Mb Osler: ökad risk för infektioner och livshotande komplikationer. Fyra fall av invasiv infektionssjukdom under samma tidsperiod beskrivs
Engelsk titel: Osler-Weber-Rendu Syndrome: increased risk of infections and life-threatening complications. Four cases of invasive infectious disease over the same period described
Läs online
Författare:
Cajander, Sara
;
Eliasson, Henrik
Email: sara.cajander@orebroll.se
Språk: Swe
Antal referenser: 16
Dokumenttyp:
Fallbeskrivning
UI-nummer: 12097362
Sammanfattning
Mb Osler, also known as hereditary hemorrhagic teleangiectasia (HHT), is a genetic disorder characterized by epistaxis, teleangiectasia and visceral arteriovenous malformations (AVM).
Symptoms are most often due to either shunting or bleeding in affected organs. Patients with HHT and pulmonary AVM are more at risk of acquiringcerebral abscesses and cerebrovascular lesions through paradoxical embolisation.
Screening for pulmonary AVM is recommended since embolisation therapy reduces neurological risks. Microbial findings in cerebral abscesses suggest dental origin. Antibiotic prophylaxis at the time of dental procedures is recommended for HHT patients with pulmonary AVM.
An association between HHT and extracerebral infection was shown in a retrospective study including 353 HHT patients. The most common pathogen in extracerebral infections is staphylococcus aureus, pointing towards nasal mucosa as the entry route for the bacteria. We present four cases of serious infectious disease in patients with HHT.