Diagnostic Value of Serum VEGF-D in Lymphangioleiomyomatosis: Results of the First Prospective Study in the Russian Federation [PDF]
Background/Objectives: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease for which serum vascular endothelial growth factor D (VEGF-D) is a recommended diagnostic biomarker. Data from the Russian Federation remain limited. We aimed to evaluate
Marina Makarova +5 more
doaj +2 more sources
Lymphangioleiomyomatosis is caused by mutations (usually sporadic, sometimes in tuberous sclerosis) of the TSC1 or TSC2 genes and results in cystic destruction of the lungs, with CT features being sufficiently characteristic to establish the diagnosis in
S.J. Bourke, D.J. Hendrick
core +4 more sources
Uterine lymphangioleiomyomatosis in a premenopausal woman with tuberous sclerosis: A case report
Lymphangioleiomyomatosis is a rare disease characterized by abnormal smooth muscle cell growth. It primarily occurs in the lungs but can also rarely occur in other organs, in which case it is classified as extrapulmonary lymphangioleiomyomatosis.
Eun Min Lee +3 more
doaj +2 more sources
Possible Novel Therapeutic Targets in Lymphangioleiomyomatosis Treatment
Lymphangioleiomyomatosis (LAM) is a rare systemic neoplastic disease that exclusively happens in women. Studies focusing on LAM and tuberous sclerosis complex (TSC) have made great progress in understanding the pathogenesis and searching for treatment ...
Ling Ye, Chengyu Yang, Hui Cai
exaly +3 more sources
A Case Series of Lymphangioleiomyomatosis, a Rare Cystic Lung Disease. [PDF]
We studied five women with a rare lung condition called LAM that typically affects only 1–5 women per 10 million. All patients had breathing difficulties and characteristic lung scans showing multiple thin‐walled air sacs. Finding five cases at one center in just 2 years suggests this condition might be more common than previously thought or often goes
Sedef S, Mutlu P.
europepmc +2 more sources
Inhibition of mTOR is the standard of care for lymphangioleiomyomatosis (LAM). However, this therapy has variable tolerability and some patients show progressive decline of lung function despite treatment.
Antonio Gomez Moruno +2 more
exaly +2 more sources
A Patient Journey:Living with lymphangioleiomyomatosis [PDF]
Dr Havi Carel was diagnosed with Lymphangioleiomyomatosis (LAM) in 2006. In the four years since she has learnt much about the adaptability of the human body and about some clinicians’ insensitivity to the quality of life issues that can be so important ...
Carel, Havi +4 more
core +3 more sources
Recent advances in the management of lymphangioleiomyomatosis [version 1; referees: 4 approved]
Lymphangioleiomyomatosis is a rare disorder that predominantly affects women and is characterized by progressive cystic changes in the lung, leading to gradually worsening shortness of breath and lung function impairment.
Kai-Feng Xu, Xinlun Tian, Jay H Ryu
doaj +2 more sources
GPNMB immunohistochemistry is a useful ancillary tool for the diagnosis of pulmonary lymphangioleiomyomatosis. [PDF]
We evaluated the diagnostic utility of GPNMB immunohistochemistry in LAM. GPNMB showed strong expression in LAM cells, while the differential diagnostic mimics showed no or only low expression. Based on its 100% sensitivity and specificity in our cohort, GPNMB is a highly reliable immunohistochemical marker for the diagnosis of LAM.
Szalai F +6 more
europepmc +2 more sources
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 +2 more sources

