1,681 results match your criteria Lymphangioleiomyomatosis


Isolated Cystic Lung Disease: An Algorithmic Approach to Distinguishing Birt-Hogg-Dubé Syndrome, Lymphangioleiomyomatosis, and Lymphocytic Interstitial Pneumonia.

AJR Am J Roentgenol 2019 Mar 19:1-5. Epub 2019 Mar 19.

1 Department of Radiology, National Jewish Health, Denver, CO.

Objective: Birt-Hogg-Dubé (BHD) syndrome, lymphangioleiomyomatosis (LAM), and lymphocytic interstitial pneumonia (LIP) frequently present as isolated cystic lung disease and can be challenging to distinguish. If imaging findings are otherwise unremarkable, the radiologist is unaided by ancillary CT findings in narrowing the diagnosis. We hypothesized that the distribution and morphologic features of lung cysts could be used to differentiate BHD syndrome, LAM, and LIP. Read More

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http://dx.doi.org/10.2214/AJR.18.20920DOI Listing

Effects of Sirolimus on Lung function in patients with Lymphangioleiomyomatosis.

Caspian J Intern Med 2019 ;10(1):7-10

Yazd Diabetes Research Center, Shaheed Sadoughi University of Medical Sciences, Yazd, Iran.

Background: Lymphangioleiomyomatosis (LAM) is a progressive lungs disease that affects women at reproductive years. Sirolimus inhibits mammalian target of rapamycin (mTOR) and its administration in past studies was hopeful in treatment of patients with LAM. The aim of this study was to evaluate sirolimus therapy on lung function in LAM patients. Read More

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http://dx.doi.org/10.22088/cjim.10.1.7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6386328PMC
January 2019
1 Read

Analysis of the MILES Cohort Reveals Determinants of Disease Progression and Treatment Response in Lymphangioleiomyomatosis.

Eur Respir J 2019 Mar 7. Epub 2019 Mar 7.

From the University of Cincinnati (FXM, NG, BCT) and Cincinnati Children's Hospital Medical Center, Cincinnati (BCT, KS), Vanderbilt University Medical Center (LRY), National Kinki-Chou Hospital, Osaka, Japan (YI), National Institutes of Health, National Heart, Lung, and Blood Institute, Bethesda (JM. and AT-D), University of Toronto, Canada (LGS), Medical University of South Carolina, Charleston (CS), Niigata University Medical and Dental Hospital, Japan (KN), Oregon Health and Science University, Portland (AFB), Cleveland Clinic Abu Dhabi, UAE (JTC), University of Texas (JMS), University of Florida, Gainesville (MLB), University of Texas Health Sciences Center, Tyler (J.M.S.), National Jewish Health and the University of Colorado, Denver and Aurora (KKB and GPD), University of California, Los Angeles, (JPL), Harvard Medical School, Boston (HJG), and University of South Florida, Tampa (HSL, JPK).

Rationale: The Multicenter International Lymphangioleiomyomatosis (LAM) Efficacy of Sirolimus (MILES) trial revealed that sirolimus stabilised lung function in patients with moderately-severe LAM. The purpose of this study was to further examine the MILES cohort for the effects of racial, demographic, clinical, and physiologic patient characteristics on disease progression and treatment response in LAM.

Methods: MILES subjects were stratified on the basis of menopausal status (premenopausal/postmenopausal), race (Asian/Caucasian), bronchodilator responsiveness (present/absent), initial FEV1 (51-70% ≤50%), and TSC-association (yes/no). Read More

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http://dx.doi.org/10.1183/13993003.02066-2018DOI Listing
March 2019
2 Reads

Angiotensin-converting enzyme inhibitors may affect pulmonary function in lymphangioleiomyomatosis.

JCI Insight 2019 Mar 7;4(5). Epub 2019 Mar 7.

Pulmonary Branch and.

Introduction: A local renin-angiotensin system exists in the pulmonary nodules of lymphangioleiomyomatosis patients. Sirolimus, the standard treatment for lymphangioleiomyomatosis, stabilizes lung function, but all patients do not respond to or tolerate sirolimus. As renin-angiotensin systems may affect tumor growth and metastasis, we questioned if angiotensin-converting enzyme inhibitors affected lymphangioleiomyomatosis disease progression. Read More

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http://dx.doi.org/10.1172/jci.insight.126703DOI Listing
March 2019
1 Read

Serum vascular endothelial growth factor-D as a diagnostic and therapeutic biomarker for lymphangioleiomyomatosis.

PLoS One 2019 28;14(2):e0212776. Epub 2019 Feb 28.

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

Background: In lymphangioleiomyomatosis (LAM), tuberous sclerosis gene mutations activate the mechanistic target of the rapamycin pathway, resulting in vascular endothelial growth factor-D (VEGF-D) overproduction. While the utility of serum VEGF-D testing for the diagnosis of LAM is outlined in ATS/JRS LAM Guidelines, the assay has not been fully validated for Asian populations. Our aims were to validate serum VEGF-D testing in Japan, by directly comparing measurements in Japan and the U. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0212776PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395035PMC
February 2019
2 Reads

Rapalog resistance is associated with mesenchymal-type changes in Tsc2-null cells.

Sci Rep 2019 Feb 28;9(1):3015. Epub 2019 Feb 28.

Division of Pediatric Nephrology, Department of Pediatrics, College of Medicine, University of Tennessee Health Sciences Center, Memphis, TN, 38103, USA.

Tuberous Sclerosis Complex (TSC) and Lymphangioleiomyomatosis (LAM) are caused by inactivating mutations in TSC1 or TSC2, leading to mTORC1 hyperactivation. The mTORC1 inhibitors rapamycin and analogs (rapalogs) are approved for treating of TSC and LAM. Due to their cytostatic and not cytocidal action, discontinuation of treatment leads to tumor regrowth and decline in pulmonary function. Read More

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http://dx.doi.org/10.1038/s41598-019-39418-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395747PMC
February 2019
1 Read

Extrapulmonary uterine lymphangioleiomyomatosis (LAM) and dysfunctional uterine bleeding: the first presentation of LAM in a tuberous sclerosis complex patient.

BMJ Case Rep 2019 Feb 25;12(2). Epub 2019 Feb 25.

Department of Cellular Pathology, New Cross Hospital, Wolverhampton, UK.

Lymphangioleiomyomatosis (LAM) is a rare disease that typically affects women of childbearing age. It most commonly affects the lungs (P-LAM) but can occasionally occur in extra-pulmonary sites (E-LAM). There is a strong association between LAM and the tuberous sclerosis complex (TSC). Read More

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http://dx.doi.org/10.1136/bcr-2018-226358DOI Listing
February 2019
2 Reads

Lymphangioleiomyoma of the Uterus and Pelvic Lymph Nodes: A Report of 3 Cases, Including the Potentially Earliest Manifestation of Extrapulmonary Lymphangioleiomyomatosis.

Int J Gynecol Pathol 2019 Feb 14. Epub 2019 Feb 14.

Departments of Pathology (H.A., H.T., R.D., S.A.) Gynecology (Y.W., K.T., C.N., K.I., R.T., R.N., S.Y., T.T., A.W.), City Hospital of Toyonaka Department of Gynecology and Obstetrics, Osaka University Hospital (M.O.), Osaka, Japan.

We present 3 cases of extrapulmonary lymphangioleiomyomatosis (LAM) identified incidentally in uterine corpus and pelvic nodes resected for other reasons. One patient, a 47-yr-old female with corpus cancer, underwent a total hysterectomy and nodal dissection; the other 2 patients, aged 44 and 49 yr, underwent simple hysterectomy for corpus leiomyomas. None of the patients had evidence of tuberous sclerosis complex or any significant lesions in other organs. Read More

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http://Insights.ovid.com/crossref?an=00004347-900000000-9912
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http://dx.doi.org/10.1097/PGP.0000000000000589DOI Listing
February 2019
6 Reads

MR findings of uterine PEComa in patients with tuberous sclerosis: report of two cases.

Abdom Radiol (NY) 2019 Feb 18. Epub 2019 Feb 18.

Department of Radiology, University of Tsukuba Hospital, 2-1-1 Amakubo, Tsukuba, Ibaraki, 305-8576, Japan.

Tuberous sclerosis complex (TSC), a rare autosomal dominant neurocutaneous disorder, is characterized by the presence of benign congenital tumors in multiple organs. Neoplasms with perivascular epithelioid cell differentiation (PEComas), including angiomyolipoma (AML) and lymphangioleiomyomatosis (LAM), can occur in association with TSC. This report describes two cases of uterine PEComas presenting characteristic MR imaging features reflecting pathological findings. Read More

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http://dx.doi.org/10.1007/s00261-019-01918-3DOI Listing
February 2019
1 Read

Correlation between the native lung volume change and postoperative pulmonary function after single lung transplantation for lymphangioleiomyomatosis: Evaluation of lung volume by three-dimensional computed tomography volumetry.

PLoS One 2019 11;14(2):e0210975. Epub 2019 Feb 11.

Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.

Purpose: Whereas native lung overinflation has been thought to happen in recipients of single lung transplantation for lymphangioleiomyomatosis because of its increased compliance, there is no study that has reported the details on the change of the native lung volume after single lung transplantation by three-dimensional computed tomography volumetry. The purpose of the present study was to evaluate the lung volume after single lung transplantation for lymphangioleiomyomatosis by three-dimensional computed tomography volumetry and investigate the correlation between the native lung volume change and postoperative pulmonary function.

Methods: We retrospectively reviewed the data of 17 patients who underwent single lung transplantation for lymphangioleiomyomatosis. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210975PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6370208PMC
February 2019
1 Read

The National Heart, Lung, and Blood Institute Lymphangioleiomyomatosis Registry: Making LAMonade out of LAMons.

Authors:
Daniel Dilling

Chest 2019 Feb;155(2):249-250

Division of Pulmonary and Critical Care Medicine, Loyola University Chicago Stritch School of Medicine Ringgold Standard Institution, Maywood, IL. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00123692183249
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http://dx.doi.org/10.1016/j.chest.2018.09.013DOI Listing
February 2019
2 Reads

Clinicopathological analysis of clinically occult extrapulmonary lymphangioleiomyomatosis in intra-pelvic and para-aortic lymph nodes associated with pelvic malignant tumors: A study of nine patients.

Pathol Int 2019 Jan;69(1):29-36

Department of Pathology, Kobe City Medical Center General Hospital, Kobe, Japan.

The clinicopathological and immunohistochemical characteristics of clinically occult extrapulmonary lymphangioleiomyomatosis in lymph nodes (LN-LAM) being dissected during surgical staging of pelvic malignancy have not been well investigated. We assessed samples from nine female patients (median age, 61). None had past or familial history of tuberous sclerosis and had LAM lesions other than LN such as lung. Read More

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http://dx.doi.org/10.1111/pin.12749DOI Listing
January 2019
1 Read

The Genetics of Pneumothorax.

Am J Respir Crit Care Med 2019 Jan 25. Epub 2019 Jan 25.

Channing Division of Network Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, United States ;

A genetic influence on spontaneous pneumothoraces - those occurring without a traumatic or iatrogenic cause - is supported by several lines of evidence: 1) Pneumothorax can cluster in families (i.e. familial spontaneous pneumothorax); 2) Mutations in the FLCN gene have been found in both familial and sporadic cases; and 3) Pneumothorax is a known complication of several genetic syndromes. Read More

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https://www.atsjournals.org/doi/10.1164/rccm.201807-1212CI
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http://dx.doi.org/10.1164/rccm.201807-1212CIDOI Listing
January 2019
15 Reads

Evidence for a role of geranylgeranylation in renal angiomyolipoma and renal epithelioid angiomyolipoma.

Oncol Lett 2019 Feb 7;17(2):1523-1530. Epub 2018 Dec 7.

State Key Laboratory of Pharmaceutical Biotechnology and Jiangsu Key Laboratory of Molecular Medicine and School of Medicine, Nanjing University, Nanjing, Jiangsu 210093, P.R. China.

Research on mevalonate kinase deficiency has revealed that it may lead to the development of renal angiomyolipomas (RAMLs). Thus, it was suspected that geranylgeranyl pyrophosphate synthase (GGPPS), a key enzyme in the mevalonate pathway, may be involved in the development of RAMLs. In the present study, the expression of GGPPS in RAMLs and renal epithelioid angiomyolipomas (REAs) was assessed, and paraffin embedded specimens from 60 patients, including 9 cases with REA and 51 cases with RAML, were examined. Read More

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http://dx.doi.org/10.3892/ol.2018.9808DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341897PMC
February 2019
2 Reads

Nontuberculous mycobacterial infection after lung transplantation: a report of four cases.

Surg Case Rep 2019 Jan 23;5(1):11. Epub 2019 Jan 23.

Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita-shi, Osaka, Japan.

Background: Nontuberculous mycobacterium (NTM) infection in a patient in an immunosuppressed state caused by increased use of immunosuppressive or biological agents is a serious clinical problem. Mycobacterium avium complex is the most common involved pathogen, followed by Mycobacterium abscessus (MABSC), while Mycobacterium kansasii is not a major concern. The rate of infection rate in lung transplant recipients is reported to range from 1. Read More

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http://dx.doi.org/10.1186/s40792-019-0565-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346695PMC
January 2019
2 Reads

Differentiation Between Lymphangioleiomyomatosis and Birt-Hogg-Dubé Syndrome: Analysis of Pulmonary Cysts on CT Images.

AJR Am J Roentgenol 2019 Jan 23:1-7. Epub 2019 Jan 23.

2 Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Republic of Korea.

Objective: The purposes of this study were to identify diagnostic imaging markers for differentiating pulmonary cysts in lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome and to identify potential risk factors for spontaneous pneumothorax in the two diseases.

Materials And Methods: This retrospective study included 44 patients with lymphangioleiomyomatosis (44 women; mean age, 35 ± 10.9 years) and 13 patients with Birt-Hogg-Dubé syndrome (nine men, four women; mean age, 45. Read More

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https://www.ajronline.org/doi/10.2214/AJR.18.20232
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http://dx.doi.org/10.2214/AJR.18.20232DOI Listing
January 2019
13 Reads
2.731 Impact Factor

Use of CT Imaging to Quantify Progression and Response to Treatment in LAM.

Chest 2019 Jan 17. Epub 2019 Jan 17.

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

Background: In lymphangioleiomyomatosis (LAM), infiltration of the lungs with smooth muscle-like LAM cells results in cystic destruction and decline in lung function, effects stabilized by sirolimus therapy. LAM lung disease is followed, in part, by high-resolution CT scans. To obtain further information from these scans, we quantified changes in lung parenchyma by analyzing image "texture. Read More

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http://dx.doi.org/10.1016/j.chest.2019.01.004DOI Listing
January 2019
4 Reads

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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329093PMC
January 2019
11 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
1 Read

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
10 Reads

Serum Vascular Endothelial Growth Factor D in Cystic Lung Disease.

JAMA 2019 Jan;321(4):401-402

Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville.

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http://jama.jamanetwork.com/article.aspx?doi=10.1001/jama.20
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http://dx.doi.org/10.1001/jama.2018.20926DOI Listing
January 2019
5 Reads

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

Adv Mater 2019 Feb 27;31(7):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
February 2019
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
2 Reads

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
3 Reads

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
2 Reads

Air travel and incidence of pneumothorax in lymphangioleiomyomatosis.

Orphanet J Rare Dis 2018 12 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
3 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
7 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
3 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
3 Reads

Efficacy and safety of low-dose Sirolimus in Lymphangioleiomyomatosis.

Orphanet J Rare Dis 2018 11 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
10 Reads
3.358 Impact Factor

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
10 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
4 Reads

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

Orphanet J Rare Dis 2018 10 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
4 Reads

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

J Obstet Gynaecol Res 2019 Feb 19;45(2):482-486. 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
February 2019
14 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
2 Reads

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

Intern Med 2019 Feb 17;58(4):569-574. 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
February 2019
4 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
17 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
13 Reads

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

Chest 2019 Mar 3;155(3):528-533. 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 (LAM).

Methods: From November 2016 to May 2018, 105 consecutive subjects with LAM 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
March 2019
19 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
10 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
6 Reads

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

Orphanet J Rare Dis 2018 09 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
15 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
13 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
February 2019
13 Reads