bluuebunny/crossref_metadata_2025 huggingface dataset:
**75** Chap. 6.
Authors: Kahn Richard J.
Published: August 2020

Barker comments on Cape Elizabeth in 1786–1812 and his visit there in March 1781, to call on the Rev. Ephraim Clark, aged fifty-five, with pneumonia requiring two bleedings. Clark had suffered frequent attacks of pulmonic inflammation which always yielded to bleeding. He continued to preach till the age of seventy-five, when he died of a pulmonic fever. Clark always carried a lancet with him on his parochial visits, as his parishioners were in the habit of being bled, in sickness and in health, as disease prevention. The remainder of this chapter deals with Barker’s patients from ages sixteen to sixty suffering with apoplexy, palsy, hemiplegia, paraplegia, and the apparent successful treatment of bloodletting. He is supported by excerpts on the subject by physicians such as Mathew Baillie and John Hunter; an article by John Collins Warren includes dissections.
**112** Chap. 8. [marked Chap. 7 in MS.]
Authors: Kahn Richard J.
Published: August 2020

Barker states that he would “hereafter describe these cancerous affections;” the remainder of this chapter is devoted to cases of anasarca, ascites, and dropsy. Cancer is discussed in chapter 9. Barker describes a number of patients who were amassing fluids and the assumed causes, such as the use of “ardent spirits” by a fifty-year-old seaman and, in the case of Mrs. Bangs of Gorham, of being “a violent dancer among the Shakers.” A case of anasarca associated with blindness is detailed. Treatments include tapping, bloodletting, and after writing Hall Jackson in Portsmouth, New Hampshire, the use of the latest treatment, digitalis (foxglove), within a year of William Withering’s publication on foxglove in Birmingham, England 1785.
Influenza or Epidemical Catarrh.
Authors: Kahn Richard J.
Published: August 2020

The chapter begins with epidemics of influenza in Maine from 1758 and is introduced by a reference to Noah Webster’s A Brief History of Epidemic and Pestilential Diseases (1799). Barker discusses his and other Maine physicians’ experience from 1780–1795 with cancer, predominantly of skin, node, and breast, seldom treated with success. He details a Portland newspaper article of 1818 graphically describing the last days of a woman’s seventeen-year battle with a lip cancer and refers to a book by a Dr. William Steward of Canaan, Maine reporting forty-seven cancers extracted “without a knife.” Finally, Barker relates his 1795 experience of obtaining a tainted quarter of veal that became palatable after soaking overnight in lye (alkali). With his reading of Lavoisier’s chemistry, Barker began to treat his patients successfully with alkalis to rid them of the “septic poisons.” He recorded his experience and sent a letter to New York City, where it was read by Professor Samuel Mitchill at Columbia University. Mitchill, an editor of the first US medical journal, the Medical Repository, published Barker’s letter, the first of many to be published in that journal over the next twenty years.
Consumption
Authors: Kahn Richard J.
Published: August 2020

This brief chapter begins with a letter dated December 20, 1831, from Samuel Emerson of Kennebunk, Maine, commending the manuscript, particularly the volume on consumption, and agreeing with the “necessity of copious and repeated bleedings.” Barker promises a history of consumptive diseases as they have appeared in Maine and some other parts of New England since 1768, to be preceded by a history of the disease back to Hippocrates by Thomas Young. He notes that earlier in the eighteenth century consumption made up only about one-tenth of the bills of mortality but by the close of the century, it appears to have made up one-fourth of all deaths. Several physicians in North America have made valuable observations on consumption, though chiefly in the middle and Southern states; Barker will provide results of his inquiries and observations as it has appeared in the northern and eastern parts of the Union. He remarks about some cures that have come about by what are called by some, “the efforts of nature.”
Tracheal Consumption.
Authors: Kahn Richard J.
Published: August 2020

Barker discusses contributing factors, symptoms, and treatment of tracheal consumption in five of his patients from Falmouth, Stroudwater, Gorham, and Westbrook. Treatments include calomel, alkalis, and opium for cough suppression. He adds a comment about ulceration of tonsils among children with reference to an 1809 article by Felix Pascalis.
**C110** Chap. 5.
Authors: Kahn Richard J.
Published: August 2020

In addition to the use of bleeding and mercurials, in this chapter Barker illustrates the beneficial effects of emetics, cathartics, alkalines, and digitalis as well as epispastics, issues, setons and cauteries, opiates, tonics, suitable diet, and salubrious air with exercise, using his own cases and those of authors in both Europe and America. He previously noted the beneficial effects of alkalines in slow fevers and epidemics and, after trials, found them useful in hectic fevers as recorded in some of his case reports since 1800 as well as those of other medical authors. He includes an excerpt of an 1802 article by Benjamin Rush on the benefits of salivation in pulmonary consumption and an 1813 article by Harvard’s John Warren in which he wrote, “The introduction of mercury, in the treatment of pulmonary diseases, after allaying inflammatory action, by venesection, and other evacuants, may be deemed one of the most important innovations, in modern practice.”
**C135** Chap. 6th
Authors: Kahn Richard J.
Published: August 2020

Barker relates some unusual cases such as empyema (pus between lung and chest wall) in a fifty-five-year-old minister who was treated, drained, and lived for an additional thirty-eight years. A president of the College of New Jersey (later Princeton University) who had long-standing lung symptoms wrote a letter stating that he was such a devotee of venesection that he bought himself a lancet for self-treatment. In 1811 Barker’s twenty-five-year-old daughter, Eliza, daughter of a consumptive mother, developed chest pain, hacking cough, fever, and wasting. She received standard treatment by a physician acquaintance but rejected her father’s and a consultant’s suggestion to be bled. Eliza deteriorated, finally agreed to be bled, and was cured after five months of symptoms. She married in June and was doing well twenty years later. In Barker’s opinion, prevention should focus on proper clothing for women for “If the breast is left open to facilitate the entrance of Cupid’s darts, it affords a more certain mark for the envenomed shafts of the grisly king of terrors” (Joseph Young, 1809).
**C207** Chap. 7th
Authors: Kahn Richard J.
Published: August 2020

After brief comments on Hippocrates, Celsus, Cullen, and others, Barker discusses “the unfortunate [John] Brown,” whose “excentric notions & heated imagination . . . fascinated a considerable number of physicians, both in Europe & America.” About 1788, when Brown’s Elements of Medicine first appeared in Maine to be read by physicians, lawyers, and divines, the lancet and other depleting means were replaced by stimulants, chiefly of spiritous liquors. People with pulmonary consumption seldom consulted physicians, preferring the stimulating plan to the lancet. Physicians also preferred the stimulating plan and rarely used the lancet until 1798, when Dr Rush’s Inquiry into the Causes and Cure of the Pulmonary Consumption, printed in 1793, was brought to Portland and read by Barker and others. But prejudice against the use of the lancet in consumption was strong, and the change didn’t take place until the beginning of the nineteenth century.
Birth of a Benefit—Brief History of the ESRD Program
Authors: Armistead Nancy C. , Perlmutter Jeffrey A.
Published: August 2020

The story of the end-stage renal disease (ESRD) entitlement program is told through progressive scientific and technological advances, selection committees, and exploration of public policy values. The legislative history shows a program thought to accommodate a limited number of patients who, returning to work, would contribute to the social security program paying for their benefits. The reality has been different. Treatment is offered to virtually all patients with ESRD, often with little regard to the individual’s likelihood of benefit from dialysis and quality of life. The program’s next chapter will tell whether patient-centered care becomes a focal point in the program’s history.
Models for Operationalizing Supportive Care in Kidney Care
Authors: Lupu Dale E. , Murphy Emma
Published: August 2020

The field of kidney supportive care is in a period of innovation, with different models emerging from local efforts to improve care. We classify emerging models into six types: embedded, mobile/visiting, chronic kidney disease case management, medical management without dialysis, concurrent hospice/dialysis, and comprehensive regional or system-wide programs. Although individual programs have demonstrated positive impact on outcomes such as advance care planning and place of death, there is not yet systematic evidence comparing the impact of model type on effectiveness or cost effectiveness. Local considerations about need, resources, opportunities, and champions are key to planning a supportive kidney care strategy. Facilitators for program success include training for nephrology providers, active collaboration between nephrology and palliative care, local champions (often nurses), sensitive messaging about medical management without dialysis, and research to demonstrate program impact.
Primary Palliative Care Education for the Multidisciplinary Nephrology Team
Authors: Teixeira J. Pedro , Combs Sara A.
Published: August 2020

Supportive care of patients with progressive kidney failure, like the care needed by all patients with chronic kidney disease and end-stage kidney disease, is most successfully provided through a multidisciplinary team. An effective kidney supportive care program requires that the multidisciplinary team be properly educated on the palliative needs of these patients and that team members develop primary palliative care skills to meet these needs. This chapter presents the case for why nephrology clinicians need to acquire primary palliative care knowledge and skills to better treat their patients. It reviews the existing state of supportive care for patients with kidney disease and of the training in primary palliative care currently provided to nephrology fellows and nephrologists. It recommends the development of a primary palliative care curriculum for members of the multidisciplinary kidney care team involved in patient care and makes suggestions on curriculum content for nephrology clinicians.
The Shared Decision-Making Process as the Recommended Standard for Treatment Decisions in Kidney Disease and Requisite Communication Skills to Implement the Process
Authors: Mandel Ernest I. , Schell Jane O. , Cohen Robert A.
Published: August 2020

Shared decision-making (SDM) is the accepted standard of care paradigm for medical decision making between patient or surrogate and clinician. In its Choosing Wisely campaign, the American Society of Nephrology (ASN) recommended SDM prior to the initiation of dialysis. Evidence suggests that SDM enhances patients’ understanding of their illness and satisfaction with the decision-making process, but at present SDM is poorly integrated into dialysis decision-making. Dialysis patients often describe a passive role in the decision to start dialysis, reinforcing the need for implementation of SDM in decision-making with patients with kidney disease. The hallmark feature of SDM is collaboration between the clinician and the patient or surrogate whereby the patient’s expertise in the realm of values and priorities is elicited while the clinician’s medical expertise is shared. The ultimate treatment decision results from the integration of their respective expertise. The Agency for Healthcare Research and Quality SHARE Approach outlines the components of SDM, and frameworks such as the Serious Illness Conversation Guide, REMAP, and SPIRES are roadmaps for those components. Communication tools and mnemonics also facilitate SDM conversations. With knowledge and application of these frameworks and tools, the nephrology community will be better positioned to fulfill the mandate embodied in the ASN Choosing Wisely campaign to employ the SDM process in renal replacement therapy decisions.
Involving Family and Friends in Palliative Care for Persons With Kidney Disease
Authors: Anderson Elizabeth , White David M.
Published: August 2020

Family-centered palliative care for patients with kidney disease shows great promise in alleviating the suffering of patients with collateral benefits to family members. Engaging family caregivers is particularly valuable for those with kidney disease, given often multiple comorbidities, impact of dialysis on quality of life, and often conflicting end-of-life decisions. Family caregivers are at high risk for burnout, increasing the likelihood that patients themselves will not receive end-of-life care or support. Important components of family-centered palliative care include assessment, emotional support, education, assertive communication skills, referrals, and grief and bereavement support. It is crucial that the care team recognize the role of cultural norms, family beliefs, and communication patterns, tailoring family-centered care to meet the needs of each individual patient.
Active Medical Management Without Dialysis for Patients With Advanced Chronic Kidney Disease
Authors: Li Kelly , Brown Mark
Published: August 2020

This chapter outlines the management of patients with advanced chronic kidney disease for whom dialysis may not be beneficial or desired. Active medical management without dialysis should be offered to patients through a shared-decision making process as a viable alternative to dialysis. This is important as patients and families wish to consider not only survival, but also symptom control and QoL in their decision to pursue a dialysis or nondialysis pathway. A multidisciplinary team delivering good quality, active, and patient-centered care that combines chronic kidney disease management with the principles of palliative care can help patients achieve good symptom management and quality of life. Active and early planning for the end-of-life phase facilitates appropriate care for patients in acute and/or unexpected deterioration and helps achieve patient and family goals.
Systematic Nonpain Symptom Assessment and Management
Authors: Yu Hana , Scherer Jennifer S.
Published: August 2020

Patients with advanced chronic kidney disease (CKD) suffer from a substantial burden of physical and emotional symptoms that adversely affect their health-related quality of life. Their number of symptoms when systematically evaluated is comparable to cancer patients. Overall symptom burden and severity are correlated directly with impaired quality of life and depression. Among the most frequent are fatigue, pain, pruritus, sleep disturbances and poor appetite. This chapter highlights one of the most common and troublesome of these symptoms affecting advanced CKD patients, particularly those with end-stage kidney disease: uremic pruritus. The epidemiology, pathophysiology, and current evidence-based treatments are discussed as a way to provide a practical guide for diagnosis and treatment. Lastly, areas for future research to improve symptom management in this growing, chronically ill population are identified.
Geriatric Nephrology Syndromes and Assessment and Management of Cognitive Impairment
Authors: Brown Edwina A. , Iyasere Osasuyi
Published: August 2020

The demographics of the renal population have evolved, such that the elderly are the fastest growing group. Consequently, the multidisciplinary renal team often encounters clinical issues associated with advancing age. In this chapter, frailty, falls, and cognitive impairment are discussed as underrecognized geriatric syndromes in patients with chronic kidney disease. A case history illustrates how these syndromes tend to co-exist and affect quality of life and survival. The risk factors, management strategies, and future research priorities are discussed, recognizing the importance of a multidisciplinary approach to the management of elderly patients with kidney disease.
Ethical Issues in the Supportive Care of Patients With Advanced Kidney Disease
Authors: Butler Catherine R. , Moss Alvin H.
Published: August 2020

Although the benefits of standard dialysis therapy for many older adults with complex comorbid conditions is equivocal, there continues to be substantial moral uncertainty in the practice of withholding and withdrawing dialysis treatment. This chapter reviews several ethical conundrums in palliative care of patients with advanced kidney disease, including uncertainty about the moral status of withholding dialysis and pursuing medical management without dialysis, challenges in decision-making when patients lack capacity to participate, and ethically relevant social and cultural factors influencing practice. Better understanding of the underlying causes of these conundrums reveals opportunities to improve quality of patient care at the individual and system levels by incorporating palliative practices. The chapter also suggests strategies for clinicians to identify and facilitate resolution of ethical conflicts around end-of-life care for patients with advanced kidney disease in clinical practice.
Transforming Practice to Support Person-Centered Care for Patients With Advanced Kidney Disease
Authors: O’Hare Ann M. , Armistead Nancy C.
Published: August 2020

Contemporary patterns of care for patients with advanced kidney disease are far from person-centered. Large changes to health systems, payment structures, quality measurement, patient and provider education, and the culture in which care is delivered will be needed to support a more person-centered approach to care for members of this population. To uphold the essence of who our patients are, efforts are needed throughout the illness trajectory to foster the development of strong patient–provider relationships and extend the reach of these relationships across settings, to educate our patients about their treatment options and what to expect in the future, to offer opportunities for patients to involve their family members and close friends in their care, and ultimately to promote a culture in which providers are flexible, creative, and tireless in working with their colleagues and with their patients and their families to fulfill the mission of person-centered care of finding the “right treatment for the right person at the right time.”
Overdiagnosis in Psychiatry
Authors: Paris Joel
Published: August 2020

This book, now revised in a second edition, examines the problem of overdiagnosis in psychiatry, focusing on problems with current diagnostic systems. It shows that diagnosis is not always a good guide to treatment selection and that diagnoses have been expanded in scope to justify currently popular methods of pharmacotherapy or psychotherapy. The most important categories that are overdiagnosed are bipolar disorders, major depression, attention deficit hyperactivity disorder, and posttraumatic stress disorder. The boundary of pathology and normality remains unclear. This edition also discusses dimensional systems that are transdiagnostic and shows how overdiagnosis is linked to the practice of aggressive psychopharmacology.
Posttraumatic Stress Disorder
Authors: Paris Joel
Published: August 2020

Posttraumatic stress disorder (PTSD) differs from other categories that are underdoing diagnostic epidemics, in that its treatment is primarily based on psychotherapy. PTSD tends to be overdiagnosed when clinicians attribute, rightly or wrongly, symptoms to a traumatic event. Yet most people who undergo trauma do not develop PTSD, and people without trauma can have similar symptoms. This chapter shows how the construct of PTSD arose historically. A problematic DSM definition, in relation both to the nature of stressors and the links between stress and outcome, causes PTSD to be overdiagnosed. The result is that many patients are receiving the wrong kind of psychotherapy for their problems.
Personality and Personality Disorder
Authors: Paris Joel
Published: August 2020

Personality traits differ among normal people, and one should only diagnose a personality disorder (PD) in the presence of a clear-cut impairment of functioning. Most of these disorders lie on a spectrum with traits, but those that cause prominent symptoms present more often in psychiatry The most clinically important category of PD is borderline personality disorder (BPD), but this condition is widely underdiagnosed. Since these patients often present with depression and/or affective instability, clinicians often see them as suffering from mood disorders, and treat them unsuccessfully with antidepressants. However, this population, which shows repetitive suicidal behavior, needs to be correctly diagnosed to be referred for specialized psychotherapy.
How Do We Know What Is Normal?
Authors: Paris Joel
Published: August 2020

Establishing the boundaries of disease with normality is somewhat easier in general medicine than in psychiatry. This chapter reviews the various definitions of normality and examines their strengths and weaknesses. It will reflect the concerns of many observers that the medicalization of human experiences can pathologize normality. It will also present a critique of an emphasis on early diagnosis, which extends the boundaries of psychiatry by treating people who are functioning within a normal range, and for whom the cost-benefit ratio of early intervention may weigh more on the negative side. Examples from general medicine are presented in which this scenario has also been played out.
Where Do We Go From Here?
Authors: Paris Joel
Published: August 2020

This chapter concludes the book by examining the implications of its overall argument. It begins by discussing the increase of consumerism in medicine, examining the good and bad sides of trends leading to less power for professionals and more power for consumers. It explains why patients and their families can become even more attached to a doubtful diagnosis that professionals who care for them. This shift has been made possible in part by wider diffusion of information through the internet. The chapter suggests strategies for clinicians interested in overcoming the tendency to overdiagnose mental disorders. They include a greater commitment to science, a healthy suspicion of fads, a tolerance for ambiguity, and a sense of humility about our current state of knowledge about mental disorders.
Injuries in sports activities in older people
Authors: Kostka Tomasz , Kostka Joanna
Published: August 2020

Regular physical activity in old age is widely recommended as an effective way to prevent chronic diseases and maintain well-being. Nevertheless, sports participation carries the risk of injury. In elderly people, the risk of injury is greater due to age-related pathophysiological changes and concomitant chronic conditions. Available data indicate an increasing number of injuries among older people, which is associated with there being more older people and an increasing number of these people are participating in sports and physical exercise. An appropriate identification of risk factors for injury and education of older people can reduce the incidence of injuries. Methods of preventing injuries include protective equipment such as helmets, warming up, and properly designed training programmes. Health benefits of participation in regular physical activity adjusted to health status and physical functioning outweigh hazards of sport-related injuries, even in advanced age.
Examining the nervous system of an older patient
Authors: Woodford Henry J. , George James
Published: August 2020

Ageing is associated with changes in the nervous system, especially the accumulation of neurodegenerative and white matter lesions within the brain. Abnormalities are commonly found when examining older people and some of these are associated with functional impairment and a higher risk of death. In order to reliably interpret examination findings it is important to assess cognition, hearing, vision, and speech first. Clarity of instruction is key. Interpretation of findings must take into account common age-related changes. For example, genuine increased tone should be distinguished from paratonia. Power testing should look for asymmetry within the individual, rather than compare to the strength of the examiner. Parkinsonism should be looked for and gait should be observed. Neurological assessment can incorporate a range of cortical abilities and tests of autonomic function, but the extent of these assessments is likely to be determined by the clinical situation and time available.
Introduction
Authors: Mitchell Andrew , De Maria Giovanni Luigi , Banning Adrian
Published: August 2020

This chapter introduces the subject of cardiac catheterization. It starts with background, basic definitions, and a history of the discipline. Indications for both cardiac catheterization and coronary angiography are explored, and the choice between cardiac computed tomography or invasive angiography is discussed. Radiology equipment is explained, along with fluoroscopy and acquisition in terms of optimizing the diagnostic picture with the lowest practicable dose. Following that, radiation safety and protective measures are defined, along with ALARA (‘as low as reasonably achievable’) and dose excess. Finally, preparation of both the patient and the catheter laboratory are covered.
The team
Authors: Mitchell Andrew , De Maria Giovanni Luigi , Banning Adrian
Published: August 2020

This chapter covers the different roles for the cardiologist, the nurse, the clinical physiologist, and the radiographer within the heart team. It describes the individuals’ responsibilities and functions from before the procedure starts to post-procedural care and patient discharge. This includes key aspects of periprocedural care such as World Health Organization checklists, sedation, patient monitoring with electrocardiograms and arterial pressure monitors, in addition to trigger points for escalation. The chapter also describes methods of training, annual requirements for procedural numbers, audit, logbooks, and record-keeping and explains some aspects of certification and recertification.
Specific conditions
Authors: Mitchell Andrew , De Maria Giovanni Luigi , Banning Adrian
Published: August 2020

This chapter covers cardiac catheterization for a number of different heart conditions, including detail on specific valve conditions such as aortic stenosis, transcatheter aortic valve implantation (TAVI) work-up for aortic stenosis, aortic regurgitation, mitral stenosis and regurgitation, and pulmonary and tricuspid valves. The chapter explores the application of cardiac catheterization for pulmonary conditions, pulmonary angiography, and specific findings and procedural detail for patients with atrial and ventricular septal defects, constrictive pericarditis, pericardial tamponade, as well as cardiomyopathies. The chapter concludes with a discussion of different causes of myocardial infarction with non-obstructed coronary arteries and the subsequent investigation of this group of patients.
Coronary artery bypass graft angiography
Authors: Mitchell Andrew , De Maria Giovanni Luigi , Banning Adrian
Published: August 2020

Most patients undergoing coronary artery bypass grafting, or CABG, in the past decade will have had a combination of arterial grafts and saphenous vein grafts (or received total arterial revascularization), as conduit patency for mammary arterial grafts has been shown to be significantly higher than that of vein grafts. This chapter covers one of the most common operations performed on patients with three-vessel coronary disease. It describes the role of pre-angiography, choosing an appropriate catheter, and the location of various grafts. It is fully illustrated with clinical images throughout, annotated to aid understanding.
Additional procedures
Authors: Mitchell Andrew , De Maria Giovanni Luigi , Banning Adrian
Published: August 2020

This chapter considers additional cardiac procedures that primary operators and team members in the cardiac catheterization laboratory will need an understanding of. This includes emergency interventions such as pericardiocentesis for pericardial tamponade, temporary pacemaker implantation for complete heart block, and cardioversion or defibrillation for cardiac arrhythmias. The chapter describes circulatory support with mechanical cardiopulmonary resuscitation, intra-aortic balloon pumps, and Impella® devices, and discusses the use of extracorporeal membrane oxygenation. Information is also included on how to perform endomyocardial biopsies. The chapter is fully illustrated with schematic diagrams as well as information on indications, contraindications, and methods for each procedure.
Post procedure
Authors: Mitchell Andrew , De Maria Giovanni Luigi , Banning Adrian
Published: August 2020

This chapter covers the actions required after the patient has undergone their procedure. It begins by explaining the role of the specialist nurse team, the checks that should be performed on the patient once they have returned to the ward, and the process for becoming ambulant. The chapter then goes on to explain how to review the results and data regarding the case. Communication of both results and potential follow-up procedures is then covered. The chapter ends by addressing discharge plans and guidance is given on completing a discharge summary.
Pathogenesis of myeloproliferative neoplasms
Authors: Fleischman Angela G. , Van Etten Richard A.
Published: August 2020

The myeloproliferative neoplasms (MPN) were first grouped together by Dameshek in 1951 based upon their similar clinical characteristics. Since that time, and particularly over the past decade, molecular biological and genetic investigation into the pathogenesis of MPN has revealed their shared stem cell nature, a common pathophysiological theme of aberrant cytokine signalling through dysregulated tyrosine kinases that drives the overproduction of maturing myeloid cells, and, as in other myeloid neoplasms, a major modifying role for loss of function mutations in epigenetic regulatory proteins. This chapter reviews the molecular pathogenesis of chronic myeloid leukaemia (CML) and the Philadelphia-negative MPN with a particular emphasis on the contribution of mouse models of MPN to our understanding of these diseases.
Molecular risk stratification of myeloproliferative neoplasms
Authors: Guglielmelli Paola , Vannucchi Alessandro M.
Published: August 2020

Recent advances in understanding the molecular landscape of chronic myeloproliferative neoplasms (MPN) have remarkably improved the diagnostic approach to these disorders. The three phenotypic driver mutations, involving JAK2 (V617F, exon 12 mutations), MPL, and CALR, are major diagnostic criteria in the World Health Organization (WHO) classification, and point to different risk categories. Subclonal mutations in genes of the epigenetic regulation and the spliceosome deserve major prognostication significance and contribute to identify categories of patients with different survival and risk of leukaemia. This chapter will address these aspects and elucidate how mutational analysis may contribute to advanced assessment of MPN patients, as well as its the impact on prognosis for those with MPN.
Essential thrombocythaemia
Authors: Besses Carlos , Bellosillo Beatriz , Alvarez-Larrán Alberto , Mughal Tariq I.
Published: August 2020

Essential thrombocythaemia is a classic myeloproliferative neoplasm characterized by thrombocytosis, increased risk of thrombotic and/or haemorrhagic complications, and a trend to transformation to myelofibrosis and acute leukaemia. Mutations in JAK2, CALR, and MPL genes besides bone marrow histology are crucial elements of diagnosis. Treatment is aimed to prevent the appearance of thrombotic complications that are the main cause of morbidity and mortality. Accordingly, thrombosis risk stratification is of the utmost importance to select the appropriate treatment. Antiplatelet therapy as primary antithrombotic prophylaxis in low-risk patients should be tailored according to the existence of extreme thrombocytosis and presence of JAK2V617F mutation and/or cardiovascular risk factors. Cytoreductive treatment options are discussed with reference to results of randomized clinical trials. Practical approach to unusual and risk situations as surgery, pregnancy, and paediatric essential thrombocythaemia are also reviewed.
Systemic mastocytosis
Authors: Gotlib Jason
Published: August 2020

The study of systemic mastocytosis (SM) is undergoing dynamic change. Understanding of the biologic underpinnings of the disease, including its various World Health Organization-defined subtypes, is being catalysed by next generation sequencing in conjunction with sophisticated molecular biology tools which have allow refined analysis of mast cell immunophenotypes and intracellular signalling networks. In turn, this has provided translational opportunities for identifying new targets of therapy and biologic correlates of response, particularly in advanced forms of disease. Because SM is a rare and heterogeneous disease, collaborative efforts are being undertaken to aggregate data from large numbers of patients to better define the natural history and prognosis of its variants. This chapter aims to summarize the major features of mast cell biology, diagnosis, classification, and treatment, including a focus on more recent developments regarding the mutational landscape of SM and novel therapeutics.
Myelodysplastic syndromes/myeloproliferative overlap neoplasms
Authors: Padron Eric , Mughal Tariq I. , Sallman David , List Alan F.
Published: August 2020

The myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are haematologically diverse stem cell malignancies sharing phenotypic features of both myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN) that display a paradoxical bone marrow phenotype hallmarked by myeloid proliferation in the context of bone marrow dysplasia and ineffective haematopoiesis. The unfolding MDS/MPN genomic landscape has revealed numerous mutations in signalling genes, such as CBL, JAK2, NRAS, KRAS, CSF3R, and others involving the spliceosome complex. These observations suggest that comutation of genes involved in dysplasia and bone marrow failure along with those of cytokine receptor signalling may, in part, explain the dual MDS/MPN phenotype. The respective MDS/MPN diseases are identified by the type of myeloid subset which predominates in the peripheral blood. Currently there are no standard treatment recommendations for most patients with MDS/MPN. To optimize efforts to improve the management and disease outcomes, it is essential to identify meaningful clinical and biologic endpoints and standardized response criteria for clinical trials.
Assessment of disease burden in patients with myeloproliferative neoplasms
Authors: Geyer Holly L. , Radia Deepti , Sarlis Nicholas , Mesa Ruben A.
Published: August 2020

The BCR-ABL-negative myeloproliferative neoplasms (MPN) collectively represent a diverse assortment of clonal myeloid malignancies with variability in both genotypic and phenotypic presentation. MPN subtypes including essential thrombocythaemia (ET), polycythaemia vera (PV), myelofibrosis (MF), systemic mast cell disorders have been best studied in terms of symptom profiles and heterogeneously incur disease burdens that impact treatment options, overall quality of life, and survival. Recent treatment strategies have aimed to incorporate disease burden assessment into the selection of therapeutic interventions. Patient-reported outcome (PRO) tools have been developed to objectively assess the MPN disease burden in both clinical and trial formats. Their development supports recent FDA efforts to promote PROs in supporting labelling claims for novel targeted agents. This chapter discusses the spectrum of symptoms inherent to MPN disorders, available risk scoring tools and PRO options, and the emerging role of patient outcomes as trial endpoints.
Clinical trials in myeloproliferative neoplasms
Authors: Barosi Giovanni , Tognoni Gianni
Published: August 2020

In this chapter we discuss the benefits and limitations of trial endpoints used for myeloproliferative neoplasms (MPN) treatment. In myelofibrosis (MF), improvement in overall survival (OS) was a secondary endpoint in three randomized trials, with confounding results related to sample size, trial duration, and treatment choices after progression. Progression-free survival was a secondary endpoint in one trial with a greater clinical impact with respect to OS, but the reflection of its benefit on OS was not demonstrated. Overall response rate was widely used in MPN, however, it reflects heterogeneous objective benefits for the patients and biological rationales. In essential thrombocythaemia and polycythaemia vera (PV), event-free survival documented the capacity of therapies to prevent thrombotic occurrences, reflecting clinically relevant benefit to patients. Patient-reported outcomes were secondary endpoints in MF and PV, and their support to change in ORR was the reason of drug approval by regulatory agencies.
Crisis management and human factors
Authors: Mercer Simon
Published: August 2020

This first chapter explains how human factors affect the management of crises. The roles human factors play in the management of emergencies and crisis situations are becoming increasingly recognized in healthcare, having gained political momentum following a concordat from the National Quality Board in 2013. A framework is presented for emergency preparation, including standard operating procedures, checklists, allocation of roles, preparing equipment and drugs, sharing mental models, and communicating with support specialists. During the incident the focus is on situational awareness, leadership and teamwork, communication, and decision-making. Post-event debriefing is discussed with the joint goals of addressing psychological stress and learning for the future. Also provided are links to available up-to-date best-practice guidelines and useful resources for further reading.
Paediatrics
Authors: Lutman Daniel
Published: August 2020

This chapter on paediatrics starts by covering neonatal resuscitation and basic and advanced paediatric life support. It then moves on to discuss the anaesthetic management of children suffering from drowning, acute severe asthma, stridor, anaphylaxis, major trauma, burns, and sepsis. Tables are provided showing normal weights and physiological parameters, and guiding choice of equipment size for different age ranges. A drug formulary is included with dose calculations for commonly used medicines. Definitions, presentation, management strategies, investigations, risk factors, exclusions, and causes, and any special considerations for each potential paediatric-related situation are given. Lists of up-to-date online resources and further reading are also provided here, offering invaluable know-how to encourage the reader to broaden their knowledge.
Obstetrics
Authors: Scrutton Mark , Kinsella Michael
Published: August 2020

This chapter covers emergency management for obstetric crises including maternal collapse, severe haemorrhage, amniotic fluid embolus, pre-eclampsia and eclampsia, total spinal, accidental dural puncture, cord prolapse, failed intubation, ruptured ectopic, retained placenta, and placenta praevia. The authors give guidance on how to perform intrauterine resuscitation and conduct anaesthesia for a category I Caesarean section. In addition, the definitions, presentation, management strategies, investigations, risk factors, exclusions and causes, and any special considerations for each potential obstetric-related situation are given. Lists of up-to-date online resources and further reading are also provided here, offering invaluable know-how to encourage the reader to broaden their knowledge.
Neurology/neurosurgery
Authors: Hunt Katharine , Waraich Manni
Published: August 2020

In this chapter on neurosurgery and anaesthesia, the authors give clear guidance for the management of time critical brain and neurological conditions, including raised intracerebral pressure, severe head injury, subarachnoid and intracerebral haemorrhage, sodium disturbances, venous air embolism, acute spinal cord injury, spinal shock, autonomic dysreflexia, dystonic reactions, and status epilepticus. In addition, the definitions, presentation, management strategies, investigations, risk factors, exclusions and causes, and any special considerations for each potential neurosurgical and neurologically related situation are given. Lists of up-to-date online resources and further reading are also provided here, offering invaluable know-how to encourage the reader to broaden their knowledge.
Regional anaesthesia
Authors: Davies Dr. Owen
Published: August 2020

There are a number of important emergencies associated with regional anaesthesia that the anaesthetist should be aware of. Life-threatening emergencies, such as local anaesthetic systemic toxicity leading to cardiorespiratory arrest can occur irrespective of the site of local anaesthetic infiltration. Specific procedures including epidural and spinal anaesthesia, eye blocks, and peripheral nerve blocks cause a range of surgical emergencies and severe complications. Neuraxial anaesthesia carries the risk of epidural haematoma and abscess while infiltration or local anaesthetic around the globe carries the risk of both retro-orbital haematoma and globe perforation, all of which may require urgent surgical intervention. Although literature supports the safety of low concentration (1 in 200 000) adrenaline as an additive to digital blocks, inadvertent injection of higher concentrations may have the potential for ischaemic injury. Finally, the assessment and diagnosis of perioperative nerve injuries when associated with a peripheral nerve block present a formidable clinical challenge.
Metabolic and endocrine
Authors: Blanshard Hannah
Published: August 2020

This chapter deals with a plethora of metabolic and endocrine abnormalities from an anaesthetic perspective. Major biochemical cation derangements are covered including high and low sodium, potassium, calcium, and magnesium levels. Guidelines are also presented for the management of anaphylaxis/severe allergy, diabetic ketoacidosis, and hyperosmolar non-ketotic coma, malignant hyperthermia, porphyric crisis, thyrotoxic storm, phaeochromocytoma, Addisonian crisis, disseminated intravascular coagulopathy, hypoglycaemia, acute liver failure, sickle cell crisis, TURP syndrome, and hypothermia. In addition, the definitions, presentation, management strategies, investigations, risk factors, exclusions and causes, and any special considerations for each potential metabolic and endocrine-related situation are given and elaborated upon. Lists of up-to-date online resources and further reading are also provided here, offering invaluable know-how to encourage the reader to broaden their knowledge.
Recovery problems
Authors: Gibson Charles
Published: August 2020

This chapter details the clinical problems anaesthetists often face with patients in the recovery unit. These include acute coronary syndromes and other causes of chest pain, acute heart failure, hypertension and hypotension, respiratory failure and hypoxia, acute confusion, lowered consciousness level and stroke, residual neuromuscular blockade, oliguria and acute renal failure, severe postoperative nausea and vomiting, and epidural problems. In addition, the definitions, presentation, management strategies, investigations, risk factors, exclusions and causes, and any special considerations for each potential recovery-related situation are given and elaborated upon. Lists of up-to-date online resources and further reading are also provided here, offering invaluable know-how to encourage the reader to broaden their knowledge.
Emergency department problems
Authors: Sutherland Kath , Blackburn Jim , Rasburn Neil
Published: August 2020

This chapter covers the common problems anaesthetists and intensive care physicians may be faced with when called to help in the emergency department. It contains guidelines for the treatment of major trauma, drug overdose (general principles and specific agents), major gastrointestinal bleeding, chemical/biological/radiation injuries, decreased level of consciousness, and sepsis. There is also a section on post-resuscitation care which supplements the life support guidelines already covered in Chapter 2 (adults) and Chapter 5 (paediatrics). In addition, the definitions, presentation, management strategies, investigations, risk factors, exclusions and causes, and any special considerations for each potential situation are given and elaborated upon. Lists of up-to-date online resources and further reading are also provided here, offering invaluable know-how to encourage the reader to broaden their knowledge.
Practical procedures
Authors: Cossey Louise , McCormick Bruce
Published: August 2020

The final chapter provides instructions for practical procedures which support advice given in other sections. Techniques for managing difficult airways are explored including front of neck access (by surgical or needle cricothyroidotomy), intubating laryngeal mask insertion, awake fibreoptic intubation, lightwand-assisted airway management, and retrograde intubation. Meanwhile, techniques for chest drain insertion and one-lung ventilation are described. Methods of administering bronchodilators by in-circuit nebulization or metered dose inhaler are offered. Advanced access techniques for the circulation are also presented, including internal jugular catheters, femoral vein catheters, intraosseous needle insertion, and venous cut-down. Pacing is discussed within the contexts of initiating new pacing (internal and external) and providing anaesthesia for patients with pre-existing implantable pacing systems and cardioverter defibrillators. A final section gives guidance on transferring critically ill patients.
A case of severe aortic stenosis: ongoing challenges with management
Authors: Kang Sok-Leng , Martin Robin
Published: August 2020

The clinical management of severe aortic stenosis in the newborn period is complex and challenging. This chapter presents case of severe aortic stenosis in a 3-day-old neonate who required multiple interventions in infancy, including balloon aortic valvuloplasty, the Ross procedure, pulmonary conduit replacement, and branch pulmonary artery stenting. The chapter outlines the clinical signs and symptoms of aortic stenosis in neonates, detailed echocardiographic and haemodynamic assessment of the morphology and severity of the lesion, and indications for intervention. Treatment options and the evidence surrounding each are also explored. Ultimately, the optimal treatment strategy and timing of intervention are likely to depend on local expertise and clinical condition at presentation.
Late presentation of the Fontan circulation
Authors: Bellsham-Revell Hannah , Bell Aaron , Head Catherine
Published: August 2020

A 16-year-old boy presented to the congenital heart disease department with oedema and chronic cough productive of casts. He had previously undergone Fontan completion for complex congenital heart disease overseas. Examination and basic investigations showed relapsed protein-losing enteropathy and plastic bronchitis, two significant complications of the Fontan circulation. He was extensively investigated, and his medical therapy optimized. Although initially referred for heart transplant assessment, he has been taken off the transplant list after good response to medical therapy alone. This case shows the complex multidisciplinary management of the adolescent Fontan patient with life-threatening complications.