A 4-year prospective evaluation of protocols to improve clinical outcomes for patients with lymphangioleiomyomatosis in a national clinical centre.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare multisystem disease. Progressive airflow limitation, pneumothorax and angiomyolipoma-related bleeding are major morbidities. As treatments are available for these complications, we prospectively audited loss of FEV1 (ΔFEV1), pneumothorax and angiomyolipoma bleeding against clinical standards over 4 years at the UK Clinical Centre. ΔFEV1 for these patients is lower than previously reported and rates of pneumothorax and angiomyolipoma haemorrhage are low. This suggests that real-time analysis of clinical data with targeted interventions can reduce morbidity in LAM. These measures could be applied as quality standards to compare the emerging LAM clinical networks worldwide.

• Authors: Janet Bee, Rupesh Bhatt, Ian McCafferty, Simon R Johnson
• Journal: Thorax (Thorax) Vol. 70 Issue 12 Pg. 1202-4 (Dec 2015) ISSN: 1468-3296 [Electronic] England
• PMID: 26123659 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Chemical References

• TOR Serine-Threonine Kinases

Topics

• Clinical Protocols
• Female
• Humans
• Lymphangioleiomyomatosis (epidemiology, physiopathology, therapy)
• Pneumothorax (epidemiology)
• Prognosis
• Quality Indicators, Health Care
• Respiratory Function Tests
• TOR Serine-Threonine Kinases (antagonists & inhibitors)
• Treatment Outcome