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Difference between revisions of "Lymphangioleiomyomatosis"
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Lymphangioleiomyomatosis (view source)
Revision as of 17:50, 10 December 2014
, 17:50, 10 December 2014→Epidemiology
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===Epidemiology=== | ===Epidemiology=== | ||
*Rare. | |||
*Usually affects women - primarily in childbearing years; case reports of LAM in men - usu. with [[TSC]].<ref name=pmid17431222>{{Cite journal | last1 = Schiavina | first1 = M. | last2 = Di Scioscio | first2 = V. | last3 = Contini | first3 = P. | last4 = Cavazza | first4 = A. | last5 = Fabiani | first5 = A. | last6 = Barberis | first6 = M. | last7 = Bini | first7 = A. | last8 = Altimari | first8 = A. | last9 = Cooke | first9 = RM. | title = Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex. | journal = Am J Respir Crit Care Med | volume = 176 | issue = 1 | pages = 96-8 | month = Jul | year = 2007 | doi = 10.1164/rccm.200610-1408CR | PMID = 17431222 }}</ref> | |||
*Associated with [[angiomyolipoma]]s. | *Associated with [[angiomyolipoma]]s. | ||
**Seen in ~30% of sporadic cases, and in ~90% of cases with tuberous sclerosis.<ref name=pmid17003820/> | **Seen in ~30% of sporadic cases, and in ~90% of cases with tuberous sclerosis.<ref name=pmid17003820/> | ||
*Associated with [[tuberous sclerosis]]<ref name=emedicine299545/> - esp. TSC2 mutations. | *Associated with [[tuberous sclerosis]]<ref name=emedicine299545/> - esp. TSC2 mutations. | ||
**In the context of [[tuberous sclerosis]], [[angiomyolipoma]] of the kidney often preceeds LAM.<ref name=pmid17003820>{{cite journal |author=Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA |title=Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors |journal=Kidney Int. |volume=70 |issue=10 |pages=1777–82 |year=2006 |month=November |pmid=17003820 |doi=10.1038/sj.ki.5001853 |url=}}</ref> | **In the context of [[tuberous sclerosis]], [[angiomyolipoma]] of the kidney often preceeds LAM.<ref name=pmid17003820>{{cite journal |author=Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA |title=Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors |journal=Kidney Int. |volume=70 |issue=10 |pages=1777–82 |year=2006 |month=November |pmid=17003820 |doi=10.1038/sj.ki.5001853 |url=}}</ref> | ||
==Gross/Radiology== | ==Gross/Radiology== | ||