title: Enteroviruses in X-Linked Agammaglobulinemia: Update on Epidemiology and Therapy.
abstract: X-linked agammaglobulinemia (XLA) has been associated with a broad range of infections, but enteroviral disease represents one of the most damaging infections. The risk of enteroviral infection in XLA is lower now than in the setting of intramuscular immunoglobulin or in patients without immunoglobulin replacement, but the rate of infection has not declined significantly in the era of intravenous immunoglobulin replacement. Enteroviruses can cause inflammation of nearly every organ, but in XLA, infections often manifest as dermatomyositis or chronic meningoencephalitis. Difficulty and delay in recognizing symptoms and lack of specific therapy contribute to the poor outcomes. Furthermore, cerebrospinal fluid detection of enteroviruses is not very sensitive. Reluctance to perform brain biopsies can lead to significant delays. The other feature compromising outcomes is the lack of specific therapy. High-dose peripheral and intraventricular immunoglobulin have been used, but failure is still common. New antienteroviral drugs are in development and show promise for immunodeficient patients with life-threatening infections with enterovirus.
authors.affiliation: Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, Pa.
authors.forename: David
authors.initials: D
authors.lastname: Bearden
authors.affiliation: ViroDefense Inc, Chevy Chase, Md.
authors.forename: Marc
authors.initials: M
authors.lastname: Collett
authors.affiliation: Center for Infection and Immunity, Mailman School of Public Health of Columbia University, New York, NY.
authors.forename: P Lan
authors.initials: PL
authors.lastname: Quan
authors.affiliation: Division of Allergy, Clinical Immunology and Rheumatology, Federal University of São Paulo, Sao Paulo, Brazil.
authors.forename: Beatriz T
authors.initials: BT
authors.lastname: Costa-Carvalho
authors.affiliation: Division of Allergy and Immunology, the Children's Hospital of Philadelphia, Philadelphia, Pa. Electronic address: sullivak@mail.med.upenn.edu.
authors.forename: Kathleen E
authors.initials: KE
authors.lastname: Sullivan
mesh_terms: D000361:Agammaglobulinemia / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000821:virology; D000998:Antiviral Agents / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging / Q000821:virology; D002675:Child, Preschool; D004770:Enterovirus / Q000235:genetics / Q000302:isolation & purification; D004769:Enterovirus Infections / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000821:virology; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000821:virology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010647:Phenyl Ethers / Q000627:therapeutic use
chemical_list: D000998:Antiviral Agents; D010647:Phenyl Ethers; C000619933:pocapavir
keywords: Agammaglobulinemia; Coxsackievirus B5; Enterovirus; Meningoencephalitis; Pleconaril; Pocapavir; Polio