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.

Extra-cerebral severe infections associated with haemorrhagic hereditary telangiectasia (Rendu-Osler-Weber Disease): five cases and a review of the literature

Di Bella S;
2014-01-01

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.
2014
Pubblicato
File in questo prodotto:
Non ci sono file associati a questo prodotto.
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11368/2934719
 Avviso

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 12
  • ???jsp.display-item.citation.isi??? ND
social impact