Case report: If it is not asthma—think of lymphangioleiomyomatosis in younger female patients
Lymphangioleiomyomatosis (LAM) is a rare lung disease predominantly affecting women, and it is characterized by the proliferation of smooth muscle cells and cystic lung destruction.
Malene Helligsø Kirkeby+3 more
doaj +1 more source
Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex. [PDF]
Background: This study evaluated the characteristics of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC) entered into the TuberOus SClerosis registry to increase disease Awareness (TOSCA).
Agranovich, Oleg+49 more
core +2 more sources
Repurposing Nitazoxanide for Potential Treatment of Rare Disease Lymphangioleiomyomatosis
Lymphangioleiomyomatosis (LAM) is a rare genetic lung disease. Unfortunately, treatment with the mTORC1 inhibitor Rapamycin only slows disease progression, and incomplete responses are common.
Stella Bähr+6 more
doaj +1 more source
The first lung transplantation at the Research Institute for Emergency named after N.V. Sklifosovsky
The paper describes the first lung transplantation at the N.V. Sklifosovsky Research Institute of Emergency Care in a female patient with endstage pulmonary lymphangioleiomyomatosis, which has shown a good result during a 6-month follow-up.
M. Sh. Khubutia+19 more
doaj +1 more source
The dual PI3K/mTOR inhibitor GSK2126458 is effective for treating solid renal tumours in Tsc2+/- mice through suppression of cell proliferation and induction of apoptosis [PDF]
Tuberous sclerosis (TSC) is an inherited tumour syndrome caused by mutations in TSC1 or TSC2 that lead to aberrant activation of mTOR. Tumour responses in TSC patients to rapamycin, an allosteric inhibitor of mTOR, or its analogs are partial and ...
Jones, Ashley+5 more
core +1 more source
Determinants of Progression and Mortality in Lymphangioleiomyomatosis.
Wenshaui Xu+23 more
semanticscholar +1 more source
Complications of lymphangioleiomyomatosis in pregnancy: a case report and review of the literature
Lymphangioleiomyomatosis is a rare cystic lung disease primarily affecting premenopausal females and may be exacerbated by pregnancy. We conducted a literature review of lymphangioleiomyomatosis during pregnancy with a specific focus on related maternal ...
Eileen Wang-Koehler, MD+2 more
doaj
Recent advances in interstitial lung disease research [PDF]
The interstitial lung diseases are a diverse collection of disorders characterized by impaired gas exchange, restricted physiology on lung function testing, and diffuse parenchymal lung infiltrates on radiography.
Vassallo, Robert
core
Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: Our interdisciplinary rheumatology-pneumology experience, and review of the literature [PDF]
Background: Interstitial lung diseases (ILDs) are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the lungs, varying from idiopathic interstitial pneumonias to secondary variants, including the ILDs associated to ...
Cerri, Stefania+9 more
core +1 more source
Pulmonary lymphangioleiomyomatosis in a male [PDF]
Hyun Wook Kang+5 more
openalex +1 more source