Prader-Willi Syndrome
Development and Manifestations

Based on a unique cohort study, this book identifies the complex care needs of people with Prader-Willi syndrome.

Joyce Whittington (Author), Tony Holland (Author)

9780521840293, Cambridge University Press

Hardback, published 22 April 2004

232 pages
25.5 x 18 x 1.9 cm, 0.57 kg

Review of the hardback: '… provides a wealth of very valuable facts to add to the jigsaw pieces that will eventually reveal a whole picture of a fascinating syndrome.' European Journal of Paediatric Neurology

Prader-Willi syndrome (PWS) is associated with an assortment of physical, behavioural and cognitive abnormalities which create a broad range of care needs. Information about the syndrome is spread across a variety of disciplines. In this book the authors seek to identify and provide the latest findings about how best to manage the complex medical, nutritional, psychological, educational, social and therapeutic needs of people with PWS. Their approach is an integrated one, centred on the PWS phenotype. Both authors have been involved in the Cambridge PWS study, which is the largest and most rounded of the cohort studies of PWS anywhere in the world. The unique data it provides is the basis of this book.

Introduction
Part I. Background: PWS, Why, What, and How To Investigate: 1. Background and historical overview
2. Biological and regulatory mechanisms in PWS
3. The Cambridge PWS project
Part II. PWS Prevalence, Phenotypic Functioning and Characteristics: 4. Prevalence, birth incidence and mortality
5. Relationship between genetic and clinical diagnosis
6. Phenotypic differences between the genetic subtypes
7. Cognitive function and attainments
8. The behavioural phenotype of PWS
9. Medical conditions affecting people with PWS
10. Psychiatric illness
11. Obsessions and compulsions
Part III. Minor Findings, Some Conclusions and Future Directions: 12. Understanding PWS
Index.

Subject Areas: Psychiatry [MMH], Paediatric medicine [MJW], Medical genetics [MFN]