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Lymphangioleiomyomatosis

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A single-center analysis of clinical features of 145 tuberous sclerosis complex—associated renal angiomyolipoma cases in China

Yushi Zhang, Xu Wang, Yang Zhao, Wenda Wang, Zhan Wang,

<title>Abstract</title> <p> <bold>Background:</bold> Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease with multiple organ system involvement. Renal angiomyolipoma (RAML) is a leading cause of death in TSC-adult patients. The aim of the study was to investigate the clinical features of tuberous sclerosis-associated renal angiomyolipoma among ... Read more >>

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Adoptive T Cell Transfer to Treat Lymphangioleiomyomatosis.

Fei Han, Emilia R Dellacecca, Levi W Barse, Cormac Cosgrove, Steven W Henning, Christian M Ankney, Dinesh Jaishankar, Alexander Yemelyanov, Vera P Krymskaya, Daniel F Dilling, I Caroline Le Poole,

Patients with lymphangioleiomyomatosis (LAM) develop pulmonary cysts associated with neoplastic, smooth muscle-like cells that feature neuroendocrine cell markers. The disease preferentially affects pre-menopausal women. Existing therapeutics do not cure LAM. As gp100 is a diagnostic marker expressed by LAM lesions, we proposed to target this immunogenic glycoprotein using TCR transgenic ... Read more >>

Am. J. Respir. Cell Mol. Biol. (American journal of respiratory cell and molecular biology)
[2020, :]

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Tuberous Sclerosis Complex: A review of the past, present and future.

Sanem Pinar Uysal, Mustafa Sahin,

Tuberous sclerosis complex (TSC) is an autosomal dominant, multisystem disorder that is characterized by cellular and tissue dysplasia in With the advent of genetic and molecular techniques, mutations in TSC1 or TSC2 genes were discovered to be responsible for mTOR overactivation, which is the underlying mechanism of pathogenesis. TSC is ... Read more >>

Turk J Med Sci (Turkish journal of medical sciences)
[2020, :]

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Fatal Hemoptysis Due to Endobronchial Aspergilloma in the Hyperinflated Native Lung after Single-Lung Transplantation for Lymphangioleiomyomatosis: A Case Report.

Asuka Yoshizaki, Masatsugu Yamamoto, Aya Hirabayashi, Yoshihiko Ono, Yukihisa Hatakeyama, Kyosuke Nakata, Daisuke Tamura, Motoko Tachihara, Hiroshi Kamiryo, Kazuyuki Kobayashi, Mari Nishio, Yoshihiro Nishimura,

Pulmonary lymphangioleiomyomatosis accounts for the majority of cadaveric lung transplantation cases. Post-transplantation management is continuingly necessary not only to prevent the progression of LAM but also to address complications. A woman with lymphangioleiomyomatosis underwent cadaveric lung transplantation. She developed post-operative native lung hyperinflation and hemoptysis with cavity shadow in the ... Read more >>

Kobe J Med Sci (The Kobe journal of medical sciences)
[2020, 65(4):E114-E117]

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A phase II clinical trial of the Safety Of Simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex.

Vera P Krymskaya, Andrew M Courtwright, Victoria Fleck, Daniel Dorgan, Robert Kotloff, Francis X McCormack, Maryl Kreider,

INRODUCTION:The mechanistic target of rapamycin inhibitors (mTORi) sirolimus and everolimus stabilize lung function in patients with pulmonary lymphangioleiomyomatosis (LAM) but do not induce remission. Pre-clinical studies suggest that simvastatin in combination with sirolimus induces LAM cell death. The objective of this study was to assess the safety of simvastatin with ... Read more >>

Respir Med (Respiratory medicine)
[2020, 163:105898]

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Effects of yoga on exercise capacity in patients with lymphangioleiomyomatosis: a nonrandomized controlled study.

Xiangfeng Li, Wenshuai Xu, Lu Zhang, Yi Zu, Yu Li, Yanli Yang, Ying Xiang, Yun Xiang, Ling Chen, Wei Liu, Lixia Chen, Kai-Feng Xu,

OBJECTIVE:To evaluate the effects of yoga on exercise capacity and quality of life in patients with lymphangioleiomyomatosis (LAM), a rare cystic lung disease in women. PATIENTS AND METHODS:This was a nonrandomized, controlled study conducted in Beijing, China (August 27, 2017 - April 26, 2018). Twenty-six participants were allocated to the ... Read more >>

Orphanet J Rare Dis (Orphanet journal of rare diseases)
[2020, 15(1):72]

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"T"eeing Up A Novel Therapy for Lymphangioleiomyomatosis.

Xiaoyun Wang, Joselyn Rojas-Quintero, Caroline A Owen,

Am. J. Respir. Cell Mol. Biol. (American journal of respiratory cell and molecular biology)
[2020, :]

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The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis.

Qi Wang, Mengqi Luo, Bo Xiang, Siyuan Chen, Yi Ji,

BACKGROUND:Lymphangioleiomyomatosis (LAM) is a rare, low-grade multisystem neoplastic disease. Most LAM patients are at a high risk of losing lung function at an accelerated rate and developing progressive dyspnea. Recently, several studies have reported their experience with pharmacological treatments for LAM. Therefore, we conducted a systematic review and meta-analysis to ... Read more >>

Respir. Res. (Respiratory research)
[2020, 21(1):55]

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Lessons on lymphangioleiomyomatosis: positivity and possibilities.

Stephanie Dreyer,

Lancet Respir Med (The Lancet. Respiratory medicine)
[2020, :]

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Quantitative analysis of computed tomography of the lungs in patients with lymphangioleiomyomatosis treated with sirolimus.

Yuki Ko, Katsuaki Asakawa, Kazunori Tobino, Tsuyoshi Oguma, Toyohiro Hirai, Toshinori Takada, Kazuhisa Takahashi, Kuniaki Seyama, ,

Objectives:We aimed to study sirolimus-related lung parenchymal changes by quantitative analysis of computed tomography (CT) of the lungs in patients with lymphangioleiomyomatosis (LAM). Methods:We studied 20 participants from the Multicenter Lymphangioleiomyomatosis Sirolimus Trial for Safety study, who had undergone both thin-section CT scans and pulmonary function tests at baseline, 12, ... Read more >>

Heliyon (Heliyon)
[2020, 6(2):e03345]

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Correction: Mutation spectrums of TSC1 and TSC2 in Chinese women with lymphangioleiomyomatosis (LAM).

Jie Liu, Weiwei Zhao, Xiaohua Ou, Zhen Zhao, Changming Hu, Mingming Sun, Feifei Liu, Junhao Deng, Weili Gu, Jiaying An, Qingling Zhang, Xiaoxian Zhang, Jiaxing Xie, Shiyue Li, Rongchang Chen, Shihui Yu, Nanshan Zhong,

[This corrects the article DOI: 10.1371/journal.pone.0226400.]. ... Read more >>

PLoS ONE (PloS one)
[2020, 15(1):e0228066]

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Neutrophil elastase from myeloid cells promotes TSC2-null tumor growth.

Manisha Taya, Maria Garcia-Hernandez, Javier Rangel-Moreno, Briaunna Minor, Erin Gibbons, Stephen R Hammes,

Chronic inflammation promotes progression of many cancers, with circulating myeloid-derived suppressor cell (MDSC) levels correlating with poor prognosis. Here we examine effects of MDSCs on lymphangioleiomyomatosis (LAM), a rare disease occurring almost exclusively in women whereby estrogen-sensitive metastatic TSC2-null tumors grow throughout the lungs, markedly reducing pulmonary function. The LAM ... Read more >>

Endocr. Relat. Cancer (Endocrine-related cancer)
[2020, :]

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Tuberous Sclerosis Complex with Lung Involvement.

Arthur Robert Kurzbuch, Jean-Yves Fournier,

J Neurosci Rural Pract (Journal of neurosciences in rural practice)
[2020, 11(1):211]

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Unconventional Estrogen Signaling in Health and Disease.

Christina L Faltas, Kira A LeBron, Marina K Holz,

Estrogen is a key hormone involved in the development and homeostasis of several tissue types in both males and females. By binding estrogen receptors, estrogen regulates essential functions of gene expression, metabolism, cell growth, and proliferation by acting through cytoplasmic signaling pathways or activating transcription in the nucleus. However, disruption ... Read more >>

Endocrinology (Endocrinology)
[2020, 161(4):]

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Zoledronic acid inhibits TSC2-null cell tumor growth via RhoA/YAP signaling pathway in mouse models of lymphangioleiomyomatosis.

Dandan Zhao, Jing Wu, Yinjuan Zhao, Wei Shao, Qi Cheng, Xiaoyan Shao, Xianwen Yuan, Juan Ye, Jianpu Gao, Meiling Jin, Chaojun Li, Xiaolin Chen, Yue Zhao, Bin Xue,

Background:This study is to investigate the effects of zoledronic acid (ZA) on TSC2-null cell proliferation and on the tumor progression and recurrence in mouse models of lymphangioleiomyomatosis (LAM). Methods:Subcutaneous mouse models and LAM mouse models were established. Immunohistochemistry and immunofluorescence were performed to detect the protein expression levels. TUNEL assay ... Read more >>

Cancer Cell Int. (Cancer Cell International)
[2020, 20:46]

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Lymphangioleiomyomatosis revealed by 68Ga-NOTA-Evans Blue PET/CT.

Guozhu Hou, Wenshuai Xu, Yuanyuan Jiang, Kai-Feng Xu, Xiaoyuan Chen, Fang Li, Wuying Cheng,

Eur. J. Nucl. Med. Mol. Imaging (European journal of nuclear medicine and molecular imaging)
[2020, :]

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Favorable survival even with high disease-specific complication rates in lymphangioleiomyomatosis after lung transplantation-long-term follow-up of a Japanese center.

Takeshi Kurosaki, Shinji Otani, Kentaroh Miyoshi, Mikio Okazaki, Seiichiro Sugimoto, Manabu Suno, Masaomi Yamane, Motomu Kobayashi, Takahiro Oto, Shinichi Toyooka,

BACKGROUND:Lung transplantation (LT) is a reliable therapeutic option for end-stage pulmonary lymphangioleiomyomatosis (LAM). Long-term outcome of LAM recipients after LT remains unknown. The aim of this study was to describe the outcomes of LT for LAM with a long-term follow-up, comparing those for other diseases in the same period. METHODS:We ... Read more >>

Clin Respir J (The clinical respiratory journal)
[2020, 14(2):116-123]

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Surgical Treatment of Chylothorax After Lung Transplantation for Lymphangioleiomyomatosis.

Francisco Javier González García, Carlos Baamonde Laborda, Anna Muñoz Fos, Paula Moreno Casado, Javier Redel Montero, Javier Algar Algar, Francisco Cerezo Madueño, David Poveda Chaves, Eloisa Ruiz López, Ángel Salvatierra Velázquez, Antonio Álvarez Kindelán,

Arch. Bronconeumol. (Archivos de bronconeumologia)
[2020, :]

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Clinical CT underestimation of the percentage volume occupied by cysts in patients with lymphangioleiomyomatosis.

Thomas C Larsen, Amir M Hasani, Shirley F Rollison, Tania R Machado, Amanda M Jones, Patricia Julien-Williams, Marcus Y Chen, Joel Moss, Han Wen,

PURPOSE:To evaluate the accuracy of cyst score measurements by standard high-resolution helical volume chest CT (HRCT) in patients with lymphangioleiomyomatosis (LAM), using a short z-length ultra-high resolution re-scan (UH re-scan) as the reference. In cystic lung diseases, cyst score is derived from CT scans and defined as the percentage of ... Read more >>

Clin Imaging (Clinical imaging)
[2020, 59(2):119-125]

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The pleura and the endocrine system.

Claudio Sorino, Stefano Negri, Antonio Spanevello, David Feller-Kopman,

The functioning of the pleura and the endocrine system are not entirely independent of each other. Some hormones can reach a greater concentration in the pleural exudate than in the blood. However, the clinical significance of this finding remains unknown. In some circumstances, hormonal changes are responsible for pathological manifestations ... Read more >>

Eur. J. Intern. Med. (European journal of internal medicine)
[2020, 72:34-37]

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Recent advances in human stem cell-based modeling of Tuberous Sclerosis Complex.

Wardiya Afshar Saber, Mustafa Sahin,

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by epilepsy, intellectual disability, and benign tumors of the brain, heart, skin, and kidney. Animal models have contributed to our understanding of normal and abnormal human brain development, but the construction of models that accurately recapitulate a human pathology remains ... Read more >>

Mol Autism (Molecular autism)
[2020, 11(1):16]

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Pulmonary Cystic Disease and Its Mimics.

Kirk D Jones,

Cystic diseases of the lung encompass a fairly broad variety of different diseases with causes including genetic abnormalities, smoking-related problems, developmental disorders, malignant neoplasms, and inflammatory processes. In addition, there are several diagnoses that closely resemble cystic lung disease, including cavitary diseases, cystic bronchiectasis, emphysema, and cystic changes in fibrosing ... Read more >>

Surg Pathol Clin (Surgical pathology clinics)
[2020, 13(1):141-163]

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Emerging Approaches to Predict Prognosis and Monitor Disease Progression in Lymphangioleiomyomatosis.

Evelyn Lynn, Marissa O'Callaghan, Abhilash Sahadevan, Cormac McCarthy,

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2019, 200(11):1424-1426]

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Total pleural coverage followed by lung transplantation in patient with lymphangioleiomyomatosis.

Do Hyung Kim, Hyo Yeong Ahn, Bong Soo Son, Joohyung Son,

Tuberous sclerosis complex lymphangioleiomyomatosis (TSC-LAM) is a rare disease, which may develop an intractable pneumothorax. Chemical or mechanical pleurodesis is a general management to prevent recurrence of pneumothorax, rendering it difficult to later dissect the pleura and control intraoperative bleeding. Since total pleural coverage (TPC) alternative to pleurodesis has been ... Read more >>

Gen Thorac Cardiovasc Surg (General thoracic and cardiovascular surgery)
[2019, :]

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Isolated sporadic uterine lymphangioleiomyoma with unusual clinical and pathological features.

Sebastian Leathersich, Mei Hui Eleanor Koay, Alireza Khani, Sangeeta Malla Bhat,

We report a unique uterine neoplasm, favoured to represent an isolated extrapulmonary lymphangioleiomyoma with unusual pathological features, in a postmenopausal woman without tuberous sclerosis complex. The large neoplasm consisted of smooth muscle fascicles and cystic spaces lined by lymphatic cells, which were negative for the melanocytic staining that is characteristically ... Read more >>

BMJ Case Rep (BMJ case reports)
[2019, 12(11):]

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