captured for the published proceedings. Nevertheless, the two Supplements to this Journal (also available together as a hard-backed book) do, over the years, embrace many of the major aspects of the study of inborn errors of metabolism and can, particularly with the Short Communications section, be used as a way into the literature on specific new topics. We hope that with judicious selection of material these supplements will continue to provide, as did the Society's earlier annual publications, a balanced record of the present state of the subject in all its facets, a record of interest to those working in allied fields as well as to the specialist. R. J. Pollitt G. M. Addison R. A. Harkness The papers listed below were also presented at the meeting. Scripts were not available by the time of publication. 1. Tangier disease and related disorders of apolipoprotein Al. G. Assmann, Munster. 2. Contribution to Ethics Symposium by M. E. Pembrey, London.

The molecular era ushered in the cloning of the growth hormone (GH) gene and the production of unlimited amounts of GH through recombinant technology. The continuing momentum of research from basic science to clinical evaluation has brought unprecedented advances to the understanding of GH biology for the clinical endocrinologist. Growth Hormone Related Diseases and Therapy: A Molecular and Physiological Perspective for the Clinician distills all the new information of relevance to the endocrinologist over the last 20 years by offering five sections: physiology, molecular genetics, GH deficiency, acromegaly and pharmacotherapy. The first section on physiology focuses on GH action. A review on the structure and function of the GH receptor is followed by a perspective on the regulatory role of ghrelin on GH secretion. The second section on genetics covers pituitary function and adenomas, including new and fascinating information on familial pituitary adenomas, their genotype and phenotype. The adult GH deficiency section spans the epidemiology and diagnosis of GH deficiency with a strong reminder for the clinician that the transition period represents a critical time of somatic maturation, which continues for years after cessation of liner growth. The section on acromegaly focuses on management, giving practical guides to the value of GH and IGF-1 measurements, the place of somatostatin analogues and of radiotherapy while reminding the reader as to why evaluating quality of life is an important part of management. Finally, the section on GH pharmacology takes the reader through innovative developments of long-acting GH formulations with some products on the threshold of clinical use. This section provides a balanced evidence based review of the effects of GH supplementation in aging and in sports where recent data indicates an enhancing effect on a selective aspect of performance. Growth Hormone Related Diseases and Therapy: A Molecular and Physiological Perspective for the Clinician integrates a wealth of information and will prove an invaluable reference for pediatric endocrinologists, adult endocrinologists, endocrine scientists and internists interested in the human biology of GH.

Breast and prostate cancers are both hormone-dependent, at least in some stages of their progression. Hormonal manipulation represents an important therapeutic approach. Although most of breast and prostate cancers initially respond to hormone therapy, most tumors reinitiate to growth. Finally, hormone-resistant and metastatic breast and prostate cancers may develop. Thus, the challenge is the dissection of mechanisms by which steroid receptor signaling pathways continue to influence cell growth and invasiveness. Compelling evidence indicates that steroid hormones elicit non-genomic responses in extra-nuclear compartment of target cells. In this cellular location, steroid-coupled receptors rapidly recruit signaling effectors or scaffold proteins and activate multiple pathways leading to proliferation, survival, migration and invasiveness. The immediate challenge is the dissection of key events regulating the steroid response of target tissues to prevent progression and improve treatment of breast and prostate cancers.

It has been known for over 150 years that hallmarks of inflammation can be observed in the wall of atherosclerotic vessels. It was, however, not clear if this inflammation is the cause or the consequence of atherogenesis. More recently, it has become evident that inflammation mediated both by innate and adaptive immunity is instrumental even in the earliest stages of the development of atherosclerotic lesions, i.e., that it plays an important pathogenetic role. In this volume, international experts in the field discuss the pathogenetic, diagnostic, preventive and possible therapeutic relevance of inflammation in atherogenesis. This book is intended for researchers and physicians in the fields of vascular biology, immunology and atherosclerosis.

The scientific advances in the physiology and pathophysiology of adipose tissue over the last two decades have been considerable. Today, the cellular and molecular mechanisms of adipogenesis are well known. In addition, adipose tissue is now recognized as a real endocrine organ that produces hormones such as the leptin acting to regulate food intake and energy balance in the central nervous system, a finding that has completely revolutionized the paradigm of energy homeostasis. Other adipokines have now been described and these molecules are taking on increasing importance in physiology and pathophysiology. Moreover, numerous works have shown that in obesity, but also in cases of lipodystophy, adipose tissue was the site of a local low-grade inflammation that involves immune cells such as macrophages and certain populations of lymphocytes. This new information is an important step in the pathophysiology of both obesity and related metabolic and cardiovascular complications. Finally, it is a unique and original work focusing on adipose tissue, covering biology and pathology by investigating aspects of molecular and cellular biology, general, metabolic, genetic and genomic biochemistry.

As the title suggests, and unlike other existing books on sleep medicine, Neuroendocrine Correlates of Sleep/Wakefulness will be devoted primarily to endocrine regulation of the behavioral state control. It will address a wide spectrum of sleep./wakefulness phenomena (both animals and humans), including pathogenesis, diagnosis, and management. From molecular biology to applied clinical therapy, sleep research has been transformed in the last few years from a research backwater to an important interdisciplinary field. Anyone who regularly reads the literature on sleep, biological rhythms, or neuroendocrinology is aware that one of the subspecialties within sleep medicine, the neuroendocrine correlates of sleep/wakefulness, has in particular experienced a growth rate that is even faster than that of the field as a whole. To a significant extent this has been due to the introduction of new research technologies. The widespread adoption of a range of new methods in sleep research has opened a window into activities at the cellular and molecular level, which previously had been tightly closed. Consequently these activities are being characterized with a degree of precision and sensitivity that is without precedent. This volume invites the reader to explore the new vistas that have been opened onto the neuroendocrine frontier of sleep medicine. The editors have selectively identified a number of key articles having a citation frequency, which is considerably above the norm or which otherwise have contributed importantly to defining the neuroendocrine perspective. This new volume on Neuroendocrine Correlates of Sleep/Wakefulness is an up-to-date resource of research summaries and reviews written by major contributors to the fields of sleep, biological rhythms and neuroendocrinology. Its coverage is broad and its basic and clinical science reviews are detailed. In this volume, an international team of experts discuss their latest ideas, concepts, methods, and interpretations with supporting examples. This volume is intended for advanced students and specialists in psychobiology, neuroscience, neuroendocrinology, and psychiatry but might also be interest to anyone concerned with understanding the Neuroendocrine correlates of sleep/wakefulness. The contributions are directed more towards providing an integrated view of the field from the perspective of the authors, rather than being a compendium of recent results. The intent is to provide a reference book for recent and future workers in this and related areas of medicine and biology. Each topic in this volume has received the attention of a panel of authors who have responded to our request to review and place into perspective the major issues, which will undoubtedly confront newcomers to the field. The topics dealt with in Neuroendocrine correlates of Sleep/wakefulness are both diverse and complex. The editors hope that this volume will provide an authoritative summary of important issues in the neuroendocrine correlates of sleep/wakefulness. We also hope that it will motivate new researchers to join the quest for solutions to the problems that have been identified by our contributing authors.

These two volumes are unique in that they take into consideration the enormous progress made in the field over the last few years. Expert knowledge is given by Professor Runnebaum, whose department was appointed WHO Collaboration Center for Research in Human Reproduction. These extensively illustrated books provide detailed information on the function and detection of new hormones and growth factors, on therapy with female sexual hormones, on environmental factors, and on the diagnostic and surgical techniques employed in reproductive medicine. This English edition of a standard German reference has been expanded to include an appendix containing a comprehensive list of pharmaceutical agents used in hormone therapy, including international and trade names and compositions.

The volume Appetite Control provides a comprehensive description of the mechanisms controlling food intake, and thereby energy balance, in the mammalian organism. During the last decade, research in this area has produced a remarkable wealth of information and has characterized the function of numerous peptides, transmitters, and receptors in appetite control. Dysfunction of these circuits leads to obesity, a growing health concern. However, the plethora of mechanistic information is in marked contrasts to an almost complete lack of anti-obesity drugs that meet the safety standards required for the chronic therapy of morbid obesity. Consequently, ongoing research aims to identify additional targets and agents for a pharmacological intervention. Thus, the mechanisms of appetite control as well as all agents interfering with its control are of considerable practical interest.

The authors of the volume are distinguished scientists who are leading experts in the field, and who have contributed important, original data to our understanding of the mechanisms of appetite control. They have quite different scientific backgrounds and, together, they represent all relevant disciplines. Thereby, the topics are presented from different points of view, not exclusively from that of pharmacology and neuroendocrinology. Thus, the volume addresses all scientists who are interested in the field of obesity research and the pathophysiology of appetite control."

Gastroparesis is an increasingly recognized disorder. Treatment can be difficult due to the several mechanisms for symptom production. Gastroparesis: Pathophysiology, Presentation and Treatment serves as a concise reference on this disorder that allows clinicians to quickly access and evaluate the necessary information for treating and managing patients with gastroparesis. Each chapter is written byexperts in their respective area. The volume will enable the reader to better understand all aspects of this disorder. The book presents current concepts in a variety of areas opening with the epidemiology, clinical presentation and pathophysiology of gastroparesis. The natural history of patients is explored as well as evaluation of patients with suspected gastroparesis. This section includes chapters on Wireless Capsule Motility, Electrogastrography and Antroduodenal Manometry. This important resource concludes with a full section on treatment including dietary, prokinetic, antiemetic, sensory, electric stimulation, and surgical methods. New developments include the use of gastric electric stimulation for treatment, the use of SmartPill for diagnosis, and a number of new agents in development for this disorder. Gastroparesis: Pathophysiology, Presentation and Treatment is the ideal reference for gastroenterologists, and also for surgeons, endocrinologists, primary care physicians and nutritionists looking to keep pace with the latest information treatment options for their patients. "

A number of chapters present the most novel research on testicular and epididymal functions or on more general fields of hormone action and molecular cell biology as it is now a tradition. However, exceptionally, the book also contains several chapters dealing with the "Approaches and Tools in the Third Millenium." The unusual inclusion of technologies as such in the 2000 edition of the workshop was an absolute necessity, as these technologies are revolutionizing the fields of biology and medicine and, in many instances, how to do research. This volume gives the scientific community essential information about the very latest technical developments and their potential for future progress.

Readers of this book can update their knowledge in the fast-moving field of endocrinology and neurobiology. Topics concerning growth and development are extensively reviewed from both basic science and clinical viewpoints. Aspects related to growth development and to the control of cellular differentiation and multiplication are discussed. Further new information is provided on: synthetic recombinant human growth hormone (rHGH); potential diagnostic and therapeutic applications of the neuropeptide, growth hormone releasing hormone (GHRH); the physiology and physiopathology of the neural control of growth hormone secretion; the diagnosis and therapy of growth hormone deficiency or excess states; and the biology, function and possible utilization of growth factors. These important new findings are relevant to progress in pediatrics, pediatric and clinical endocrinology, neuroendocrinology and physiology.

The rapid developments in molecular genetics have clarified many of the muta tions in monogenic thyroid diseases over the last two decades; now the target of molecular thyroid genetics has become the oligogenic thyroid diseases. These include the autoimmune thyroid diseases and familial thyroid cancers, both of which are much commoner than the monogenic diseases. However, the method ological approach to the genetics of these more complex diseases is still far from being well established. Although the discovery of susceptibility genes has been partially accomplished in complex diseases such as asthma, Crohn's dis ease, and types I and II diabetes mellitus, the elucidation of susceptibility genes in complex diseases remains a major challenge. This volume contains papers presented at the International Symposium on the Genetics of Complex Thyroid Diseases. This meeting was held in association with the International Thyroid Congress in Kyoto in October 2000 and sup ported in part by the Japan Intractable Diseases Research Foundation and Knoll Pharmaceuticals Inc. The symposium was the first international symposium con cerning the genetics of complex thyroid diseases and was restricted to the study of the autoimmune thyroid diseases and familial thyroid cancer. Twenty distin guished researchers from the United States, the United Kingdom, France, Ger many, Italy, and Japan were invited. Each presentation precipitated intense dis cussion and there was much consensus during the meeting. Nevertheless, this volume will leave the reader with a clear understanding of how little we still know.

This book provides a concise summary across all aspects of endocrine surgery, covering the major endocrine organs and discussing aspects of surgical care. It comprises five sections covering the major endocrine organs/systems, each with a specialist section editor. Within each section there are a number of brief (2-3 page) chapters, written in a combination of prose and bullet points, a format that is conducive to both detailed reading and quicker, "dipping in", review. Each chapter contains a "Pearls and Pitfalls" summary box. The text takes into account the latest evidence and current expert opinion from an internationally renowned list of authors.

Sex matters! Are there differences between the sexes when it comes to brain function and the behaviours that result? This volume attempts to answer this fundamental question. If the answer is 'yes' then this should impact upon our approach to treating mental illness in humans, and to modelling it in animals, as we look for aetiological and pharmacological solutions.

In mammals, a robust physiologic system acts to maintain relative constancy of weight. A key element of this system is leptin. The nature of this "brain-somatic" cross talk is as yet poorly understood, but it is likely to have important implications for the pathophysiology and treatment of obesity, diabetes and other metabolic disorders.

Professor Egon Diczfalusy, a pioneer in gynaecological endocrinology and in particular female contraception, celebrated his 80th birthday with a scientific symposium. The participants were scientists from all over the world who had been scholars and close collaborators at different points in time. The interdisciplinary meeting consisted of lectures on the epidemiological impacts of ageing, its challenge for the pharmaceutical industry, philosophical aspects of the future of mankind, and the history and future of contraception. Three round table discussions addressed the hormonal control of reproduction and ageing, new approaches and future perspectives of contraception for women and men, and hormone replacement in the elderly.

Different genetic diagnostic and treatment options are used worldwide to improve routine IVF procedures for the benefit of patients. This handbook updates the new genetic diagnostic technologies that have been translated to the clinic, aiming to improve outcomes in the clinic and result in a healthy baby in the home. Chapters cover the use of genetic technologies in a personalized manner to unravel the possible genetic risks for the couple wishing to conceive, in terms of sperm, the embryo, the endometrium, miscarriage, and finally the fetus. This expanded new edition covers the range of the latest genetic diagnostic technologies being translated into practice internationally to improve routine IVF procedures for the benefit of patients. Bringing together international experts to discuss their work, this text gives a context for the developments in this very fast-moving area of research and offers a comprehensive and rounded appraisal of hot topics.

This volume contains the proceedings of the third in a series of conferences entitled, The International Symposium on Biological Reactive Intermediates. The first was held at the University of Turku in Finland, in 1975, the second at the University of Surrey in the United Kingdom, in 1980 and the most recent at the University of Maryland in the United States, in 1985. The significance of these conferences has been emphasized by the rapid growth of mechanistic toxicology over the last decade. These conferences were initially stimulated by the attempt to uncover the significance behind the observations that the toxicity of carcinogenic responses produced by many chemicals was associated with the observation that their metabolism led to the formation of chemcially reactive electrophiles which covalently bound to nucleophilic sites in cells such as proteins, nucleic acid or fats. Recently, newer concepts have arisen which have necessitated the expansion of subjects covered by the conference. For example, the application of newer knowledge of the role of active oxygen species in reactive metabolite formation, the concept of suicide substrates, examination of the function of glutathione in cells, application of immunological techniques and molecular biological probes to the solution of toxicological problems all had an impact on the study of the biological reactive intermediates.

Proceedings of the 2nd World Conference - Hormonal and Genetic Basis of Sexual Differentiation Disorders and Hot Topics in Endocrinology. The meeting took place at The Eden Roc Hotel in Miami Beach, Florida, 1/15/10 - 1/17-10. Endocrinology and more specifically, the area of sexual differentiation disorders is an evolving field of medicine. The diagnosis and treatment of Disorders of Sex Development (DSD) is multi-faceted.

Sugar consumption is suspected to play an important role in the pathogenesis of diabetes, cardiovascular disorders, fatty liver disease, and some forms of cancers. Dietary sugars-fructose in particular-also have a potential role in obesity and metabolic diseases. Dietary Sugars and Health presents all aspects of dietary sugars as they relate to health and disease. It provides a review of the current science related to dietary sugars, ranging from historical and cultural perspectives to food science and production to basic research, animal trials, human pathophysiology, epidemiology, and public health policy implications. Each chapter features a concise, thorough summary of the current knowledge, including an overview of cutting-edge research, controversies, and future perspectives. The book provides a balanced approach through global and multidisciplinary contributions as well as input from various sectors, from academia to the food and beverage industry. The overall collection provides readers with a balanced and complete view of the science related to dietary sugars and health. This book is an invaluable reference for food scientists, nutrition scientists, clinical and translational researchers, obesity researchers, physiologists, public health scientists, and policy makers.

The molecular era ushered in the cloning of the growth hormone (GH) gene and the production of unlimited amounts of GH through recombinant technology. The continuing momentum of research from basic science to clinical evaluation has brought unprecedented advances to the understanding of GH biology for the clinical endocrinologist. Growth Hormone Related Diseases and Therapy: A Molecular and Physiological Perspective for the Clinician distills all the new information of relevance to the endocrinologist over the last 20 years by offering five sections: physiology, molecular genetics, GH deficiency, acromegaly and pharmacotherapy. The first section on physiology focuses on GH action.

A review on the structure and function of the GH receptor is followed by a perspective on the regulatory role of ghrelin on GH secretion. The second section on genetics covers pituitary function and adenomas, including new and fascinating information on familial pituitary adenomas, their genotype and phenotype. The adult GH deficiency section spans the epidemiology and diagnosis of GH deficiency with a strong reminder for the clinician that the transition period represents a critical time of somatic maturation, which continues for years after cessation of liner growth. The section on acromegaly focuses on management, giving practical guides to the value of GH and IGF-1 measurements, the place of somatostatin analogues and of radiotherapy while reminding the reader as to why evaluating quality of life is an important part of management. Finally, the section on GH pharmacology takes the reader through innovative developments of long-acting GH formulations with some products on the threshold of clinical use. This section provides a balanced evidence based review of the effects of GH supplementation in aging and in sports where recent data indicates an enhancing effect on a selective aspect of performance. Growth Hormone Related Diseases and Therapy: A Molecular and Physiological Perspective for the Clinician integrates a wealth of information and will prove an invaluable reference for pediatric endocrinologists, adult endocrinologists, endocrine scientists and internists interested in the human biology of GH."

A panel of outstanding investigators surveys and explains the major cutting-edge methods used in thryroid receptor (TR) research and explains their practical experimental details. Described in step-by-step detail to ensure robust experimental results, the techniques presented cover a wide variety of key areas, including TR in development and knockout (mouse and Xenopus), transcriptional regulation by TRs in both cell-free systems and in living cells, and TR mutant analysis of patients. Additional methods provide powerful tools for the isolation of TR-regulated protein complexes, for studying the oncogene v-Erba in blood cell differentiation, and for target gene analysis in the brain. Microarray chip methods are also presented for analyzing the organs of transgenic mice to identify target genes in the liver.

The use of cells for the treatment of a variety of diseases is no longer a dream. Today, blood transfusion, bone marrow transplantation, the use of ex vivo cultured skin in wound healing, and peripheral stern cell transplantation, including the ex vivo expansion of hematopoietic stern cells after high-dose chemo/radiation therapy, are routine. This high standard of knowledge and skills in cell transplantation might also re sult in tackling hitherto untreatable diseases. Organ transplantation is presently the only life-saving treatment for a variety of conditions. Important findings in cell and molecular biol ogy, the identification of hematopoietic, mesenchymal and neuronal stern cells, together with breakthroughs in the methodology for isolat ing, purifying, expanding, and storing human cells could make cellular therapy an alternative to organ transplantation in certain diseases within the next decade. Placental blood may be the source of choice in isolating naive progenitor cells for allogeneic transplantation. Immunotherapy is the most hopeful strategy to date for the treat ment of tumors resistant to chemotherapy, radiation therapy, or hor mone therapy. It includes the use of tumor-infiltrating lymphocytes, ex vivo activated memory T lymphocytes, and cell-based vaccines."

The Temporal Lobe, Volume 187 covers the exponential growth of studies on the relationships between brain and language/cognition, many of which involved the temporal lobe. This volume summarizes research on the anatomy and function of the temporal lobe under both normal and pathological conditions. In addition, it discusses the interactions of the temporal lobe with other brain structures. The book highlights the role of the temporal lobe in language processing as well as vision, object, face recognition and processing. The book also discusses the temporal lobe's role in reading, speech and the processing of color, music, action and memory. Temporal lobe disorders, assessments and treatments are also covered, including encephalitis, Alzheimer's, epilepsy, Korsakov's syndrome, and more.

to the Endothelin System David M. Pollock ascular endothelial cells form a monolayer lining in all of the blood vessels of V the circulation. Initially, they were thought to function as a crude filter allow- ing nutrients from the blood stream to diffuse through to the underlying tissues without letting proteins or blood cells escape. We now know that endothelial cells are important regulators of circulatory function, due in large measure to their rec- ognized ability to synthesize and release many factors that regulate vascular smooth muscle tone. Endothelial-derived factors, including relaxing and contracting sub- stances such as prostacyclin, nitric oxide (NO), and endothelin (ET), have been identified as important contributors in the regulation of vascular tone. Endothe- lial cells have a highly active metabolic function and are involved in clearing a number of agents from circulating blood. In addition, they have the enzyme that inactivates bradykinin and converts angiotensin I into the very potent pressor agent, angiotensin II (Ang II). Endothelial cells also generate various proteins like von Willebrand's factor, tissue plasminogen activator, growth promoting factors and lipids such as platelet activating factor. It is now clear that in addition to the regu- lation of vascular tone and hemodynamics, endothelial cells playa critical role in regulating growth and proliferative processes, inflammation and hemostasis.