Frontiers in Immunology (Nov 2020)
Different Clinical Presentations and Outcomes of Disseminated Varicella in Children With Primary and Acquired Immunodeficiencies
- Paul Bastard,
- Paul Bastard,
- Aurélien Galerne,
- Alain Lefevre-Utile,
- Alain Lefevre-Utile,
- Alain Lefevre-Utile,
- Coralie Briand,
- André Baruchel,
- André Baruchel,
- Philippe Durand,
- Philippe Durand,
- Philippe Durand,
- Judith Landman-Parker,
- Elodie Gouache,
- Nathalie Boddaert,
- Nathalie Boddaert,
- Nathalie Boddaert,
- Despina Moshous,
- Despina Moshous,
- Despina Moshous,
- Joel Gaudelus,
- Joel Gaudelus,
- Robert Cohen,
- Georges Deschenes,
- Alain Fischer,
- Alain Fischer,
- Alain Fischer,
- Alain Fischer,
- Stéphane Blanche,
- Stéphane Blanche,
- Loïc de Pontual,
- Loïc de Pontual,
- Bénédicte Neven,
- Bénédicte Neven,
- Bénédicte Neven
Affiliations
- Paul Bastard
- Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France
- Paul Bastard
- Service d’Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France
- Aurélien Galerne
- Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France
- Alain Lefevre-Utile
- Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France
- Alain Lefevre-Utile
- INSERM U976—Human Systems Immunology and Inflammatory Networks, Institut de Recherche de Saint Louis, Paris, France
- Alain Lefevre-Utile
- Université de Paris, Paris, France
- Coralie Briand
- Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France
- André Baruchel
- Université de Paris, Paris, France
- André Baruchel
- Département d’Hématologie Pédiatrique, Hôpital Robert-Debré, AP-HP, Paris, France
- Philippe Durand
- Service de Réanimation Pédiatrique, Hôpital du Kremlin-Bicêtre, Kremlin-Bicêtre, France
- Philippe Durand
- Université Paris XI, AP-HP, Paris
- Philippe Durand
- Université Paris Saclay, Saint-Aubin, France
- Judith Landman-Parker
- Sorbonne Université, Service de d’Hématologie Oncologie Pédiatrique, Hôpital Armand Trousseau, AP-HP, Paris, France
- Elodie Gouache
- Sorbonne Université, Service de d’Hématologie Oncologie Pédiatrique, Hôpital Armand Trousseau, AP-HP, Paris, France
- Nathalie Boddaert
- Université de Paris, Paris, France
- Nathalie Boddaert
- 0Service de Radiologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Université de Paris, Paris, France
- Nathalie Boddaert
- 1INSERM U1163, Institut IMAGINE, Paris, France
- Despina Moshous
- Service d’Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France
- Despina Moshous
- Université de Paris, Paris, France
- Despina Moshous
- 1INSERM U1163, Institut IMAGINE, Paris, France
- Joel Gaudelus
- Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France
- Joel Gaudelus
- 2Sorbonne Paris Nord University, Bobigny, France
- Robert Cohen
- 3ACTIV Centre Hospitalier Intercommunal de Créteil, Créteil, France
- Georges Deschenes
- 4Service de Néphrologie Pédiatrique, Hôpital Robert-Debré, AP-HP, Paris, France
- Alain Fischer
- Service d’Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France
- Alain Fischer
- Université de Paris, Paris, France
- Alain Fischer
- 1INSERM U1163, Institut IMAGINE, Paris, France
- Alain Fischer
- 5Experimental Medicine, Collège de France, Paris, France
- Stéphane Blanche
- Service d’Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France
- Stéphane Blanche
- Université de Paris, Paris, France
- Loïc de Pontual
- Service de Pédiatrie, Hôpital Jean Verdier, Bondy, AP-HP (Assistance-Publique-Hôpitaux de Paris), France
- Loïc de Pontual
- 2Sorbonne Paris Nord University, Bobigny, France
- Bénédicte Neven
- Service d’Immunologie et Hématologie Pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France
- Bénédicte Neven
- Université de Paris, Paris, France
- Bénédicte Neven
- 1INSERM U1163, Institut IMAGINE, Paris, France
- DOI
- https://doi.org/10.3389/fimmu.2020.595478
- Journal volume & issue
-
Vol. 11
Abstract
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.
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