the effects of GH and insulin-like growth factor on brain growth and development. Short-term data suggest positive effects of GH treatment on cognitive functioning in children with PWS. The...

Context: Infants and toddlers with Prader-Willi Syndrome (PWS) have a mental and motor developmental delay. Short-term data suggest a positive effect of growth hormone (GH) on mental and motor development in infants and children with PWS. There are, however, no longer-term results about the effects of GH treatment on mental and motor development.Objective: To investigate the longer-term effects of GH on psychomotor development in infants and toddlers wit...

Background: Growth hormone treatment (GH) in children with PWS results in an improvement in height velocity, body composition and mental and motor development. Discontinuation of GH after attainment of adult height (AH) leads to a decrease in lean body mass and an increase in body fat percentage, which results in an increased risk of impaired glucose tolerance (IGT) and diabetes mellitus type 2 (DM2). Studies in adults with PWS suggest positive effects of GH, but GH is known t...

Context: Most children with Prader-Willi syndrome (PWS) develop scoliosis. Scoliosis has a prevalence of 80% in children with PWS older than 10 years, who were not treated with growth hormone (GH). GH is an approved treatment for children with PWS and improves psychomotor development and body composition. The onset and progression of scoliosis are generally associated with an accelerated growth velocity and GH treatment might cause accelerated growth. Sinc...

Background: In children with Prader-Willi syndrome (PWS), the benefits of growth hormone (GH) treatment are well established. GH has substantially changed the phenotype of children with PWS. Discontinuation of GH after adult height (AH) attainment leads to a decrease in lean body mass and an increase in body fat percentage. Due to their abnormal body composition, adults with PWS are predisposed to develop impaired glucose tolerance (IGT) and diabetes mellitus type 2 (T2DM). Re...

Introduction: Individuals with Prader-Willi syndrome (PWS) suffer from hyperphagia, hypotonia and hypothalamic dysfunction, leading to a variety of pituitary hormone deficiencies. Central adrenal insufficiency (CAI) has been reported in PWS, while each of these studies used different testing modalities and cut-off values. Therefore, reported prevalence of CAI ranges from 0% to 60%. It has been speculated that CAI might be responsible, at least in part,...

Context: In children with Prader-Willi syndrome (PWS), the benefits of growth hormone (GH) treatment are well established. Currently, when young adults with PWS have attained adult height (AH), they have to stop GH treatment. Several one year studies have shown that GH treatment is also beneficial for adults with PWS, improving body composition. However, little is known about the longer-term effects.Objective: To investi...

Introduction: Recombinant Growth Hormone (GH) has changed the lives of many patients with Prader-Willi Syndrome (PWS). GH treatment has beneficial effects on body composition, physical performance, cognition, psychomotor development, respiratory function and quality of life of patients with PWS. Due to the narrow therapeutic range, GH treatment is subject to strict limits. Clinicians measure serum immunoreactive Insulin-like Growth Factor 1 ('total IGF-I&#...