Linked Life Data

8729995

Source:http://linkedlifedata.com/resource/pubmed/id/8729995

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
1996-10-22
pubmed:abstractText
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder that affects women and can lead to serious respiratory impairment. Since Bourneville's tuberous sclerosis (TS) was first reported, the striking similarities between the two entities have led many to believe that LAM is a forme fruste of TS. This is suggested by reports that angiomyolipomas, rare tumours in themselves, are reported in 40-80% of TS patients and occur in 15-30% of LAM patients. A retrospective chart review was conducted of 14 patients that presented to our institution with a diagnosis of LAM. We sought to document the clinical manifestations, particularly the incidence and location of extrathoracic tumours, in order to further support the hypothesis that LAM is a forme fruste of TS. Twelve patients had premenopausal onset of symptoms and two postmenopausal. The diagnosis was confirmed histologically (n = 12) and/or by computed tomography (CT) scan of the thorax (n = 12). Imaging investigations revealed extrathoracic tumours in 12 of 14 patients (86%). Eight of the 14 patients (57%) had renal tumours consistent with angiomyolipomas (bilateral in five patients). Only one patient had renal symptoms (flank pain and haematuria). All had normal serum creatinine, one had a reduced creatinine clearance. Extrathoracic nonrenal tumours were discovered in the pancreas, adrenals and uterus, findings previously unreported in LAM. In summary, the incidence of extrathoracic tumours in lymphangioleiomyomatosis patients is much higher than previously reported in the literature. This increased association supports the theory that lymphangioleiomyomatosis and tuberous sclerosis represent part of a spectrum of a similar disease process.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
0903-1936
pubmed:author
pubmed:issnType
Print
pubmed:volume
9
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
402-5
pubmed:dateRevised
2005-11-17
pubmed:meshHeading
pubmed:year
1996
pubmed:articleTitle
Extrathoracic angiomyolipomas in lymphangioleiomyomatosis.
pubmed:affiliation
Toronto Hospital, University of Toronto, Canada.
pubmed:publicationType
Journal Article