QJM Advance Access published online on December 8, 2005
QJM, doi:10.1093/qjmed/hci148
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
1 From the Department of Internal Medicine and Public Health, Section of Internal Medicine, University of Bari, Bari, Italy
* To whom correspondence should be addressed. Background: Hereditary haemorrhagic telangiectasia (HHT) is a systemic autosomal dominant vascular disease. Although the clinical picture is that of a chronic disabling disease, vascular malformations can suddenly lead to life-threatening conditions. Aim: To assess the frequency and type of emergency acute complications in HHT. Design: Retrospective case-note review. Methods: From August 2000 to December 2004, our specialized HHT centre saw 139 patients (74 males, 65 females, mean age 45.5 years, range 14-77) with a definite diagnosis of HHT. We reviewed their clinical files and recorded all visits for acute complications (massive nosebleeds, haematemesis, melaena, haematochezia, haemothorax, haemoptysis, TIA/ischaemic stroke, haemorrhagic stroke, brain abscess). Results: Fifty patients (35.9%) had at least one acute complication. There were a total of 93 visits potentially involving the emergency department. Most commonly, patients sought urgent medical attention for nosebleeds and gastrointestinal bleeding (63.4%), but there were also disorders of the brain, lung, heart and liver. Discussion: Acute complications of HHT are not uncommon and can be severe and wide-ranging. Physicians should be aware of HHT and its major complications, as a prompt diagnosis is essential to direct patients to the most appropriate therapies, and to suggest screening for visceral involvement in their relatives.
Received August 4, 2005
Revised November 7, 2005
Original paper
Emergencies in hereditary haemorrhagic telangiectasia
M. Gallitelli 1,
G. Pasculli 1,
T. Fiore 2,
A. Carella 3,
and
C. Sabbà 4 *
2 From the Department of Neurological and Psychiatric Sciences, Section of Neuroradiology, University of Bari, Bari, Italy; From the Department of Emergency and Transplantation, University of Bari, Bari, Italy; From the Center for Hereditary Haemorrhagic Telangiectasia, University of Bari, Bari, Italy
3 From the Department of Emergency and Transplantation, University of Bari, Bari, Italy; From the Center for Hereditary Haemorrhagic Telangiectasia, University of Bari, Bari, Italy
4 From the Department of Internal Medicine and Public Health, Section of Internal Medicine, University of Bari, Bari, Italy; From the Department of Neurological and Psychiatric Sciences, Section of Neuroradiology, University of Bari, Bari, Italy; From the Department of Emergency and Transplantation, University of Bari, Bari, Italy; From the Center for Hereditary Haemorrhagic Telangiectasia, University of Bari, Bari, Italy
C. Sabbà, E-mail: c.sabba{at}dimimp.uniba.it
Abstract 
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  S. Al-Saleh, M. Mei-Zahav, M. E. Faughnan, I. B. MacLusky, S. Carpenter, M. Letarte, and F. Ratjen Screening for pulmonary and cerebral arteriovenous malformations in children with hereditary haemorrhagic telangiectasia Eur. Respir. J., October 1, 2009; 34(4): 875 - 881. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Emergencies in Patients with Hereditary Hemorrhagic Telangiectasia Journal Watch Dermatology, May 9, 2006; 2006(509): 4 - 4. [Full Text]
|
|