Journal article
Primary immune deficiencies - principles of care
- Abstract:
- Primary immune deficiencies (PIDs) are a growing group of over 230 different disorders caused by ineffective, absent or an increasing number of gain of function mutations in immune components, mainly cells and proteins. Once recognized, these rare disorders are treatable and in some cases curable. Otherwise untreated PIDs are often chronic, serious, or even fatal. The diagnosis of PIDs can be difficult due to lack of awareness or facilities for diagnosis, and management of PIDs is complex. This document was prepared by a worldwide multi-disciplinary team of specialists; it aims to set out comprehensive principles of care for PIDs. These include the role of specialized centers, the importance of registries, the need for multinational research, the role of patient organizations, management and treatment options, the requirement for sustained access to all treatments including immunoglobulin therapies and hematopoietic stem cell transplantation, important considerations for developing countries and suggestions for implementation. A range of healthcare policies and services have to be put into place by government agencies and healthcare providers, to ensure that PID patients worldwide have access to appropriate and sustainable medical and support services.
- Publication status:
- Published
- Peer review status:
- Peer reviewed
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(Preview, Version of record, pdf, 493.0KB, Terms of use)
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- Publisher copy:
- 10.3389/fimmu.2014.00627
Authors
- Publisher:
- Frontiers Media
- Journal:
- Frontiers in Immunology More from this journal
- Volume:
- 5
- Pages:
- 627
- Publication date:
- 2014-12-15
- DOI:
- EISSN:
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1664-3224
- ISSN:
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1664-3224
- Language:
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English
- Keywords:
- UUID:
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uuid:7eb6ba5a-1961-4c53-9d07-55e4daecf946
- Local pid:
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pubs:502625
- Source identifiers:
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502625
- Deposit date:
-
2015-01-16
Terms of use
- Copyright holder:
- Chapel, et al
- Copyright date:
- 2014
- Notes:
- Copyright: © 2014 Chapel, Prevot, Gaspar, Español, Bonilla, Solis, Drabwell and The Editorial Board for Working Party on Principles of Care at IPOPI. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
- Licence:
- CC Attribution (CC BY)
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