Different Clinical Presentations and Outcomes of Disseminated Varicella in Children With Primary and Acquired Immunodeficiencies.

Autor:	Bastard P; Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France.; Service d'Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France., Galerne A; Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France., Lefevre-Utile A; Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France.; INSERM U976-Human Systems Immunology and Inflammatory Networks, Institut de Recherche de Saint Louis, Paris, France.; Université de Paris, Paris, France., Briand C; Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France., Baruchel A; Université de Paris, Paris, France.; Département d'Hématologie Pédiatrique, Hôpital Robert-Debré, AP-HP, Paris, France., Durand P; Service de Réanimation Pédiatrique, Hôpital du Kremlin-Bicêtre, Kremlin-Bicêtre, France.; Université Paris XI, AP-HP, Paris.; Université Paris Saclay, Saint-Aubin, France., Landman-Parker J; Sorbonne Université, Service de d'Hématologie Oncologie Pédiatrique, Hôpital Armand Trousseau, AP-HP, Paris, France., Gouache E; Sorbonne Université, Service de d'Hématologie Oncologie Pédiatrique, Hôpital Armand Trousseau, AP-HP, Paris, France., Boddaert N; Université de Paris, Paris, France.; Service de Radiologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Université de Paris, Paris, France.; INSERM U1163, Institut IMAGINE, Paris, France., Moshous D; Service d'Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France.; Université de Paris, Paris, France.; INSERM U1163, Institut IMAGINE, Paris, France., Gaudelus J; Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France.; Sorbonne Paris Nord University, Bobigny, France., Cohen R; ACTIV Centre Hospitalier Intercommunal de Créteil, Créteil, France., Deschenes G; Service de Néphrologie Pédiatrique, Hôpital Robert-Debré, AP-HP, Paris, France., Fischer A; Service d'Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France.; Université de Paris, Paris, France.; INSERM U1163, Institut IMAGINE, Paris, France.; Experimental Medicine, Collège de France, Paris, France., Blanche S; Service d'Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France.; Université de Paris, Paris, France., de Pontual L; Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France.; Sorbonne Paris Nord University, Bobigny, France., Neven B; Service d'Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France.; Université de Paris, Paris, France.; INSERM U1163, Institut IMAGINE, Paris, France.
Jazyk:	angličtina
Zdroj:	Frontiers in immunology [Front Immunol] 2020 Nov 05; Vol. 11, pp. 595478. Date of Electronic Publication: 2020 Nov 05 (Print Publication: 2020).
DOI:	10.3389/fimmu.2020.595478
Abstrakt:	Primary infection with varicella-zoster virus (VZV) causes chickenpox, a benign and self-limited disease in healthy children. In patients with primary or acquired immunodeficiencies, primary infection can be life-threatening, due to rapid dissemination of the virus to various organs [lung, gastrointestinal tract, liver, eye, central nervous system (CNS)]. We retrospectively described and compared the clinical presentations and outcomes of disseminated varicella infection (DV) in patients with acquired (AID) ( n = 7) and primary (PID) ( n = 12) immunodeficiencies. Patients with AID were on immunosuppression (mostly steroids) for nephrotic syndrome, solid organ transplantation or the treatment of hemopathies, whereas those with PID had combined immunodeficiency (CID) or severe CID (SCID). The course of the disease was severe and fulminant in patients with AID, with multiple organ failure, no rash or a delayed rash, whereas patients with CID and SICD presented typical signs of chickenpox, including a rash, with dissemination to other organs, including the lungs and CNS. In the PID group, antiviral treatment was prolonged until immune reconstitution after bone marrow transplantation, which was performed in 10/12 patients. Four patients died, and three experienced neurological sequelae. SCID patients had the worst outcome. Our findings highlight substantial differences in the clinical presentation and course of DV between children with AID and PID, suggesting differences in pathophysiology. Prevention, early diagnosis and treatment are required to improve outcome. (Copyright © 2020 Bastard, Galerne, Lefevre-Utile, Briand, Baruchel, Durand, Landman-Parker, Gouache, Boddaert, Moshous, Gaudelus, Cohen, Deschenes, Fischer, Blanche, de Pontual and Neven.)
Databáze:	MEDLINE
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