In Hereditary Haemorrhagic Telangiectasia Pathological Nailfold Capillaroscopy is Associated with Pulmonary Arteriovenous Malformations
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چکیده
1Department of Internal Medicine, Hospital Sierrallana, Torrelavega, Spain 2Biomedical Research Centre Network for Rare Diseases (CIBERER), Spain 3Research Institute Valdecilla (IDIVAL), Santander, Spain 4Department of Rheumatology, Hospital Sierrallana, Spain 5Quality Unit, Marqués de Valdecilla University Hospital, Santander, Spain 6Department of Genetics, Marqués de Valdecilla University Hospital, Spain 7Department of Cardiology, Marqués de Valdecilla University Hospital, Spain 8Biological Research Center, Superior Council of Scientifi c Investigations (CSIC), Madrid, Spain 9Department of Radiology, Marqués de Valdecilla University Hospital, Spain
منابع مشابه
Impact of Pulmonary Arteriovenous Malformations on Respiratory–Related Quality of Life in Patients with Hereditary Haemorrhagic Telangiectasia
Fifteen to fifty percent of patients with hereditary haemorrhagic telangiectasia have pulmonary arteriovenous malformations. The objective of this study was to measure the effect of the presence of pulmonary arteriovenous malformations and of their embolisation on respiratory-related quality of life (QoL). We prospectively recruited patients with a diagnosis of hereditary haemorrhagic telangiec...
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Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder, characterised by the presence of vascular malformations. The pulmonary vascular complications of HHT include pulmonary arteriovenous malformations, pulmonary hypertension associated with high-output heart failure and liver vascular malformations and, finally, pulmonary arterial hypertension secondary to HHT. In ...
متن کاملNeonatal pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasia.
A 3 week old infant presented with persistent hypoxaemia and was diagnosed with pulmonary arteriovenous malformations. Her family history was positive for hereditary haemorrhagic telangiectasia. She was treated successfully with coil embolotherapy at the age of 4 months. Transcatheter embolisation may be considered the primary treatment for pulmonary arteriovenous malformations in infancy.
متن کاملPulmonary arteriovenous malformations and other pulmonary aspects of hereditary haemorrhagic telangiectasia
Pulmonary arteriovenous malformations (PAVMs) are vascular structures that provide a direct capillary-free communication between the pulmonary and systemic circulations. The majority of patients have no PAVM-related symptoms, but are at risk of major complications that can be prevented by appropriate interventions. More than 90% of PAVMs occur as part of hereditary haemorrhagic telangiectasia (...
متن کاملPositional oximetry to screen for pulmonary arteriovenous malformations in HHT.
Orthodeoxia has been described in patients with hereditary haemorrhagic telangiectasia (HHT) and pulmonary arteriovenous malformations (PAVM), but its usefulness in screening children for PAVMs is unknown. We show here that while oximetry is overall lower in children with significant PAVMs, supine and upright oximetry is of no additional diagnostic value indicating that positional oximetry is n...
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تاریخ انتشار 2017