bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cognitive behavioural therapies in the treatment of functional somatic symptoms
Authors: Sharpe Michael 
Published: November 2023

Abstract
In this introductory chapter, Dr Sharpe outlines the historical development of cognitive and behavioural treatment and then discusses the cognitive behavioural model of aetiology. He applies this model to the understanding of functional somatic symptoms and discusses the efficacy of this approach. He concludes with practical guidelines for the treatment of patients with functional somatic symptoms using cognitive behavioural psychotherapy.
Chest pain and palpitations
Authors: Bass Christopher , Mayou Richard 
Published: November 2023

Abstract
In this chapter Drs Bass and Mayou draw attention to the high prevalence of com plaints of non-cardiac chest pain and benign palpitations in different medical set tings. They also point out the important covariation between these symptoms and other functional complaints and describe the poor outcome in terms of continued symptoms and disability. The contribution of pathological, physiological, and psychological factors to aetiology is reviewed. Emotional disorders (especially symptoms of anxiety and panic) occur in a proportion of patients but many have no conspicuous psychiatric morbidity.
A worldwide overview: the epidemiology of HLA-B27 and associated spondyloarthritides
Authors: Khan Muhammad Asim 
Published: November 2023

Abstract
The discovery of a remarkable association between HLA-B27 and ankylosing spondylitis (AS) and related disorders a quarter of a century ago helped to revitalize interest in the clinical features, pathogenesis, and genetic epidemiology of these disorders, and also broadened our understanding of their clinical spectrum (Khan and van der Linden 1990b, 1990c). The concept of spondyloarthropathies no longer necessarily implies the presence of sacroiliitis or spondylitis, but rather a spectrum that may start with features such as enthesitis, dactylitis, or oligoarthritis on the one hand, and sacroiliitis and spondylitis on the other, that may also be accompanied by certain extra-articular features, including acute anterior uveitis or mucocutaneous lesions.
The bowel and spondylarthritis: a clinical approach
Authors: Mielants H , Veys E M 
Published: November 2023

Abstract
The close relationship between the bowel and the locomotor system (peripheral joints, enthesis, sacroiliac joints, and axial skeleton) is suggested by many clinical observations and several experimental animal models. Gastrointestinal abnormalities can be present in several rheumatological diseases. Among the inflammatory connective tissue diseases, these are largely restricted to scleroderma. In most other rheumatic diseases (for example, rheumatoid arthritis, polyarteritis nodosa, systemic lupus erythematosus, Henoch-Schi:inlein purpura) the intestinal manifestations are either restricted to a systemic arteritis involving intestinal blood vessels or related to drug toxicity.
Animal models of the spondyloarthropathies
Authors: Taurog Joel D 
Published: November 2023

Abstract
The study of the pathogenesis, treatment, and prevention of human disease is invaluably aided by animal models. Animal models of human disease have traditionally been divided operationally into two groups, those occurring spontaneously and those requiring experimental induction. However, diseases occurring in genetically manipulated animals, for example transgenic or ‘gene knockout’ animals, can logically be considered in either of these categories. Ultimately, classification is less important that the utility a particular type of animal or experimental system provides in understanding important aspects of human disease.
Ankylosing spondylitis-the patient’s point of view
Authors: Rogers Fergus J 
Published: November 2023

Abstract
Ankylosing spondylitis (AS) is such a variable condition that every patient’s experience is different. The first major problem encountered by patients worldwide is misdiagnosis. For the young spondylitic patient with a recent disease onset this can become a lonely, painful, and humiliating experience: an ideal sequence of events would be the following: Upon diagnosis the patient is given only a brief description of the disorder, most of which is rapidly forgotten. The primary physician knows little about the condition and either cannot give advice or gives inappropriate advice. This leads some patients to suspect that the doctor is withholding information to protect them against knowledge of an appalling prognosis. The patient is not referred to a patient organization.
Cranial nerves
Authors: Baraitser Michael 
Published: November 2023

Abstract
This syndrome is also called anosmia with hypogonadotropic hypogonadism. The anosmia is due to an agenesis of the olfactory lobes. Cleft lip and palate might be part of the syndrome. Renal agenesis should be sought. A large number of those with the condition are obese. Congenital mirror movements are an interesting associated feature, which points towards a disorder of neuronal outgrowth in the motor system, particularly of the inhibitory callosal fibres (Danek et al.,1992).
Spinal malformations
Authors: Baraitser Michael 
Published: November 2023

Abstract
Five kindred have been described with well-documented syringomyelia, and there have been both recessive and dominant modes of inheritance described (Bentley et al., 1975; Busis and Hochberg, 1985; Colombo and Cislaghi, 1993). The main problem seems to be the possible variable expression of the gene involved, resulting, at one end of the spectrum in syringomyelia with or without syringobulbia, and at the other end, in minor radiological abnormalities such as occipital dysplasia, basilar impression, or Amold-Chiari malformation type I. Patients with syringomyelia might have bulbar weakness, upper limb weakness, and pyramidal tract involvement in the lower limbs. There is mostly a dissociated sensory loss—see also Chiari malformation, below.
Cortical dysplasias
Authors: Baraitser Michael 
Published: November 2023

Abstract
Classification based on Dobyns and Truwit (1995). Lissencephaly (agyria/pachygyria diffuse with abnormally thick cortex and only four layers). Also called lissencephaly type 1: classical: Miller-Dieker syndrome; Lissencephaly sequence; lissencephaly with very small head including a small cerebellum; Cobblestone lissencephaly or lissencephaly type II (a mixture of agyria, pachygyria and polymicrogyria, enlarged ventricles, small brainstem, cerebellun, especially vermis and cerebellar polymicrogyria): Hydrocephalus, agyria, retinal dysplasia (HARD) (seep. 56); the congenital muscular dystrophies (seep. 218);
Craniosynostosis
Authors: Baraitser Michael 
Published: November 2023

Abstract
The previous classification dividing the craniosynostoses into acrocephalysyndactyly and acrocephalypolysyndactyly is no longer useful. The same is probably true for the subdivisions into type I, III, etc. The gene and mutations in the FGFR2 gene have been identified in Apert’s syndrome but the diagnosis is clinically straightforward and gene mutation analysis is not needed for diagnostic purposis. Prenatal diagnosis for the offspring of those who are affected is usually possible given the severe hand and foot syndactyly and ultrasonography is probably all that is needed.
Cerebellar ataxia
Authors: Baraitser Michael 
Published: November 2023

Abstract
Major inroads have now been made by molecular geneticists into the differentiation of the many syndromes of cerebellar ataxia. In general, it is still useful to use age of onset and associated features as a starting point. Congenital onset: cerebellar hypoplasia recessive; cerebellar hypoplasia dominant; cerebellar hypoplasia affecting the granular layer; carbohydrate-deficient glycoprotein syndrome; pontoneocerebellar hypoplasia; Congenital onset affecting especially the vermis: Joubert’s syndrome;
Pyramidal tract disorders
Authors: Baraitser Michael 
Published: November 2023

Abstract
Pure X-linked spastic paraplegia, MASA, and X-linked hydrocephalus due to an acqueduct stenosis, seem to be the result of the mutations of the same gene called Li-CAM. The same mutation in a single family can result in different phenotypes. There are metabolic conditions which must be excluded, especially in single cases. Adrenoleukodystrophy and arginase deficiency are two such conditions.
Metabolic disorders/degenerative diseases of childhood
Authors: Baraitser Michael 
Published: November 2023

Abstract
Synonyms: (Pomes’ disease: Acid maltase deficiency—infantile
Abstract: This condition might initially be diagnosed as Werdnig-Hoffmann disease as the infants are floppy, weak, and areflexic. Unlike Spinal muscular atrophy, type I SMA/1 there might be joint contractures and the muscles appear on palpation to be firm and hypertrophied. Fasciculations might be seen, and swallowing and sucking is usually poor. The heart is enlarged, the myocardium is thick, and the ECG will show giant QRS complexes. Death occurs within the first few years of life.
The Management of Head Injuries
Authors: Currie David G , Ritchie Ewan , Stott Stephen 
Published: November 2023

Abstract
Head injuries are commonplace. Though most patients survive, the consequences, both physical and social, can be devastating. The methods of initial assessment and management can have a crucial effect on determining the outcome of the injury, and long-term effects. Only a minority of head-injured patients, however, are cared for by Neurosurgeons. The majority are seen by non-specialists, often junior doctors and nurses, and treated in district hospitals where specialist neurosurgical assistance is unavailable. As a guide for doctors and nurses working in the A & E department, or the Orthopaedic or General Surgical Ward, the emphasis of this book is on the early management of the head-injured patient. New to the second edition are chapters on managing the ‘disturbed’ head injured patient, a chapter of special value to nurses (who often have to deal with this difficult task). A new chapter on anaesthesia and head injuries has also been added, with other chapters significantly revised to reflect the crucial role played by anaesthetists in head injury management. Providing practical advice on the early treatment of minor head injuries, and the management of major head injuries, the book provides advice that will be essential in ensuring effective treatment of these patients.
Limiting violence through good design
Authors: Francis Susan 
Published: November 2023

Abstract
The relationship between space and human behaviour is an issue that has been explored in various disciplines over time. Whilst now widely accepted that there is a relationship between them, the extent to which the environment can actually deter mine behaviour is more debatable. Some have tried to link environment and behav iour in a determinstic way, suggesting that physical space can direct behaviour. Many people acknowledge that the design of the environment can influence how people react and how they feel. Indeed, it can support certain work patterns and make others more difficult to achieve. So, the idea that the environment can play a part in limiting violence has currency both in literature and practice.
Attacks on doctors and nurses
Authors: Cembrowicz Stefan , Ritter Sue , Wright Steve 
Published: November 2023

Abstract
Violence in the UK is widespread. A rate of 667 violent offences per 100 000 population was recorded by police in England and Wales in 1997 (Home Office 1998). The British Crime Survey (BCS) found that just over 5% of survey respondents had been victims of violent crime in the previous year (Mirlees-Black et al. 1996). Both of these figures are likely to under-represent the true prevalence of violence. Violence is clearly a major public health problem (Golding 1996; Shepherd and Farrington 1993), and health-service workers are not immune.
Genetics of Stone Forming Diseases
Authors: Strazzullo Pasquale , Vuotto Pietro 
Published: November 2023

Abstract
Nephrolithiasis is a common and heterogeneous disease manifesting itself most commonly during middle age (Preminger 1992). In particular, calcium nephrolithiasis is a health problem and a source of disability in all affluent countries with a reported prevalence between 4 and 10 per cent (Smith 1989).
Economic Evaluation in Health Care
Authors: Drummond Michael , McGuire Alistair 
Published: November 2023

Abstract
To accompany the hugely sccessful Methods for Economic Evaluation of Health Care Programmes 2e, this book is a thorough and rigorous discussion of the methodological principles and recent advances in the rapidly advancing field of theory and practice of economic evaluation in health care. Written by an internationally acclaimed group of authors, the book provides an in-depth discussion of the latest theoretical advances and gives comprehensive reviews of the available literature. The book covers the main areas of economic evaluation, including the methods for measuring costs and outcomes, the collection of data alongside clinical studies, ways of handling uncertainty, discounting and issues relating to the transferability of economic data.
Output measures and valuation in health
Authors: Dolan Paul 
Published: November 2023

Abstract
In all countries throughout the world, there is a considerable degree of government involvement in the market for health care. If governments are going to deploy the resources at their disposal where they will be of greatest benefit, then information on the benefits associated with alternative allocations is required. This raises questions about how benefit is to be defined and measured and how it is to be allocated amongst the population. This chapter addresses these questions from a normative perspective. That is, it is concerned with how health outcomes ought to be measured and with how benefits ought be weighted according to who receives them.
Costing in economic evaluations
Authors: Brouwer Werner , Rutten Frans , Koopmanschap Marc 
Published: November 2023

Abstract
In this chapter the methods related to costing in economic evaluation are discussed. Costing involves identifying, measuring and valuing all resource changes that occur as a certain health care intervention is carried out. The aim is to value the use of scarce resources (materials, drugs, time of physicians, time of patients etc.) that is needed to produce a certain health effect - the outcome of the intervention. Then, we may be able to weigh the sacrifices against the gains of the intervention and thus determine the relative desirability of such an intervention. In Figure 4.1 we indicate which resource uses may in general be distinguished and need to be identified, estimated in quantitative terms and valued monetarily.
Roles of parathyroid hormone-related peptide and the PTH/PTHrP receptor in skeletal development: implications for growth in children with end-stage renal disease
Authors: Jü Harald , Segre Gino V 
Published: November 2023

Abstract
The actions of two distinct peptides, parathyroid hormone (PTH) and PTH-related peptide (PTHrP), are mediated through the PTH/PTHrP receptor (also referred to as PTH1R), a G protein-coupled receptor which signals through at least two different second messenger pathways, adenylate cyclase (AC)/protein kinase A (PKA) and phospholipase C (PLC)/protein kinase C (PKC).1–3 The PTH/PTHrP receptor is most abundantly expressed in kidney, bone, and the metaphyseal growth plate cartilage, and it is thus likely to have a central role in numerous biological actions.4–6 In kidney and bone, the PTH/PTHrP receptor mediates the endocrine actions of PTH, thus enhancing the tubular reabsorption of calcium, promoting the renal excretion of phosphate, and mobilizing calcium (and phosphate) from bone. Together with 1,25(OH)2 vitamin D3 (1,25(OH)2D3), whose production it regulates, PTH is therefore the most important physiological regulator of calcium and phosphorus homeostasis.
Abnormal PTH synthesis, secretion, and metabolism; pathological parathyroid cell growth and apoptosis in chronic renal failure
Authors: Rodriguez Mariano , Drüeke Tilman B 
Published: November 2023

Abstract
Serum calcium and 1,25(OH)2 vitamin D3 (calcitriol) have long been recognized as important regulators of parathyroid cell function. They act by binding to specific receptors, namely the calcium-sensing receptor (CaR) and the vitamin D receptor (VDR) which are expressed abundantly in the parathyroid cell. More recent work has revealed that extracellular phosphate is another element which directly affects parathyroid function. Alterations in the metabolism of calcitriol, calcium, and phosphate may lead to abnormalities in parathyroid cell function.
Cytokines and growth factors in renal bone disease
Authors: González Esther A , Martin Kevin J 
Published: November 2023

Abstract
Renal bone disease or renal osteodystrophy is a common complication of chronic renal failure and represents a spectrum of skeletal abnormalities ranging from high turnover states, such as osteitis fibrosa which is seen with secondary hyperparathyroidism, to states of low bone turnover which include osteomalacia and the increasingly common adynamic bone disease. While each of these abnormalities may be predominant in a specific patient, combinations of these patterns are commonly present. Histologically, osteitis fibrosa is characterized by increased bone turnover with increased osteoblast and osteoclast number and activity leading to increased bone formation and increased bone resorption. An additional feature of osteitis fibrosa, as the name implies, is the presence of endosteal and marrow fibrosis. In contrast, in adynamic bone disease, there is decreased bone turnover, with decreased number and activity of osteoblasts and osteoclasts.
Osteoporosis in uremia
Authors: Fournier Albert , Grados Franck , Mazouz Hakim , Fardellone Patrice , Oprisiu Roxana , Brazier Michel , Solal Martine Esther Cohen , Ghazali Abderahman 
Published: November 2023

Abstract
As recalled by Eastell et al.,1 World Health Organization (WHO) proposed an operational definition of osteoporosis based on bone mineral density (BMD) measurement, namely BMD of 2.5 SD below its peak value, also called ‘T score at –2.5’. This definition has however limitations in clinical practice since it raises a risk factor for fracture to the status of a definition while it ignores the importance of other determinants of bone strength or of fractures like the risk of falls. Nevertheless it has the advantage to be universally accepted. When at least one fracture is present the osteoporosis is considered to be severe. The T score has to be distinguished from the Z score which is the number of SD above or below the mean BMD of an age, sex, and race-matched population. A T score between –1 and –2.5 SD defines osteopenia. These definitions have been justified by the pragmatic concern of the clinician when to start preventive intervention. Thus while ‘osteopenia’ requires only dietary and behaviorial preventive measures, ‘osteoporosis’ justifies pharmacological intervention to decrease the risk of fracture. It should be noted that the WHO definition has been validated only in women whereas there is no evidence that it is relevant in men.
Soft tissue calcification in chronic renal failure
Authors: Rostand Stephen G , Thornley-Brown Denyse 
Published: November 2023

Abstract
Since the days of Virchow1 soft tissue (extra-osseous or heterotopic) calcification has been observed in autopsy examinations of patients with chronic renal disease. However, with the advent of newer and better dialysis techniques that have improved the life expectancy of patients with end-stage renal disease (ESRD), clinicians have become more aware of the potential serious clinical consequences of soft tissue calcification, particularly those involving the cardiovascular and pulmonary systems. Moreover, the present demography of patients with advanced renal insufficiency, particularly their ever-increasing age and longevity on dialytic therapy, together with more sophisticated non-invasive diagnostic techniques, suggest that almost every patient with end-stage renal disease has at least some ectopic calcification. In this chapter we will discuss the types and distribution of soft tissue calcification in renal failure, risk factors for such calcification, and the possible cellular mechanisms underlying extra-osseous bone formation. Last, having identified some of these underlying risks, we will address the difficult and unsettled questions of prevention and management.
Estimating the costs of tobacco use
Authors: Lightwood James , Collins David , Lapsley Helen , Novotny Thomas E 
Published: November 2023

Abstract
Reliable estimates of the costs of tobacco use are valuable to policy-makers, particularly in planning health service provision and other items of public expenditure. However, such estimates are difficult to obtain because the methods used by different researchers vary and, in some respects, are controversial. Four types of cost analyses are compared here and the implications of different methods for results are explored. The literature on the healthcare costs of smoking is more extensive than for other types of cost and therefore forms the focus of this chapter. Estimates of the gross healthcare costs of smoking (that is, all the expenditures assodated with treating diseases attributable to smoking) for high-income countries range between CJ.10°/o and 1.1% of gross domestic product (GDP).The higher estimates occur in countries where healthcare costs account for a relatively large share of GDP. In low-income and middle-income countries, fewer studies have been performed, and often with very limited data, but the exisdn1 studies suggest that the gross costs of smoking can be as high as those in high-income countries. Studies of the net healthcare costs of smoking—which compare the lifetime healthcare costs of smokers and non-smokers and take account of the fact that smokers’ lives are usually shorter than non-smokers’ —reach more heterogeneous conclusions. This is because of major variations in the methods and assumptions used. However, the majority of these studies indicate that there are net costs from smoking. There is a clear need for refinement of the methods for making cost estimates, particularly for application in developing countries where the tobacco epidemic has yet to peak.