Organelle Diseases: clinical features, diagnosis, pathogenesis and management. Edited by D. A. Applegarth, J. E. Dimmick and J. G. Hall

we have classiÐed metabolic diseases on the basis of the biochemical Traditionally nature of the metabolites involved. This volume makes a novel but timely approach by focusing on the major organelles where the disease primarily manifests itself at the subcellular level. During the last three decades we have witnessed major advances in our knowledge of disorders of the lysosome, the peroxisome and now the mitochondrion ; maybe the Golgi apparatus will be next. When a text is impressively sandwiched between a Foreword by Charles Scriver and a Postscript by Victor McKusick, we can be pretty sure that the contents will be worth savouring. This is certainly the case here and we are not disappointed. There is a wealth of information on lysosomal disorders, peroxisomal disorders and mitochondrial disorders. Each section covers the basic science and function of the organelle, clinical diagnosis of the disorders, their biochemical diagnosis and Ðnally the pathology. The mitochondrial section, however, only covers disorders of the respiratory chain and mtDNA mutations. Other mitochondrial disorders such as fatty acid oxidation defects, and various other organic acid or urea cycle defects, are deliberately not included. A fourth section deals with practical issues such as therapy, taking a family history, physical examination and the metabolic autopsy. Finally, four appendices deal with consent for DNA banking, addresses of various support groups, useful web sites and a glossary. The strength of this book is that it has been written by authors who clearly have considerable Ðrst-hand experience of the various topics covered. The layout is excellent and it is easy to Ðnd your way around. It is well illustrated with clear tables, Ñow charts and clinical photographs as well as light and electron micrographs. Apart from one Ðgure that seems to have been duplicated and a couple of incorrect references, it would be hard to be critical of this book. In conclusion, this is an excellent volume and one that is recommended to all those with an interest in the diagnosis and management of organelle disorders.