Human Haemoglobin Variants And Their Characteristics

Completely revised new edition of the definitive reference on disorders of hemoglobin.

Since the dawn of the era of molecular biology, hemoglobin has been subjected to more scrutiny than any other protein, and Bunn, Forget, and Ranney can each lay claim to major contributions to the saga of hemoglobin. Their well-organized, comprehensive, and superbly illustrated work is an excellent review of the abnormal hemoglobin field. Early chapters deal with the structure and function of human hemoglobin and the way in which this is modified in various disease states. Later sections deal with the various structural hemoglobin variants and their associated clinical manifestations, the thalassaemias, and the acquired disorders of hemoglobin. The sections that deal with the modification of hemoglobin function in various disease states are particularly good. The book contains an extensive and up-to-date bibliography and is remarkably free from errors of fact or type--the best standard of reference on the subject as of the year 1977.

Research on abnormal human hemoglobins (protein in blood that carries oxygen), has taught us about the inheritance, biochemistry, and distribution of these traits. This knowledge, coupled with mathematical research using computer models of population genetics, has enabled researchers to marry biological fact and genetic theory. This volume places medical understanding in an evolutionary framework. Using published data on the frequencies of abnormal hemoglobins in the world's populations, Livingston analyzes and interprets these frequencies in the light of world distribution of different forms of diseases such as malaria. He further develops the genetic theory of the evolutionary homeostasis. Livingston discusses the relation of abnormal hemoglobins to endemic malaria and, shows how natural selection pressures explain the known distribution of these traits. Where non-coinciding distributions arise, the book presents other genetic, anthropological, evolutionary, and epidemiological evidence to explain these discrepancies. This classic work remains a useful sourcebook for professors and graduate students of anthropology, genetics, epidemiology, and hematology.

In the new edition of this successful and authoritative book, the thalassaemias are reviewed in detail with respect to their clinical features, cellular pathology, molecular genetics, prevention and treatment. It is aimed at specialists in haematology in the laboratory or clinical setting, particularly in areas where thalassaemia is common either in the native population or in immigrant communities. The fourth edition has been both updated and re-organized. Three new chapters have been added on the link between alpha-thalassaemia and mental retardation, on avoidance and population control and on global epidemiology. Considerable emphasis is placed on molecular pathology reflecting the huge burst of information to have come out of this field in the last few years.

Volume 1 of the Prevention Book presents the principles of a programme for the prevention of the thalassaemia and other haemoglobin disorders, including a description of the various types of disorders requiring prenatal diagnosis, the strategies used for carrier screening, and a number of annexes listing upto date epidemiological and mutation data on thalassaemia. This book was written for use in combination with Volume 2, which describes many of the laboratory protocols in great detail.