The management of Prader-Willi syndrome from paediatric to adult care
Ann Christin Lindgren
Subjects with Prader-Willi syndrome (PWS) need a comprehensive care of a multi-disciplinary team from childhood to adulthood. It is not only growth, body composition and sexual maturation that are compromised in PWS. The complications due to overweight like Type-2 Diabetes and cardio-vascular disorders need to be prevented already during childhood. The psychiatric well-being of individuals with PWS is important to focus on and also to discover co-morbidities like autism to be able to find the appropriate education, care and understanding for the individuals. Improvement of quality of life in individuals with PWS is the main goal with the care.
The international approval for treatment with Growth hormone (GH) in PWS during childhood has made a difference. Treatment with GH has beneficial effect on linear growth with increased final height and also improvement of body composition and bone density.
Studies of subjects with growth hormone deficiency have shown that it is important to continue GH treatment during transition from late adolescence to early adulthood to achieve peak bone mass (PMB) after completion of linear growth and puberty. This is probably true in adolescents with PWS as many have a GH deficiency and a hypogonadism.
In addition to increased linear growth and increased bone density, maintenance of a favourable body composition in PWS is an important goal with the GH treatment in combination with sex steroid replacement. The dose of GH needed to achieve PMB and maintaining body composition in individuals with PWS, as well as, the dose and timing of introduction of sexual hormones are important topics that need to be further studied.