the management of genetic disorders in the series “progress in clinical and biological...

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Early Human Development, 5 (1981) 217-224 Elsevier/North-Holland Biomedical Press 217 BOOK REVIEWS The Management of Genetic Disorders in the series “‘Progress in Clinical and Biological Research”, Vol. 34 C.S. Bartsocas and C.J. Papadatos (eds.) Alan R. Liss, Inc., New York, 1979 (430 pages; US $ 32.00) Amongst the sciences that inform clinical practice, genetics has been surpris- ingly slow in assuming its rightful place bearing in mind that our age will be remembered in scientific history as that in which the genetic code was first cracked. This may be because practising physicians need to be optimistic about treatment and as Alan Emery puts it in the first chapter of the Man- agement of Genetic Disorders, genetic disease is invariably serious, rarely treatable, and never curable. This is, however, a pessimistic view that is partly contradicted later in the volume by Leon Rosenberg who, in his chap- ter on therapeutic modalities for genetic diseases, states in the introduction that the simplistic notion that all diseases are either inherited or acquired has led illogically to the idea that acquired disorders are treatable even curable whereas inherited ones are not. To find such basically disparate views between the covers of one book is the inevitable consequence of its genesis, which was as the record of the proceedings of the Second International Clinical Genetics Seminar on the Management of Genetic Disorders held in Athens, Greece, in June 1979, and inevitably raises the question of whether the ephemerides of such a conference are properly to find their way into Karl Popper’s third realm of knowledge on the Library shelves. Of course we would all like to be invited to such conferences but they have to be kept small if they are to be at all useful and we should perhaps regard the records of their proceedings in the same light as newspapers, keeping us up to date with day to day events but in no sense authoratative as history. The great merit of this volume is that it is only one year out of date and for this one can forgive.the editors its infelicities of style and printing which are in any case almost inevitable when so many contributors are speaking and writing in a language other than their own. As one might imagine would be the case in such a compilation, contributions vary a great deal in their matter, style, value and emphasis - ranging from an overview of genetic councelling in the U.S.S.R. at one end of the scale to inborn errors of membrane transport, mechanisms and implications for treatment at the other - and it is therefore difficult to define the readership that the book deserves or will get. It is im- possible in any relatively brief review even to list the 34 different chapters which with author and subject index and lists of references of various degrees of comprehensiveness that make up its 419 pages. But it would be roughly

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Page 1: The management of genetic disorders in the series “progress in clinical and biological research”, vol. 34: C.S. Bartsocas and C.J. Papadatos (eds.) Alan R. Liss, Inc., New York,

Early Human Development, 5 (1981) 217-224 Elsevier/North-Holland Biomedical Press

217

BOOK REVIEWS

The Management of Genetic Disorders in the series “‘Progress in Clinical and Biological Research”, Vol. 34 C.S. Bartsocas and C.J. Papadatos (eds.) Alan R. Liss, Inc., New York, 1979 (430 pages; US $ 32.00)

Amongst the sciences that inform clinical practice, genetics has been surpris- ingly slow in assuming its rightful place bearing in mind that our age will be remembered in scientific history as that in which the genetic code was first cracked. This may be because practising physicians need to be optimistic about treatment and as Alan Emery puts it in the first chapter of the Man- agement of Genetic Disorders, genetic disease is invariably serious, rarely treatable, and never curable. This is, however, a pessimistic view that is partly contradicted later in the volume by Leon Rosenberg who, in his chap- ter on therapeutic modalities for genetic diseases, states in the introduction that the simplistic notion that all diseases are either inherited or acquired has led illogically to the idea that acquired disorders are treatable even curable whereas inherited ones are not. To find such basically disparate views between the covers of one book is the inevitable consequence of its genesis, which was as the record of the proceedings of the Second International Clinical Genetics Seminar on the Management of Genetic Disorders held in Athens, Greece, in June 1979, and inevitably raises the question of whether the ephemerides of such a conference are properly to find their way into Karl Popper’s third realm of knowledge on the Library shelves. Of course we would all like to be invited to such conferences but they have to be kept small if they are to be at all useful and we should perhaps regard the records of their proceedings in the same light as newspapers, keeping us up to date with day to day events but in no sense authoratative as history. The great merit of this volume is that it is only one year out of date and for this one can forgive.the editors its infelicities of style and printing which are in any case almost inevitable when so many contributors are speaking and writing in a language other than their own. As one might imagine would be the case in such a compilation, contributions vary a great deal in their matter, style, value and emphasis - ranging from an overview of genetic councelling in the U.S.S.R. at one end of the scale to inborn errors of membrane transport, mechanisms and implications for treatment at the other - and it is therefore difficult to define the readership that the book deserves or will get. It is im- possible in any relatively brief review even to list the 34 different chapters which with author and subject index and lists of references of various degrees of comprehensiveness that make up its 419 pages. But it would be roughly

Page 2: The management of genetic disorders in the series “progress in clinical and biological research”, vol. 34: C.S. Bartsocas and C.J. Papadatos (eds.) Alan R. Liss, Inc., New York,

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true to state that amongst aevelopmental biologists, those engaged in clinical paediatric practice and in genetic councelling would be most likely to find material of value and interest. There are very helpful chapters on the man- agement of children with Turner’s syndrome by John Crawford and of the Mucopolysaccharidoses and Allied Disorders by Jules Leroy, a useful review of the Hereditary Renal Diseases and their medical management by Samuel Thier and of genetic thyroid disorders by Demetrios Koutras, and up to date accounts of the treatment of a number of inborn errors of metabolism including phenylketonuria, alpha-1-antitrypsin deficiency, Wilson’s disease beta-thalassemia and Refsum’s and Fabry’s diseases. Professor Lenz contri- butes a thoughtful chapter on genetic councelling in limb malformations, on which he is the obvious authority, and Jean Frezal’s chapter on genetic regulations in man, though hard going, is well worth working through. Alto- gether this is a better and more useful book than one might have expected given its origins and most paediatricians and some geneticists would find it worthwhile dipping into if they can persuade their libraries to buy it. Per- haps they should keep it out of the hands of their psychiatric colleagues in case it should reinforce the belief, now entrenched in academic psychiatry, that the origins of emotional disturbance are to be found in nature rather than nurture -with the inevitable reference back to Professor Emery’s open- ing remarks quoted above. Altogether this book justifies its place amongst the 34 volumes of Progress in Clinical and Biological Research and the place of medical genetics as one of the most important clinical disciplines.

J.A. DAVIS

High-Risk Infants and Children: Adult and Peer Interactions T.M. Field (ed.) Academic Press, New York, 1980 (387 pages; US $ 27.00)

This is one of a well produced series of books on developmental psychology. Its 36 contributors include distinguished members of some of the foremost academic departments of psychology and child development in the United States. As would be expected there is a high standard of writing and of bibliography.

It is a little difficult to understand the organisation of the volume but in essence it consists of two parts: the first concerned with interactions of cer- tain infants with their parents and the second with interactions of similar children with their peers and teachers. The first section is divided into four parts each consisting of between two and four chapters. Infants or children experiencing failure to thrive, abuse or family disruption, at risk for devel- oping autism, at risk for developmental delay and children with perceptual- motor handicaps are dealt with in successive parts. The connection between