title: Cholecystitis and nephrotic syndrome complicating Epstein-Barr virus primary infection.
abstract: Epstein-Barr virus (EBV) infection results in a spectrum of clinical manifestations. The host immune response to EBV plays a key role in the extent and degree of clinical features, which in children under 4 years of age are usually mild, non-specific and self-limiting. A 2-year-old boy in whom no known immune disorder could be found presented with acute acalculous cholecystitis, renal dysfunction with massive proteinuria, ascites, pleural effusion, minimal peripheral oedema and a severe systemic inflammatory response. Improvement occurred after initiation of corticosteroids and antiviral treatment with gancyclovir. In severely symptomatic or complicated EBV infection, a primary immunodeficiency must be suspected. If a primary immunodeficiency has been ruled out, the correct management of severe EBV infection in the immunocompetent host remains controversial.
authors.affiliation: a Departments of Pediatrics.
authors.forename: Diana
authors.initials: D
authors.lastname: Rodà
authors.affiliation: a Departments of Pediatrics.
authors.forename: Malka
authors.initials: M
authors.lastname: Huici
authors.affiliation: a Departments of Pediatrics.
authors.forename: Sílvia
authors.initials: S
authors.lastname: Ricart
authors.affiliation: b Department of Nephrology.
authors.forename: Jordi
authors.initials: J
authors.lastname: Vila
authors.affiliation: c Infectology Unit , Department of Pediatrics.
authors.forename: Clàudia
authors.initials: C
authors.lastname: Fortuny
authors.affiliation: d Department of Allergy and Clinical Immunology , Hospital Sant Joan de Déu , Barcelona , Spain.
authors.forename: Laia
authors.initials: L
authors.lastname: Alsina
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D000998:Antiviral Agents / Q000008:administration & dosage; D002675:Child, Preschool; D002764:Cholecystitis / Q000150:complications* / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D015774:Ganciclovir / Q000008:administration & dosage; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications* / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents; D000998:Antiviral Agents; D015774:Ganciclovir
keywords: Acalculous cholecystitis; Epstein–Barr virus; Haemophagocytic lymphohistiocytosis syndrome; Nephrotic syndrome