We report a case of sporadic pulmonary lymphangioleiomyomatosis presenting with chylothorax and with parietal pleural involvement by the disease. The implications for the anatomic distribution of lesions in pulmonary lymphangioleiomyomatosis is discussed.
Shunei Asao, B.A.+2 more
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Differential diagnosis of chylothorax in therapeutic practice
The article is devoted to the differential diagnosis of chylous pleural effusion. The spectrum of traumatic and non-traumatic causes of chylothorax is discussed in detail.
Marina A. Makarova+2 more
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A rare cause of recurrent spontaneous pneumothorax: Birt-hogg-dube syndrome [PDF]
Birt-Hogg-Dube (BHD) syndrome is an unusual disorder characterized by the triad of cutaneous lesions, renal tumors and lung cysts. In cases with BHD syndrome, the frequency of recurrent pneumothorax is increased due to presence of multiple lung cysts. It
Demirci, M.+3 more
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VATS therapy of chylothorax caused by leiomyomatosis complicated with tuberous sclerosis complex
Lymphangioleiomyomatosis with tuberous sclerosis complex is a rare disease. One of the most frequent complications of lymphangioleiomyomatosis is pleural effusion (chylothorax) wich can be treated with the use of VATS.
Adrienn Csiszkó+5 more
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Identification of a novel TSC2 c.3610G > A, p.G1204R mutation contribute to aberrant splicing in a patient with classical tuberous sclerosis complex: a case report [PDF]
Background: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by hamartomas in any organ systems. Mutations in the TSC1 or TSC2 gene lead to the dysfunction of hamartin or tuberin proteins, which cause tuberous sclerosis ...
Bottillo, I+7 more
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Lymphangioleiomyomatosis (LAM) is a rare disease that mainly affects women, particularly at fertile age. It is sporadic or associated with tuberous sclerosis complex. It is characterised by an abnormal proliferation of immature smooth muscle cells (SMC), which grow aberrantly in the airway, parenchyma, lymphatics and pulmonary blood vessels and which ...
Ansotegui Barrera, Emilio+3 more
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Management of lymphangioleiomyomatosis [PDF]
Lymphangioleiomyomatosis (LAM), a multisystem disease affecting almost exclusively women, is characterized by cystic lung destruction and presents with dyspnea, recurrent pneumothoraxes, chylous effusions, lymphangioleiomyomas, and angiomyolipomas.
Angelo M. Taveira-DaSilva, Joel Moss
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Anesthesia for Suboccipital Craniotomy in a Patient with Lymphangioleiomyomatosis: A Case Report [PDF]
Lymphangioleiomyomatosis (LAM) is a rare pulmonary condition often presenting with spontaneous pneumothorax. Imaging or biopsy confirm the diagnosis.
Luman, Emily+2 more
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Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study [PDF]
Rationale Mechanistic target of rapamycin inhibitors reduce loss of lung function in lymphangioleiomyomatosis (LAM), although their benefit varies between individuals.
Janet Bee+9 more
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Doxycycline reduces the migration of tuberous sclerosis complex-2 null cells - effects on RhoA-GTPase and focal adhesion kinase [PDF]
& Sons Ltd and Foundation for Cellular and Molecular Medicine. Lymphangioleiomyomatosis (LAM) is associated with dysfunction of the tuberous sclerosis complex (TSC) leading to enhanced cell proliferation and migration.
Black, JL+5 more
core +1 more source