The articles in Issue 4 of JOURNAL OF INHERITED METABOLIC DISEASE, Volume 16 (1993) contain the main lectures presented at the 30th Annual Symposium of the Society for the Study of Inborn Errors of Metabolism (SSIEM), Leuven, Belgium, 1992, which was dedicated to Inherited Metabolic Diseases and the Brain'. Topics discussed included: Inborn errors and brain fluids, neurotransmitter disorders, inborn errors and demyelination, and recent developments. Participants from many countries provided a state-of-the-art review which will be of interest to clinicians and research workers alike in many different disciplines.

Knowledge of tumours of the thyroid gland has advanced consider- ably in the 22 years that have elapsed since work was started on the first edition of Histological Typing of Thyroid Tumours. In the intro- duction to that volume it was recognized that the definitions and clas- sifications put forward would need revision in time, and the present text differs substantially from the first edition. As far as is possible, however, the framework of the classification proposed remains the same, as the original classification was widely accepted and proved useful in many studies. The link between the morphological type of thyroid tumour and its epidemiology, natural history, function, prognosis and response to therapy has been further strengthened since the first edition. In par- ticular, the decision taken to separate papillary and follicular carcino- mas and exclude a mixed papillary follicular type has been well justi- fied. One of the major changes has been the recognition that many tu- mours regarded 20 years ago as small cell carcinoma were really ma- lignant lymphoma, and this development has been incorporated into this edition, with increased importance given to primary malignant lymphoma of the thyroid. Much work has also been done on medul- lary carcinoma of the thyroid, its link with mUltiple endocrine neo- plasia syndromes, and its association in its inherited form with C-cell hyperplasia; this too is recognized by an expanded section on this tu- mour.

In recent years, the clinical evolution of thyroid diseases has been studied exhaustively as new discoveries have been made about thyroid malfunction and the effective management of patients. Thyroid Diseases presents a comprehensive exposition of a range of disorders caused by thyroid dysfunction. With contributions by well-known experts in the field, the book is the first compendium in several decades to assemble a vast amount of data and research into one volume. Following a methodological format, each chapter explores a different malady or condition. After defining the dysfunction or disease, the text presents the epidemiology, etiopathogenesis, clinical features, first and second level tests for diagnosis, first and second line therapies, prognosis, and recommendations for follow-up. Highlighted topics include: Transient forms of hyper- and hypothyroidism Subclinical hyper- and hypothyroidism Thyroid dysfunction during gestation and postpartum The influence of iodine on the thyroid during pregnancy The importance of drugs that modify thyroid function Thyroid disrupters that affect thyroid homeostasis The bimodal and/or transient development of some thyroid diseases The evolution of diagnosis, priorities of therapies, and lifelong monitoring of patients All of the contributors to this text pursued long periods of training in elite research laboratories. They are active investigators and well known in the international arena. The information presented in this volume is invaluable for practicing clinicians and those training in internal medicine, endocrinology, surgery, and other areas of medicine.

An intimate relation between hormones and lipoprotein metabolism has been known for a long time especially from hormone-deficiency or -overproduction syndromes. The mechanisms through which hormones influence lipid metabolism have become a field of major interest in sci ence. The more we learn about cellular hormonal actions the better we understand regulatory processes and phenomena occuring in patients. The present book summarizes data discussed at an International Meet ing at Marburg, Germany, on "Hormones in Lipoprotein Metabolism." It was an attempt to pull together knowledge in basic science from the mode of action of hormones all the way to clinical appearance of hor monal disorders involving lipoprotein metabolism. Thus data on molec ular biology, on hormonal regulation of apolipoprotein synthesis, on lipoprotein receptors and enzyme induction are discussed together with the large field of oral contraceptive use, postmenopausal estrogen sub stitution, lipid disorders in diabetes mellitus, in thyroid dysfunction, in adrenal insufficiency and in glucocorticoid application. We are aware of the fact that such an overview cannot be complete but should serve as a collection of data and ideas for those interested in hormonal regulation of lipid metabolism. We thank all authors for taking an extra effort in writing up their presentations and thus making this edition possible. We also thank Bristol-Myers-Squibb for generous support of the meeting and of the final publication. Marburg, Germany ARMIN STEINMETZ JURGEN SCHNEIDER HANS KAFFARNIK Contents Molecular Biology Chairmen: D. J. Rader, A."

The articles in Issue 4 of Journal of Inherited Metabolic Disease, Vol. 14 (1991) contain the main lectures presented at the 28th Annual Symposium of the Society for the Study of Inborn Errors of Metabolism, Birmingham, UK, 1990, which was dedicated to `The Liver and Inherited Metabolic Disease' with a half-day workshop on `Screening and Economics'. The subjects covered include metabolic functions of the liver, bile acids, alpha-1-antitrypsin deficiency, tyrosinaemia type I, Crigler-Najjar disease type I and Niemann-Pick disease type C, providing updates on a wide range of metabolic disorders and illustrating the importance of the complementary contributions from professionals in different disciplines. Also covered in detail is the exciting potential of liver transplantation as treatment for several inborn errors of metabolism. This state-of-the-art review will be of interest to clinicians and research workers alike.

Thyroid diseases are relatively common, occurring in about 10% of the population in the Western world. Many are simple to diagnose and treat, while others are not. In this comprehensive work, the author draws upon personal experience, teaching, and a distillation of the literature to provide clinicians with advice on how to handle specific patient-related problems. The book begins with a summary of basic information on the structure, function, development, abnormal development, and physiology of the thyroid gland. It then reviews tests of thyroid function and clinical examinations before proceeding to chapters on disease states. Chapters on clinical problems include hyper- and hypothyroidism, thyroid nodules, thyroid cancer, thyroiditis, goitre, and iodine deficiency disorders. The chapters on hyper- and hypothyroidism are designed to aid the physician in defining the specific cause and management of patients with these conditions. Evaluation and treatment of thyroid nodules and thyroid cancer arouse considerable differences of opinion; these differences are reviewed and specific recommendations are outlined. Flow diagrams are included for the work up of different clinical situations, and each chapter concludes with a section of key facts. The last two chapters cover clinically related issues. Since non-thyroid illnesses, both physical and mental, can distort thyroid function tests, the author provides advice on how to avoid a misdiagnosis. Because some diagnostic tests and treatments involve administering radioactive iodine to the patient, the final chapter discusses radiation biology and the thyroid. This cohesive volume will be of value to endocrinologists and clinicians who treat patients with thyroid disease, as well as to medical students and physicians in training.

Iron is indispensable for the growth, development and well-being of almost all living organisms. Biological systems from bacteria, fungi and plants to humans have evolved systems for the uptake, utilisation, storage and homeostasis of iron. Its importance for microbial growth makes its uptake systems a natural target for pathogenic microorganisms and parasites. Uniquely, humans suffer from both iron deficiency and iron overload, while the capacity of iron to generate highly reactive free radicals, causing oxidative stress, is associated with a wide range of human pathologies, including many neurodegenerative diseases. Whereas some essential metal ions like copper and zinc are closely linked with iron metabolism, toxic metals like aluminium and cadmium can interfere with iron metabolism. Finally, iron metabolism and homeostasis are key targets for the development of new drugs for human health. The 4th edition of Iron Metabolism is written in a lively style by one of the leaders in the field, presented in colour and covers the latest discoveries in this exciting area. It will be essential reading for researchers and students in biochemistry, molecular biology, microbiology, cell biology, nutrition and medical sciences. Other interested groups include biological inorganic chemists with an interest in iron metabolism, health professionals with an interest in diseases of iron metabolism, or of diseases in which iron uptake systems are involved (eg. microbial and fungal infections, cancer, neurodegenerative disorders), and researchers in the pharmaceutical industry interested in developing novel drugs targeting iron metabolism/homeostasis.

Current knowledge relevant to the understanding of thyroid autoimmunity is summarized in this book. Some of the world's leading scientists in this field review recent data from four subject areas: immunogenetics, thyroid peroxidase as an antigen, environmental factors, and cytokines. Much ongoing and as yet unpublished work is described, especially in the discussion included after each article. This information is of particular interest since the body of knowledge in these fields is growing very rapidly. The book is based on a workshop in which researchers from 13 countries took part. One major aim was to raise controversial points and to develop ideas for their potential solution. At the workshop it became apparent that a great deal of the information given in textbooks is outdated. This is an important update for researchers who are interested in the above topics and in understanding the etiology and pathogenesis of thyroid autoimmunity.

Hypoglycemia simply means "low blood-sugar," but without concrete symptoms it's very hard to diagnose. It is nevertheless a condition that should be watched over carefully. People react differently to low blood sugar as well as to the treatment they receive. Hypoglycemia for Dummies explores this fickle condition and shows you how to manage your blood sugar to feel better. This no-nonsense, plain-English guide lays out the facts you need to maintain a healthy body. It offers expert advice on identifying symptoms, changing lifestyles, and also extensive coverage on diet, exercise, alternative treatments, and the link between low blood sugar and diabetes. This expanded 2nd edition provides: * A thorough explanation of hypoglycemia and how it affects your body * Exercise routines that lead to a healthier lifestyle * Diet suggestions on what to eat and how often * A basis for choosing a doctor that's right for you * Vitamins and supplements that treat your symptoms * Ways to manage hypoglycemia in the workplace * An explanation of how hypoglycemia affects family and friends * Methods to de-stress yourself Complete with tips on helping other hypoglycemics and myth-debunking facts about the disease, Hypoglycemia for Dummies is the fast and simple way to learn and treat the condition, with the help of the most up-to-date medical information available. Escape the blood sugar blues and starting feeling better in no time!

394 finding by Dr C. Jakobs, Amsterdam, was elevated plasma galactitol and/or sorbitol levels in some cataract patients with quite normal activities of the galactose-degrading enzymes and sorbitol dehydrogenase in RBC. Inherited disorders of glycoprotein metabolism were reviewed by Dr M. Cantz, Heidelberg, followed by detailed presentations on selected disorders. The meeting was closed by two exciting lectures, given by Dr J. R. Hobbs, London, and Dr F. Ledley, Houston, on the outcome of bone marrow transplantation and on future aspects of gene therapy in patients with inborn errors of metabolism. Each year the 'Mini' Symposium preceding the main topics attracts increasing numbers and in Munich more than half of the 281 active participants also attended on "Maternal Phenylketonuria," organized by Dr the highly interesting workshop D. Brenton, London. This four-hour workshop included international practical experiences in the treatment of maternal phenylketonuria as well as the results of amino acid transport and animal experiments.

Dementia of Alzheimer type (DAT), multiinfarct dementia (MID) and dementia occurring in the course of Parkinson's disease (PD +D) now make up one of the largest categories of chronic diseases in the elderly. In addition to the burden those illnesses impose on the affected individuals and their families they consume large socio-economic re- sources. In the light of all the above mentioned features, it seemed to us that a Symposium on behalf of the 125th Anniversary of Birth of Aloys Alzheimer was particularly well-suited to help to advance research on Alzheimer's disease and other dementias. This International Symposium combined with a Satellite Symposium about "Clinical Aspects of Alz- heimer Dementias" took place in June 1989 in Wiirzburg and has been organized by the Psychiatric Departments of the Universities of Wiirzburg and Munich (H. Beckmann, K. Maurer, P. Riederer, H. Hippius and H. Lauter) and the Department of Pathochemistry and General Neurochemistry of the University of Heidelberg (S. Hoyer). In the chapters which follow, thorough reviews of recognized authorities in the field of dementia are given in the four main fields of epidemiology, neuropathology, neurochemistry and clinics. This Symposium and the edition of this book would not have been possible without the generous support of E. Merck, Darmstadt. In particular we gratefully acknowledge the efforts undertaken by Mr. Hernandez-Meyer to organizing this symposium and to publish this book. Furthermore we are grateful to the secretarial help by Mrs. Moeslein, Miss Philipp and Miss Grabner.

Endorphins and other endogenous opioids appear to be the connecting link between reproductive functions and stress adaption of the human organism. This book contains the con- tributions of an international group of biologists, bioche- mists, and endocrinologists on the opioidergic control me- chanisms in reproduction and stress physiology. Main topics covered are: endogeneous opioids and the pituitary-gonadal system; ovarian endorphinsecretion; pregnancy-associated changes of plasma endorphin; and opioid control of the hypo- thalamo-pituitary-adrenal axis. Although all chapters give an excellent review on various studies in basic opioid re- search, there are numerous clinical implications mentioned in this book.

The European School of Oncology came into existence to respond to a need for informa tion, education and training in the field of the diagnosis and treatment of cancer. There are two main reasons why such an initiative was considered necessary. Firstly, the teaching of oncology requires a rigorously multidisciplinary approach which is difficult for the Univer sities to put into practice since their system is mainly disciplinary orientated. Secondly, the rate of technological development that impinges on the diagnosis of cancer has been so rapid that it is not an easy task for medical faculties to adapt their curricula flexibly. With its residential courses for organ pathologies and its seminars on new techniques or on the principal therapeutic controversies, it is the ambition of the European School of Oncology to fill a cultural and scientific gap and, thereby, create a bridge between the Uni versity and Industry and between these two and daily medical practice. One of the more recent initiatives of the ESO is the Intercity Meetings. The concept of Inter city Meetings was first put forward with the idea of creating total coverage throughout Europe of certain tumours at the forefront of research and, in some cases, controversy."

International experts are brought together in this book to give basic as well as clinical data on obesity and cachexia, and an integrated picture of the regulation of intermediary metabolism in both situations. The data provide evidence that tissue catabolism and anabolism are regulated by similar mechanisms, which again are the target of different hormonal and metabolic factors. This joint discussion of the similarities evident in both areas will greatly benefit future understanding and research in obesity and cachexia.

1.1 Outline of the Study In this study the influence of psychotropic drugs on anterior pituitary hormone (APR) secretion was investigated in healthy subjects and in depressed patients. The basis for this study is formed by pharmacological investigations which indicate that the therapeutic effect of psychotropic drugs can be attributed to their functional influence on central nervous aminergic impulse transmission (stimuli) as well as by endocrinological investigations which show that central nervous aminergic neurons influence APR secretion in man. The purpose was to frod out if psychotropic drugs with differing pharmacological actions also influence APR secretion differently, even though the complex processes of central nervous stimuli transmission, the effect of psychotropic drugs on these processes, as well as the action of aminergic neuronal systems on APR secretion are not completely understood. Furthermore, it was investigated if it is possible to reach conclusions on the effects of drugs on central nervous aminergic neurons based upon APR secretion data. Specifically, the effects of various antidepressants, neuroleptics, and benzodiazepine-type tranquilizers on the secretion of growth hormone (GR), prolactin (PRL), adrenocorticotropic hormone (ACTR), and cortisol were investigated in healthy male subjects. The first part of this study presents in detail the results of these experiments and also incorporates the results of other investigators. It is endeavored to answer the question whether and to what extent the different psychotropic drugs influence APR secretion and whether the APR secretion profiles of the different psychotropic drugs can serve as a possible pharmacoendocrinological model in humans.

This book contains the necessary knowledge and tools to incorporate nutrition into primary care practice. As a practical matter, this effort is led by a dedicated primary care physician with the help of motivated registered dietitians, nurses, psychologists, physical therapists, and office staff whether within a known practice or by referral to the community. It is essential that the nutrition prescription provided by the physician be as efficient as possible. While many team members have superior knowledge in the areas of nutrition, exercise, and psychology, the health practitioner remains the focus of patient confidence in a therapy plan. Therefore, the endorsement of the plan rather than the implementation of the plan is the most important task of the physician. This book proposes a significant change in attitude of primary health care providers in terms of the power of nutrition in prevention and treatment of common disease. It features detailed and referenced information on the role of nutrition in the most common conditions encountered in primary care practice. In the past, treatment focused primarily on drugs and surgery for the treatment of disease with nutrition as an afterthought. Advanced technologies and drugs are effective for the treatment of acute disease, but many of the most common diseases such as heart disease, diabetes, and cancer are not preventable with drugs and surgery. While there is mention of prevention of heart disease, this largely relates to the use of statins with some modest discussion of a healthy diet. Similarly, prevention of type 2 diabetes is the early introduction of metformin or intensive insulin therapy.

Starting with the morphology of the myoendocrine cell and the biochemistry and physiology of cardiac hor mones, the book describes pathophysiological findings and discuss their clinical relevance in congestive heart failure, arterial hypertension, renal insufficiency, and cirrhosis of the liver. Furthermore, evidence is presented for the role of the heart as a target organ for various endocrine factors such as thyroid hormones, catecholamines, renin-angiotensin, growth hormones and endogenous opioids.

The islets of Langerhans, the primary source of hormone production in the pancreas, have been the focus of research into the nature of diabetes for decades. In recent years, the molecular biology of this multiendocrine organ has been intensively investigated, with a corresponding increase in our understanding of the normal and pathological functioning of islet cells. Molecular Biology of the Islets of Langerhans provides a comprehensive treatment of molecular studies on the synthesis of insulin, glucagon, somatostatin and pancreatic polypeptide. In addition, this volume presents contemporary hypotheses and explanations, at the molecular level, of the defects in islet cells that may lead to diabetes. The material presented here will be essential reading for researchers in human endocrinology and, in particular, for those working in the area of diabetes research.

Ein Screening auf angeborene endokrine und metabole Stoerungen ermoeglicht die Fruherkennung von Krankheiten wie Hypothyreose, AGS (adrenogenitales Syndrom), Phenylketonurie, Galaktosamie, Biotinidasemangel, Cystische Fibrose oder Homocystinurie. Je nach Land und politischen Bestimmungen unterscheiden sich jedoch die routinemassig durchgefuhrten Untersuchungen. Dieses Handbuch beschreibt praxisbezogen die biochemischen Grundlagen, die Grundsatze der Labor- und Bestatigungsdiagnostik sowie Therapiemoeglichkeiten zu insgesamt zwoelf Krankheiten. Daruber hinaus werden praanalytische Fragen, wie Zeitpunkt und Art der Blutentnahme oder moegliche Stoerfaktoren, umfassend behandelt. Der allgemeine Teil geht auf ethische und psychosoziale Aspekte des Screenings ein. Ein Verzeichnis der Screeningzentren und jener Labors, die fur die Bestatigungsdiagnostik Dienste anbieten, sowie ein Adressenverzeichnis von Elterngruppen und Informationsquellen im Internet komplettieren dieses Buch.

A number of clinical and epidemiological studies have shown that disorders of lipoprotein metabolism constitute one of the most important risk factors for the development of atherosclerosis and coronary heart disease. This volume examines the state of the art of lipoprotein subclass metabolism and its relation to these diseases. The authors also report on new developments concerning the role of lipoprotein recptors, macrophages and apolipoprotein E polymorphism in cholesterol homeostasis. The combination of general outline form and very specific aspects of cholesterol transport will interest those in other disciplines following developments in the field, as well as those directly involved in lipoprotein research.

This book records the proceedings of the 5th International Symposium on cancer topics organised in collaboration with the Association for International Cancer Research, a cancer charity based in the United Kingdom. The Symposium was held at the Klinikum Steglitz, Free University of West Berlin, Germany, between 1 and 3 October 1987. The Organising and Scientific Committees are deeply grateful to the President of the Free University, Professor Heckelmann, for allowing us to meet in the Klinikum Steglitz and for the University's generous financial support. It was a special pleasure to have a positive and generous input from the West Berlin Senate. Their support was crucial in making the Symposium a scientific success. The Symposium received financial support from a number of sponsors, and we are indeed happy to acknowledge our gratitude to them: Behringwerke AG, Bert- hold AG, Boehringer Mannheim GmbH, the British Council, Deutsche Abbott GmbH, Deutsche Bank AG, Du Pont De Nemours GmbH, Knoll AG, Krewel- werke GmbH, Nunc GmbH, Schering AG, Schwarz GmbH and Varian GmbH. The Symposium was the most ambitious so far mounted by the Association for International Cancer Research. The organisation and planning were carried through by an Organising Committee including Professor J. Hammerstein, Profes- sor K. D. Asmus, Barbara Steiger and Renate Nigam. The Scientific Committee included Dr. D. C. H. McBrien, Professor T. Dormandy, Professor K. R. Rees, Pro- fessor M. U. Dianzani and Professor H. Esterbauer. We thank these colleagues very much for their dedication, hard work and expert input.

Crash Course - your effective every-day study companion PLUS the perfect antidote for exam stress! Save time and be assured you have the essential information you need in one place to excel on your course and achieve exam success. A winning formula now for over 20 years, each series volume has been fine-tuned and fully updated - with an improved full-colour layout tailored to make your life easier. Especially written by senior students or junior doctors - those who understand what is essential for exam success - with all information thoroughly checked and quality assured by expert Faculty Advisers, the result are books which exactly meet your needs and you know you can trust. Each chapter guides you succinctly through the full range of curriculum topics, integrating clinical considerations with the relevant basic science and avoiding unnecessary or confusing detail. A range of text boxes help you get to the hints, tips and key points you need fast! A fully revised self-assessment section matching the latest exam formats is included to check your understanding and aid exam preparation. The accompanying enhanced, downloadable eBook completes this invaluable learning package. Series volumes have been honed to meet the requirements of today's medical students, although the range of other health students and professionals who need rapid access to the essentials of metabolism and nutrition will also love the unique approach of Crash Course. Whether you need to get out of a fix or aim for a distinction Crash Course is for you! Provides the exam syllabus in one place - saves valuable revision time Written by senior students and recent graduates - those closest to what is essential for exam success Quality assured by leading Faculty Advisors - ensures complete accuracy of information Features the ever popular 'Hints and Tips' boxes and other useful aide-memoires - distilled wisdom from those in the know Updated self-assessment section matching the latest exam formats - confirm your understanding and improve exam technique fast