Ciba Foundation Symposium Research Articles

The fetus and independent life the ciba foundation symposium no. 86

Many clinical features of autosomal centronuclear myopathies (CNM) and X-linked myotubular myopathy (XLMTM) are common to congenital myasthenic syndromes (CMS). We describe three children whose clinical and electrophysiological findings originally suggested CMS, in whom CNM was diagnosed pathologically, though not yet genetically characterised. A fourth case, with XLMTM, also showed electrophysiological features of a neuromuscular transmission defect. Three (including the XLMTM case) showed improved strength with acetylcholinesterase inhibitor treatment. We also studied neuromuscular junction structure and function in the MTM1 knockdown zebrafish model of XLMTM, demonstrating abnormal neuromuscular junction organization; anticholinesterase therapy resulted in marked clinical response.These observations suggest that a neuromuscular transmission defect may accompany CNM and contribute to muscle weakness. Muscle biopsy should be considered in infants suspected to have CMS, especially if treatment response is incomplete, or no CMS gene mutation is identified. Treatment with acetylcholinesterase inhibitors may benefit some CNM patients. This warrants further confirmation.

Book Reviews

Book reviews in this article: Tracing Neural Connections with Horseradish Peroxidase edited by Mesulam. Development of the Autonomic Nervous System: Ciba Foundation Symposium 83 edited by G. Burnstock. Glutamate: Transmitter in the Central Nervous System edited by P. J. Roberts, J. Storm‐Mathisen, and G. A. R. Johnston.

Adhesion and microorganism pathogenicity: Edited by KATHERINE ELLIOT, MAEVE O'CONNOR and JULIE WHELAN. 1981. Ciba Foundation Symposium No. 80. Pitman Medical, London. Pp. x and 346.

Adhesion and microorganism pathogenicity: Edited by KATHERINE ELLIOT, MAEVE O'CONNOR and JULIE WHELAN. 1981. Ciba Foundation Symposium No. 80. Pitman Medical, London. Pp. x and 346.

Book Review

Books reviewed in this article: Peptides of the Pars Intermedia: Ciba Foundation Symposium 81 edited by D. Evered and G. Lawrenson

Development of Mammalian Absorptive Processes (review)

This is a book that can be read by any biologist, or any scientist for that matter. But as I read it, I thought how nice it would be if it could be placed on some universal short list of required reading for young clinical neurologists. The author's earlier book Charles Sherrington: An Appraisal would also be on the list. From the two one can learn in a few evenings about the transformation of Fechner's "outer psychophysics" to what was inaccessible to Fechner, though he knew it would come into existence some day. He called it "inner psychophysics." It is what we now call neurophysiology. As a further characterization of the physical side of Fechner's equation, how much closer does neurophysiology take us to mind? In answering that question it is easy to talk nonsense. Granit deals with it quietly and sensibly, yielding in the end, so far as I can gather, to the sign ignorabimus on the wall, but not to the temptations that have snared a good many other biologists. There is no reference here to an entity called "conscious awareness" which analyzes and interprets patterns of nerve impulses in the cerebral cortex. Nor will you find in this book any claim to the effect that neurophysiology is on its way to setting up energy equivalents for thoughts, and that when this is done the problem of consciousness will no longer exist. It is probably superfluous to say that I like very much the idea that Professor Granit's book is on my shelf—next to his book on Sherrington and his monographs on the retina, the receptor organs in general, and on the physiology of motor control. If any of these were not there where tliey belong, I would be restless and discontent until they were replaced. REFERENCE 1. Roe, A., and Simpson, G. G. (eds.). Behavior and evolution. New Haven, Conn.: Yale Univ. Press, 1958. Sidney Schulman Division ofBiological Sciences University of Chicago Development ofMammalianAbsorptive Processes. CIBA Foundation Symposium, n.s. 70. Amsterdam: Excerpta Medica, 1979. Pp. xii+338. These proceedings consist of 16 papers and related discussion periods presented at an international symposium in London, England, in January 1979. The goal of the meeting was to examine critically past and current experimental research on intestinal maturation and to define promising areas for future study. While most of the data were derived from laboratory animals, serious efforts were made to relate the findings to man—as emphasized in the transcribed interactions which follow each paper and in a 14-page concluding general discussion . The symposium was chaired byJohn T. Harries of the Institute of Child Health in London, who emphasized that recent scientific advances have resulted in a marked increase in the survival of very young premature infants and that a Perspectives in Biology and Medicine, 25, 3 ¦ Spring 1982 507 clearer understanding of the development of fetal and neonatal absorptive processes should help even further in this regard. The authoritative coverage includes intestinal morphogenesis, epithelial cell differentiation, intestinal enzyme development, bile acid metabolism, the early transport of macromolecules, hormonal factors in gut maturation, the nutritional needs of the premature infant, and perinatal changes in the absorption of trace elements. Each article starts with an informative abstract, and the clearly written text is supplemented by 85 figures and 23 tables. The bibliographies provide useful references for background reading, while the separately referenced discussion periods cite many articles that were still in press in early 1979. As a result, this book can be recommended as an excellent starting point for students or research workers interested in developmental aspects of human nutrition. It should certainly also be of much interest to perinatologists, pediatric gastroenterologists, and others concerned with all aspects of intestinal function in prematurity, health, and disease. Stimulating ideas and suggestions for needed investigation abounded at this meeting; the proceedings make fascinating reading. Sumner C. Kraft Department of Medicine University of Chicago A Century of Surgery: The History of the American Surgical Association. By Mark M. Ravitch. 2 vols. Philadelphia: J. B. Lippincott Co., 1981. Pp. lvii+1737. $195.00. In 1980 the American Surgical Association had its one hundredth annual meeting. To mark...

BOOK REVIEWS

Clinical and Experimental Pharmacology and PhysiologyVolume 9, Issue 1 p. 115-118 Free Access BOOK REVIEWS First published: February 1982 https://doi.org/10.1111/j.1440-1681.1982.tb00787.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Abstract Perspectives in Clinical Endocrinology. Edited by Walter B. Essman Environmental Chemicals, Enzyme Function and Human Disease, Ciba Foundation Symposium No. 76 (New Series). Membrane Physiology and Cell Excitation. By Bruce Hendry. Central Neurotransmitter Turnover. Edited by C.J. Pycock and R.V. Taberner. Volume9, Issue1February 1982Pages 115-118 RelatedInformation

Protein Degradation in Health and Disease (Ciba Foundation Symposium 75, New Series)

Book Review| February 01 1982 Protein Degradation in Health and Disease (Ciba Foundation Symposium 75, New Series) Protein Degradation in Health and Disease (Ciba Foundation Symposium 75, New Series). Excerpta MedicaAmsterdam, Oxford and New York 1980. pp. 417, $70 J. M. EVANSON J. M. EVANSON Search for other works by this author on: This Site PubMed Google Scholar Biochem Soc Trans (1982) 10 (1): 63. https://doi.org/10.1042/bst0100063a Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Cite Icon Cite Get Permissions Citation J. M. EVANSON; Protein Degradation in Health and Disease (Ciba Foundation Symposium 75, New Series). Biochem Soc Trans 1 February 1982; 10 (1): 63. doi: https://doi.org/10.1042/bst0100063a Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search Dropdown Menu nav search search input Search input auto suggest search filter All ContentAll JournalsBiochemical Society Transactions Search Advanced Search This content is only available as a PDF. © 1982 Biochemical Society1982 Article PDF first page preview Close Modal You do not currently have access to this content.

Adhesion and Microorganism Pathogenicity. Ciba Foundation Symposium 80. Pp. 346. (Pitman Medical, 1981.) £19.50

<i>Adhesion and Microorganism Pathogenicity</i>. Ciba Foundation Symposium 80. Pp. 346. (Pitman Medical, 1981.) £19.50

Trends in enzyme histochemistry and cytochemistry Ciba Foundation Symposium No. 73 (new series)

Trends in enzyme histochemistry and cytochemistry Ciba Foundation Symposium No. 73 (new series)

Protein degradation in health and disease. Ciba Foundation Symposium 75, 417 pp, Excerpta Medica, Amsterdam, The Netherlands, 1980. $70.75

Muscle & NerveVolume 4, Issue 6 p. 532-532 Book Review Protein degradation in health and disease. Ciba Foundation Symposium 75, 417 pp, Excerpta Medica, Amsterdam, The Netherlands, 1980. $70.75 R. H. T. Edwards PhD, FRCP, R. H. T. Edwards PhD, FRCP London, EnglandSearch for more papers by this author R. H. T. Edwards PhD, FRCP, R. H. T. Edwards PhD, FRCP London, EnglandSearch for more papers by this author First published: November/December 1981 https://doi.org/10.1002/mus.880040614AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume4, Issue6November/December 1981Pages 532-532 RelatedInformation

Resolution of the need for continuous nocturnal feedingin a patient with severe type I glycogen storage disease

Rahimtoola J: Neutralizing antibodies against E. coli and V. cholera enterotoxins in human milk from a developing country, Scand J lmmunol 5:867, 1976. 3. Wyatt RG, Garcia B, C~ceres A, and Mata LJ: Immunoglobulins and antibodies in colostrum and milk of Guatemalan mayan women, Arch Latinoamer yutr 22:629, 1972. 4. Fubara ES, and Freter R: Protection against enteric bacterial infection by secretory IgA antibodies, J Immunol 111:395, 1973. 5. Ahlstedt S, Carlsson B, Fallstr~m SP, Hanson LA, Holmgren J, Lidin-Janson G, Lindblad BS, Jodal U, Kaijser B, Sohl-Akedund A, and Wadsworth C: Antibodies in human serum and milk induced by enterobacteria and food proteins, in: Immunology of the gut, CIBA Foundation Symposium 46, 1977, p 115. 6. Walker WA: Antigen handling by the gut, Arch Dis Child 53:527, 1978. 7. Urrutia JJ, Garcia B, Carballo M, DeMaeyer E, Hanson LA, and Cruz JR: Daily milk outputs produced by Guatemalan women of three different socioeconomic characteristics (submitted to Am J Clin Nutr). 8. Sohl-Akerlund AS, Hanson LA, Ahlstedt S, and Carlsson B: A sensitive method for specific quantitation of secretory IgA, Scand J Immunol 6:1275, 1977. 9. Ahlstedt S, Carlsson B, Hanson LA, Kaijser B, Mattsby Baltzer I, and Sohl-/~kerlund A: Application of the ELISA for determination of immunoglobulin class-specific Escherichia coli antibodies, Scand J Immunol 8(Suppl 7):119, 1978. 10. Hanson LA, Carlsson B, Cruz JR, Garcia B, Holmgren J, Khan SR, Lindblad BS, Svennerholm A-M, Svennerholm B, and Urrutia J: Immune response in the mammary gland, in Ogra PL, and Dayton DH, editors: Immunology of breast milk, New York, 1979, Raven Press, p 145.

Trends in Enzyme Histochemistry and Cytochemistry (CIBA Foundation Symposium 73, New Series)

Trends in Enzyme Histochemistry and Cytochemistry (CIBA Foundation Symposium 73, New Series)

Book Reviews

Human genetics: Possibilities and realities. Ciba Foundation Symposium 66 (new series). Pp. xi + 425. Price: $51.25 (Dfl. 105.00).

Book Reviews

Book reviewed in this article:CARBOHYDRATE METABOLISM IN PREGNANCY AND THE NEWBORN 1978, edited by H. W. Sutherland and J. M. Stowers; 1979.PAEDIATRIC EMERGENCIES, edited by J. A. Black; 1979.PEDIATRIC DIFFERENTIAL DIAGNOSIS. A Problem‐Oriented Approach, by Stephen H. Sheldon; 1979.THE ORTHOPAEDIC MANAGEMENT OF SPINA BIFIDA CYSTICA, Second Edition, by Malcolm Menelaus; 1980.CEREBRAL ANEURYSMS. Advances in Diagnosis and Therapy, edited by H. W. Pia, C. Langmaid and J. Zierski; 1979.DEVELOPMENT OF MAMMALIAN ABSORPTIVE PROCESSES. Ciba Foundation Symposium 70 (new series); 1979.CLINICAL PERINATOLOGY, edited by Silvio Aladjem, Audrey K. Brown and Claude Sureau, 2nd edition; 1980.DIAGNOSIS AND MANAGEMENT OF THE FETUS AND NEONATE AT RISK, by S. Gorham Babson, Martin L. Pernoll, Gerda I. Benda and Katherine Simpson, 4th edition; 1980.EMERGENCY TRANSFER OF THE HIGH‐RISK NEONATE, by Angelo Ferrara and Anantham Harin; 1980.HEART DISEASE IN INFANTS AND CHILDREN, edited by Gerald Graham and Ettore Rossi; 1980.

Advances in Genetics Edited by E. W. Caspari (review)

Advances in Genetics. Vol. 20. Edited by E. W. Caspari. New York: Academic Press, 1979. Pp. 464. $41.00. Advances in Genetics was launched 20 volumes ago to cope with the rapid progress in the diverse areas of life sciences impacted by genetic research. As genetic principles, concepts, and techniques were applied to these areas—including medicine—either by geneticists or converts to the genetic approach, specialized bodies of knowledge were gradually isolated or insulated from the mainstream of genetics. In contrast to the annual reviews which are frequently annotated bibliographies , lavane« in Genetics presents minimonographs with depth, breadth, and frequently considerable scholarship. Each volume attempts a broad coverage. In volume 20, the minimonographs cover influenza virus genetics (C. Scholtissek); applied microbiology (V. S. Malik); plant tissue culture and breeding (I. K. Vasil, M. R. Ahuja, and V. Vasil); mechanisms of sex determination, gonadal sex differentiation, and germ-cell development in animals (J. R. McCarrey and U. K. Abbott); recent advances in histocompatibility (G. D. Snell); and hereditary anemias of the mouse (E. S. Russell). The minomonographs range from 28 to 87 pages and include extensive references to publication date. Geneticists are likely to weigh price and specialized contents against their specific interests in adding this volume to the personal library. On the other hand, each volume is a worthwhile and perhaps necessary addition to departmental and institutional libraries. Volume 20 maintains the high standards in this important series of great interest to geneticists and nongeneticists attempting to keep abreast of the knowledge rather than the information explosion in their fields. Human Genetics: Possibilities and Realities. CIBA Foundation Symposium 66 (New Series). New York: Elsevier North-Holland Publishing Co., 1979. Pp. 437. $51.25. Many geneticists are unaware of the riches to be found in the human species. The application of the concepts and tools of molecular genetics and biology to an unlikely experimental species, man, has already yielded extraordinary returns for both basic genetic theory and human biology, including medicine. The CIBA Symposium 66 is indeed devoted to the possibilities and realities of human genetics. The chairman's closing remarks concluded with a valid and frank statement: "We have had a very exciting meeting and our symposium will be a landmark even if it records our confused perception of the future of genetics and human biology." The 16 contributed papers range from "Cultural Change and Its Relevance for Human Genetics" to "Genetics in an Oocyte," Three papers were concerned with immunology and three with cancer, indicating the weight of these areas in current research. Each contributor was assigned an area compatible with his expertise, and each contribution had a broader than usual view of the relationship between genetics and the area. Frank discussions follow each presentation and illuminate each area, highlighting differences of opinions among Perspectives in Biology and Medicine · Spring 1981 | 505 ...

Health and Disease in Tribal Societies. Ciba Foundation Symposium 49

<b>Health and Disease in Tribal Societies</b>. <i>Ciba Foundation Symposium 49</i>

Drug Concentrations in Neuropsychiatry. Ciba Foundation Symposium 74 (new series). (Pp. 266; illustrated; $45.75.) Excerpta Medica: Amsterdam. 1980. - Pharmacokinetics of Psychoactive Drugs. Edited by L. A. Gottschalk (Pp. 158; illustrated; £14.00.) SP Medical &amp; Scientific Books: London. 1980. - Long-Term Effects of Neuroleptics. Edited by F. Cattabeni, G. Racagni, P. F. Spano and E. Costa. (Pp. 619; illustrated; $78.88.)

Drug Concentrations in Neuropsychiatry. Ciba Foundation Symposium 74 (new series). (Pp. 266; illustrated; $45.75.) Excerpta Medica: Amsterdam. 1980. - Pharmacokinetics of Psychoactive Drugs. Edited by L. A. Gottschalk (Pp. 158; illustrated; £14.00.) SP Medical &amp; Scientific Books: London. 1980. - Long-Term Effects of Neuroleptics. Edited by F. Cattabeni, G. Racagni, P. F. Spano and E. Costa. (Pp. 619; illustrated; $78.88.)

Human Genetics: Possibilities and Realities (Ciba Foundation Symposium 66; new series)

Book Review| February 01 1981 Human Genetics: Possibilities and Realities (Ciba Foundation Symposium 66; new series) Human Genetics: Possibilities and Realities (Ciba Foundation Symposium 66; new series). Excerpta MedicaAmsterdam 1979 pp. 425, $51.25 S. MALCOLM S. MALCOLM Search for other works by this author on: This Site PubMed Google Scholar Biochem Soc Trans (1981) 9 (1): 180. https://doi.org/10.1042/bst0090180 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Share Icon Share Twitter LinkedIn Cite Icon Cite Get Permissions Citation S. MALCOLM; Human Genetics: Possibilities and Realities (Ciba Foundation Symposium 66; new series). Biochem Soc Trans 1 February 1981; 9 (1): 180. doi: https://doi.org/10.1042/bst0090180 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search nav search search input Search input auto suggest search filter All ContentAll JournalsBiochemical Society Transactions Search Advanced Search This content is only available as a PDF. © 1981 Biochemical Society1981 Article PDF first page preview Close Modal You do not currently have access to this content.

Trends in enzyme histochemistry and cytochemistry. Ciba foundation symposium 73. Edited by David Evered and Maeve O'Connor. Excerpta Medica, Amsterdam, 1980. $53.75. p 314

Trends in enzyme histochemistry and cytochemistry. Ciba foundation symposium 73. Edited by David Evered and Maeve O'Connor. Excerpta Medica, Amsterdam, 1980. $53.75. p 314

Announcement

Environmental Clemicals, Enzyme Function and Human Disease. Edited by David Evered and Geralyn Lawrenson. Ciba Foundation Symposium 76 (new series). (Amsterdam: Excerpta Medica, 1980.)The Principles and Methods of Modern Toxicology. Edited by C. L. Galli, S. D, Murphy and R. Paoletti. (Amsterdam: Elsevier, 1980.)