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. 2019 Oct;39(7):702-712.
doi: 10.1007/s10875-019-00658-9. Epub 2019 Aug 10.

A 1-Year Prospective French Nationwide Study of Emergency Hospital Admissions in Children and Adults With Primary Immunodeficiency

Hélène Coignard-Biehler  1   2   3 Nizar Mahlaoui  2   3   4   5 Benoit Pilmis  1   2 Vincent Barlogis  6 Pauline Brosselin  2 Nathalie De Vergnes  2 Marianne Debré  4 Marion Malphettes  7 Pierre Frange  4   8   9 Emilie Catherinot  10 Isabelle Pellier  2   11 Isabelle Durieu  12 Antoinette Perlat  13 Bruno Royer  14 Alain Le Quellec  15 Eric Jeziorski  16 Alain Fischer  2   3   4   17 Olivier Lortholary  18   19   20 CEREDIH French PID study groupLaurent AaronDaniel AdoueClaire AguilarNathalie AladjidiAlexandre AlcaisZahir AmouraPhilippe ArletCorinne Armari-AllaBrigitte Bader-MeunierSophie BayartYves BertrandBoris BienvenuStéphane BlancheDamien BodetBernard BonnotteRaphaël BoriePatrick BoutardClaire BriandetJean-Paul BrionJacques BrouardSarah Cohen-BeaussantLaurence CostesLouis-Jean CoudercPierre CougoulVirginie CourteilleGeneviève de Saint BasileCatherine DevoldereAnne DevilleJean DonadieuEric DoreFabienne DulieuChristine EdanNatacha Entz-WerleClaire FieschiAmandine ForestierFanny FouyssacVincent GajdosLionel GalicierVirginie GandemerMartine GardembasCatherine GaudGaelle GuillermEric HachullaMohamed HamidouOlivier HermineCyrille HoarauSébastien HumbertArnaud JaccardSerge JacquotJean-Philippe JaisRoland JaussaudPierre-Yves JeandelKamila KebailiAnne-Sophie KorganowOlivier LambotteFanny LanternierClaire LarrocheAnne-Sophie LascauxEmmanuelle Le MoigneVincent Le MoingYvon LebranchuMarc LecuitGuillaume LefevreRichard LemalValérie Li Thiao TeAude Marie-CardineNicolas Martin SilvaAgathe MasseauChristian MassotFrançoise MazingueEtienne MerlinGérard MichelFrédéric MillotBéatrice MonlibertFabrice MonpouxDespina MoshousLuc MouthonMartine MunzerBénédicte NevenRaphaëlle Nove-JosserandEric OksenhendlerMarie Ouachée-ChardinCaroline OudotAnne PagnierJean-Louis PasqualiMarlène PasquetYves PerelCapucine PicardChristophe PiguetDominique PlantazJohan ProvotPierre QuartierFrédéric Rieux-LaucatPascal RoblotPierre-Marie RogerPierre-Simon RohrlichHervé RubieValéry SalleFrançoise Sarrot-ReynauldAmélie ServettazJean-Louis StephanNicolas SchleinitzFelipe SuarezLaure SwiaderSophie TaqueCaroline ThomasOlivier TournilhacCaroline ThumerelleFrançois TronJean-Pierre VannierJean-François Viallard
Affiliations

A 1-Year Prospective French Nationwide Study of Emergency Hospital Admissions in Children and Adults With Primary Immunodeficiency

Hélène Coignard-Biehler et al. J Clin Immunol. .

Abstract

Purpose: Patients with primary immunodeficiency (PID) are at risk of serious complications. However, data on the incidence and causes of emergency hospital admissions are scarce. The primary objective of the present study was to describe emergency hospital admissions among patients with PID, with a view to identifying "at-risk" patient profiles.

Methods: We performed a prospective observational 12-month multicenter study in France via the CEREDIH network of regional PID reference centers from November 2010 to October 2011. All patients with PIDs requiring emergency hospital admission were included.

Results: A total of 200 admissions concerned 137 patients (73 adults and 64 children, 53% of whom had antibody deficiencies). Thirty admissions were reported for 16 hematopoietic stem cell transplantation recipients. When considering the 170 admissions of non-transplant patients, 149 (85%) were related to acute infections (respiratory tract infections and gastrointestinal tract infections in 72 (36%) and 34 (17%) of cases, respectively). Seventy-seven percent of the admissions occurred during winter or spring (December to May). The in-hospital mortality rate was 8.8% (12 patients); death was related to a severe infection in 11 cases (8%) and Epstein-Barr virus-induced lymphoma in 1 case. Patients with a central venous catheter (n = 19, 13.9%) were significantly more hospitalized for an infection (94.7%) than for a non-infectious reason (5.3%) (p = 0.04).

Conclusion: Our data showed that the annual incidence of emergency hospital admission among patients with PID is 3.4%. The leading cause of emergency hospital admission was an acute infection, and having a central venous catheter was associated with a significantly greater risk of admission for an infectious episode.

Keywords: Primary immunodeficiency; emergency hospital admission; infection; prophylaxis.

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