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https://www.readbyqxmd.com/read/28219570/living-donor-lung-transplantation-after-bone-marrow-transplantation-for-chediak-higashi-syndrome
#1
Tetsu Yamada, Toyofumi F Chen-Yoshikawa, Shigeharu Oh, Rieko Ito-Taniguchi, Fumiaki Gochi, Masaaki Sato, Akihiro Aoyama, Hiroshi Date
An 8-year-old girl with Chediak-Higashi syndrome (CHS) had pulmonary complications after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) and eventually underwent single living-donor lobar lung transplantation (LDLLT). Electron micrographic findings showed vagus nerve tissue in extracted lung having granular inclusions, which are pathognomonic for CHS. Because her mother was the donor for both hematopoietic stem cell and lung transplantations, she was weaned from immunosuppression and is doing well 3 years after lung transplantation...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28217691/%C3%AE-1-antitrypsin-deficiency-a-misfolded-secretory-protein-variant-with-unique-effects-on-the-endoplasmic-reticulum
#2
David H Perlmutter
In the classical form of α1-antitrypsin deficiency (ATD) a point mutation leads to accumulation of a misfolded secretory glycoprotein in the endoplasmic reticulum (ER) of liver cells and so ATD has come to be considered a prototypical ER storage disease. It is associated with two major types of clinical disorders, chronic obstructive pulmonary disease (COPD) by loss-of-function mechanisms and hepatic cirrhosis and carcinogenesis by gain-of-function mechanisms. The lung disease predominantly results from proteolytic damage to the pulmonary connective tissue matrix because of reduced levels of protease inhibitor activity of α1-anitrypsin (AT) in the circulating blood and body fluids...
September 2016: Endoplasmic Reticulum Stress in Diseases
https://www.readbyqxmd.com/read/28195247/type-ii-congenital-pulmonary-airway-malformation-associated-with-intralobar-pulmonary-sequestration-report-of-a-case-and-review-of-classification-criteria
#3
M G Mastrogiulio, A Barone, M G Disanto, A Ginori, M R Ambrosio, S F Carbone, D Spina
Pulmonary congenital abnormalities are rare disorders including congenital pulmonary airway malformations (CPAM) and pulmonary sequestration (PS). CPAM is a lesion characterized by the presence of anomalous bronchiolar or acinar structures, variable in size, either cystic or not cystic. PS is generally defined as nonfunctioning lung tissue that is not in normal continuity with the tracheobronchial tree and that derives its blood supply from systemic vessels. We describe a case of a baby girl with a very rare association between CPAM type 2 and intralobar pulmonary sequestration (IPS) focusing on the cystic lesions typical of CPAM and on the lymphatic and blood vessels...
March 2016: Pathologica
https://www.readbyqxmd.com/read/28179988/ips-derived-mscs-from-an-expandable-bank-to-deliver-a-prodrug-converting-enzyme-that-limits-growth-and-metastases-of-human-breast-cancers
#4
M Ullah, Y Kuroda, T J Bartosh, F Liu, Q Zhao, C Gregory, R Reger, J Xu, R H Lee, D J Prockop
One attractive strategy to treat cancers is to deliver an exogenous enzyme that will convert a non-toxic compound to a highly toxic derivative. The strategy was tested with viral vectors but was disappointing because the efficiency of transduction into tumor cells was too low. Recent reports demonstrated that the limitation can be addressed by using tissue-derived mesenchymal stromal cells (MSCs) to deliver enzyme/prodrug systems that kill adjacent cancer cells through bystander effects. Here we addressed the limitation that tissue-derived MSCs vary in their properties and are difficult to generate in the large numbers needed for clinical applications...
2017: Cell Death Discovery
https://www.readbyqxmd.com/read/28161607/targeting-the-canonical-nf-%C3%AE%C2%BAb-pathway-with-a-high-potency-ikk2-inhibitor-improves-outcomes-in-a-mouse-model-of-idiopathic-pneumonia-syndrome
#5
Kenneth A Fowler, Corey M Jania, Stephen L Tilley, Angela Panoskaltsis-Mortari, Albert S Baldwin, Jonathan S Serody, James M Coghill
Idiopathic pneumonia syndrome (IPS) is a non-infectious, inflammatory disorder of the lungs that occurs most often after fully myeloablative allogeneic hematopoietic stem cell transplantation (HSCT). IPS can be severe and is associated with high one year mortality rates despite existing therapies. The canonical NF-κB signaling pathway has previously been linked to several inflammatory disorders of the lung including asthma and lung allograft rejection. It has never been specifically targeted as a novel IPS treatment approach, however...
February 1, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28138192/a-rare-cause-of-recurrent-chest-infection-in-children-bronchopulmonary-sequestration
#6
Shabbir Hussain, Shoaib Ahmed, Syed Awais Ul Hassan Shah, Saba Haider Tarar
Bronchopulmonary sequestration is one of the rare thoracic congenital anomalies. We report the case of a 6 year old boy with history of recurrent episodes of chest infection and breathing difficulty. This time admitted with fever and cough. Investigations revelaed neutrophilic leucocytosis, raised C-reactive protein and a retrocardiac opacity on chest radiographs. Contrast enhanced Computed Topography(CT scan) revealed a large, well defined, left lower lobe multi-loculated cystic mass with a vessel arising from the descending aorta supplying sequestrated lung portion...
February 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28006781/allogeneic-compact-bone-derived-mesenchymal-stem-cell-transplantation-attenuates-the-severity-of-idiopathic-pneumonia-syndrome-in-a-murine-bone-marrow-transplantation-model
#7
Shu-Kai Qiao, Han-Yun Ren, Yong-Jin Shi, Wei Liu
BACKGROUND/AIMS: Idiopathic pneumonia syndrome (IPS) is a serious and life-threatening lung complication following allogeneic hematopoietic stem cell transplantation (allo-HSCT) and currently no effective therapies exist. This study was designed to determine whether transplantation of allogeneic murine compact bone derived- mesenchymal stem cells (CB-MSCs) could prevent the development of IPS. METHODS: We tested the effects of CB-MSCs transplantation on IPS using an established murine model of C57BL/6 (H-2b)→BALB/c (H-2d)...
2016: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/27938474/human-muse-cells-non-tumorigenic-pluripotent-like-stem-cells-have-the-capacity-for-liver-regeneration-by-specific-homing-and-replenishment-of-new-hepatocytes-in-liver-fibrosis-mouse-model
#8
Masahiro Iseki, Yoshihiro Kushida, Shohei Wakao, Takahiro Akimoto, Masamichi Mizuma, Fuyuhiko Motoi, Ryuta Asada, Shinobu Shimizu, Michiaki Unno, Gregorio Chazenbalk, Mari Dezawa
Muse cells, a novel type of non-tumorigenic pluripotent-like stem cells reside in the bone marrow, skin and adipose tissue, are collectable as cells positive for pluripotent surface marker SSEA-3. They are able to differentiate into cells representative of all three germ layers. The capacity of intravenously injected human bone marrow-Muse cells to repair the liver fibrosis model of immunodeficient mice was evaluated in this study. They exhibited the ability for differentiation spontaneously into hepatoblast/hepatocyte-lineage cells and high migration toward the serum and liver tissue of carbon tetrachloride-treated mice in vitro...
2, 2016: Cell Transplantation
https://www.readbyqxmd.com/read/27922138/mitomycin-c-treated-human-induced-pluripotent-stem-cells-as-a-safe-delivery-system-of-gold-nanorods-for-targeted-photothermal-therapy-of-gastric-cancer
#9
Meng Yang, Yanlei Liu, Wenxiu Hou, Xiao Zhi, Chunlei Zhang, Xinquan Jiang, Fei Pan, Yuming Yang, Jian Ni, Daxiang Cui
Human-induced pluripotent stem cells (iPS) possess an intrinsic tumor tropism ability. However, iPS cells are impeded in clinical applications of tumor therapy due to the formation of teratomas and their survival in normal organs such as the liver, lungs, spleen and kidneys. Mitomycin C (MMC) can overcome this limitation by suppressing iPS proliferation. Herein, we fabricated a safe delivery system of iPS cells treated with MMC loading with gold nanorods (AuNRs) for the targeted photothermal treatment of gastric cancer...
January 7, 2017: Nanoscale
https://www.readbyqxmd.com/read/27895584/induced-pluripotent-stem-cells-inhibit-bleomycin-induced-pulmonary-fibrosis-in-mice-through-suppressing-tgf-%C3%AE-1-smad-mediated-epithelial-to-mesenchymal-transition
#10
Yan Zhou, Zhong He, Yuan Gao, Rui Zheng, Xiaoye Zhang, Li Zhao, Mingqi Tan
Pulmonary fibrosis is a progressive and irreversible fibrotic lung disorder with high mortality and few treatment options. Recently, induced pluripotent stem (iPS) cells have been considered as an ideal resource for stem cell-based therapy. Although, an earlier study demonstrated the therapeutic effect of iPS cells on pulmonary fibrosis, the exact mechanisms remain obscure. The present study investigated the effects of iPS cells on inflammatory responses, transforming growth factor (TGF)-β1 signaling pathway, and epithelial to mesenchymal transition (EMT) during bleomycin (BLM)-induced lung fibrosis...
2016: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/27705974/myeloperoxidase-anti-neutrophil-cytoplasmic-antibody-positive-interstitial-pneumonia-associated-with-granulomatosis-with-polyangiitis-diagnosed-by-surgical-lung-biopsy
#11
Haruka Chino, Eri Hagiwara, Hideya Kitamura, Tomohisa Baba, Hideaki Yamakawa, Tamiko Takemura, Takashi Ogura
Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis that often involves the lung. However, interstitial pneumonia (IP) is rarely seen in GPA patients. We herein report 3 cases of IP associated with GPA diagnosed by surgical lung biopsy. High-resolution CT showed uniform subpleural reticular opacity with traction bronchiectasis. Biopsies from all 3 patients revealed neutrophilic capillaritis, microabscesses with giant cells, and coexisting histological findings of usual IP pattern or fibrosing nonspecific IP pattern...
2016: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/27628133/human-pluripotent-stem-cells-ipsc-generation-culture-and-differentiation-to-lung-progenitor-cells
#12
Mahboobe Ghaedi, Laura E Niklason
Induced pluripotent stem (iPS) cells are the product of adult somatic cell reprogramming to an embryonic-like state by inducing a "forced" expression of specific genes. They are similar to natural pluripotent stem cells, such as embryonic stem (ES) cells, in many aspects, such as the expression of certain stem cell genes and potency and differentiability. Human iPS cells are invaluable resource for basic research, cell therapy, drug discovery, and human organ tissue engineering. iPS cells can be derived from the patient to be treated and thus are genetically identical cells that may avoid immune rejection...
September 15, 2016: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27468336/rare-presentation-of-intralobar-pulmonary-sequestration-associated-with-repeated-episodes-of-ventricular-tachycardia
#13
D Sheshagiri Rao, Ramachandra Barik
Arterial supply of an intralobar pulmonary sequestration (IPS) from the coronary circulation is extremely rare. A significant coronary steal does not occur because of dual or triple sources of blood supply to sequestrated lung tissue. We present a 60-year-old woman who presented to us with repeated episodes of monomorphic ventricular tachycardia (VT) in last 3 mo. Radio frequency ablation was ineffective. On evaluation, she had right lower lobe IPS with dual arterial blood supply, i.e., right pulmonary artery and the systemic arterial supply from the right coronary artery (RCA)...
July 26, 2016: World Journal of Cardiology
https://www.readbyqxmd.com/read/27448666/loss-of-ccr2-signaling-alters-leukocyte-recruitment-and-exacerbates-%C3%AE-herpesvirus-induced-pneumonitis-and-fibrosis-following-bone-marrow-transplantation
#14
Stephen J Gurczynski, Megan C Procario, David N O'Dwyer, Carol A Wilke, Bethany B Moore
CCR2-expressing leukocytes are required for the progression of fibrosis in models of induced lung injury as well as models of bone marrow transplant (BMT)-related idiopathic pneumonia syndrome. Infection with murid γ-herpesvirus-68 (γHV-68) results in severe pneumonitis and pulmonary fibrosis following syngeneic BMT; however, the roles that various proinflammatory leukocyte populations play in this process remain unclear. Deletion of CCR2 in both non-BMT and BMT mice increased early lytic viral replication and resulted in a reduction in the numbers of lung-infiltrating GR1+,F4/80+ and CXCR1+ cells, while maintaining robust neutrophil infiltration...
September 1, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/27421574/high-resolution-ct-findings-of-idiopathic-pneumonia-syndrome-after-haematopoietic-stem-cell-transplantation-based-on-the-updated-concept-of-idiopathic-pneumonia-syndrome-by-the-american-thoracic-society-in-2011
#15
REVIEW
N Tanaka, Y Kunihiro, T Kobayashi, T Yujiri, S Kido, K Ueda, N Matsunaga
Idiopathic pneumonia syndrome (IPS) is an acute lung dysfunction of non-infectious aetiology and a severe complication following haematopoietic stem cell transplantation (HSCT). Recently, the American Thoracic Society (ATS) updated the concept of IPS and extended the concept to a wider range; it defined IPS as "an idiopathic syndrome of pneumopathy after HSCT, with evidence of widespread alveolar injury and in which infectious aetiologies and cardiac dysfunction, acute renal failure, or iatrogenic fluid overload have been excluded...
October 2016: Clinical Radiology
https://www.readbyqxmd.com/read/27342496/-clinical-characteristics-of-30-hodgkin-s-lymphoma-patients-with-extranodal-involvement
#16
Yan-Fen Li, Yu Zhao, Jian Bo, Ying Zhou, Chao Ma, Quan-Shun Wang, Li Yu, Dai-Hong Liu
OBJECTIVE: To investigate the clinical characteristics, treatment outcomes, prognosis, delayed toxicity of Hodgkin's lymphoma with extranodal Involvement. METHODS: Thirty patients newly diagnosed as Hodgkin's lymphoma with extranodal involvement from April 2008 to September 2014 were retrospectively analyzed. RESULTS: Twenty-seven patients suffered from the advanced-stage diseases, their major pathological changes were nodular sclerosis and mixed cellular type, the most commonly involeved extranodal sites were the lung and bones, followed by the liver, stomach and intestine...
June 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27341307/positive-feedback-loop-of-oct4-and-c-jun-expedites-cancer-stemness-in-liver-cancer
#17
Kung-Kai Kuo, King-Teh Lee, Ker-Kong Chen, Ya-Han Yang, Ying-Chu Lin, Ming-Ho Tsai, Kenly Wuputra, Yen-Liang Lee, Chia-Chen Ku, Hiroyuki Miyoshi, Yukio Nakamura, Shigeo Saito, Chun-Chieh Wu, Chee-Yin Chai, Richard Eckner, Chen-Lung Steve Lin, Sophie S-W Wang, Deng-Chyang Wu, Chang-Shen Lin, Kazunari K Yokoyama
The network of stemness genes and oncogenes in human patient-specific reprogrammed cancer stem cells (CSCs) remains elusive, especially in liver cancer. HepG2-derived induced pluripotent stem cell-like cells (HepG2-iPS-like cells) were generated by introducing Yamanaka factors and the knockdown vector shTP53. They exhibited features of stemness and a higher tumorigenesis after xenograft transplantation compared with HepG2 cells. The cancerous mass of severe combined immunodeficiency (SCID) mice derived from one colony was dissected and cultured to establish reprogrammed HepG2-derived CSC-like cells (designated rG2-DC-1C)...
June 24, 2016: Stem Cells
https://www.readbyqxmd.com/read/27324730/influence-of-stapling-the-intersegmental-planes-on-lung-volume-and-function-after-segmentectomy
#18
Hiroyuki Tao, Toshiki Tanaka, Tatsuro Hayashi, Kumiko Yoshida, Masashi Furukawa, Koichi Yoshiyama, Kazunori Okabe
OBJECTIVES: Dividing the intersegmental planes with a stapler during pulmonary segmentectomy leads to volume loss in the remnant segment. The aim of this study was to assess the influence of segment division methods on preserved lung volume and pulmonary function after segmentectomy. METHODS: Using image analysis software on computed tomography (CT) images of 41 patients, the ratio of remnant segment and ipsilateral lung volume to their preoperative values (R-seg and R-ips) was calculated...
October 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/27296616/engineering-a-perfusable-3d-human-liver-platform-from-ips-cells
#19
Arnout Schepers, Cheri Li, Arnav Chhabra, Benjamin Tschudy Seney, Sangeeta Bhatia
In vitro models of human tissue are crucial to our ability to study human disease as well as develop safe and effective drug therapies. Models of single organs in static and microfluidic culture have been established and shown utility for modeling some aspects of health and disease; however, these systems lack multi-organ interactions that are critical to some aspects of drug metabolism and toxicity. Thus, as part of a consortium of researchers, we have developed a liver chip that meets the following criteria: (1) employs human iPS cells from a patient of interest, (2) cultures cells in perfusable 3D organoids, and (3) is robust to variations in perfusion rate so as to be compatible in series with other specialized tissue chips (e...
July 5, 2016: Lab on a Chip
https://www.readbyqxmd.com/read/27214296/venous-sampling-for-cushing-disease-comparison-of-internal-jugular-vein-and-inferior-petrosal-sinus-sampling
#20
Martin G Radvany, Alfredo Quinones-Hinojosa, Gary L Gallia, Gary S Wand, Roberto Salvatori
OBJECTIVE: Because magnetic resonance imaging (MRI) fails to detect many adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas, inferior petrosal sinus sampling (IPSS) is considered the gold standard to differentiate Cushing disease (CD) from ectopic ACTH secretion syndrome (EAS). Some authors have suggested internal jugular vein sampling (IJVS) as an alternative to IPSS. METHODS: We simultaneously compared IJVS to IPSS in 30 consecutive patients referred for ACTH-dependent Cushing syndrome and equivocal MRI exams...
September 2016: Endocrine Practice
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