acyclovir and Agammaglobulinemia

Chronic enteroviral meningoencephalitis is a well-recognized complication in patients with X-linked agammaglobulinemia (XLA). The majority of published cases refers to its occurrence in patients on no replacement therapy or on only intramuscular immunoglobulin. The advent of intravenous immunoglobulin (IVIg) in the early 1980s and its widespread use in XLA was thought to have virtually eradicated enteroviral meningoencephalitis in these patients. We describe the development of echovirus meningoencephalitis in an 11-year-old boy on regular IVIg replacement whose serum IgG levels were maintained at between 6 and 8 g/L (NR 6-13 g/L). Treatment with daily high-dose IVIg was commenced, with significant clinical improvement being noted within a few weeks in association with a reduction in blood-brain barrier permeability. The persistence of live virus, however, necessitated the use of intraventricular immunoglobulin. The virus proved resistant to two courses of specific intraventricular immunoglobulin and a 6-week course of oral ribavirin and eventually proved fatal 5 months after presentation. In view of the therapeutic uncertainties we have reviewed the use of immunoglobulin in the treatment of enteroviral meningoencephalitis over the past 6 years.

Topics: Acyclovir; Agammaglobulinemia; Child, Preschool; Echovirus Infections; Humans; Immunoglobulins, Intravenous; Male; Meningoencephalitis; Ribavirin

To describe atypical varicella zoster virus (VZV) retinitis in a patient with Good syndrome.. A 63-year-old patient with Good syndrome presented with bilateral necrotizing retinitis starting from the posterior pole. He had a history of thymoma status post thymectomy 4 years previously, left-sided sinusitis, and recent pulmonary aspergillosis. Qualitative PCR was performed on aqueous fluid.. Immunological investigations revealed reduced levels of CD4. Good syndrome should be considered in a patient with opportunistic infections and history of thymoma in the absence of human immunodeficiency virus. Atypical retinitis can occur in patients with Good syndrome and quantitative PCR is important for accurate diagnosis.

Topics: Acyclovir; Agammaglobulinemia; Antiviral Agents; Aqueous Humor; CD4 Lymphocyte Count; DNA, Viral; Eye Infections, Viral; Fluorescein Angiography; Herpes Zoster Ophthalmicus; Herpesvirus 3, Human; Humans; Male; Middle Aged; Polymerase Chain Reaction; Retinitis; Thymoma; Thymus Neoplasms; Valacyclovir; Valine; Visual Acuity

Chronic mucocutaneous or cutaneous herpes simplex is a rare syndrome associated with immunosuppression. We report the first case associated with X-linked hypogammaglobulinemia and illustrate the effectiveness of oral acyclovir in this potentially serious illness.

Topics: Acyclovir; Administration, Oral; Agammaglobulinemia; Child; Chronic Disease; Genetic Linkage; Herpes Simplex; Humans; Male; Sex Chromosome Aberrations; Skin Diseases; X Chromosome

Topics: Acyclovir; Agammaglobulinemia; Antiviral Agents; Bromodeoxyuridine; Child; Drug Resistance, Microbial; Guanine; Humans; Male; Mutation; Simplexvirus; Thymidine Kinase