Results 41 to 50 of about 9,034 (208)

Unusual cause of dyspnea in patient with Myelofibrosis: The Ruxolitinib lung

Respiratory Medicine Case Reports
Although pulmonary complications are frequent in patients suffering from hematological diseases, secondary pulmonary alveolar proteinosis is a very rare complication of myelofibrosis.
Antoine El Kik, Maarten Vander Kuylen, Benjamin Bailly, Jennifer Fallas, Benjamin Bondue   +4 more
doaj   +1 more source

Proteinose alveolar congénita.

Acta Médica Portuguesa, 2005
Pulmonary Alveolar Proteinosis (PAP) is a rare entity characterized by the intra-alveolar accumulation of proteinaceous PAS positive and diastase resistant material. Its pathogenesis remains unclear, although a surfactant protein B deficiency and changes
Susana Pissarra, Gustavo Rocha, Inês Acevedo, Hercília Guimarães   +3 more
doaj   +1 more source

Assessment of Surfactant Protein A (SP-A) dependent agglutination [PDF]

, 2010
Background: Monomers of the collectin surfactant associated protein-A (SP-A) are arranged in trimers and higher oligomers. The state of oligomerization differs between individuals and likely affects SP-A's functional properties.
Stefanie M Heinrich, Matthias Griese
core   +3 more sources

Expert‐Designed Fact Sheets and AI‐Based Analysis of Patient Symptoms to Combat Diagnostic Delays in Inherited Metabolic Diseases

Journal of Inherited Metabolic Disease, Volume 49, Issue 2, March 2026.
ABSTRACT The importance of early diagnosis of inherited metabolic diseases (IMDs) is well known, as it allows early intervention to prevent or reduce complications and improve prognosis, since many of these disorders are treatable. However, diagnosis can still be delayed, and many patients remain undiagnosed. Reducing diagnosis delays is a primary goal
Aline Cano, Xiaoyi Chen, Azza Khemiri, Anais Brassier, Arnoux Jean‐Baptiste, Roseline Froissart, Juliette Bouchereau, Célia Hoebeke, Karin Mazodier, Bénédicte Héron, Philippe Labrune, Catherine Caillaud, David Cheillan, Yann Nadjar, Samia Pichard, Apolline Imbard, Magali Pettazzoni, Claire Douillard, Belmatoug Nadia, Anna‐Line Calatayud, Mounira Zerguini, Nicolas Garcelon, Jean‐François Benoist, Cécile Acquaviva, Pascale De Lonlay, the other members of the expert group consortium, Marie‐Thérèse Abi‐Warde, Cécile Acquaviva, Jean‐Baptiste Arnoux, Stéphanie Badiou, Magalie Barth, Nadia Belmatoug, Jean‐François Benoist, Juliette Bouchereau, Anais Brassier, Arnaud Bruneel, Catherine Caillaud, Aline Cano, Brigitte Chabrol, David Cheillan, Emmanuelle Corbe‐Guillard, Christelle Corne, Lena Damaj, Myriam Dao, Pascale De Lonlay, Anne‐Frédérique Dessein, Dries Dobbelaere, Claire Douillard, Thierry Dupré, François Feillet, Roseline Froissart, Margaux Gaschignard, Magali Gorce, Laurent Gouya, Anne‐Sophie Guemann, Bénédicte Héron, Célia Hoebeke, Apolline Imbard, Elsa Kaphan, François Labarthe, Philippe Labrune, Pascal Laforet, Thierry Levade, Elise Lebigot, Edouard Le Guillou, Olivier Lidove, Julien Maquet, Wladimir Mauhin, Clothilde Marbach, Karin Mazodier, Karine Mention, Fanny Mochel, Caroline Moreau, Yann Nadjar, Esther Noel, Mickael Obadia, Cécile Pagan, Magali Pettazzoni, Samia Pichard, Clement Pontoizeau, Aurélia Poujois, Isabelle Redonnet‐Vernhet, Frédérique Sabourdy, Manuel Schiff, Christine Serratrice, Aude Servais, Caroline Sevin, Anne Spraul, Bénédicte Sudrié, Marine Tardieu, Sandrine Vuillaumier, Camille Wicker, Arnaud Wiedemann‐Fode, Vincent Barlogis, Nathalie Boddaert, Kanetee Busiah, Annabelle Chaussenot, Dominique Debray, Céline Falaise, Muriel Girard, Dalila Habes, Annie Harroche, Florence Lacaille, Mehdi Oualha, Caroline Ovaert, Rachel Reynaud, Caroline Rousset‐Rouvière, Cécile Rouzier, Karim Wahbi   +108 more
wiley   +1 more source

Serial bronchoscopic lung lavage in pulmonary alveolar proteinosis under local anesthesia

Lung India, 2015
Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by alveolar accumulation of surfactant composed of proteins and lipids due to defective surfactant clearance by alveolar macrophages.
K Rennis Davis, D Thomas Vadakkan, E V Krishnakumar, A Muhammed Anas   +3 more
doaj   +1 more source

Autoimmune hyperphosphatemic tumoral calcinosis in a patient with FGF23 autoantibodies [PDF]

, 2018
Hyperphosphatemic familial tumoral calcinosis (HFTC)/hyperostosis-hyperphosphatemia syndrome (HHS) is an autosomal recessive disorder of ectopic calcification due to deficiency of or resistance to intact fibroblast growth factor 23 (iFGF23). Inactivating
Burbelo, Christopher J. Romero, Daniela Egli-Spichtig, Emily Farrow, Farzana Perwad, Ichikawa, Lori C. Guthrie, Mary Scott Roberts, Michael J. Econs, Michael T. Collins, Peter D. Burbelo, Rachel I. Gafni, Shimada, Shoji Ichikawa, Uchida   +14 more
core   +1 more source

Whole lung lavage combined with Granulocyte-macrophage colony stimulating factor inhalation for an adult case of refractory pulmonary alveolar proteinosis [PDF]

, 2014
BACKGROUND: Whole-lung lavage (WLL) is classically the first-line treatment for symptomatic pulmonary alveolar proteinosis (PAP). However, some patients require multiple WLLs because of refractory nature of their PAP. In this instance, these patients may
Hong yan Yu, Hui Huang, Xue feng Sun, Yan xun Wang, Zuo jun Xu   +4 more
core   +2 more sources

Innate immune activation by inhaled lipopolysaccharide, independent of oxidative stress, exacerbates silica-induced pulmonary fibrosis in mice [PDF]

, 2012
Acute exacerbations of pulmonary fibrosis are characterized by rapid decrements in lung function. Environmental factors that may contribute to acute exacerbations remain poorly understood.
AG Heppleston, AJ Ghio, B Yucesoy, Bernhard Ryffel, C Dostert, C Shimbori, CA Beamer, CA Beamer, CA Beamer, Cheryl L. Fattman, CL Wohlford-Lenane, David M. Brass, DM Brass, DM Brass, DM Brass, DS Kim, DS Kim, DW Porter, EN Potts-Kant, Erin Potts-Kant, F Martinon, H Li, HB Yu, HY Cho, IC Papanikolaou, IN Park, JB Kornum, Jennifer C. Spencer, JF Woessner Jr, JM Peters, John W. Hollingsworth, Joseph D. Latoche, JW Song, K Boon, KD Srivastava, KM Vannella, M Corbel, M Sarih, M Selman, Mary K. Dunkel, MH Lowry, P Li, RF Hamilton Jr, Richard L. Auten, RJ Langley, RM Gilberti, S Lo Re, Sarah M. Reilly, SH Rosen, SL Cassel, TR McMillan, V Hornung, V Rabolli, Y Kondoh, YW Tang, Zhuowei Li   +55 more
core   +9 more sources

Genetic determinants of risk in autoimmune pulmonary alveolar proteinosis

Nature Communications, 2021
Autoimmune pulmonary alveolar proteinosis (aPAP) is a complex lung disease caused by abnormal surfactant homeostasis. Here, the authors carry out a genome-wide association study of aPAP in a Japanese cohort, finding variants in the MHC and suggesting ...
Saori Sakaue, Etsuro Yamaguchi, Yoshikazu Inoue, Meiko Takahashi, Jun Hirata, Ken Suzuki, Satoru Ito, Toru Arai, Masaki Hirose, Yoshinori Tanino, Takefumi Nikaido, Toshio Ichiwata, Shinya Ohkouchi, Taizou Hirano, Toshinori Takada, Satoru Miyawaki, Shogo Dofuku, Yuichi Maeda, Takuro Nii, Toshihiro Kishikawa, Kotaro Ogawa, Tatsuo Masuda, Kenichi Yamamoto, Kyuto Sonehara, Ryushi Tazawa, Konosuke Morimoto, Masahiro Takaki, Satoshi Konno, Masaru Suzuki, Keisuke Tomii, Atsushi Nakagawa, Tomohiro Handa, Kiminobu Tanizawa, Haruyuki Ishii, Manabu Ishida, Toshiyuki Kato, Naoya Takeda, Koshi Yokomura, Takashi Matsui, Masaki Watanabe, Hiromasa Inoue, Kazuyoshi Imaizumi, Yasuhiro Goto, Hiroshi Kida, Tomoyuki Fujisawa, Takafumi Suda, Takashi Yamada, Yasuomi Satake, Hidenori Ibata, Nobuyuki Hizawa, Hideki Mochizuki, Atsushi Kumanogoh, Fumihiko Matsuda, Koh Nakata, Tomomitsu Hirota, Mayumi Tamari, Yukinori Okada   +56 more
doaj   +1 more source

Macrophages and lipid metabolism [PDF]

, 2018
Distinct macrophage populations throughout the body display highly heterogeneous transcriptional and epigenetic programs. Recent research has highlighted that these profiles enable the different macrophage populations to perform distinct functions as ...
Remmerie, Anneleen, Scott, Charlotte
core   +1 more source