Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood |
| |
| Authors: | Nizar Mahlaoui Alison Jones Sarita Workman Andrew Cant |
| |
| Affiliation: | 1.French National Reference Center for Primary Immune Deficiencies (CEREDIH),Necker Enfants Malades University Hospital,Paris,France;2.Pediatric Immuno-Haematology and Rheumatology Unit,Necker Enfants Malades University Hospital,Paris,France;3.INSERM UMR 1163, Laboratory of Human Genetics of Infectious Diseases,Paris,France;4.Sorbonne Paris Cité, Imagine Institute,Paris Descartes University,Paris,France;5.Center for Chronic Immunodeficiency (CCI), Medical Center—University of Freiburg, Faculty of Medicine,University of Freiburg,Freiburg,Germany;6.Immunology Department,Great Ormond Street Hospital for Children NHS Foundation Trust,London,UK;7.Department of Immunology,Royal Free London NHS Foundation Trust,London,UK;8.Great North Children’s Hospital, & Institute for Cellular Medicine University of Newcastle,Newcastle upon Tyne,UK |
| |
| Abstract: | Primary immunodeficiencies (PIDs) are a widely heterogeneous group of inherited defects of the immune system consisting of many clinical phenotypes with at least 300 underlying genetic deficits currently known. Patients with PIDs can present with, or develop during the course of their life, a susceptibility to recurrent and chronic infection along with autoimmune, allergic, inflammatory, and/or proliferative disorders, all potentially leading to end-organ damage. In recent years, a combination of basic and clinical research has greatly improved understanding of the underlying immunological and genetic defects in PIDs, leading to improved diagnosis, classification, and treatment approaches. In this review, we consider some of the key understandings that should direct diagnostic and treatment approaches in PID and offer insights into current and emerging management approaches and the lifelong care of patients from childhood through to adulthood. |
| |
| Keywords: | |
| 本文献已被 SpringerLink 等数据库收录！ |
|
