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J Clin Immunol. 2019 Aug 10. doi: 10.1007/s10875-019-00658-9. [Epub ahead of print]

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

Coignard-Biehler H1,2,3, Mahlaoui N2,3,4,5, Pilmis B1,2, Barlogis V6, Brosselin P2, De Vergnes N2, Debré M4, Malphettes M7, Frange P4,8,9, Catherinot E10, Pellier I2,11, Durieu I12, Perlat A13, Royer B14, Le Quellec A15, Jeziorski E16, Fischer A2,3,4,17, Lortholary O18,19,20; CEREDIH French PID study group, Aaron L, Adoue D, Aguilar C, Aladjidi N, Alcais A, Amoura Z, Arlet P, Armari-Alla C, Bader-Meunier B, Bayart S, Bertrand Y, Bienvenu B, Blanche S, Bodet D, Bonnotte B, Borie R, Boutard P, Briandet C, Brion JP, Brouard J, Cohen-Beaussant S, Costes L, Couderc LJ, Cougoul P, Courteille V, de Saint Basile G, Devoldere C, Deville A, Donadieu J, Dore E, Dulieu F, Edan C, Entz-Werle N, Fieschi C, Forestier A, Fouyssac F, Gajdos V, Galicier L, Gandemer V, Gardembas M, Gaud C, Guillerm G, Hachulla E, Hamidou M, Hermine O, Hoarau C, Humbert S, Jaccard A, Jacquot S, Jais JP, Jaussaud R, Jeandel PY, Kebaili K, Korganow AS, Lambotte O, Lanternier F, Larroche C, Lascaux AS, Le Moigne E, Le Moing V, Lebranchu Y, Lecuit M, Lefevre G, Lemal R, Te VLT, Marie-Cardine A, Silva NM, Masseau A, Massot C, Mazingue F, Merlin E, Michel G, Millot F, Monlibert B, Monpoux F, Moshous D, Mouthon L, Munzer M, Neven B, Nove-Josserand R, Oksenhendler E, Ouachée-Chardin M, Oudot C, Pagnier A, Pasquali JL, Pasquet M, Perel Y, Picard C, Piguet C, Plantaz D, Provot J, Quartier P, Rieux-Laucat F, Roblot P, Roger PM, Rohrlich PS, Rubie H, Salle V, Sarrot-Reynauld F, Servettaz A, Stephan JL, Schleinitz N, Suarez F, Swiader L, Taque S, Thomas C, Tournilhac O, Thumerelle C, Tron F, Vannier JP, Viallard JF.

Author information

1
Service de Maladies Infectieuses et Tropicales, Centre d'Infectiologie Necker Pasteur, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Université de Paris, Paris, France.
2
Centre de Référence Déficits Immunitaires Héréditaires (CEREDIH), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France.
3
Institut Imagine, Université de Paris, 149 Rue de Sèvres, 75015, Paris, France.
4
Unité d'Immuno-Hématologie et Rhumatologie Pédiatrique, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France.
5
Laboratoire de Biostatistiques, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France.
6
Assistance Publique-Hôpitaux de Marseille, Service de Pédiatrie et Hématologie Pédiatrique, CHU de Marseille, Hôpital de la Timone, Marseille, France.
7
Assistance Publique-Hôpitaux de Paris, Immunologie-Hématologie, Hôpital Saint Louis, Paris, France.
8
Laboratoire de Microbiologie Clinique, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France.
9
Unité de Recherche EA 7327, Université de Paris, Paris, France.
10
Hôpital Foch, Service de Pneumologie, Suresnes, France.
11
Immunologie-Hématologie-Oncologie Pédiatrique, Centre Hospitalo-Universitaire Angers, Angers, France.
12
Centre Hospitalo-Universitaire Lyon, Service de Médecine Interne et Pathologie Vasculaire, Centre Hospitalier Lyon Sud, Pierre-Bénite, France.
13
Service de Médecine Interne, Centre Hospitalo-Universitaire Pontchaillou, Rennes, France.
14
Service d'Hématologie Clinique et de Thérapie Cellulaire, Centre Hospitalo-Universitaire, Amiens, France.
15
Service de Médecine Interne, Centre Hospitalo-Universitaire, Montpellier, France.
16
Service de Pédiatrie Générale, Infectiologie et Immunologie Clinique, Centre Hospitalo-Universitaire, Montpellier, France.
17
Collège de France, Paris, France.
18
Service de Maladies Infectieuses et Tropicales, Centre d'Infectiologie Necker Pasteur, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Université de Paris, Paris, France. olivier.lortholary@aphp.fr.
19
Centre de Référence Déficits Immunitaires Héréditaires (CEREDIH), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. olivier.lortholary@aphp.fr.
20
Institut Imagine, Université de Paris, 149 Rue de Sèvres, 75015, Paris, France. olivier.lortholary@aphp.fr.

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

PMID:
31401750
DOI:
10.1007/s10875-019-00658-9

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