title: Extra-cerebral severe infections associated with haemorrhagic hereditary telangiectasia (Rendu-Osler-Weber Disease): five cases and a review of the literature.
abstract: Hereditary haemorrhagic telangiectasia (HHT) is one of the most common autosomal dominant disorders and is characterized by genetically determined abnormalities of vascular structure. People affected by HHT are predisposed to severe infections such as cerebral abscesses, typical of patients with pulmonary arteriovenous malformations, and extra-cerebral infections such as bacteraemia, septic arthritis, osteomyelitis, hepatic abscesses, skin infections and infective endocarditis. We present a retrospective series of severe bacterial extra-cerebral infections in five patients affected by HHT, admitted to our Institute from January 2007 to June 2013. We also reviewed the literature of the last five years concerning infectious complications in people affected by HHT. Our study shows that HHT patients with infectious complications exclusively localized in extra-cerebral sites are usually fragile, old and affected by comorbidities. Moreover, we recognized a trend of Staphylococcus aureus (SA) severe infection recurrence in such patients, both in our series and in the literature. In our opinion these results suggest the need to evaluate the possible benefits of SA nasal colonization screening and decolonization in such patients.
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Musso
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Capone
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Pierangelo
authors.initials: P
authors.lastname: Chinello
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Di Bella
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Galati
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Pasquale
authors.initials: P
authors.lastname: Noto
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Taglietti
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Simone
authors.initials: S
authors.lastname: Topino
authors.affiliation: Second Division of Infectious Diseases, National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Petrosillo
mesh_terms: D000368:Aged; D001424:Bacterial Infections / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*