This book is dedicated to an update on metabolic disorders and their consequences on the developing nervous system. In the last ten years, major scientific advances in biochemical diagnosis have enabled the identification of many new disease entities, as well as a better classification of clinical symptoms and their correlation to biochemical defects. This volume provides an introduction to basic sciences specifically relevant to this disease group, then focuses on each subgroup in turn: hyperphenylalaninaemias, mitochondrial encephalomyopathies, organic acidoses, urea cycle defects, lysosomal storage and peroxylsomal disorders. Finally, a discussion of long term follow-up and family related issues also reports the results of a four-year research project led by the Mariani Foundation.

Sufficient levels of muscle strength, defined as the maximal force an individual is capable of exerting during a voluntary contraction, is a critical component to overall physical health and well-being. Muscle strength generally increases with age throughout childhood and adolescence. This book discusses specific methods of assessment and factors affecting muscle strength in children. In addition, many other factors including gender and physical activity levels have previously been reported to affect the structure or metabolic characteristics of skeletal muscle. This book reviews the literature describing the maladaptations that occur in skeletal muscle in response to the most common lifestyle associated chronic diseases and discusses the mechanisms behind the maladaptations, as well as potential therapies to halt or reverse the changes. This book also examines the impact of ageing on muscle strength, as well as reviews the physiological and mechanical properties of muscle architecture essential to the production of muscle force under different loading conditions.