title: Anti-infective prophylaxis for primary immunodeficiencies: what is done in Italian Primary Immunodeficiency Network centers (IPINet) and review of the literature.
abstract: Primary immunodeficiencies (PIDs) are rare diseases characterized by an increased susceptibility to infections. Early diagnosis and appropriate treatment are critical for reducing morbidity and mortality. Based on available data, the efficacy of antibiotic administration for the prophylaxis of infections remains uncertain, and recommendations supporting this practice are poor. The use of antimicrobial prophylaxis is mainly based on single institution-specific experience without controlled measurements of patient safety and quality health outcomes. To address this issue an Italian Network on Primary Immunodeficiencies (IPINet) has been set up in 1999 within the Italian Association of Pediatric Hematology and Oncology (AIEOP) to increase the awareness of these disorders among physicians. Further, diagnostic and treatment guideline recommendations have been established to standardize the best clinical assistance to all patients, including antibiotic prophylaxis, and for a national epidemiologic monitoring of PIDs. The aim of this review is not only to give a scientific update on the use of antimicrobial prophylaxis in selected congenital immunological disorders but also to draw a picture of this practice in the context of the Italian Primary Immunodeficiency Network (IPINet). Controlled multicenter studies are necessary to establish if, when and how you should start an efficacious antimicrobial prophylaxis.
authors.affiliation: Department of Pediatrics - Policlinico Tor Vergata, University of Rome Tor Vergata, Rome, Italy.
authors.forename: V
authors.initials: V
authors.lastname: Moschese
authors.affiliation: Department of Biomedicine and Evolutive Age, University of Bari, Bari, Italy.
authors.forename: B
authors.initials: B
authors.lastname: Martire
authors.affiliation: Department of Pediatrics, University of Brescia, Brescia, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Soresina
authors.affiliation: Department of Pediatrics - Policlinico Tor Vergata, University of Rome Tor Vergata, Rome, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Chini
authors.affiliation: Department of Pediatrics - Policlinico Tor Vergata, University of Rome Tor Vergata, Rome, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Graziani
authors.affiliation: Department of Pediatrics - Policlinico Tor Vergata, University of Rome Tor Vergata, Rome, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Monteferrario
authors.affiliation: San Raffaele Telethon Institute for Gene Therapy (HSR-TIGET), Division of Regenerative Medicine, Stem Cell and Gene Therapy, San Raffaele Scientific Institute, Milan, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Bacchetta
authors.affiliation: Department of Pediatrics, Bambino Gesu' Children Hospital-University of Rome Tor Vergata, Rome, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Cancrini
authors.affiliation: Department of Clinical Immunology, Sapienza University of Rome, Rome, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Fiorilli
authors.affiliation: Department of Sciences for Woman and Childs Health, Anna Meyer Childrens Hospital, University of Florence, Florence, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Gambineri
authors.affiliation: Paediatric Oncology and Hematology Unit Lalla Seragnoli, University of Bologna, Bologna, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Pession
authors.affiliation: Department of Translational Medicine, Federico II University, Naples, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Pignata
authors.affiliation: Department of Clinical Immunology, Sapienza University of Rome, Rome, Italy.
authors.forename: I
authors.initials: I
authors.lastname: Quinti
authors.affiliation: Paediatric Oncology and Hematology Unit Lalla Seragnoli, University of Bologna, Bologna, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Rondelli
authors.affiliation: Department of Pediatrics, Bambino Gesu' Children Hospital-University of Rome Tor Vergata, Rome, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Rossi
authors.affiliation: Department of Pediatrics, Bambino Gesu' Children Hospital, Rome, Italy.
authors.forename: A G
authors.initials: AG
authors.lastname: Ugazio
authors.affiliation: Department of Pediatrics, University of Brescia, Brescia, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Plebani
authors.affiliation: IRCSS Foundation, University of Milan, Milan, Italy.
authors.forename: M C
authors.initials: MC
authors.lastname: Pietrogrande
mesh_terms: D019072:Antibiotic Prophylaxis; D017074:Common Variable Immunodeficiency / Q000150:complications; D004062:DiGeorge Syndrome / Q000150:complications; D006105:Granulomatous Disease, Chronic / Q000150:complications; D006801:Humans; D017098:IgA Deficiency / Q000150:complications; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D053632:X-Linked Combined Immunodeficiency Diseases / Q000150:complications