1. Different Clinical Presentations and Outcomes of Disseminated Varicella in Children With Primary and Acquired Immunodeficiencies
- Author
-
Paul Bastard, Aurélien Galerne, Alain Lefevre-Utile, Coralie Briand, André Baruchel, Philippe Durand, Judith Landman-Parker, Elodie Gouache, Nathalie Boddaert, Despina Moshous, Joel Gaudelus, Robert Cohen, Georges Deschenes, Alain Fischer, Stéphane Blanche, Loïc de Pontual, Bénédicte Neven, Human genetics of infectious diseases : Mendelian predisposition (Equipe Inserm U1163), Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Groupe de Pathologie Infectieuse Pédiatrique [Paris] (GPIP), Société Française de Pédiatrie (SFP), Hôpital Jean Verdier [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Louis Mourier - AP-HP [Colombes], Hôpital Robert Debré, Institut de Chimie des Substances Naturelles (ICSN), Institut de Chimie du CNRS (INC)-Université Paris-Saclay-Centre National de la Recherche Scientifique (CNRS), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Nutrition, croissance et cancer (U 1069) (N2C), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), CHU Necker - Enfants Malades [AP-HP], Centre Hospitalier Intercommunal de Créteil (CHIC), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Gestionnaire, HAL Sorbonne Université 5, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)
- Subjects
Male ,0301 basic medicine ,lcsh:Immunologic diseases. Allergy ,Pediatrics ,medicine.medical_specialty ,[SDV.IMM] Life Sciences [q-bio]/Immunology ,Adolescent ,medicine.medical_treatment ,Fulminant ,Immunology ,Disease ,Antiviral Agents ,03 medical and health sciences ,Fatal Outcome ,0302 clinical medicine ,varicella ,medicine ,Humans ,Immunology and Allergy ,Child ,innate immunity ,Immunodeficiency ,Original Research ,Lung ,Chickenpox ,business.industry ,Immunologic Deficiency Syndromes ,Infant ,Immunosuppression ,primary immumunodeficiencies ,medicine.disease ,Rash ,3. Good health ,030104 developmental biology ,medicine.anatomical_structure ,Child, Preschool ,Varicella Zoster Virus Infection ,disseminated varicella ,[SDV.IMM]Life Sciences [q-bio]/Immunology ,Female ,medicine.symptom ,business ,lcsh:RC581-607 ,Nephrotic syndrome ,030215 immunology ,steroids - Abstract
International audience; 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.
- Published
- 2020