bluuebunny/crossref_metadata_2025_split huggingface dataset:
Acquired coagulation disorders
Authors: Warkentin T.E. 
Published: February 2020

Abstract
Acquired disorders of coagulation may be the consequence of many underlying conditions, and although they may share abnormality of a coagulation test, for example, a prolonged prothrombin time, their clinical effects are diverse and often opposing. General clinical approach: diagnosis—most acquired disorders of coagulation can be identified by screening haemostasis tests, including (1) prothrombin time; (2) activated partial thromboplastin time; (3) thrombin clotting time; (4) fibrinogen degradation products, including (5) the cross-linked fibrin assay (D-dimer); and (6) complete blood count with examination of a blood film. Few bleeding disorders give normal results in all these tests, but disorders predisposed to thrombosis as a result of deficiency of natural anticoagulants (e.g. antithrombin, protein C, and protein S) or certain mutations (e.g. factor V Leiden) must be specifically sought. Treatment—patients with coagulopathies who are bleeding or who require surgery are usually treated with blood products such as platelets and frozen plasma. Other treatments used in particular circumstances include (1) vitamin K—required for the post-translational modification of factors II, VII, IX, and X as well as the anticoagulant factors, protein C, and protein S; (2) cryoprecipitate—used principally for the treatment of hypofibrinogenaemia; (3) concentrates of specific factors—used in isolated deficiencies (e.g. of factors VIII, IX, XI, VIIa, or fibrinogen); (4) antifibrinolytic agents (e.g. ε-aminocaproic acid and tranexamic acid); (5) desmopressin (1-deamino-8-d-arginine vasopressin)—increases factor VIII and von Willebrand factor.
Blood transfusion
Authors: Giovanniello D.S. , Snyder E.L. 
Published: February 2020

Abstract
Transfusion of blood components is a life-saving treatment for patients with severe haemorrhage and can also be used to replace coagulation factors and to ameliorate the effects of severe anaemia, thrombocytopenia, and impaired platelet function. With greater understanding of red cell, platelet, and leucocyte antigen structure and function, transfusion therapy has improved. In addition, understanding current and emerging infectious agents has ensured patient safety. Transfusion medicine has expanded over recent decades to include multiple disciplines, such as therapeutic apheresis, cellular therapy, and tissue banking. One of the most important technological improvements in transfusion therapy was the development of sterile, disposable, and flexible plastic containers that allow separation of whole blood into cellular (e.g. red cells, platelets) and noncellular (e.g. plasma, cryoprecipitate) components, known as apheresis. This technology allows the blood of a donor or patient to pass through an apparatus that separates out one particular constituent and returns the remainder to the circulation. Anticoagulants and additives currently used to collect blood allow storage of liquid suspensions of concentrated red cells for 35 to 42 days. These advances have essentially eliminated the use of whole blood. Blood transfusion is used to treat patients with severe anaemia, haemorrhage, thrombocytopenia, and coagulation disorders. Although the hazards of blood replacement are relatively small, the expected benefit of a transfusion must outweigh the risk to the patient. Therefore, a thorough understanding of the indications of blood transfusion is required to minimize unnecessary blood replacement and to prevent wastage of limited blood resources. Clinicians who prescribe blood transfusion must also be familiar with the risks and be able to recognize and treat transfusion reactions.
Structure and function of skin
Authors: McGrath John A. 
Published: February 2020

Abstract
Skin provides a mechanical barrier against the external environment, but has further roles in thermoregulation, metabolism, and the regulation of fluid balance, as well as being socially important in contributing to physical and chemical attraction between individuals.
There are more than 1,000 different skin diseases, although relatively few are commonly encountered in general medical practice. However, several skin conditions can reflect a general medical problem such as a systemic infection, or a cutaneous manifestation of internal disease (e.g. patients with underlying malignancy, endocrine disorders, or chronic inflammatory diseases). An understanding of skin structure and function is also important in dealing with the common clinical scenario of ‘skin failure’ resulting from adverse drug reactions, burns, or extensive trauma.
Papulosquamous disease
Authors: Griffiths Christopher E.M. 
Published: February 2020

Abstract
Papulosquamous diseases are characterized by well-demarcated areas of papules and scale, typically on an erythematous background. The differential diagnosis includes psoriasis, lichen planus, mycosis fungoides, discoid lupus erythematosus, eczema/dermatitis, drug eruptions, tinea, pityriasis versicolor, secondary syphilis, and pityriasis rosea. The presence of significant pruritus is a useful marker to help with the differential diagnosis: lichen planus and discoid eczema are typically pruritic, whereas others, such as psoriasis, are less so. The distribution is also key to diagnosis, with psoriasis often showing characteristic symmetrical involvement of the extensor surfaces, scalp, and nails. Histology can be essential to reach a diagnosis and plan an appropriate approach to management.
Dermatitis/eczema
Authors: Friedmann Peter S. , Arden-Jones Michael J. , Hay Roderick J. 
Published: February 2020

Abstract
Eczema is a characteristic pattern of skin inflammation that has many subtypes, with some induced by external factors such as irritants or skin sensitizers. Atopic eczema is due partly to a genetic susceptibility, which programmes altered immune responses and skin physiology, together with reactions to exogenous allergens and microbes, but several eczema patterns do not appear to have external causes. Management requires identification and avoidance of provoking factors. The inflammation is treated with topical steroids of different potencies, supplemented with moisturizers. Newer therapies include topical calcineurin antagonists, with a range of systemic therapies being used to control the most severe types of disease.
Infections of the skin
Authors: Hay Roderick J. 
Published: February 2020

Abstract
A huge variety of different organisms exist on healthy skin, the normal microbiome. Under certain circumstances, microbes can actively infect the skin as a primary or secondary event in cutaneous or systemic disease. The most common causes of bacterial infection of the skin are Staphylococcus aureus or β-haemolytic streptococci. There are increasing reports of both hospital- and community-acquired infection by methicillin-resistant S. aureus. Mycobacterium tuberculosis remains a common skin infection in many tropical areas, and syphilis, leprosy, and leishmaniasis are important skin infections in some parts of the world. Dermatophytosis, or ringworm, is caused by mould fungi that can digest keratin (keratinophilic). The diagnosis can be confirmed in the laboratory by examining scrapings or clippings of skin, hair, or nails mounted in potassium hydroxide. Occasionally, in the immunocompromized patient these and other herpes virus infections can disseminate, both to other parts of the skin as well as internally.
Sebaceous and sweat gland disorders
Authors: Layton Alison M. 
Published: February 2020

Abstract
Cutaneous glands in humans include holocrine or sebaceous glands and merocrine or sweat glands. Merocrine glands are subdivided into apocrine, eccrine, and apoeccrine glands. Disorders of each of these cutaneous glands have been associated with disease. Apocrine glands in adults are found predominantly in the axillae and anogenital regions, with a few located in the ear canal (ceruminous glands) and eyelids (Moll’s glands). Disorders associated with apocrine glands include hidradenitis suppurativa, Fox–Fordyce disease, bromhidrosis, trimethylaminuria, and chromhidrosis. Eccrine glands are the sweat-producing glands of the skin. Many drugs and systemic diseases can influence the degree of sweating, such as thyroid disease, infection, carcinoid, and cholinergic drugs. In cystic fibrosis, the concentration of sodium chloride in sweat is increased. Meanwhile, acne is a common inflammatory skin disease often associated with significant psychosocial morbidity.
Cutaneous reactions to drugs
Authors: Walsh Sarah , Creamer Daniel , Lee Haur Yueh 
Published: February 2020

Abstract
Adverse reactions to medications are common and important cause of iatrogenic illness. Severe cutaneous adverse drug reactions include toxic epidermal necrolysis, Stevens–Johnson syndrome, drug reaction with eosinophilia and systemic symptoms, and acute generalized exanthematous pustulosis, which together constitute 2% of all adverse drug reactions and may be life-threatening. Less severe drug-induced skin reactions such as exanthems, urticaria, lichenoid drug rashes, and fixed drug eruptions are more common, sometimes termed benign cutaneous adverse reactions, and generally resolve without sequelae. Drugs may also cause adverse events due to alteration of the normal function of the skin or its appendages. This may take the form of photosensitivity, abnormal pigmentation, or disrupted growth of hair or nails.
Alzheimer’s disease and other dementias
Authors: Schott Jonathan M. 
Published: February 2020

Abstract
Dementia is defined as a syndrome consisting of progressive impairment in memory and other cognitive deficits (aphasia, apraxia, agnosia, or disturbance in executive function) in the absence of another explanatory central nervous system disorder, depression, or delirium, sufficient to interfere with activities of daily living. In recent years there has been a move to consider and classify patients with less severe forms of cognitive impairment—so-called mild cognitive impairment—and non-memory presentations, with the latest DSM-V criteria identifying individuals with major or minor neurocognitive syndromes. Ongoing research using biomarkers suggest that there is a long presymptomatic phase for many causes of dementia.
Epilepsy in later childhood and adulthood
Authors: Sen Arjune , Johnson M.R. 
Published: February 2020

Abstract
Epilepsy is a common, serious neurological disease, with prevalence of 1% and a cumulative lifetime risk of 5%. An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive, synchronous neuronal activity. Epilepsy is defined as a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiological, cognitive, psychological, and social consequences of this condition. Traditionally epilepsy was diagnosed after a patient had two or more unprovoked seizures. However, a more modern definition of epilepsy would also include patients who have had an isolated seizure and have evidence for an enduring alteration in the brain that increases the likelihood of future seizures such as an ‘epileptiform’ electroencephalogram abnormality or an appropriate lesion on structural brain imaging (CT or MRI). Epilepsy cannot, though, be diagnosed unless there has been at least one clinical event compatible with an unprovoked seizure.
Narcolepsy
Authors: Walker Matthew C. 
Published: February 2020

Abstract
Narcolepsy with cataplexy is a specific syndrome of daytime sleepiness, disrupted nocturnal sleep, and episodes of sudden loss of muscle tone—provoked by the anticipation of emotions (in particular laughter)—leading to a tendency to fall, mouth opening, dysarthria or mutism, and facial muscle jerking. It is associated with loss of hypocretin (orexin) neurons in the hypothalamus, hypocretin concentrations in the cerebrospinal fluid below 110 pg/ml, and the HLA genotype DQ B1*0602. A less common form of narcolepsy without cataplexy probably has a different, as yet unknown, pathogenesis. Once established, narcolepsy is lifelong; spontaneous recovery does not occur. Symptomatic treatment—which is essential for school performance, work, driving ability, and quality of life—is with stimulant (e.g. amphetamine) and anticataplectic (e.g. clomipramine) drugs. More recently, sodium oxybate, an anaesthetic, has been used to induce deep sleep overnight, resulting in improvements in all symptoms.
Sleep disorders
Authors: Reading Paul J. 
Published: February 2020

Abstract
Dysfunctional sleep is an important cause of morbidity and is associated with numerous long-term health problems. Sleep-related symptoms can loosely be divided into insomnias, disorders causing excessive daytime sleepiness, and parasomnias, with some conditions having elements of all three categories. The need to sleep is imperative, reflecting the fact that sleepiness, similar to hunger and thirst, is a true drive state. Although its function remains largely elusive, disordered sleep can be associated with profound adverse effects on cognition, mental health, and physical well-being. Moreover, sleep-related symptoms are very common, with 25% of people reporting problems that significantly and regularly impact on daily activities.
Syncope
Authors: Larner Andrew J. 
Published: February 2020

Abstract
Syncope is the most common identified cause of transient loss of consciousness, being ten times more frequent than epilepsy. It is a consequence of cerebral hypoperfusion due to reduced cardiac output, often related to reduced venous return due to decreased peripheral vascular resistance with pooling of blood volume in dependent body parts. Diagnosis is clinical, based on history of the circumstances of the event obtained from the patient and reliable eyewitness(es). In most patients, particularly under 45 years of age, the condition is benign and self-limiting, with an excellent prognosis, requiring little investigation beyond physical examination and electrocardiogram to exclude heart disease. Cardiac causes of syncope may require specific treatment.
Brainstem death and prolonged disorders of consciousness
Authors: Ercole Ari , Hutchinson Peter J. , Pickard John D. 
Published: February 2020

Abstract
Advances in resuscitation and the advent of modern intensive care techniques to support the circulation challenge the simple definition of death in terms of loss of spontaneous circulation (‘cardiac death’). Instead, death is now better regarded as an irreversible loss of the capacity for consciousness combined with irreversible loss of the capacity to breathe. Since the brainstem is required for both consciousness and spontaneous breathing, irreversible loss of brainstem function (e.g. after trauma, haemorrhage, or hypoxia/ischaemia) defines the state of ‘brainstem death’. Clinical criteria for the diagnosis of brainstem death have been published, but practice varies around the world. Brainstem death lies at the extreme end of this spectrum and is, by definition, permanent. Unlike those with brainstem death, patients with prolonged disorders of consciousness may survive for many years without physiological support. The care of such patients has huge social, societal, ethical, and economic implications.
Visual pathways
Authors: Ajina Sara , Kennard Christopher 
Published: February 2020

Abstract
This chapter investigates visual disturbances, which may be caused by diseases of the optic disc, optic nerve, optic chiasm, optic tract, lateral geniculate nucleus, optic radiations, and occipital lobe of the brain, as well as other brain areas involved in complex visual processing. Diagnosis of disturbances of the visual pathways requires both knowledge of their anatomy and physiology, and the ability to carry out a thorough neuro-ophthalmological examination which should enable (1) documentation of the character and extent of the visual disturbance, and (2) topographic localization of the lesion, so that the relevant investigative techniques, such as radiological imaging, can be appropriately requested.
Eye movements and balance
Authors: Strupp Michael , Brandt Thomas 
Published: February 2020

Abstract
The disorders underlying vertigo and dizziness are often combined with disturbances of eye movements; reciprocal effects occur because of the anatomical and functional overlap of the vestibular and ocular motor systems. Therefore, both systems must always be tested in patients complaining of vertigo and dizziness. Using a systematic approach it is possible to make a correct diagnosis in more than 90% of patients. The history is of special importance and one should already have an idea, from the symptoms reported by the patient, what the underlying cause of the vertigo is in order to differentiate the different forms of peripheral, central and functional vestibular vertigo/dizziness and non-vestibular forms.
Traumatic brain injury
Authors: Lawrence Tim , Watkins Laurence 
Published: February 2020

Abstract
Traumatic brain injury is one of the leading causes of death and disability worldwide. It is an extremely heterogenous condition with respect to mechanism, pathophysiology, injury pattern, and investigation findings, with highly variable outcomes, posing a significant challenge to clinicians treating it.
Essential to the management of traumatic brain injury is an integrated, multidisciplinary approach from rapid resuscitation and early intervention through to rehabilitation. The pathophysiology can be divided into primary and secondary injury, where primary represents the injury at the point of trauma and secondary the progression of injury due to a cascade of downstream events occurring as a consequence of the primary injury and subsequent physiological insults.
Viral infections
Authors: McGill Fiona , Farrar Jeremy , Wills Bridget , De Jong Menno , Warrell David A. , Solomon Tom 
Published: February 2020

Abstract
This chapter looks at how enteroviruses are responsible for most cases of viral meningitis where a pathogen is identified; many other viruses can also cause meningitis with considerable geographical and seasonal variation. Their typical presentation is with sudden onset of fever, headache, neck stiffness, and photophobia. There is no change in conscious level. Prognosis is generally good, though recent data suggest not always. Viruses invade and damage the central nervous system in two ways: directly, by infecting the leptomeninges, brain, and spinal cord; and, indirectly, by inducing an immunological reaction resulting in para and postinfectious diseases. In both cases, the terms ‘meningitis’, ‘encephalitis’, and ‘myelitis’ are used alone or in combination.
Intracranial abscesses
Authors: Lawrence Tim , Kerr Richard S.C. 
Published: February 2020

Abstract
An intracranial abscess is a life-threatening condition. Although the incidence is low in countries where antimicrobial treatment for infections is widespread, they remain frequent causes of space-occupying masses in developing countries and, therefore, an important cause of death and disability. Early diagnosis and intervention is vital in reducing potential subsequent sequelae. This chapter describes how they can be broadly defined into three categories based on their anatomical location: extradural, subdural, and intraparenchymal. Aside from supportive care and (where possible) identification and treatment of any underlying cause, treatment requires (1) abscess drainage by image-guided surgical aspiration or excision by craniotomy, and (2) long-term antimicrobial therapy. Early intervention offers the best chance of recovery. Without intervention, intracranial abscesses are fatal. With appropriate treatment overall mortality is between 6.6% and 12.7%, depending on the surgical method used (aspiration compared with craniotomy, respectively). Long-term complications from neurological deficit and epilepsy remain frequent in survivors.
Diseases of the autonomic nervous system
Authors: Mathias Christopher J. , Low David A. 
Published: February 2020

Abstract
The autonomic nervous system innervates all organs, producing predominantly involuntary and automatic actions that are mediated by two principal efferent pathways, the sympathetic and parasympathetic, which are neurochemically and anatomically distinct. Numerous synaptic relays and neurotransmitters allow the autonomic control of organ function at local and central levels to be integrated with the requirements of the whole body. The peripheral and central components of the autonomic nervous system are frequently affected by diseases, conditions, or toxins. Autonomic disorders are described as (1) primary—without defined cause, including multiple system atrophy and acute/subacute dysautonomias; or (2) secondary—with specific defects or as a consequence of other conditions, including diabetes mellitus, Riley–Day syndrome, amyloid neuropathy, dopamine β-hydroxylase deficiency, spinal cord injury, and many drugs.
The motor neuron diseases
Authors: Jenkins Tom , Brockington Alice , Shaw Pamela J. 
Published: February 2020

Abstract
The motor neuron diseases can be considered an extended family of conditions with pathology affecting the lower and/or upper motor neurons, leading to clinical features of limb and/or bulbar weakness. Accurate diagnosis is essential to guide management, in particular, treatment options, any genetic implications, and prognosis. Amyotrophic lateral sclerosis is the family prototype and is a diffuse neurodegenerative disorder characterized by both upper and lower motor neuron cell death, causing progressive paralysis of limb, bulbar (speech and swallowing) and respiratory muscles, and resulting in death from respiratory failure. The cardinal clinical feature of combined upper and lower motor neuron signs in various body regions (bulbar, arms, trunk, legs) forms the basis for diagnostic criteria. There is no cure and the disease progresses relentlessly, with few patients surviving beyond 5 years from symptom onset.
Inherited neurodegenerative diseases
Authors: Sathe Swati 
Published: February 2020

Abstract
Many disorders of the nervous system, especially the degenerative conditions, have a genetic basis, which is usually due to a mutated gene resulting in decreased production of a critical structural or regulatory protein. Inherited neurodegenerative disorders present an enormous challenge because of the complexity of the nervous system, the broad clinical and genetic heterogeneity characteristic of these diseases, and the progressive and generally irreversible nature of their neuropathology. This chapter reviews and provides a guideline for inherited neurodegenerative disease. It is organized in a manner that a good neurological examination would be organized (i.e. systemic disorders followed by neurological disorders), discussed in a top-down manner (i.e. from cortex to muscle). Discussion of individual disorders starts with the molecular genetics, followed by molecular pathology, histology, clinical features, investigational findings, and management.
Disorders of the neuromuscular junction
Authors: Hilton-Jones David , Palace Jacqueline 
Published: February 2020

Abstract
This chapter looks at how two fundamentally different pathological processes are associated with disease at the neuromuscular junction: acquired disorders in which autoantibodies are directed against nerve or muscle receptor or ion channels; rare inherited conditions in which the defect may be pre- or postsynaptic. The acquired neuromuscular junction disorders are associated with antibodies directed against one of the ion channels. The fact that there are three autoimmune disorders known to affect such a small region may be explained by the neuromuscular junction, unlike the peripheral nerve, not being contained within the blood–nerve barrier, which stops just short of the nerve terminal, and thus being potentially exposed to circulating humoral attack. The inherited disorders may affect presynaptic processes (acetylcholine resynthesis, packaging, or release), acetylcholinesterase binding, or postsynaptic function (acetylcholine receptor numbers or localization). Pathogenic mechanisms are considered in more detail when discussing individual disorders.