Current Transition Practice for Primary Immunodeficiencies and Autoinflammatory Diseases in Europe: a RITA-ERN Survey.
Por:
Israni M, Nicholson B, Mahlaoui N, Obici L, Rossi-Semerano L, Lachmann H, Hayward G, Avramovic MZ, Guffroy A, Dalm V, Rimmer R, Solis L, Villar C, Gennery AR, Skeffington S, Nordin J, Warnatz K, Korganow AS, Anton-Lopez J, Cattalini M, Amin T, Berg S, Soler-Palacin P, Burns SO and Campbell M
Publicada:
1 ene 2023
Ahead of Print:
12 oct 2022
Resumen:
BACKGROUND: Due to the absence of curative treatments for inborn errors of immunity (IEI), children born with IEI require long-term follow-up for disease manifestations and related complications that occur over the lifespan. Effective transition from pediatric to adult services is known to significantly improve adherence to treatment and long-term outcomes. It is currently not known what transition services are available for young people with IEI in Europe. OBJECTIVE: To understand the prevalence and practice of transition services in Europe for young people with IEI, encompassing both primary immunodeficiencies (PID) and systemic autoinflammatory disorders (AID). METHODS: A survey was generated by the European Reference Network on immunodeficiency, autoinflammatory, and autoimmune diseases Transition Working Group and electronically circulated, through professional networks, to pediatric centers across Europe looking after children with IEI. RESULTS: Seventy-six responses were received from 52 centers, in 45 cities across 17 different countries. All services transitioned patients to adult services, mainly to specialist PID or AID centers, typically transferring up to ten patients to adult care each year. The transition process started at a median age of 16-18 years with transfer to the adult center occurring at a median age of 18-20 years. 75% of PID and 68% of AID centers held at least one joint appointment with pediatric and adult services prior to the transfer of care. Approximately 75% of PID and AID services reported having a defined transition process, but few centers reported national disease-specific transition guidelines to refer to. CONCLUSIONS: Transition services for children with IEI in Europe are available in many countries but lack standardized guidelines to promote best practice.
Filiaciones:
Israni M:
Department of Immunology, Royal Free London NHS Foundation Trust, London, UK
Nicholson B:
Department of Immunology, Royal Free London NHS Foundation Trust, London, UK
Mahlaoui N:
Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
Obici L:
Fondazione IRCCS Policlinico San Matteo, Centro Per Lo Studio E La Cura Delle Amiloidosi Sistemiche, Pavia, Italy
Rossi-Semerano L:
Department of Pediatric Rheumatology, National Reference Centre for Auto-Inflammatory Diseases and Amyloidosis of Inflammatory Origin (CEREMAIA), Bicêtre hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Le Kremlin Bicêtre, France
Lachmann H:
Division of Medicine, National Amyloidosis Centre, University College London, London, UK
Hayward G:
Paediatric and Adult Rheumatology, Leeds General Infirmary and Chapel Allerton Hospital, Leeds, UK
Avramovic MZ:
Department for Allergology, Rheumatology and Clinical Immunology, University Children's Hospital Ljubljana, Ljubljana, Slovenia
Guffroy A:
Department of Clinical Immunology and Internal Medicine, Tertiary Center for Primary Immunodeficiency, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Hôpitaux Universitaires de Strasbourg, 67000, Strasbourg, France
Université de Strasbourg, INSERM UMR - S1109, 67000, Strasbourg, France
Dalm V:
Department of Internal Medicine, Division of Clinical Immunology, Erasmus University Medical Center, Rotterdam, the Netherlands
Department of Immunology, Erasmus University Medical Center, Rotterdam, the Netherlands
Rimmer R:
Rare Autoinflammatory Conditions Community - UK (RACC - UK), Oxford, UK
Solis L:
International Patient Organisation for Primary Immunodeficiencies (IPOPI), Brussels, Belgium
Villar C:
Barcelona PID Foundation, Barcelona, Spain
Gennery AR:
Paediatric Haematopoietic Stem Cell Transplant Unit, Great North Children's Hospital (GNCH), Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK
Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, NE2 4HH, UK
Skeffington S:
Irish Vasculitis Organisation, Dublin, Ireland
Nordin J:
International Patient Organisation for Primary Immunodeficiencies (IPOPI), Brussels, Belgium
Warnatz K:
Center for Chronic Immunodeficiency, Medical Center, Faculty of Medicine, University of Freiburg, University of Freiburg, Freiburg, Germany
Department of Rheumatology and Clinical Immunology, Division of Immunodeficiency, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany
Korganow AS:
Department of Clinical Immunology and Internal Medicine, Tertiary Center for Primary Immunodeficiency, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Hôpitaux Universitaires de Strasbourg, 67000, Strasbourg, France
Université de Strasbourg, INSERM UMR - S1109, 67000, Strasbourg, France
Anton-Lopez J:
Department of Pediatric Rheumatology, Pediatric Immune Dysfunction Disease Study Group (GEMDIP), Institut de Recerca Sant Joan de Déu, Sant Joan de Déu Hospital, Barcelona, Spain
Cattalini M:
Pediatrics Clinic, University of Brescia, ASST Spedali Civili Di Brescia, Brescia, Italy
Amin T:
Department of Paediatric Rheumatology, Leeds Children's Hospital, Leeds Teaching Hospitals Trust, Leeds, UK
Berg S:
Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
Department of Pediatrics, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden
Soler-Palacin P:
Pediatric Infectious Diseases and Immunodeficiencies Unit, Hospital Universitari Vall d'Hebron, Universitat Autonoma de Barcelona, Bellaterra, Spain
Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Barcelona, Catalonia, Spain
Burns SO:
Department of Immunology, Royal Free London NHS Foundation Trust, London, UK.
University College London Institute of Immunity and Transplantation, London, UK.
hybrid, Green Published
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