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Lymphangioleiomyomatosis and Langerhans cell histiocytosis - two case reports from our practice [PDF]

Srpski Arhiv za Celokupno Lekarstvo, 2022
Introduction. Lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are the most common pulmonary cystic diseases. Although they differ in pathogenesis, they share several features.
Milenković Ana, Ilić Miroslav, Javorac Jovan, Savić Nevena, Lalić Nensi   +4 more
doaj   +1 more source

Lymphangioleiomyomatosis biomarkers linked to lung metastatic potential and cell stemness [PDF]

, 2016
Lymphangioleiomyomatosis (LAM) is a rare lung-metastasizing neoplasm caused by the proliferation of smooth muscle-like cells that commonly carry loss-of-function mutations in either the tuberous sclerosis complex 1 or 2 (TSC1 or TSC2) genes.
Aguilar, Helena, Ancochea Bermúdez, Julio, Boni, Jacopo, Campos, Miriam, Casanova, Álvaro, Climent, Fina, Cordero, Alex, Esteller, Manel, Extremera, Ana I., Falo, Catalina, Fernández, Adela, García, Nadia, Gil, Miguel, González Suárez, Eva, Gumà i Garcia, Anna Maria, Gómez Baldó, Laia, Hernández-Losa, Javier, Herranz, Carmen, Laporta, Rosalía, Llatjós, Roger, Llorente, Alicia, López, José Ignacio, Mateo González, Francesca, Molina Molina, María, Morilla, Idoia, Ortega, Raúl, Pernas, Sònia, Petit, Anna, Pla Farnós, María Jesús, Pujana Genestar, M. Ángel, Román, Antonio, Ruiz de Garibay, Gorka, Sanchez-Mut, Jose Vicente, Serra-Musach, Jordi, Ussetti, Piedad, Valenzuela, Claudio, Varela Rodríguez, Mar, Vidal-Bel, August, Xaubet Mir, Antonio   +38 more
core   +1 more source

Recurrent pneumothoraces with diffuse small ground‐glass and solid nodules

Respirology Case Reports, 2021
Multifocal micronodular pneumocyte hyperplasia (MMPH) is the lesser known pulmonary manifestation of tuberous sclerosis. It manifests radiologically as diffuse small ground‐glass and solid nodules.
Li Yan Sandra Hui, Xin Min Cheng, Ing Xiang Soo, Su Ying Low   +3 more
doaj   +1 more source

Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: a phase 2 trial [PDF]

, 2011
This study showed sustained regression of renal angiomyolipomas in patients with tuberous sclerosis or sporadic LAM receiving 2 years of sirolimus treatment.
Cox, J A, Cross, J J, Davies, D M, de Vries, P J, Doyle, T, Howe, C J, Johnson, S R, Kingswood, J C, McCartney, D L, Pointon, K, Sampson, J R, Serra, A L, Tattersfield, A E, Watson, P C   +13 more
core   +1 more source

A rare lung disease diagnosed by CT in a young pregnant woman

Clinical Case Reports, 2021
Lymphangioleiomyomatosis (LAM) is an unusual lung disease which can be diagnosed by its characteristic appearance on a high‐resolution CT, and may not always require a biopsy.
Sunil Persad, Nadiya Dowlath, Michael Prasad, Vanessa Harry   +3 more
doaj   +1 more source

Anesthesia for Suboccipital Craniotomy in a Patient with Lymphangioleiomyomatosis: A Case Report [PDF]

, 2012
Lymphangioleiomyomatosis (LAM) is a rare pulmonary condition often presenting with spontaneous pneumothorax. Imaging or biopsy confirm the diagnosis.
Luman, Emily, Martuza, Robert Lawrence, Peterfreund, Robert Andrew   +2 more
core   +3 more sources

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Orphanet Journal of Rare Diseases, 2021
Background Lymphangioleiomyomatosis (LAM) is a rare disease that almost exclusively affects women of reproductive age. Patients are warned of the increased risks if they become pregnant.
Lisha Shen, W. Xu, Jinsong Gao, Jun Wang, Jiannan Huang, Yani Wang, Yudi He, Yanli Yang, X. Tian, Kaifeng Xu   +9 more
semanticscholar   +1 more source

Identification of a novel TSC2 c.3610G > A, p.G1204R mutation contribute to aberrant splicing in a patient with classical tuberous sclerosis complex: a case report [PDF]

, 2018
Background: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by hamartomas in any organ systems. Mutations in the TSC1 or TSC2 gene lead to the dysfunction of hamartin or tuberin proteins, which cause tuberous sclerosis ...
Bottillo, I, Han, Y, Lang, Y, Liu, X, Shao, L., Wang, J, Wang, Q, Zhang, R   +7 more
core   +2 more sources

Assessment of Lung Structure and Regional Function Using 0.55 T MRI in Patients With Lymphangioleiomyomatosis

Investigative Radiology, 2021
Objectives Contemporary lower-field magnetic resonance imaging (MRI) may offer advantages for lung imaging by virtue of the improved field homogeneity. The aim of this study was to evaluate the utility of lower-field MRI for combined morphologic imaging ...
Ipshita Bhattacharya, R. Ramasawmy, A. Javed, Margaret Lowery, Jennifer L. Henry, C. Mancini, T. Machado, A. Jones, P. Julien‐Williams, R. Lederman, R. Balaban, Marcus Y. Chen, J. Moss, Adrienne E Campbell-Washburn   +13 more
semanticscholar   +1 more source

Successful management of bilateral refractory chylothorax after double lung transplantation for lymphangioleiomyomatosis

Annals of Thoracic Medicine, 2014
Lymphangioleiomyomatosis (LAM) is a rare disease that leads to airways and lymphatic channels obstruction due to abnormal smooth muscle proliferation. It presents with dyspnea, pneumothorax or chylothorax.
Mohammed Hussein, Yasser M Aljehani, Imran Nizami, Waleed Saleh   +3 more
doaj   +1 more source