OBJECTIVES, Hereditary hemorrhagic telangectasia, also known as the Rendu-Osler-Weber syndrome, is a rare au- tosomal dominant form of fibrovascular dysplasia with in- complete penetrance. It is characterized by structural al- terations at the microcirculatory level (capillaries and venules) related to the loss of supportive tissues and po- tential hemorrhagic involvement of all organs. MATERIALS AND METHODS. This report provides a review of the litera- ture on the pathogenesis, clinical manifestations, and treatment options Rendu-Osler-Weber syndrome and de- scribes a case recently treated in the Oral Medicine ward of the University of Milan (Bicocca). RESULTS AND CONCLU- SIONS. The syndrome is manifested by multiple small telangectasias involving the skin and mucosa (e.g., that of the gastrointestinal tract or other organs) associated with recurrent episodes of bleeding at involved sites and frank or occult melena. Treatment is supportive and aimed at the prevention of complications. In patients di- agnosed with hereditary telangectasia, broad spectrum antibiotic prophylaxis is essential before all procedures, particularly those that are invasive (e.g., extractions, im- plants, etc.). The authors suggest the use of amoxicillin 1 g every 12 h, starting the day before the procedure. Treatment should be continued for 5 more days.
OBIETTIVI. Descrivere la Teleangectasia emorragica eredita- ria o sindrome di Rendu-Osler-Weber, malattia a trasmis- sione autosomica dominante a penetranza incompleta che determina alterazioni a carico dei vasi del microcirco- lo (capillari e venule), dovute alla perdita dei tessuti di so- stegno che normalmente circondano i vasi sanguigni, ed emorragie che possono interessare potenzialmente ogni organo. MATERIALI E METODI. È stata fatta una revisione bi- bliografica dell’eziopatogenesi, delle manifestazioni clini- che e delle opzioni terapeutiche di tale malattia ed è stata presentata una relazione sul caso di un paziente con que- sta sindrome, in cura presso il reparto di Medicina orale dell’Universtità di Milano Bicocca. RISULTATI E CONCLUSIONI. La sindrome si manifesta clinicamente con teleangectasie multiple di piccole dimensioni a livello della cute e delle mucose, per esempio del tratto gastrointestinale o di altri organi, associata a episodi ricorrenti di sanguinamento delle sedi colpite e da melena evidente od occulta. Il trat- tamento è di supporto e aiuta a prevenire le complicanze. Una volta posta diagnosi di telangectasia emorragica, pri- ma di effettuare ogni tipo di intervento, in particolare un’a- zione invasiva (come per esempio un’estrazione o un in- tervento di implantologia ecc.), risulta fondamentale effet- tuare una profilassi antibiotica ad ampio spettro. Gli auto- ri consigliano amoxicillina da 1 g ogni 12 ore da iniziare il giorno precedente la terapia odontoiatrica e da continua- re dopo per altri 5 giorni
MANIFESTAZIONI CLINICHE INTRA ED EXTRA ORALI DELLA SINDROME DI RENDU-OSLER-WEBER
LAURITANO D
Primo
Conceptualization
;
2010
Abstract
OBJECTIVES, Hereditary hemorrhagic telangectasia, also known as the Rendu-Osler-Weber syndrome, is a rare au- tosomal dominant form of fibrovascular dysplasia with in- complete penetrance. It is characterized by structural al- terations at the microcirculatory level (capillaries and venules) related to the loss of supportive tissues and po- tential hemorrhagic involvement of all organs. MATERIALS AND METHODS. This report provides a review of the litera- ture on the pathogenesis, clinical manifestations, and treatment options Rendu-Osler-Weber syndrome and de- scribes a case recently treated in the Oral Medicine ward of the University of Milan (Bicocca). RESULTS AND CONCLU- SIONS. The syndrome is manifested by multiple small telangectasias involving the skin and mucosa (e.g., that of the gastrointestinal tract or other organs) associated with recurrent episodes of bleeding at involved sites and frank or occult melena. Treatment is supportive and aimed at the prevention of complications. In patients di- agnosed with hereditary telangectasia, broad spectrum antibiotic prophylaxis is essential before all procedures, particularly those that are invasive (e.g., extractions, im- plants, etc.). The authors suggest the use of amoxicillin 1 g every 12 h, starting the day before the procedure. Treatment should be continued for 5 more days.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
