bluuebunny/crossref_metadata_2025_split huggingface dataset:
Fabry’s disease and the lipidoses
Authors: Desnick Robert J , Eng Christine M 
Published: November 2023

Abstract
Originally described as a dermatologic curiosity by Fabry (1898), and independently by Anderson in the same year (1898), Fabry’s disease is now recognized as an inborn error of metabolism resulting from the defective activity of the lysosomal enzyme, cx-galactosidase A. The enzymatic defect, transmitted by an X-linked recessive gene, leads to the progressive deposition of neutral glycosphingolipids with terminal cx-galactosyl moieties in the plasma and lysosomes of endotheli al, perithelial, and smooth-muscle cells of the cardiovascular-renal system and, to a lesser extent, in reticuloendothelial, myocardial, and connective-tissue cells. Epithelial cells in the kidney, cornea, and other tissues contain the lysosomal glycolipid deposits, as do ganglion and perineural cells of the autonomic nervous system.
Nephropathic cystinosis: growing into adulthood
Authors: Gallo Linda K , Gahl William A 
Published: November 2023

Abstract
Although nephropathic cystinosis has long been considered a renal disease of childhood, two major therapeutic interventions have transformed it into an adult disorder as well. First, renal transplantation has prolonged the lives of many cystinosis patients into the third and fourth decades. In particular, the 70-80 North American patients who had received a renal allograft prior to 1985 (Gahl et al. 1986) have now reached adulthood. Second, the cystine-depleting agent, cysteamine, has so favourably altered the natural course of cystinosis that many patients are now approaching adulthood without requiring a renal allograft (Gahl et al. 1987; Clark et al. 1992; Markello et al. 1993). Consequently, adult nephrologists and other physicians are increas ingly called upon to manage the many renal and non-renal complications of long-standing cystinosis. These will be discussed below, but first it is helpful to become familiar with the disease in children.
The amyloidoses and familial Mediterranean fever
Authors: Nelson S R , Pras M 
Published: November 2023

Abstract
The understanding of’amyloid’ is advancing on several fronts, particularly the molecular genet ics of the inherited forms of this condition. These inherited forms of amyloid may have their prodrome in childhood but most have their clinical manifestations in adolescent and adult life. Rokitansky (1842) and others described physical deposits of a waxy, lard-like substance before Virchow (1854) termed them ‘amyloid’. But it is Virchow who is recognized as the discoverer of the disease entity ‘amyloidosis’ because of his clinical description and observation that the deposits take up iodine in a manner similar to starch or cellulose. On the basis of this staining, Virchow concluded that they were made up of carbohydrate. A few years later, the chemists Kekule and Schmidt (1858) noted that they contained so much nitrogen that they were albumi nous in nature. For many years the protein component has been the focus of amyloid research but recently the presence of the carbohydrate and glycosaminoglycan components has been re examined. Manipulation of the latter may offer therapeutic options for the future in addition to present therapies.
Legal Problems in Emergency Medicine
Authors: Montague Alan , Hopper Andrew 
Published: November 2023

Abstract
Legal Problems in Emergency Medicine is a small, practical book which covers a large, complex subject. It is the first book to offer practical guidance specifically for the doctor, nurse, manager, or lawyer faced with legal difficulties arising out of a medical emergency. 'Ignorance of the law is no excuse', and all professionals now require a working knowledge of the law relating to their subject. Those practising Emergency Medicine are no exception, whether casualty officers, hospital specialists, general practitioners, or nursing or paramedical staff. The first part of this book offers guidance, with examples, for those 'on the spot' in difficult situations, whilst the second part consists of a condensed but authoritative summary of the various legal topics comprising emergency medicine law. Written in plain language, it makes several complex and difficult areas of the law accessible to the legal layman-yet it does not avoid more complicated issues and is fully reference so that the truth of each statement can be validated. Readers will benefit from the extensive working knowledge of a unique team of authors: an emergency physician who has practised at the English Bar, and a practising solicitor.
Local difficulties
Authors: Montague Alan , Hopper Andrew 
Published: November 2023

Abstract
The service of an emergency department can only be as good as its hospital will allow. Overtired or over-confident junior members of in-patient teams may be tempted to take advantage of the inexperience of a casualty officer. He may be dissuaded from making a formal referral against his better judgement or the reluctant response may be unacceptably slow.
Medical negligence and vicarious liability
Authors: Montague Alan , Hopper Andrew 
Published: November 2023

Abstract
The civil law exists to redress wrongs, not to punish wrongdoers. It is principally concerned with parties who fall out over business agreements, children and property of families who fall apart, and with those who commit and those who suffer civil wrongs (or torts). In National Health Service work there are no contracts between doctors and patients and so the bulk of medical litigation is in the realm of tort.
Police powers
Authors: Montague Alan , Hopper Andrew 
Published: November 2023

Abstract
The law of England and the British Constitution developed with traditions of the rights of man and the liberty of the subject. These have been eroded as governments have sought to protect us from antisocial and lawless elements, in part by conferring special powers on police constables. It is fundamental that a constable has no general or inherent power to enter premises, detain people, seize things, or obtain answers to his questions.
In vitro models in the study of renal cystogenesis
Authors: Mangoo-Karim Roberto , Sullivan Lawrence P , Ye Min , Grantham Jared J 
Published: November 2023

Abstract
Epithelial cyst configuration is a physiological design in the function of certain organs such as ovarian follicles, thyroid follicles, and blastocysts. Similarly, the cylindrical design of nephrons and collecting ducts and the juxtaposition of renal tubules and microvessels are essential for proper renal function. In the kidney, however, the cyst phenotype deriving from tubules is distinctly abnormal. These pathological structures distort the functional, vascular, and structural integrity of the kidney, and are associated in many instances with the progressive deterioration of renal function. Polycystic kidney disease encompasses a spectrum of clinical conditions that have renal enlargement secondary to cyst expansion as a common feature.
Pathogenesis of polycystic kidney disease: altered cellular function
Authors: Wilson Patricia D 
Published: November 2023

Abstract
There may be several different mechanisms leading to the formation of cysts in kidneys, which can be broadly divided into those of genetic and non-genetic in origin. Within the genetic group, to date, no exact animal model has been shown to reflect a human genetic polycystic kidney disease (PKD) as judged by chromosome assignment, inheritance trait or rate of disease progression. Nevertheless, animal models have been of value in analysing cellular mechanisms involved in renal cyst formation (Table 6.1), and are particularly useful when viewed in comparison to findings in human tissues (Table 6.2).
Hypertension in polycystic disease
Authors: Watson .A1ichael L 
Published: November 2023

Abstract
Reports of hypertension in associatton with polycystic kidney disease (PKD) began to appear in the literature in the early part of the twentieth century (Braasch 1916; Schacht 1931). Most of these reports related to patients who had chronic renal failure as a consequence of cystic kidney disease (Braasch and Schacht 1933; Rall and Odell 1949; Higgins 1952), and it was noted that in these patients the incidence of hypertension was very much higher than in age- and sex-matched controls with end-stage renal disease secondary to pyelonephritis (Schacht 1931).
Miscellaneous renal and systemic complications of autosomal dominant polycystic kidney disease including infection
Authors: Elzinga Lawrence W , Bennett William M 
Published: November 2023

Abstract
As a multiorgan genetic disease, autosomal dominant polycystic kidney disease (ADPKD) has protean manifestations. Many of the more common and better understood complications and issues are covered separately in this monograph, including hypertension, renal failure, cystic liver disease, and cerebral aneurysms. This chapter addresses miscellaneous additional topics, including the very important subject of renal and hepatic infection.
Particular problems in childhood and adolescence in autosomal dominant polycystic kidney disease
Authors: Chapman Arlene B 
Published: November 2023

Abstract
The original nomenclature for Autosomal dominant polycystic kidney disease (ADPKD) was ‘adult polycystic kidney disease’ due to the usual age of presentation. However, the disease is autosomal dominantly inherited and the number of neonates, infants, and children diagnosed with ADPKD has increased dramatically during the past 20 years. The increased recognition of ADPKD in the paediatric age group is due to a number of factors.
Counselling and ethical considerations in autosomal dominant polycystic kidney disease
Authors: Macnicol Anne M , Wright Alan F 
Published: November 2023

Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is a common and serious genetic disorder with onset of symptoms generally after the reproductive age resulting in the propagation of the disease over many generations. It there fore presents an important opportunity to genetic counsellors to inform affected and ‘at-risk’ family members about the nature of the disorder and the treatment options, the recurrence risks to offspring and the advantages and disadvantages of pre-symptomatic testing. Advances in treatment have gradually led to an improved prognosis. Specific treatment is not available in ADPKD but early diagnosis leading to the prevention of hypertension is beneficial.
What causes kidney failure? Causes as yet without a specific treatment
Authors: Cameron Stewart 
Published: November 2023

Abstract
Fortunately, we already know from Chapter 3 that not all forms of glomemlonephritis lead to kidney failure, and that of those types that do, not everyone affected takes this course. However, in all groups of young adults with kidney failure who have been studied, the commonest single cause of kidney de .. struction are one or another of the group of conditions known collectively as glomerulonephritis. This is not true of children, or of the elderly, as we have noted above.
People with special needs: diabetics, paraplegics, children, and the elderly
Authors: Cameron Stewart 
Published: November 2023

Abstract
Facing up to kidney failure is bad enough, but is doubly difficult if, in addition, you have a condition which affects other parts of your body and may have been creating problems for you during many years. In addition, people at the extremes of life—the very young and the very old-have their own special problems.
An overview of treatments for kidney failure
Authors: Cameron Stewart 
Published: November 2023

Abstract
There were, at the end of 1992, just under 600 000 people being maintained by some form of dialysis, and about 200 000 people with functioning transplanted kidneys. Thus in the next year or two the number of people alive who would otherwise have died will exceed one million. Which form of treatment is best? Of course there is no answer to this question, because it depends entirely upon what individual people want, and what may be best for them medically.
The development of new drugs
Authors: Hobbs Richard , Millson David 
Published: November 2023

Abstract
The main objectives of clinical novel therapeutic research are to demonstrate the efficacy, safety, and metabolism of a drug or device in defined clinical indications, with the dosage range and proposed market formulation defined, and culminating in a successful approval from drug regulatory authorities. More recently, there has been a greater expectation that novel drugs will demonstrate efficacy over and above that seen in existing therapies and also provide some evidence on cost-effectiveness.
Licensing and marketing of drugs
Authors: Langman Michael , Wells, Frank , Leach Frank 
Published: November 2023

Abstract
One inevitable consequence of drugs being developed by a commercial pharmaceutical industry is that, like other commodities, they are bought and sold for profit. However, they are a commodity with some very special features which make the market for drugs unique, in particular with regard to the extent to which it is stringently regulated. There are good reasons for the stringent regulation of the market in drugs.
Impact of the UK healthcare reforms on prescribing
Authors: Walley Tom , Ferguson, John , Carter Yvonne 
Published: November 2023

Abstract
Prescribing by GPs has come in for extensive attention in recent years, because of the rising costs, concerns about the quality of much prescribing, and value for money. This increased attention must, however, be seen in the context of extensive reforms in the NHS over the same period, the biggest upheaval since its inception in the 1940s.
Non-prescription or over-the-counter medicines and the role of the pharmacist
Authors: Bond Christine , Bradley Colin 
Published: November 2023

Abstract
In the UK, the retail and wholesale dealings of all medicines and poisons are regulated primarily by the Medicines Act 1968, together with the Poisons Act 1972 and the Misuse of Drugs Act 1971. Sale and supply of medicines (by which is meant ‘medicinal products’ and includes any substance or article sold or supplied for administration to human beings or animals for a medicinal purpose) is controlled by the Part III of the Medicines Act 1968, and by orders made under it.
Introduction
Authors: Gronwall Dorothy , Wrightson, Philip , Waddel Peter 
Published: November 2023

Abstract
In recent years the road toll has tended to edge downward in developed countries perhaps because of an increasing awareness of the need to build safety features into motor vehicles, and perhaps due to an improvement in the education of drivers and law enforcers. The hidden road toll, the effect on the drivers, passengers, and pedestrians who suffer head injuries in traffic accidents, has also edged downward. Unfortunately there is still a horrendously high number of head injuries overall. People are still head injured in falls, in sports, particularly contact sports, in industry, and in violence on the streets. Young men still make up the majority of these cases, with small children and the elderly also at risk.
Some specific consequences
Authors: Gronwall Dorothy , Wrightson, Philip , Waddel Peter 
Published: November 2023

Abstract
Some or all of the after-effects which we talked about in the last chapter can be seen in anyone who has had a closed head injury, whether it was mild or severe. You will remember that in Chapter Z we described how in this sort of injury damage occurs to widespread areas of the brain because of the distortion and twisting which occurs. Penetrating injuries or blood clots on the other hand cause more localized damage, with different effects. These often look like the results of a stroke, perhaps with paralysis of one side of the body or loss of speech. Often both sorts of damage are present at the same time, which makes them more difficult to deal with.
Providing a head-injury service
Authors: Gronwall Dorothy , Wrightson, Philip , Waddel Peter 
Published: November 2023

Abstract
Up to now we have been talking about the effects of head injury and how the problems are dealt with. We have agreed that many of the problems are very difficult to solve. Though nowadays the standard of acute medical care is usually good, we have also suggested that the other services that the authorities provide are often inadequate, both because they have not understood what is needed and because they have not given the requirements of head-injured people enough priority in their budgets.
Autoantibodies and lupus nephritis
Authors: Rahman M A Anisur , Isenberg David A 
Published: November 2023

Abstract
Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease with an incidence of approximately four cases per 10 people per year in the UK. The peak age of onset is between the ages of 20 and 40 and women are affected approximately 10 times more often than men. The condition is commoner in some racial groups than others. In Birmingham (UK) the prevalence of SLE in women is 206 per 10 in Afro-Caribbeans, 91 per lOS in Asians, and 36 per 10 in Caucasians. For men the corresponding figures are 9.3 per 105, 2.6 per 10, and 3.4 per 10 for these three ethnic groups, respectively.
Clinical manifestations of lupus nephritis
Authors: Cameron J Stewar 
Published: November 2023

Abstract
Lupus is an archetypcal multisystem disease, and almost every tissue or organ may be affected. Indeed, its diagnosis conventionally depends upon multisystem involvement, as well as the presence of antibodies directed against various nuclear antigens. Thus it is best regarded as a syndrome—the lupus syndrome—rather than a disease entity. Although clinical renal disease, when present, is a major determinant of prognosis it must be seen in the context of this multisystem involvement. Renal disease is rarely the first manifestation of lupus, even when it may come later to dominate the clinical picture.
Pregnancy in women with lupus nephritis
Authors: Hou SusanH 
Published: November 2023

Abstract
Pregnancy in a woman with lupus nephritis is fraught with hazards: she has a disease which is immunologically mediated and modulated by changes in sex hormones; there are major hormonal and immunologic changes during pregnancy; her illness may affect her kidneys and heart which ordinarily function at increased levels during pregnancy; and she may require treatment which carries added risks during pregnancy. The fetus is at risk from the direct effects of the disease, the effects of the medications used to treat it, and the infectious complications of these medications. To move from conception to birth with two healthy individuals requires traversing a medical minefield filled with sudden and unpredictable dangers for both mother and child.
Gender Issues: Freudian and feminist stories
Authors: Sayers Janet 
Published: November 2023

Abstract
Freud notoriously narrated both sexes’ psychology in terms of the male-centred myths of Oedipus and Narcissus. He rejected the suggestion that women’s psychology might be better encompassed by the female-centred myth of Elektra. And he tended to focus on individual and intrapsychic issues to the relative neglect of interrelational and interpersonal issues now recognized by post-Freudian and feminist theorists to be crucial in shaping our adult lives and loves. In this chapter, I seek to highlight the latter issues, and the rather different stories women and men often tell about themselves, with examples from culturally shared myths; published autobiography and fiction; dream stories anonymously written by pupils attending two parallel, single-sex, state secondary schools; and from the stories patients have told me in therapy.
Sacred tales
Authors: Sims Andrew 
Published: November 2023

Abstract
In this chapter we will look at some of the stories behind the sources of energy in modern British health care. I see us, like the observers at an old-fashioned battle, standing on a hillside looking at and recognizing the prominent individuals as they approach us. We have difficulty identifying them as they leap into our view, and we may only be able to recognize them by their tails as they disappear once again. So, as the new National Health Service was unveiled before us in the early 1990s, we knew that we were to be confronted with purchasers and providers but it was not so easy to see at that stage how market forces would come to distort certain services. We have come to realize that it is ‘only by their tails that ye shall know them’.
Thinking skills: ethical reasoning and problem solving in psychiatric ethics
Authors: Dickenson Donna , Fulford Bill (Kwm) 
Published: November 2023

Abstract
In Chapter 1 we found that the ethical problems we face in psychiatry are often a good deal trickier, both practically and theoretically, than those arising in other areas of medicine. We thus need sharper tools to tackle them effectively. The tools for ethical problem solving in medicine come from a variety of sources — psychology, social science, anthropology, political theory, law, and many other disciplines. We will be drawing on some of these later on in thinking about the cases in this book. Perhaps most important of all, though, are the thinking skills — the strategies for analysing and thinking through ethical problems — that we get from philosophy.
Human Growth after Birth
Authors: Sinclair David , Dangerfield Peter 
Published: November 2023

Abstract
Growth is a process which affects every individual from the hour of birth to the hour of death and forms a background to almost every medical discipline, and Human Growth After Birth has been extremely successful in five editions. This book is written in a clear and approachable style and is the only publication which provides an elementary overview of the various manifestations of human growth. Professor David Sinclair, Professor of Human Anatomy, University of Aberdeen, wrote the first five editions, the last of which was published in 1989. Dr Peter Dangerfield, Department of Human Anatomy, University of Liverpool has updated the book for this new edtion while maintaining the unique style and appeal of the text. The new edition covers recent concepts in cell growth, the cell cycly and growth inhibitors. This book also contains the latest information on genes and the human genome. The sections on the growth of the CNS and on reproduction and infertility have been revised in line with current thinking. The references have been fully updated and boxed further reading has been added. Key points are highlighted throughout, and there are a number of new illustrations.
Definitions and epidemiology
Authors: Wrightson Philip , Gronwall Dorothy 
Published: November 2023

Abstract
Mild head injuries make substantial demands on health services. They take up a good deal of time in EDs, and they may need expensive imaging and sometimes short term use of hospital beds. Later a small proportion of patients have persisting symptoms which keep them off work for long periods and require costly rehabilitation and disability pay. For both economic and clinical audits it is important to know how many injuries there are, how they are managed and why, and for how long patients are disabled.
However, there are problems in defining what is meant by mild head injury. Plainly it is part of a continuum extending from injuries that are trivial to those which threaten life. Where we place the marker which separates the ‘mild’ injury from a severe one will depend on our needs and interests.
Mechanisms and pathology
Authors: Wrightson Philip , Gronwall Dorothy 
Published: November 2023

Abstract
The mechanical factors in mild head injury and the cellular pathology that follows have been difficult to unravel. Human studies, of necessity, have been limited to clinical observation and to the imaging and neurophysiological studies that have been ethically justifiable, with occasional autopsy material available from people who have had a mild head injury and have died from other causes. Experimental injury in subhuman primates has worked out the important mechanical factors and some of the pathology, but the model is an expensive one and there have been ethical difficulties, so that its usefulness is probably over.