Fatal adenovirus type 7b infection in a child with Smith-Lemli-Opitz syndrome

Adenovirus type 7 causes worldwide respiratory tract infections, mainly in children. Severe systemic infections can occur, especially in immunocomprom ised patients and in patients with underlying chronic diseases. This report describes the first case of a fatal disseminated adenovirus type 7 infecti on in a child with Smith-Lemli-Opitz syndrome, a rare autosomal recessive d isorder due to a primary enzymatic defect in cholesterol metabolism. Nasoph aryngeal secretions and autopsy specimens including liver, lung, pleural fl uid, and rectum were collected for viral culture. Adenovirus serotype 7 str ains were obtained from all anatomic sites, except the liver. All these cli nical isolates were analyzed using restriction enclonuclease digestion of t he genome, identifying them as genome type 7b, a virulent type. In this cas e, the fatal evolution could have been accelerated by the presence of an im munodeficiency although immunodeficiency is not included in the definition of Smith-Lemli-Opitz syndrome. The frequent recurrent banal infections in S mith-Lemli-Opitz syndrome could be prevented by a cholesterol supplementati on regimen. Finally, this report emphasizes the need for efficient therapy for disseminated adenovirus infections, especially for virulent genome type s. (C) 2001 Wiley-Liss, Inc.