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Sickle Cell Anemia by Fernando Ferreira Costa,Nicola Conran Pdf
Although sickle cell anemia was the first molecular disease to be identified, its complex and fascinating pathophysiology is still not fully understood. A single mutation in the beta-globin gene incurs numerous molecular and cellular mechanisms that contribute to the plethora of symptoms associated with the disease. Our knowledge regarding sickle cell disease mechanisms, while still not complete, has broadened considerably over the last decades. Sickle Cell Anemia: From Basic Science to Clinical Practice aims to provide an update on our current understanding of the disease’s pathophysiology and use this information as a basis to discuss its manifestations in childhood and adulthood. Current therapies and prospects for the development of new approaches for the management of the disease are also covered.
Sickle Cell Pain is a panoramic, in-depth exploration of every scientific, human, and social dimension of this cruel disease. This comprehensive, definitive work is unique in that it is the only book devoted to sickle cell pain, as opposed to general aspects of the disease. The 752-page book links sickle cell pain to basic, clinical, and translational research, addressing various aspects of sickle pain from molecular biology to the psychosocial aspects of the disease. Supplemented with patient narratives, case studies, and visual art, Sickle Cell Pain’s scientific rigor extends through its discussion of analgesic pharmacology, including abuse-deterrent formulations. The book also addresses in great detail inequities in access to care, stereotyping and stigmatization of patients, the implications of rapidly evolving models of care, and recent legislation and litigation and their consequences.
The EBMT Handbook by Nicolaus Kröger,Mohamad Mohty,Carlo Dufour Pdf
This Open Access edition of the European Society for Blood and Marrow Transplantation (EBMT) handbook addresses the latest developments and innovations in hematopoietic stem cell transplantation and cellular therapy. Consisting of 93 chapters, it has been written by 175 leading experts in the field. Discussing all types of stem cell and bone marrow transplantation, including haplo-identical stem cell and cord blood transplantation, it also covers the indications for transplantation, the management of early and late complications as well as the new and rapidly evolving field of cellular therapies. This book provides an unparalleled description of current practices to enhance readers' knowledge and practice skills. This work was published by Saint Philip Street Press pursuant to a Creative Commons license permitting commercial use. All rights not granted by the work's license are retained by the author or authors.
Understanding Sickle Cell Disease by Miriam Bloom Pdf
Although more is known about sickle cell disease than about any other inherited disease, no cure for it exists. In America alone, about one in 375 who are of African ancestry is born with sickle cell disease. A smaller number of Americans descended from families from the Mediterranean area, the Middle East, and India also are affected. In addition, about eight percent of black Americans who do not suffer from the disease itself carry the gene for it that can be transmitted to their children. Sickle cell disease is of enormous biological, social, and historic importance. It was first described in medical literature almost a century ago. Improvements during the past two decades in our understanding of the disease and in medical care are permitting those afflicted to live longer, more comfortable and more productive lives. This book was written for all who are interested in this disease--those who have it, their families, the carriers of the sickle cell gene, teachers, and those who wish to update their information about it. This overview of sickle cell disease explains what it is and how it is inherited, as well as the relationship between the sickle cell gene and its geographic origins, the way the gene has been spread throughout history, and the effect of sickle cell hemoglobin on red blood cells that carry it. Understanding Sickle Cell Disease describes the variety of symptoms in both children and adults and details the emotional aspects of the disease. Of particular interest is a chapter on the care, especially the home care, of those who are affected. This book explains how it is possible today for couples carrying the genes to raise families free of the disease. Although there is no known cure for sickle cell disease, there is little doubt that one will ultimately be devised. This volume surveys current research efforts and the promise they hold.
Within the last few years, iron research has yielded exciting new insights into the under standing of normal iron homeostasis. However, normal iron physiology offers little protec tion from the toxic effects of pathological iron accumulation, because nature did not equip us with effective mechanisms of iron excretion. Excess iron may be effectively removed by phlebotomy in hereditary hemochromatosis, but this method cannot be applied to chronic anemias associated with iron overload. In these diseases, iron chelating therapy is the only method available for preventing early death caused mainly by myocardial and hepatic iron toxicity. Iron chelating therapy has changed the quality of life and life expectancy of thalassemic patients. However, the high cost and rigorous requirements of deferoxamine therapy, and the significant toxicity of deferiprone underline the need for the continued development of new and improved orally effective iron chelators. Such development, and the evolution of improved strategies of iron chelating therapy require better understanding of the pathophysiology of iron toxicity and the mechanism of action of iron chelating drugs. The timeliness of the present volume is underlined by several significant develop ments in recent years. New insights have been gained into the molecular basis of aberrant iron handling in hereditary disorders and the pathophysiology of iron overload (Chapters 1-5).
Sickle Cell Disease in Clinical Practice by Jo Howard,Paul Telfer Pdf
Sickle Cell Disease is the most common genetic disease world wide and in the UK. It has marked geographical variation in its distribution in the UK, with a concentration in London and other major conurbations (Birmingham and Manchester). In these areas, specialist centres have become established offering expert, up to date care for both inpatients and out patients with Sickle Cell Disease. Although patient numbers are increasing outside these areas, the expertise of health professionals can be patchy. This book aims to provide a user friendly, accessible resource for areas with smaller numbers of patients, to allow them to provide equitable care with the larger well established centres. Sickle Cell Disease can be associated with acute life threatening complications, when clear, easily available advice is needed, and with chronic long term complications which may need liaison with other health professionals. Clear treatment protocols for all the common complications of sickle cell disease, are outlined here, with summaries of key evidence and references.
Author : Mark T. Gladwin,Gregory Kato,Enrico M. Novelli Publisher : McGraw Hill Professional Page : 715 pages File Size : 52,8 Mb Release : 2021-01-05 Category : Medical ISBN : 9781260458602
Sickle Cell Disease by Mark T. Gladwin,Gregory Kato,Enrico M. Novelli Pdf
The most comprehensive, current sickle cell disease resource—for both clinicians and researchers A Doody's Core Title for 2023! The first and only resource of its kind, Sickle Cell Disease examines this blood disorder through both clinical and research lenses. More than 80 dedicated experts in the field present their combined clinical knowledge of basic mechanisms, screening, diagnosis, management, and treatment of myriad complex complications of a single base point mutation in the human genome. Case studies with “How I Treat” authoritative insights provide overviews of common and rare complications, and Key Facts offer at-a-glance high-yield information. Filled with clinical photos, illustrations, numerous original diagrams, and with free updates available online, this unmatched resource covers: Mechanisms of sickle cell disease Historic and current research approaches The latest work in gene therapy and editing Guidelines for patient care, diagnosis, unique cases, and therapies Rare and common complications, including domestic and internationally relevant topics Psychosocial and supportive care The newest standards of therapy and future treatment options in children and adults Cardiopulmonary complications
M D George R Buchanan,M D M P H Araba N Afenyi-Annan,M D Samir K Ballas
Author : M D George R Buchanan,M D M P H Araba N Afenyi-Annan,M D Samir K Ballas Publisher : Createspace Independent Publishing Platform Page : 0 pages File Size : 53,7 Mb Release : 2014-09-09 Category : Sickle cell anemia ISBN : 1502452782
Evidence-Based Management of Sickle Cell Disease by M D George R Buchanan,M D M P H Araba N Afenyi-Annan,M D Samir K Ballas Pdf
Sickle cell disease can be severe and disabling. When properly treated, patients live longer and with better quality life. This is a US government publication intended to provide evidence-based guidelines for the care of these patients for the use of all concerned providers as well as patients and family members. This book is available in print here for convenience.
Although it strikes individuals from a variety of backgrounds, sickle cell anemia has always been known as a "black" disease in America. In the Blood argues that ever since the discovery in 1910 and subsequent scientific analysis of the disease, sickle cell anemia has been manipulated to serve social ends-as a tool for securing white identity and a way to establish a hierarchy based on European heritage. Tapper shows how sickle cell anemia was used to promote the superiority of racial purity, to characterize the black body as contaminated, and even to support the notion that modern humans evolved from multiple origins.
Sickle cell disease (SCD), an inherited hemolytic anemia, is associated with multiple acute and chronic complications such as painful vasoocclusive events, cerebral vasculopathy, priapism, and renal or lung disease. These complications are variable and unpredictable, and can be associated with significant morbidity and poor quality of life. This book covers several areas regarding pathology, diagnosis, complications, signs, symptoms and medical treatments. There are few studies in literature on the role of physiotherapy as a resource to prevent and treat locomotor system disorders, respiratory problems and painful crises in SCD individuals. This book highlights the role of physiotherapy in sickle cell anemia. A comprehensive and authoritative monograph, this book will be equally interesting to both established researchers and to graduate students interested in both genetics and the physical therapy field.
Sickle cell anemia is a genetic disease of the blood. It is caused by a defect in one gene of a person. Genes are the elements in cells that carry the information that determines traits, such as hair or eye color. In sickle cell anemia, a defect in the gene controls how hemoglobin is made. This defect can be passed from parents to their children. Students explore the history of sickle cell anemia, the pioneering doctors who studied its cause, and early treatments. They also investigate hemoglobin S, who gets sickle cell, and how the gene mutation is inherited. They learn about the different types of sickle cell disease and treatments, including blood transfusions and bone marrow transplants, and some of the promising new research in medicines and gene therapy.
Modeling the Heart and the Circulatory System by Alfio Quarteroni Pdf
The book comprises contributions by some of the most respected scientists in the field of mathematical modeling and numerical simulation of the human cardiocirculatory system. The contributions cover a wide range of topics, from the preprocessing of clinical data to the development of mathematical equations, their numerical solution, and both in-vivo and in-vitro validation. They discuss the flow in the systemic arterial tree and the complex electro-fluid-mechanical coupling in the human heart. Many examples of patient-specific simulations are presented. This book is addressed to all scientists interested in the mathematical modeling and numerical simulation of the human cardiocirculatory system.
The Management of Sickle Cell Disease by U. S. Department of Health Pdf
#1 Best Seller on Sickle Cell Disease (SCD). Sickle cell disease is a group of blood disorders passed down from parents to children. Sickle cell anemia shortens life expectancy by 30 years via bacterial infections, painful swellings, fever, arthritis, leg ulcers, eye, lung & heart damage. Over 100,000 people, mostly African-Americans, in the United States have sickle cell disease. Over 2 million people have sickle cell trait in America. It is estimated that more than 300,000 children are born each year with SCD around the world. This edition of The Management of Sickle Cell Disease (SCD) is organized into four parts: 1. Diagnosis and Counseling 2. Health Maintenance 3. Treatment of Acute and Chronic Complications 4. Special Topics. The original intent was to incorporate evidence-based medicine into each chapter, but there was variation among evidence-level scales, and some authors felt recommendations could be made, based on accepted practice, without formal trials in this rare disorder. The best evidence still is represented by randomized, controlled trials (RCTs), but variations exist in their design, conduct, endpoints, and analyses. It should be emphasized that selected people enter a trial, and results should apply in practice specifically to populations with the same characteristics as those in the trial. Randomization is used to reduce imbalances between groups, but unexpected factors sometimes may confound analysis or interpretation. In addition, a trial may last only a short period of time, but long-term clinical implications may exist. Another issue is treatment variation, for example, a new pneumococcal vaccine developed after the trial, which has not been tested formally in a sickle cell population. Earlier trial results may be accepted, based on the assumption that the change is small. In some cases, RCTs cannot be done satisfactorily (e.g., for ethical reasons, an insufficient number of patients, or a lack of objective measures for sickle cell "crises"). Thus the bulk of clinical experience in SCD still remains in the moderately strong and weaker categories of evidence. Not everyone has an efficacious outcome in a clinical trial, and the frequency of adverse events, such as with long-term transfusion programs or hematopoietic transplants, might not be considered. Thus, an assessment of benefit-to-risk ratio should enter into translation of evidence levels into practice recommendations. A final issue is that there may be two alternative approaches that are competitive (e.g., transfusions and hydroxyurea). In this case the pros and cons of each course of treatment should be discussed with the patient. This book is B&W copy of the government agency publication.
Explores the scientific discoveries that led to an understanding of the genetic basis of the disease, explaining how a single mutation in hemoglobin multiplies into the many symptoms of sickle cell disease. This book describes treatments that help an individual manage sickle cell disease symptoms.