title: 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.affiliation: National Centre for Lymphangioleiomyomatosis, Nottingham University Hospitals NHS Trust, Nottingham, Nottinghamshire, UK.
authors.forename: Janet
authors.initials: J
authors.lastname: Bee
authors.affiliation: National Centre for Lymphangioleiomyomatosis, Nottingham University Hospitals NHS Trust, Nottingham, Nottinghamshire, UK Renal Tumour Service, University Hospital Birmingham, Birmingham, West Midlands, UK.
authors.forename: Rupesh
authors.initials: R
authors.lastname: Bhatt
authors.affiliation: National Centre for Lymphangioleiomyomatosis, Nottingham University Hospitals NHS Trust, Nottingham, Nottinghamshire, UK Renal Tumour Service, University Hospital Birmingham, Birmingham, West Midlands, UK.
authors.forename: Ian
authors.initials: I
authors.lastname: McCafferty
authors.affiliation: National Centre for Lymphangioleiomyomatosis, Nottingham University Hospitals NHS Trust, Nottingham, Nottinghamshire, UK Division of Respiratory Medicine and Respiratory Research Unit, University of Nottingham, Nottingham, Nottinghamshire, UK.
authors.forename: Simon R
authors.initials: SR
authors.lastname: Johnson
mesh_terms: D002985:Clinical Protocols; D005260:Female; D006801:Humans; D018192:Lymphangioleiomyomatosis / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy*; D011030:Pneumothorax / Q000453:epidemiology; D011379:Prognosis; D019984:Quality Indicators, Health Care; D012129:Respiratory Function Tests; D058570:TOR Serine-Threonine Kinases / Q000037:antagonists & inhibitors; D016896:Treatment Outcome
chemical_list: D058570:TOR Serine-Threonine Kinases
keywords: Rare lung diseases