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This book provides information on a wide variety of issues ranging from genetics to clinical description of the syndromes, genetic testing and counseling, and clinical management including surveillance, surgical and prophylactic interventions, and chemoprevention.

This is the first single-authored monograph on Roald Dahl since 1994. Remarkably, in spite of Dahl's commercial success, very little scholarly work has been produced. This study focuses on the critical context, texts and paratexts that make up the packaging of "Dahl". It offers the first thorough overview of the criticism and language employed to discuss Dahl since the 1970s. It delves into the relationship between Dahl's children's and adult fiction by exploring the common traits and patterns that bring his whole work together. It also examines how Dahl constructed himself as a children's...