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Difference between revisions of "Medical lung diseases"
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→Lymphangioleiomyomatosis: format +ref, more
(→Lymphangioleiomyomatosis: +image) |
(→Lymphangioleiomyomatosis: format +ref, more) |
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*Clinical: dyspnea, recurrent pneumothorax. | *Clinical: dyspnea, recurrent pneumothorax. | ||
*May be an indication for lung transplantation. | *May be an indication for lung transplantation. | ||
*Non-neoplastic muscle proliferation vs. tumour that can metastasize.<ref name=pmid20235883>{{Cite journal | last1 = Taveira-DaSilva | first1 = AM. | last2 = Pacheco-Rodriguez | first2 = G. | last3 = Moss | first3 = J. | title = The natural history of lymphangioleiomyomatosis: markers of severity, rate of progression and prognosis. | journal = Lymphat Res Biol | volume = 8 | issue = 1 | pages = 9-19 | month = Mar | year = 2010 | doi = 10.1089/lrb.2009.0024 | PMID = 20235883 }}</ref> | |||
===Epidemiology=== | ===Epidemiology=== | ||
*Associated with [[angiomyolipoma]]s.<ref name=emedicine299545>[http://emedicine.medscape.com/article/299545-overview http://emedicine.medscape.com/article/299545-overview]</ref> | *Associated with [[angiomyolipoma]]s.<ref name=emedicine299545>[http://emedicine.medscape.com/article/299545-overview http://emedicine.medscape.com/article/299545-overview]</ref> | ||
*Associated with [[tuberous sclerosis]]<ref name=emedicine299545/> - abnormality in same gene (TSC2). | *Associated with [[tuberous sclerosis]]<ref name=emedicine299545/> - abnormality in same gene (TSC2). | ||
* | *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> | ||
*Rare. | *Rare. | ||
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===IHC=== | ===IHC=== | ||
*HMB-45 +ve | *HMB-45 +ve. | ||
*ER +ve | *ER +ve. | ||
*PR +ve | *PR +ve. | ||
*SMA +ve | *SMA +ve. | ||
==Pulmonary alveolar proteinosis== | ==Pulmonary alveolar proteinosis== | ||