Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): otorhinolaryngological manifestations.
Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder affecting blood vessels of the skin, mucous membrane and viscera. The otorhinolaryngologist is the commonest clinician involved in management as epistaxis occurs in 93% of the patients. As marked advances have recently been made regarding the pathogenesis and management of the condition, the otorhinolaryngological perspective is reviewed.
Pau
H
H
Department of Otorhinolarynogology, Leicester Royal Infirmary, Leicester, UK. hpau@globalnet.co.uk
Carney
A S
AS
Murty
G E
GE
D000281:Administration, Intranasal; D000287:Administration, Topical; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D002881:Chromosomes, Human, Pair 12 / Q000235:genetics; D002899:Chromosomes, Human, Pair 9 / Q000235:genetics; D004621:Embolization, Therapeutic; D004967:Estrogens / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D015870:Gene Expression; D006801:Humans; D053685:Laser Therapy; D008297:Male; D008967:Molecular Biology / Q000379:methods; D009297:Nasal Mucosa / Q000473:pathology*; D013517:Otorhinolaryngologic Surgical Procedures / Q000379:methods; D011374:Progesterone / Q000008:administration & dosage / Q000627:therapeutic use; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000235:genetics* / Q000473:pathology* / Q000628:therapy
D004967:Estrogens; D011374:Progesterone