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Immune Responses In Patients With Lysosomal Storage Disorders Treated With Enzyme Replacement Therapy And Haemopoietic Stem Cell Transplantation Book in PDF, ePub and Kindle version is available to download in english. Read online anytime anywhere directly from your device. Click on the download button below to get a free pdf file of Immune Responses In Patients With Lysosomal Storage Disorders Treated With Enzyme Replacement Therapy And Haemopoietic Stem Cell Transplantation book. This book definitely worth reading, it is an incredibly well-written.
Author : Muhammad A. Saif
Publisher :
Page : pages
File Size : 32,76 MB
Release : 2013
Category :
ISBN :
Author : Michelle Kenyon
Publisher : Springer
Page : 318 pages
File Size : 28,4 MB
Release : 2018-03-14
Category : Medical
ISBN : 3319500260
This book is open access under a CC BY 4.0 license. This textbook, endorsed by the European Society for Blood and Marrow Transplantation (EBMT), provides adult and paediatric nurses with a full and informative guide covering all aspects of transplant nursing, from basic principles to advanced concepts. It takes the reader on a journey through the history of transplant nursing, including essential and progressive elements to help nurses improve their knowledge and benefit the patient experience, as well as a comprehensive introduction to research and auditing methods. This new volume specifically intended for nurses, complements the ESH-EBMT reference title, a popular educational resource originally developed in 2003 for physicians to accompany an annual training course also serving as an educational tool in its own right. This title is designed to develop the knowledge of nurses in transplantation. It is the first book of its kind specifically targeted at nurses in this specialist field and acknowledges the valuable contribution that nursing makes in this area. This volume presents information that is essential for the education of nurses new to transplantation, while also offering a valuable resource for more experienced nurses who wish to update their knowledge.
Author : Pedro A. de Alarcón
Publisher : Cambridge University Press
Page : 501 pages
File Size : 28,98 MB
Release : 2021-02-18
Category : Medical
ISBN : 1108488986
An essential guide to the pathogenesis, diagnosis and management of hematologic problems in the neonate, covering erythrocyte disorders, leukocyte disorders, immunologic disorders and hemostatic disorders. Guidance is practical, including blood test interpretation, advice on transfusions and reference ranges for hematological values.
Author :
Publisher :
Page : 0 pages
File Size : 39,94 MB
Release : 2013
Category :
ISBN :
Author : John A. Barranger
Publisher : Springer Science & Business Media
Page : 563 pages
File Size : 41,92 MB
Release : 2007-10-16
Category : Science
ISBN : 0387709096
The knowledge of lysosomal biology and the consequences of its dysfunction have increased dramatically in the past 60 years. This book describes the nature of the lysosomal dysfunction and diseases as well as potential future treatments and therapies. Disease specific chapters provide thorough reviews of the clinical features of lysosomal storage disorders, their molecular basis and the commercial or experimental therapeutic approaches sought in this area. This is an invaluable resource for researchers in biochemical and molecular genetics, enzyme therapy, and gene transfer.
Author : Juan M. Pascual
Publisher : Cambridge University Press
Page : 507 pages
File Size : 13,73 MB
Release : 2017-04-20
Category : Medical
ISBN : 1107042054
A review of childhood neurodegenerative and other progressive but non-degenerative disorders to guide their diagnosis and management.
Author : Aubrey Milunsky
Publisher : John Wiley & Sons
Page : 1392 pages
File Size : 37,9 MB
Release : 2021-03-30
Category : Science
ISBN : 1119676975
"The time is fast approaching when virtually all the culprit genes and their mutations for 7,000 rare monogenic disorders1 will be known. Thus far causal single genes and their mutations have been determined for 5,6732 genetic disorders, enabling pre-implantation genetic testing or prenatal genetic diagnosis. These advances using chromosomal microarrays, whole exome sequencing and even whole genome sequencing together with fetal imaging, and non-invasive prenatal testing, expand the era in which all couples have the option of avoiding or preventing having children with irreversible, irremediable, crippling, or lethal monogenic disorders. Primary care physicians, and those in all medical specialties, will need to inform their patients of this key option. This imperative is already partly in current practice. Missing is the requirement of physicians to request and obtain the precise name of the genetic disorder in question or an existing DNA report on a family member, for prospective parents to benefit from available options"--
Author : Seung Hyun Choi
Publisher :
Page : 0 pages
File Size : 39,6 MB
Release : 2022
Category : Immunologic diseases
ISBN :
Primary immune disorders (PIDs) are rare genetic disorders that impair the immune system. There are two types of PIDs: primary immunodeficiency (PIDD), which is characterized by absent or poor function of the immune system, and primary immune regulatory disorders (PIRDs), which present with immunemediated pathology. Allogeneic hematopoietic stem cell transplant (HSCT) is the only curative option for the majority of PIDD and PIRD. Overall survival of PIDD patients has improved significantly over the years with earlier diagnosis and advances in HSCT, but PIRD patients continue to have inferior transplant outcomes with lower survival, higher incidence of graft versus host disease (GvHD), and autoimmune complications. To understand the differences in transplant outcomes between PIDD and PIRD and to identify factors that contribute to worse transplant outcomes in PIRD, we performed a singlecenter retrospective analysis of transplant outcomes in 109 PIDD patients and 21 PIRD patients at Boston Children's Hospital. We found that PIRD patients were more likely to be a lot older, have autoimmunity, and receive reduced intensity conditioning (RIC) than PIDD patients at the time of transplant. After transplant, PIRD patients had much lower survival, higher incidence of autoimmunity, greater dependence on IVIG/IgRT, and had poorer donor chimerism and immune reconstitution than PIDD patients. We found statistically significant relationships between pretransplant conditions and transplant outcomes. Having pretransplant autoimmunity and receiving RIC regimen were associated with lower survival, occurrence of posttransplant autoimmunity, and slower immune reconstitution, and in PIDD, older age was associated with delayed immune reconstitution. Together, our results suggest that PIRD patients have worse transplant outcomes than PIDD due to older age, RIC regimen, and autoimmune manifestations. Our study emphasizes the need to consider the three important pretransplant factors in improving transplant outcomes in PIRD patients.
Author : YP Munjal
Publisher : JP Medical Ltd
Page : 1450 pages
File Size : 30,49 MB
Release : 2015-08-30
Category : Medical
ISBN : 9351524159
This is the tenth edition of the authoritative API Textbook of Medicine, completely revised, updated and expanded, with 28 brand new chapters. The textbook is comprised of two volumes, divided into 29 sections. Beginning with an introduction to the practice of medicine, and a disease profile and epidemiology of communicable and non-communicable diseases, each subsequent section covers a separate medical specialty. The second section on ‘Clinical Approach to Key Manifestation’ has been expanded with six new chapters, including the appropriate selection of imaging modalities. Other new topics in this edition include advanced cardiac life support system, life-style changes in the management of diabetes, diabetes in the elderly, prevention of cardiovascular disease, acute and chronic pancreatitis, and tumours of the liver. Chapters on chronic and sleep-related pulmonary disorders have been completely re-written to highlight their increased prevalence, and a new chapter on pulmonary rehabilitation has been added. An entirely new section on the ‘Future of Medicine’ including regenerative medicine, nanotechnology and nanomedicine, robotic surgery, and an introduction to ‘space medicine’, brings the API Textbook of Medicine to its conclusion. With 1090 full colour images and illustrations, spanning over 3000 pages, this all-encompassing textbook is a comprehensive guide to the practice of medicine, brought fully up-to-date for physicians, surgeons and post-graduate medical students. Key Points New edition of this comprehensive, two volume textbook Fully revised, updated and expanded with 28 new chapters New section on the future of medicine 1090 full colour images and illustrations Previous edition published 2012
Author : Frederick J. Suchy
Publisher : Cambridge University Press
Page : 875 pages
File Size : 30,45 MB
Release : 2021-03-18
Category : Medical
ISBN : 1108911374
Liver disease in children is increasing in prevalence, placing a huge burden on healthcare systems and often requiring long-term management. Offering an integrative approach to the science and clinical practice of pediatric hepatology, this is the definitive reference text for improved diagnosis and treatment strategies. In the new edition of this authoritative text, chapters have been thoroughly revised in line with major advances in the field, such as recognizing the increased frequency of fatty liver disease, and how genetic testing has the potential to establish earlier diagnoses for a variety of diseases. Disorders covered include cholestasis, metabolic disorders and hepatitis, with their presentation across the spectrum of infancy, childhood and adolescence discussed. The indications and surgical aspects of liver transplant are explained and post-transplant care is described in detail. This is a valuable resource for pediatricians, hepatologists, gastroenterologists and all clinicians involved in the care of children with liver diseases.