Cholecystitis and nephrotic syndrome complicating Epstein-Barr virus primary infection

• Published in: Paediatrics and international child health Vol. 37; no. 1; pp. 74 - 77
• Main Authors: Rodà, Diana, Huici, Malka, Ricart, Sílvia, Vila, Jordi, Fortuny, Clàudia, Alsina, Laia
• Format: Journal Article
• Language: English
• Published: England 01-02-2017
• Subjects: Adrenal Cortex Hormones - administration & dosage; Anti-Inflammatory Agents - administration & dosage; Antiviral Agents - administration & dosage; Child, Preschool; Cholecystitis - complications; Cholecystitis - drug therapy; Cholecystitis - etiology; Cholecystitis - pathology; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - diagnosis; Epstein-Barr Virus Infections - drug therapy; Epstein-Barr Virus Infections - pathology; Ganciclovir - administration & dosage; Herpesvirus 4, Human - isolation & purification; Humans; Male; Nephrotic Syndrome - complications; Nephrotic Syndrome - drug therapy; Nephrotic Syndrome - etiology; Nephrotic Syndrome - pathology; Treatment Outcome; Haemophagocytic lymphohistiocytosis syndrome; Acalculous cholecystitis; Nephrotic syndrome; Epstein–Barr virus
• ISSN: 2046-9047; 2046-9055
• DOI: 10.1080/20469047.2015.1135561

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.