Linked Life Data

16707400

Source:http://linkedlifedata.com/resource/pubmed/id/16707400

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
5
pubmed:dateCreated
2006-5-18
pubmed:abstractText
Lymphangioleiomyomatosis (LAM) is a rare disease of the lungs and lymphatics, which can occur sporadically or in association with tuberous sclerosis. LAM almost exclusively affects females, generally developing before the menopause. The disease is characterised by progressive pulmonary cystic change, recurrent pneumothorax, chylous pleural collections and, in most cases, progressive respiratory failure. Abdominal manifestations include lymphadenopathy, cystic lymphatic masses (lymphangioleiomyomas), chylous ascites and angiomyolipoma (a benign tumour). Survival in LAM is approximately 70% at 10 yrs, although this is highly variable since long-term survivors have been described. Diagnosis is made by a combination of clinical features and computed tomography scanning or, in cases of doubt, lung biopsy. In patients with rapidly progressive disease, hormone treatment (predominantly progesterone) has been used, although no firm evidence supports its use. Otherwise, treatment is aimed at complications including pneumothorax, chylous collections and extrapulmonary manifestations. The only treatment for severe LAM is currently lung transplantation. Recent developments in the cell biology of lymphangioleiomyomatosis have shown that these patients have somatic mutations in the genes linked to tuberous sclerosis and that rapamycin may correct the resulting cellular abnormality. Trials of rapamycin in lymphangioleiomyomatosis are currently underway and offer hope of evidence-based treatment for the disease.
pubmed:commentsCorrections
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
0903-1936
pubmed:author
pubmed:issnType
Print
pubmed:volume
27
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1056-65
pubmed:dateRevised
2007-3-1
pubmed:meshHeading
pubmed:year
2006
pubmed:articleTitle
Lymphangioleiomyomatosis.
pubmed:affiliation
Division of Therapeutics and Molecular Medicine, University Hospital, Queens Medical Centre, Nottingham NG7 2UH, UK. simon.johnson@nottingham.ac.uk
pubmed:publicationType
Journal Article, Review