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Management of Prader-Willi Syndrome is the first book to provide a comprehensive source of knowledge about Prader-Willi Syndrome and to offer common-sense guidelines for management. It consists of contributions from professionals in many health and allied disciplines who have worked with this special population. The book focuses on clinical, social, familial, and community issues related to care. It is directed to health, education, and other specialists in academic, clinical, and community settings. Management of Prader-Willi Syndrome describes strategies for management which are appropriate to an interdisciplinary approach.
Management of Prader-Willi Syndrome brings together the contributions of professionals with considerable expertise in diagnosis and management of PWS. Clinical, social, family, and community issues are explored and management strategies identified. The text presents historical, medical, and genetic information to orient the reader. The major portion deals with pragmatic guidelines, rather than research and diagnosis, and is directed to health and educational specialists in academic, clinical, and community settings. This manual is endorsed by The Prader-Willi Syndrome Association, which is recognized world-wide.
First multi-year cumulation covers six years: 1965-70.
Although Prader-Willi syndrome was first described 35 years ago, it was following detection of an interstitial chromosome 15q deletion in some affected patients ten years ago that it became a major focus of multidisciplinary scientific interest. This interest was compounded by the later determination that some patients with a clinically distinct disorder, Angelman syndrome, apparently also had the same chromosome 15q deletion. Subsequently, molecular genetic studies showed that some cytogenetically normal patients with both disorders have uniparental disomy, maternal in Prader-Willi syndrome and paternal in Angelman syndrome. Genetic imprinting has been implicated in this unusual phenomenon....
The management of and attitudes toward children and adults with Down syndrome have undergone considerable changes in the course of the condi tion's long history (Zellweger, 1977, 1981, Zellweger & Patil, 1987). J. E. D. Esquirol (1838) and E. Seguin (1846) were probably the first physicians to witness the condition without using currently accepted diagnostic designa tions. Seguin coined the terms furfuraceus or lowland cretinism in contradis tinction to the goiterous cretinism endemic at that time in the Swiss Alps. Esquirol, as well as Seguin, had a positive attitude toward persons who were mentally ill or mentally subnormal. Esquirol pioneered a more humane treatment in mental institutions...