1,661 results match your criteria Lymphangioleiomyomatosis


Safe and effective use of nivolumab for treating lung adenocarcinoma associated with sporadic lymphangioleiomyomatosis: a rare case report.

BMC Pulm Med 2019 Jan 11;19(1):12. Epub 2019 Jan 11.

Service d'Oncologie Thoracique, Hôpital Bichat, 46 rue Henri Huchard, 75018, Paris, France.

Background: Sporadic lymphangioleiomyomatosis (LAM) is a rare form of diffuse parenchymal lung disease. PD-1 blocking antibodies constitute an essential treatment option for advanced non-small-cell lung cancer (NSCLC). The effect of immune checkpoint inhibitors in lymphangioleiomyomatosis patients with non-small cell lung cancer is unknown: concomitant symptomatic interstitial lung disease or the use of immunosuppressors was a key exclusion criterion in the original studies of immune checkpoint inhibitors, especially regarding the risk of interstitial lung disease exacerbation. Read More

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https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890
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http://dx.doi.org/10.1186/s12890-018-0775-5DOI Listing
January 2019
4 Reads

Concentration of Serum Vascular Endothelial Growth Factor (VEGF-D) and Its Correlation with Functional and Clinical Parameters in Patients with Lymphangioleiomyomatosis from a Brazilian Reference Center.

Lung 2019 Jan 8. Epub 2019 Jan 8.

Divisao de Pneumologia, Instituto do Coracao, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Avenida Doutor Enéas de Carvalho Aguiar, 44, São Paulo, São Paulo, 05403-900, Brazil.

Introduction: Serum vascular endothelial growth factor-D (VEGF-D) is a lymphangiogenic growth factor that is considered a valuable tool in the diagnosis of lymphangioleiomyomatosis (LAM). Previous studies have reported a wide variability in VEGF-D serum levels in LAM patients and it seems to be associated with pulmonary impairment and lymphatic involvement.

Methods: We conducted a cross-sectional study from 2009 to 2017 that evaluated VEGF-D serum levels in a cohort of LAM patients who were never treated with mTOR inhibitors and compared them to healthy age-matched volunteers. Read More

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http://dx.doi.org/10.1007/s00408-018-00191-3DOI Listing
January 2019

Intrapulmonary metastasis from primary pulmonary meningioma presenting as multiple cystic lesions: a case report.

BMC Pulm Med 2019 Jan 8;19(1). Epub 2019 Jan 8.

Department of Thoracic Surgery, West China Hospital, Sichuan University, Chengdu, 610041, People's Republic of China.

Background: Cystic lung lesions involving both lungs include a variety of diseases, such as pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis and pulmonary metastasis. Primary pulmonary meningioma accompanied with intrapulmonary metastasis was extremely rare and we were not aware of previous studies reporting with cystic radiological manifestation.

Case Presentation: A 64-year-old female patient was admitted to our department for a mass located in right posterior mediastinum with multiple cystic pulmonary lesions. Read More

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http://dx.doi.org/10.1186/s12890-018-0773-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6325864PMC
January 2019
2 Reads

Rationally Designed 3D Hydrogels Model Invasive Lung Diseases Enabling High-Content Drug Screening.

Adv Mater 2018 Dec 27:e1806214. Epub 2018 Dec 27.

Department of Chemical Engineering and Applied Chemistry, Institute of Biomaterials and Biomedical Engineering, University of Toronto, Toronto, Ontario, M5S 3G9, Canada.

Cell behavior is highly dependent upon microenvironment. Thus, to identify drugs targeting metastatic cancer, screens need to be performed in tissue mimetic substrates that allow cell invasion and matrix remodeling. A novel biomimetic 3D hydrogel platform that enables quantitative analysis of cell invasion and viability at the individual cell level is developed using automated data acquisition methods with an invasive lung disease (lymphangioleiomyomatosis, LAM) characterized by hyperactive mammalian target of rapamycin complex 1 (mTORC1) signaling as a model. Read More

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http://dx.doi.org/10.1002/adma.201806214DOI Listing
December 2018
1 Read

Mitochondrial dysfunction is a key determinant of the rare disease lymphangioleiomyomatosis and provides a novel therapeutic target.

Oncogene 2018 Dec 20. Epub 2018 Dec 20.

Department of Pharmaceutical Biotechnology, School of Pharmacy, University of Pecs, Pecs, Hungary.

Lymphangioleiomyomatosis (LAM) is a rare and progressive systemic disease affecting mainly young women of childbearing age. A deterioration in lung function is driven by neoplastic growth of atypical smooth muscle-like LAM cells in the pulmonary interstitial space that leads to cystic lung destruction and spontaneous pneumothoraces. Therapeutic options for preventing disease progression are limited and often end with lung transplantation temporarily delaying an inevitable decline. Read More

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http://www.nature.com/articles/s41388-018-0625-1
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http://dx.doi.org/10.1038/s41388-018-0625-1DOI Listing
December 2018
1 Read

Lymphangioleiomyomatosis Mortality in Patients with Tuberous Sclerosis Complex.

Ann Am Thorac Soc 2018 Dec 20. Epub 2018 Dec 20.

University of Cincinnati, Division of Pulmonary, Critical Care and Sleep Medicine. Department of Internal Medicine, Cincinnati, Ohio, United States ;

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http://dx.doi.org/10.1513/AnnalsATS.201807-471RLDOI Listing
December 2018
1 Read

Pregnancy unmasking symptoms of undiagnosed lymphangioleiomyomatosis: Case report and review of literature.

Respir Med Case Rep 2019 23;26:63-67. Epub 2018 Nov 23.

Centegra Health Care System, USA.

Cystic lung diseases are a group of disorders that appear similar on radiological studies on chest computed tomography. Each disorder is characterized by its own etiology, pathophysiology, course of progression and manifestation. Lymphangioleiomyomatosis (LAM) is one of the cystic lung diseases that can either be hereditary or sporadic. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.11.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6277247PMC
November 2018
1 Read

Air travel and incidence of pneumothorax in lymphangioleiomyomatosis.

Orphanet J Rare Dis 2018 Dec 13;13(1):222. Epub 2018 Dec 13.

Service de pneumologie, Centre hospitalier universitaire vaudois, PMU BU44.07, Rue du Bugnon 44, 1011, Lausanne, Switzerland.

Background: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of women characterized by multiple lung cysts leading to respiratory insufficiency and frequent pneumothorax (PT). Air travel (AT) could increase the risk of PT in LAM through rupture of subpleural cysts induced by atmospheric pressure changes in aircraft cabin. To determine whether AT increases the risk of PT in LAM, we performed a retrospective survey of members of European LAM patient associations. Read More

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http://dx.doi.org/10.1186/s13023-018-0964-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6293523PMC
December 2018
1 Read

Can we predict the existence of extrarenal feeders to renal angiomyolipomas?

Eur Radiol 2018 Dec 12. Epub 2018 Dec 12.

Department of Radiology, School of Medicine, Juntendo University, Tokyo, Japan.

Objectives: To identify factors predicting the presence of extrarenal feeders to renal angiomyolipomas (AMLs) METHODS: This is a retrospective study of 44 patients with 58 renal AMLs embolized in our department. Arteriography obtained during embolization and CT angiography obtained before and after embolization were reviewed to characterize AMLs with and without extrarenal feeders. Tumor characteristics were compared between the two groups. Read More

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http://dx.doi.org/10.1007/s00330-018-5877-1DOI Listing
December 2018

miR‑124 targets retinoid X receptor α to reduce growth of TSC2‑deficient lymphangioleiomyomatosis.

Oncol Rep 2019 Feb 7;41(2):1342-1350. Epub 2018 Dec 7.

Jiangsu Key Laboratory of Molecular Medicine, School of Medicine, Nanjing University, Nanjing, Jiangsu 210093, P.R. China.

Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease that leads to progressive destruction of lung function. However, the mechanisms underlying the progression of LAM remain unknown. Recent studies demonstrated that miR‑124‑3p (hereinafter referred to as miR‑124) is a downregulated miRNA in tumors and it is still unclear whether miR‑124 participates in LAM. Read More

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http://dx.doi.org/10.3892/or.2018.6916DOI Listing
February 2019
2 Reads

Coexistent sarcoidosis and lymphangioleiomyomatosis in a patient with cystic lung disease.

Respirol Case Rep 2019 Feb 28;7(2):e00389. Epub 2018 Nov 28.

Department of Respiratory Medicine Cork University Hospital Ireland.

A 45-year-old lady presented acutely with pleuritic chest pain, haemoptysis, and dyspnoea. Her background was significant for a 1.4 cm renal angiomyolipoma, and she was an ex-smoker without any relevant family history. Read More

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http://doi.wiley.com/10.1002/rcr2.389
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http://dx.doi.org/10.1002/rcr2.389DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6261928PMC
February 2019
2 Reads

[Sporadic lymphangioleiomyomatosis: A rare cause of bilateral spontaneous pneumothorax in young woman].

Rev Pneumol Clin 2018 Dec 23;74(6):497-501. Epub 2018 Nov 23.

Département de chirurgie thoracique, CHU Hassan II, Fés, Maroc; Faculté de médecine et de pharmacie, université Sidi Mohamed Ben Abdallah, Fés, Maroc.

Sporadic lymphangioleiomyomatosis is an orphan disease of the young woman. Its clinical manifestations are mainly respiratory, including spontaneous pneumothorax. The management is multidisciplinary. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07618417183014
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http://dx.doi.org/10.1016/j.pneumo.2018.10.005DOI Listing
December 2018
2 Reads

[The start of the Hungarian lung transplantation program and the first results].

Orv Hetil 2018 11;159(46):1859-1868

Mellkassebészeti Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.

The first lung transplantation in Hungary was performed on 12th of December, 2015. It was a joint effort of the National Institute of Oncology and the Semmelweis University. Hereby we summarise the results and experiences from the first three years. Read More

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http://dx.doi.org/10.1556/650.2018.31300DOI Listing
November 2018
1 Read

Efficacy and safety of low-dose Sirolimus in Lymphangioleiomyomatosis.

Orphanet J Rare Dis 2018 Nov 14;13(1):204. Epub 2018 Nov 14.

Departments of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-Ro 43-Gil, Songpa-Gu, Seoul, 05505, Republic of Korea.

Background: Lymphangioleiomyomatosis is a rare disease caused by unregulated activation of mammalian target of rapamycin (mTOR) signalling pathway. Sirolimus showed efficacy in a phase 3 trial of patients with lymphangioleiomyomatosis, but the optimal dose remains unclear.

Methods: We investigated the efficacy and safety of low-dose compared with conventional-dose sirolimus. Read More

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https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0
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http://dx.doi.org/10.1186/s13023-018-0946-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6236936PMC
November 2018
5 Reads

Lymphangioleiomyomatosis (LAM) presenting as recurrent pneumothorax in an infant with tuberous sclerosis: treated successfully with sirolimus.

BMJ Case Rep 2018 Nov 8;2018. Epub 2018 Nov 8.

Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

Lymphangioleiomyomatosis (LAM) either sporadic or a part of tuberous sclerosis complex is rare in paediatric age group. Here, we report a case of LAM with tuberous sclerosis in an infant. She was referred to our institute at the age of 4 months as a case of recurrent bilateral pneumothorax requiring intercostal tube drainage. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22624
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http://dx.doi.org/10.1136/bcr-2018-226244DOI Listing
November 2018
6 Reads

Vitamin D binding protein: a new biomarker of disease severity in lymphangioleiomyomatosis.

Eur Respir J 2018 Nov 1;52(5). Epub 2018 Nov 1.

Division of Pulmonary and Critical Care Medicine, Dept of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.

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http://erj.ersjournals.com/lookup/doi/10.1183/13993003.01886
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http://dx.doi.org/10.1183/13993003.01886-2018DOI Listing
November 2018
2 Reads

Comorbid connective tissue diseases and autoantibodies in lymphangioleiomyomatosis: a retrospective cohort study.

Orphanet J Rare Dis 2018 Oct 20;13(1):182. Epub 2018 Oct 20.

Clinical Research Centre, National Hospital Organization Kinki-Chuo Chest Medical Centre, 1180 Nagasone-cho, Kita-ku, Sakai City, Osaka, 591-8555, Japan.

Background: Lymphangioleiomyomatosis (LAM) and connective tissue diseases (CTDs) occur more frequently among women than men. We investigated the frequency of comorbid CTD and positive serum autoantibody findings in patients with LAM.

Methods: A total of 152 patients with LAM were prospectively and consecutively registered in the National Hospital Organization Kinki-Chuo Chest Medical Centre cohort. Read More

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http://dx.doi.org/10.1186/s13023-018-0933-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6195983PMC
October 2018
3 Reads

A case of uterine lymphangioleiomyomatosis in a young woman that was identified via peritonitis.

J Obstet Gynaecol Res 2018 Oct 19. Epub 2018 Oct 19.

Department of Obstetrics and Gynecology, Fujita Health University School of Medicine, Toyoake, Japan.

Although lymphangioleiomyomatosis is often observed with tuberous sclerosis, uterine lymphangioleiomyomatosis is rare. Our patient was 36 years old (gravida 0, para 0). She had a history of tuberous sclerosis, and many myometrial cystic lesions were identified during assisted reproductive therapy. Read More

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http://doi.wiley.com/10.1111/jog.13822
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http://dx.doi.org/10.1111/jog.13822DOI Listing
October 2018
8 Reads

Incidental lymphangioleiomyomatosis in the lymph nodes of gynecologic surgical specimens.

Eur J Obstet Gynecol Reprod Biol 2018 Dec 10;231:93-97. Epub 2018 Oct 10.

Department of Gynecology, National Cancer Center Hospital, Tokyo 104-0045, Japan.

Objectives: Incidentally discovered lymphangioleiomyomatosis (LAM) in sampled lymph nodes are infrequent but intractable issues for gynecologists. The aims of this study were to elucidate the prevalence of incidental nodal LAM in a consecutive cohort of gynecologic surgical specimens from Japanese patients, to document clinicopathological features of nodal LAM cases, and to investigate the association between the subsequent development of pulmonary LAM and tuberous sclerosis complex (TSC).

Study Design: We retrospectively reviewed 1732 consecutive Japanese patients who underwent gynecologic surgery with lymph node sampling in the National Cancer Center Hospital between January 2004 and April 2017. Read More

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http://dx.doi.org/10.1016/j.ejogrb.2018.10.027DOI Listing
December 2018
1 Read

Successful Sirolimus Treatment of Lymphangioleiomyomatosis in a Hepatitis B Virus Carrier.

Intern Med 2018 Oct 17. Epub 2018 Oct 17.

Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan.

A 34-year-old woman experiencing shortness of breath was referred to our hospital. The patient was diagnosed with sporadic lymphangioleiomyomatosis based on the observation of bilateral diffuse multiple thin-walled cysts on computed tomography of the chest, chylous effusion, elevated serum vascular endothelial growth factor-D levels and transbronchial biopsy findings. This patient was a hepatitis B virus (HBV) carrier. Read More

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https://www.jstage.jst.go.jp/article/internalmedicine/advpub
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http://dx.doi.org/10.2169/internalmedicine.1329-18DOI Listing
October 2018
3 Reads

mTOR inhibitor therapy as a disease modifying therapy for tuberous sclerosis complex.

Am J Med Genet C Semin Med Genet 2018 Sep 11;178(3):365-373. Epub 2018 Oct 11.

Department of Pediatrics, Division of Child Neurology, Cincinnati Childrens Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.

Between 1993 and 2003, through experiments involving Drosophila sp., cancer biologists identified the protein kinase known as the mammalian target of rapamycin, its pathway, and its relationship to the genes responsible for tuberous sclerosis. Thereafter, clinical research has resulted in regulatory approval of mTOR inhibitors for four distinct manifestations of the disease: giant cell astrocytoma, angiomyolipoma, lymphangioleiomyomatosis, and epilepsy. Read More

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http://doi.wiley.com/10.1002/ajmg.c.31655
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http://dx.doi.org/10.1002/ajmg.c.31655DOI Listing
September 2018
15 Reads

Renal manifestation of tuberous sclerosis complex.

Am J Med Genet C Semin Med Genet 2018 Sep 11;178(3):338-347. Epub 2018 Oct 11.

Cardiology Clinical Academic Group, Molecular and Clinical Sciences Research Centre, St. Georges University of London, London, United kingdom.

Tuberous sclerosis complex (TSC) is a tumor predisposition syndrome with significant renal cystic and solid tumor disease. It commonly causes several types of cystic disease and benign tumors (angiomyolipomata) in the kidneys that can both lead to significant premature loss of glomerular filtration rate. The main risks of angiomyolipomata, severe bleeding, loss of renal function, and pulmonary lymphangioleiomyomatosis, can be ameliorated by active surveillance and preemptive therapy with mTOR inhibitors. Read More

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http://doi.wiley.com/10.1002/ajmg.c.31654
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http://dx.doi.org/10.1002/ajmg.c.31654DOI Listing
September 2018
12 Reads

Chest CT Scan at Radiation Dose of a Posteroanterior and Lateral Chest Radiograph Series: A Proof of Principle in Lymphangioleiomyomatosis.

Chest 2018 Oct 3. Epub 2018 Oct 3.

Cardiovascular and Pulmonary Branches, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD. Electronic address:

Background: Given the rising utilization of medical imaging and the risks of radiation, there is increased interest in reducing radiation exposure. The objective of this study was to evaluate, as a proof of principle, CT scans performed at radiation doses equivalent to that of a posteroanterior and lateral chest radiograph series in the cystic lung disease lymphangioleiomyomatosis.

Methods: From November 2016 to May 2018, 105 consecutive subjects with lymphangioleiomyomatosis received chest CT scans at standard and ultra-low radiation doses. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00123692183248
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http://dx.doi.org/10.1016/j.chest.2018.09.007DOI Listing
October 2018
16 Reads

Dysregulation of the endothelin pathway in lymphangioleiomyomatosis with no direct effect on cell proliferation and migration.

Sci Rep 2018 Oct 2;8(1):14698. Epub 2018 Oct 2.

Université de Lyon, Université Claude Bernard Lyon 1, INRA, EPHE, IVPC, Viral Infections and Comparative Pathology, UMR754, F69007, Lyon, France.

LAM is a rare low-grade metastasizing lung neoplasm. Inhibitors of mTOR improve clinical outcome of LAM patients by preventing loss of lung function. Nevertheless, other cell targets may be of interest for drug development. Read More

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http://dx.doi.org/10.1038/s41598-018-32795-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6168484PMC
October 2018
8 Reads
5.080 Impact Factor

Vps34-mediated macropinocytosis in Tuberous Sclerosis Complex 2-deficient cells supports tumorigenesis.

Sci Rep 2018 Sep 21;8(1):14161. Epub 2018 Sep 21.

Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Tuberous Sclerosis Complex (TSC), a rare genetic disorder with mechanistic target of rapamycin complex 1 (mTORC1) hyperactivation, is characterized by multi-organ hamartomatous benign tumors including brain, skin, kidney, and lung (Lymphangioleiomyomatosis). mTORC1 hyperactivation drives metabolic reprogramming including glucose and glutamine utilization, protein, nucleic acid and lipid synthesis. To investigate the mechanisms of exogenous nutrients uptake in Tsc2-deficient cells, we measured dextran uptake, a polysaccharide internalized via macropinocytosis. Read More

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http://dx.doi.org/10.1038/s41598-018-32256-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6155086PMC
September 2018
4 Reads

Efficacy of sirolimus for the prevention of recurrent pneumothorax in patients with lymphangioleiomyomatosis: a case series.

Orphanet J Rare Dis 2018 Sep 21;13(1):168. Epub 2018 Sep 21.

Department of Pulmonary and Critical Care Medicine, The Second Xiangya Hospital, Central South University, 139 Renmin Road, Changsha, China.

Pneumothorax is one of the most common symptoms in patients with lymphangioleiomyomatosis (LAM). However, current management strategies for patients with LAM who present with recurrent pneumothorax remain inadequate. Here, we describe the successful prevention of recurrent pneumothorax by sirolimus treatment in five women with LAM. Read More

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https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0
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http://dx.doi.org/10.1186/s13023-018-0915-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6150971PMC
September 2018
13 Reads

Lymphangioleiomyomatosis: a case report and review of diagnosis and treatment.

Onco Targets Ther 2018 31;11:5339-5347. Epub 2018 Aug 31.

Department of Lung Cancer Surgery, Tianjin Key Laboratory of Lung Cancer Metastasis and Tumor Microenvironment, Tianjin Lung Cancer Institute, Tianjin Medical University General Hospital, Tianjin 300052, China,

Lymphangioleiomyomatosis (LAM) is a rare disease that generally affects young women and involves the abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs (pulmonary LAM) and extrapulmonary sites (extrapulmonary LAM). This disease is rare in males. It is hard to distinguish between lung cancer and pulmonary LAM, especially during early stages. Read More

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https://www.dovepress.com/lymphangioleiomyomatosis-a-case-re
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http://dx.doi.org/10.2147/OTT.S161360DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126514PMC
August 2018
8 Reads

Angiomyolipoma rebound tumor growth after discontinuation of everolimus in patients with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis.

PLoS One 2018 7;13(9):e0201005. Epub 2018 Sep 7.

Schlumberger Moscow Research Center, Moscow Research Institute of Pediatrics and Pediatric Surgery, Moscow, Russian Federation.

Introduction: The EXIST-2 (NCT00790400) study demonstrated the superiority of everolimus over placebo for the treatment of renal angiomyolipomas associated with tuberous sclerosis complex (TSC) or sporadic lymphangioleiomyomatosis (LAM). This post hoc analysis of EXIST-2 study aimed to assess angiomyolipoma tumor behavior among patients who submitted to continued radiographic examination following discontinuation of everolimus in the noninterventional follow-up phase.

Methods: For patients who discontinued everolimus at the completion of extension phase for reasons other than angiomyolipoma progression, a single CT/MRI scan of the kidney was collected after 1 year of treatment discontinuation. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0201005PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6128468PMC
September 2018
3 Reads

Could Immunotherapy Sink Its Teeth into Lymphangioleiomyomatosis?

Am J Respir Cell Mol Biol 2018 Dec;59(6):663-665

1 Regenerative Medicine Program Ottawa Hospital Research Institute Ottawa, Ontario, Canada.

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https://www.atsjournals.org/doi/10.1165/rcmb.2018-0251ED
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http://dx.doi.org/10.1165/rcmb.2018-0251EDDOI Listing
December 2018
13 Reads

Less common manifestations in TSC.

Am J Med Genet C Semin Med Genet 2018 Sep 29;178(3):348-354. Epub 2018 Aug 29.

Department of Pediatric Radiology, Hospital San Joan de Déu, Barcelona.

Tuberous sclerosis complex (TSC) is due to pathogenic variants in TSC1 or TSC2 genes resulting in hyperactivation of the mTOR pathway. Many organ systems can be affected, such as brain, skin, eye, heart, bone, kidney, or lung. Typical lesions of TSC usually are those included as major criteria, including angiofibromas, hypomelanotic macules, tubers, subependymal nodules, angiomyolipomas, cardiac rhabdomyomas, and lymphangioleiomyomatosis. Read More

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http://doi.wiley.com/10.1002/ajmg.c.31648
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http://dx.doi.org/10.1002/ajmg.c.31648DOI Listing
September 2018
15 Reads

Circulating Biomarkers From the Phase 1 Trial of Sirolimus and Autophagy Inhibition for Patients With Lymphangioleiomyomatosis.

Chest 2018 Nov 23;154(5):1070-1082. Epub 2018 Aug 23.

Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA. Electronic address:

Background: We have previously conducted the Sirolimus and Autophagy Inhibition in LAM (SAIL) trial, a phase 1 dose-escalation study of the combination of sirolimus and hydroxychloroquine in patients with lymphangioleiomyomatosis (LAM). The goal of the present study was to analyze sera from the SAIL trial to identify novel biomarkers that could shed light into disease pathogenesis and response to therapy.

Methods: We used the DiscoveryMAP platform from Rules Based Medicine to simultaneously measure 279 analytes in sera collected at each visit from subjects enrolled in the SAIL trial. Read More

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http://dx.doi.org/10.1016/j.chest.2018.08.1029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6224706PMC
November 2018
5 Reads

COPD assessment test as a possible tool for evaluating health-related quality of life in lymphangioleiomyomatosis.

Respir Investig 2018 Nov 22;56(6):480-488. Epub 2018 Aug 22.

Divisions of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-Ku, Tokyo 113-8431, Japan. Electronic address:

Background: Lymphangioleiomyomatosis (LAM) is a progressive, cystic lung disease that causes an obstructive ventilatory impairment similar to chronic obstructive pulmonary disease (COPD) and impairs the health-related quality of life (HRQoL). Here, we extended the use of the COPD assessment test (CAT) to patients with chronic respiratory diseases other than COPD. Specifically, the CAT was administered to patients with LAM for the first time. Read More

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http://dx.doi.org/10.1016/j.resinv.2018.07.004DOI Listing
November 2018
1 Read

Lymphangioleiomyomatosis (LAM).

Am J Respir Crit Care Med 2018 Aug;198(4):P7-P8

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http://dx.doi.org/10.1164/rccm.1984P7DOI Listing
August 2018
2 Reads

The efficacy and adverse events of mTOR inhibitors in lymphangioleiomyomatosis: systematic review and meta-analysis.

Orphanet J Rare Dis 2018 Aug 14;13(1):134. Epub 2018 Aug 14.

Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.

Background: Lymphangioleiomyomatosis (LAM) is a rare lung disease and the mammalian target of the rapamycin (mTOR) inhibitors has been used as an effective therapy. Here we conducted a systematic review and meta-analysis with the aims to quantify the efficacy and safety of mTOR inhibitors in LAM patients.

Methods: The following databases were searched for clinical trials regarding LAM patients treated with mTOR inhibitors until December 2017: Pubmed, Embase, Cochrane Library and OVID medicine. Read More

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https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0
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http://dx.doi.org/10.1186/s13023-018-0874-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6092843PMC
August 2018
9 Reads

Immune Checkpoint Ligand PD-L1 Is Upregulated in Pulmonary Lymphangioleiomyomatosis.

Am J Respir Cell Mol Biol 2018 Dec;59(6):723-732

3 Penn Center for Pulmonary Biology, Pulmonary, Allergy and Critical Care Division, and.

Pulmonary lymphangioleiomyomatosis (LAM) is a slow-progressing metastatic disease that is driven by mutations in the tumor suppressor tuberous sclerosis complex 1/2 (TSC1/2). Rapamycin inhibits LAM cell proliferation and is the only approved treatment, but it cannot cause the regression of existing lesions and can only stabilize the disease. However, in other cancers, immunotherapies such as checkpoint blockade against PD-1 and its ligand PD-L1 have shown promise in causing tumor regression and even curing some patients. Read More

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https://www.atsjournals.org/doi/10.1165/rcmb.2018-0123OC
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http://dx.doi.org/10.1165/rcmb.2018-0123OCDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6293078PMC
December 2018
19 Reads

The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis.

Eur Respir J 2018 Nov 1;52(5). Epub 2018 Nov 1.

Division of Respiratory Medicine, University of Nottingham and National Institute for Health Research, Respiratory Biomedical Research Centre, Nottingham, UK.

Lymphangioleiomyomatosis (LAM) is a rare disease of women. Decline in lung function is variable, making appropriate targeting of therapy difficult. We used unbiased serum proteomics to identify markers associated with outcome in LAM. Read More

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http://erj.ersjournals.com/lookup/doi/10.1183/13993003.00951
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http://dx.doi.org/10.1183/13993003.00951-2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214577PMC
November 2018
19 Reads

Smoking-Related Diffuse Cystic Lung Disease.

Chest 2018 Aug;154(2):e31-e35

Division of Pathology & Laboratory Medicine and Perinatal Institute, Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Department of Pathology & Laboratory Medicine, University of Cincinnati, Cincinnati, OH.

Exposure to cigarette smoke can lead to a variety of parenchymal lung diseases, including diffuse cystic lung diseases (DCLDs). Lymphangioleiomyomatosis (LAM) is the prototypical DCLD and has a characteristic appearance on high-resolution CT (HRCT). We present a series of four patients with DCLD on HRCT who were referred to our institution with a presumed diagnosis of LAM and who were found instead to have smoking-related injury of the small airways on histopathological analysis. Read More

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http://dx.doi.org/10.1016/j.chest.2018.02.027DOI Listing
August 2018
4 Reads

Thoracoabdominal imaging of tuberous sclerosis.

Pediatr Radiol 2018 08 4;48(9):1307-1323. Epub 2018 Aug 4.

Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., MLC 5031, Cincinnati, OH, 45229, USA.

Imaging of tuberous sclerosis complex has rapidly evolved over the last decade in association with increased understanding of the disease process and new treatment modalities. Tuberous sclerosis complex is best known for the neurological symptoms and the associated neuroimaging findings, and children with tuberous sclerosis complex require active surveillance of associated abnormalities in the chest, abdomen and pelvis. Common findings that require regular imaging surveillance are angiomyolipomas in the kidneys and lymphangioleiomyomatosis in the chest. Read More

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http://dx.doi.org/10.1007/s00247-018-4123-yDOI Listing
August 2018
2 Reads

Pulmonary manifestations in tuberous sclerosis complex.

Am J Med Genet C Semin Med Genet 2018 Sep 28;178(3):326-337. Epub 2018 Jul 28.

Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts.

Tuberous sclerosis complex has manifestations in many organ systems, including brain, heart, kidney, skin, and lung. The primary manifestations in the lung are lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH). LAM affects almost exclusively women, and causes cystic lung destruction, pneumothorax, and chylous pleural effusions. Read More

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http://dx.doi.org/10.1002/ajmg.c.31638DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6209521PMC
September 2018
1 Read

[F]Fluorocholine and [F]Fluoroacetate PET as Imaging Biomarkers to Assess Phosphatidylcholine and Mitochondrial Metabolism in Preclinical Models of TSC and LAM.

Clin Cancer Res 2018 Dec 27;24(23):5925-5938. Epub 2018 Jul 27.

Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

Purpose: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by inactivating mutations of the or gene, characterized by neurocognitive impairment and benign tumors of the brain, skin, heart, and kidneys. Lymphangioleiomyomatosis (LAM) is a diffuse proliferation of α-smooth muscle actin-positive cells associated with cystic destruction of the lung. LAM occurs almost exclusively in women, as a TSC manifestation or a sporadic disorder ( somatic mutations). Read More

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http://clincancerres.aacrjournals.org/lookup/doi/10.1158/107
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http://dx.doi.org/10.1158/1078-0432.CCR-17-3693DOI Listing
December 2018
6 Reads

Effect of everolimus on renal function in patients with tuberous sclerosis complex: evidence from EXIST-1 and EXIST-2.

Nephrol Dial Transplant 2018 Jul 19. Epub 2018 Jul 19.

Royal Sussex County Hospital, Brighton, UK.

Background: A reduction in renal angiomyolipoma volume observed with everolimus (EVE) treatment in patients with tuberous sclerosis complex (TSC) has been postulated to translate to clinical benefit by reducing the risk of renal hemorrhage and chronic renal failure.

Methods: The long-term effects of EVE on renal function (∼4 years of treatment) were examined in patients treated with EVE in the Phase 3 EXIST-1 and EXIST-2 studies. Patients in EXIST-1 had TSC and subependymal giant cell astrocytoma (SEGA), and patients in EXIST-2 had renal angiomyolipoma and a definite diagnosis of TSC or sporadic lymphangioleiomyomatosis. Read More

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http://dx.doi.org/10.1093/ndt/gfy132DOI Listing
July 2018
5 Reads

Roles of human epidermal growth factor receptor family in pulmonary lymphangioleiomyomatosis.

Hum Pathol 2018 Nov 18;81:121-130. Epub 2018 Jul 18.

Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. Electronic address:

Lymphangioleiomyomatosis (LAM) is a rare and refractory disease that affects women of reproductive age. Several target therapies are used to manage LAM, but no curative modes of treatment have been reported yet. Therefore, in this study, we focused on targeting human epidermal growth factor receptor (HER) family proteins as a treatment strategy for LAM. Read More

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http://dx.doi.org/10.1016/j.humpath.2018.07.002DOI Listing
November 2018
18 Reads

The history of lung transplantation in Hong Kong.

J Thorac Dis 2018 Jun;10(Suppl 16):S1899-S1904

Department of Cardiothoracic Surgery, Queen Mary Hospital, Hong Kong, China.

Clinical lung transplant was first performed in Hong Kong in 1995. In the early years, the volume of activity was very low. There has been a clear trend of increasing volume in the past few years. Read More

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http://dx.doi.org/10.21037/jtd.2018.04.118DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6035933PMC
June 2018
14 Reads

An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit.

Case Rep Pathol 2018 29;2018:5749421. Epub 2018 May 29.

Department of Radiology, Clarion Hospital, Clarion, PA 16214, USA.

Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. Read More

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http://dx.doi.org/10.1155/2018/5749421DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5996408PMC
May 2018
10 Reads

Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.

Am J Surg Pathol 2018 10;42(10):1370-1383

Department of Pathology, Massachusetts General Hospital, Boston, MA.

Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms that may show overlapping morphology and immunohistochemistry with uterine smooth muscle tumors. In this study, we evaluated the morphologic, immunohistochemical, and molecular features of 32 PEComas, including 11 with aggressive behavior. Two distinct morphologies were observed: classic (n=30) and those with a lymphangioleiomyomatosis appearance (n=2). Read More

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http://Insights.ovid.com/crossref?an=00000478-201810000-0001
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http://dx.doi.org/10.1097/PAS.0000000000001119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6133752PMC
October 2018
30 Reads

Impact of flat chest on cadaveric lung transplantation: postoperative pulmonary function and survival.

Eur J Cardiothorac Surg 2018 Jul 6. Epub 2018 Jul 6.

Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Objectives: Thoracic cage flatness, the so-called flat chest, is a chest wall deformity associated with end-stage lung disease requiring lung transplantation. There is little information on the impact of flat chest on lung transplantation. The purpose of this study was to investigate whether flat chest directly influences respiratory function and survival after cadaveric lung transplantation. Read More

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http://dx.doi.org/10.1093/ejcts/ezy248DOI Listing
July 2018
7 Reads

Have Noninvasive Imaging Studies Supplanted the Need for Invasive Hemodynamics: Lessons Learned from Lymphangioleiomyomatosis.

Authors:
Joel A Strom

J Am Soc Echocardiogr 2018 Aug 3;31(8):902-904. Epub 2018 Jul 3.

Florida Polytechnic University, Lakeland, Florida. Electronic address:

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http://dx.doi.org/10.1016/j.echo.2018.06.003DOI Listing
August 2018
3 Reads