Consensus of the Italian Primary Immunodeficiency Network on transition management from pediatric to adult care in patients affected with childhood-onset inborn errors of immunity

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

卷:146

Article

Cirillo, Emilia¹  Giardino, Giuliana¹  Ricci, Silvia^2,3  Moschese, Viviana⁴  Lougaris, Vassilios⁵  Conti, Francesca⁶  Azzari, Chiara^2,3  Barzaghi, Federica⁷  Canessa, Clementina^2,3  Martire, Baldassarre⁸  Badolato, Raffaele⁵  Dotta, Laura⁵  Soresina, Annarosa⁵  Cancrini, Caterina^9,10  Finocchi, Andrea^9,10  Montin, Davide¹¹  Romano, Roberta¹  Amodio, Donato^9,10  Ferrua, Francesca⁷  Tommasini, Alberto^12,13  Baselli, Lucia Augusta¹⁴  Dellepiane, Rosa Maria¹⁴  Polizzi, Agata¹⁵  Chessa, Luciana¹⁶  Marzollo, Antonio¹⁷  Cicalese, Maria Pia⁷  Putti, Maria Caterina¹⁷  Pession, Andrea⁶  Aiuti, Alessandro⁷  Locatelli, Franco¹⁸  Plebani, Alessandro⁵  Pignata, Claudio¹ 

[1] Univ Naples Federico II, Dept Translat Med Sci, Pediat Sect, Via S Pansini 5, I-80131 Naples, Italy

[2] Univ Florence, Dept Hlth Sci, Div Pediat Immunol, Florence, Italy

[3] Meyer Childrens Hosp, Florence, Italy

[4] Univ Roma Tor Vergata, Pediat Immunopathol & Allergol Unit, Rome, Italy

[5] Univ Brescia & Dept Pediat, Dept Clin & Expt Sci, ASST Spedali Civili Brescia, Brescia, Italy

[6] Univ Bologna, Unit Pediat, St Orsola Univ Hosp, Bologna, Italy

[7] IRCCS San Raffaele Sci Inst, San Raffaele Telethon Inst Gene Therapy & Pediat, Milan, Italy

[8] Mons AR Dimiccoli Hosp, Maternal Infant Dept, Unit Pediat & Neonatol, Barletta, Italy

[9] Bambino Gesu Pediat Hosp, Acad Dept Pediat, Unit Immunol & Infect Dis, Rome, Italy

[10] Univ Roma Tor Vergata, Dept Syst Med, Rome, Italy

[11] Univ Turin, Regina Margherita Children Hosp, Dept Publ Hlth & Pediat, Div Pediat Immunol & Rheumatol, Turin, Italy

[12] IRCCS Burlo Garofolo, Inst Maternal & Child Hlth, Dept Pediat, Trieste, Italy

[13] Univ Trieste, Dept Med Surg & Hlth Sci, Trieste, Italy

[14] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pediat, Milan, Italy

[15] Univ Catania, Dept Educ Sci, Catania, Italy

[16] Sapienza Univ Rome, Dept Clin & Mol Med, Rome, Italy

[17] Univ Padua, Dept Womens & Childrens Hlth, Pediat Hematol Oncol Unit, Padua, Italy

[18] Univ Rome, IRCCS Bambino Gesu Childrens Hosp, Dept Pediat Hematol & Oncol, Rome, Italy

关键词: Transitional care;    inborn errors of immunity;    primary immunodeficiency;    humoral immune defects;    DiGeorge syndrome;    combined immunodeficiency;    innate immune defects;    DNA repair syndromes;    Italian Network of Primary Immunodeficiencies;   

DOI  :  10.1016/j.jaci.2020.08.010

来源: Elsevier

【 摘 要 】

Medical advances have dramatically improved the long-term prognosis of children and adolescents with inborn errors of immunity (IEIs). Transfer of the medical care of individuals with pediatric IEIs to adult facilities is also a complex task because of the large number of distinct disorders, which requires involvement of patients and both pediatric and adult care providers. To date, there is no consensus on the optimal pathway of the transitional care process and no specific data are available in the literature regarding patients with IEIs. We aimed to develop a consensus statement on the transition process to adult health care services for patients with IEIs. Physicians from major Italian Primary Immunodeficiency Network centers formulated and answered questions after examining the currently published literature on the transition from childhood to adulthood. The authors voted on each recommendation. The most frequent IEIs sharing common main clinical problems requiring full attention during the transitional phase were categorized into different groups of clinically related disorders. For each group of clinically related disorders, physicians from major Italian Primary Immunodeficiency Network institutions focused on selected clinical issues representing the clinical hallmark during early adulthood.

【 授权许可】

Free