Literature DB >> 24276214

[Osler's disease].

F Ahlhelm1, J Lieb, G Schneider, U Müller, S Ulmer.   

Abstract

Osler's disease, also known as hereditary hemorrhagic telangiectasia (HHT) and Osler-Weber-Rendu syndrome, is an autosomal dominant disorder leading to abnormal blood vessel formation in the skin, mucous membranes and often in organs, such as the lungs, liver and brain (arteriovenous malformations AVM). Various types are known. Patients may present with epistaxis. Teleangiectasia can be identified by visual inspection during physical examination of the skin or oral cavity or by endoscopy. Diagnosis is made after clinical examination and genetic testing based on the Curacao criteria. Modern imaging modalities, such as computed tomography (CT) or magnetic resonance imaging (MRI) have become more important as they can depict the AVMs. Pulmonary AVMs can be depicted in CT imaging even without the use of a contrast agent while other locations including the central nervous system (CNS) usually require administration of contrast agents. Knowledge of possible clinical manifestations in various organs, possible complications and typical radiological presentation is mandatory to enable adequate therapy of these patients. Interventional procedures are becoming increasingly more important in the treatment of HHT patients.

Entities:  

Mesh:

Year:  2013        PMID: 24276214     DOI: 10.1007/s00117-013-2552-z

Source DB:  PubMed          Journal:  Radiologe        ISSN: 0033-832X            Impact factor:   0.635


  28 in total

Review 1.  Helical biphasic contrast-enhanced CT of the liver: technique, indications, interpretation, and pitfalls.

Authors:  J H Oliver; R L Baron
Journal:  Radiology       Date:  1996-10       Impact factor: 11.105

2.  Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: Doppler sonographic screening in a large family.

Authors:  E Buscarini; L Buscarini; C Danesino; M Piantanida; G Civardi; P Quaretti; S Rossi; M Di Stasi; M Silva
Journal:  J Hepatol       Date:  1997-01       Impact factor: 25.083

3.  Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia.

Authors:  C O Maher; D G Piepgras; R D Brown; J A Friedman; B E Pollock
Journal:  Stroke       Date:  2001-04       Impact factor: 7.914

4.  Nontumorous arterioportal shunt mimicking hypervascular tumor in cirrhotic liver: two-phase spiral CT findings.

Authors:  T K Kim; B I Choi; J K Han; J W Chung; J H Park; M C Han
Journal:  Radiology       Date:  1998-09       Impact factor: 11.105

5.  Pial arteriovenous fistula in children as presenting manifestation of Rendu-Osler-Weber disease.

Authors:  R García-Mónaco; W Taylor; G Rodesch; H Alvarez; P Burrows; P Coubes; P Lasjaunias
Journal:  Neuroradiology       Date:  1995-01       Impact factor: 2.804

6.  Bleeding from cerebral arteriovenous malformations as part of their natural history.

Authors:  C J Graf; G E Perret; J C Torner
Journal:  J Neurosurg       Date:  1983-03       Impact factor: 5.115

7.  Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy.

Authors:  R I White; A Lynch-Nyhan; P Terry; P C Buescher; E J Farmlett; L Charnas; K Shuman; W Kim; M Kinnison; S E Mitchell
Journal:  Radiology       Date:  1988-12       Impact factor: 11.105

8.  Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment.

Authors:  J Remy; M Remy-Jardin; L Wattinne; C Deffontaines
Journal:  Radiology       Date:  1992-03       Impact factor: 11.105

9.  Should asymptomatic patients with hereditary haemorrhagic telangiectasia (HHT) be screened for cerebral vascular malformations? Data from 22,061 years of HHT patient life.

Authors:  A J Easey; G M F Wallace; J M B Hughes; J E Jackson; W J Taylor; C L Shovlin
Journal:  J Neurol Neurosurg Psychiatry       Date:  2003-06       Impact factor: 10.154

10.  Hereditary hemorrhagic telangiectasia: multi-detector row helical CT assessment of hepatic involvement.

Authors:  Amato Antonio Stabile Ianora; Maurizio Memeo; Carlo Sabba; Anna Cirulli; Antonio Rotondo; Giuseppe Angelelli
Journal:  Radiology       Date:  2003-11-26       Impact factor: 11.105

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