Results 11 to 20 of about 61,025 (285)

Pulmonary alveolar proteinosis [PDF]

Respirology, 2020
ABSTRACTPAP is an ultra‐rare disease in which surfactant components, that impair gas exchange, accumulate in the alveolae. There are three types of PAP. The most frequent form, primary PAP, includes autoimmune PAP which accounts for over 90% of all PAP, defined by the presence of circulating anti‐GM‐CSF antibodies.
Jouneau, Stéphane, Menard, Cedric, Lederlin, Mathieu   +2 more
semanticscholar   +6 more sources

Autoimmune pulmonary alveolar proteinosis successfully treated with lung lavage in an adolescent patient: a case report

Journal of Medical Case Reports, 2021
Background Pulmonary alveolar proteinosis is a rare interstitial lung disease characterized by accumulating surfactant materials in the alveoli. The autoimmune form is by far the most common in adults, while in the pediatric age group, the vast majority ...
Abdalla Mohmed Alasiri, Reem Abdullah Alasbali, Meaad Ali Alaqil, Aishah Marei Alahmari, Nouf Dagash Alshamrani, Rabab Nasir Badri   +5 more
doaj   +2 more sources

Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies

Frontiers in Immunology, 2023
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder that is characterized by the abnormal accumulation of surfactant within the alveoli. Alveolar macrophages (AMs) have been identified as playing a pivotal role in the pathogenesis of PAP ...
Xinmei Huang, Xinmei Huang, Mengshu Cao, Mengshu Cao, Mengshu Cao, Mengshu Cao, Yonglong Xiao, Yonglong Xiao   +7 more
doaj   +2 more sources

Autoimmune pulmonary alveolar proteinosis in children

ERJ Open Research, 2022
In childhood, a multitude of causes lead to pulmonary alveolar proteinosis (PAP), an excessive surfactant accumulation in the alveolar space, limiting gas exchange.
Matthias Griese, Panagiota Panagiotou, Effrosyni D. Manali, Mirjam Stahl, Nicolaus Schwerk, Vanessa Costa, Konstantinos Douros, Maria Kallieri, Ruth Maria Urbantat, Horst von Bernuth, Lykourgos Kolilekas, Lurdes Morais, Ana Ramos, Kerstin Landwehr, Katrin Knoflach, Florian Gothe, Karl Reiter, Vassiliki Papaevangelou, Athanasios G. Kaditis, Christina Kanaka-Gantenbein, Spyros A. Papiris   +20 more
doaj   +2 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   +2 more sources

Congenital Pulmonary Alveolar Proteinosis [PDF]

Case Reports in Pediatrics, 2013
Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been
Saber Hammami, Khaled Harrathi, Khaled Lajmi, Samir Hadded, Chebil Ben Meriem, Mohamed Néji Guédiche   +5 more
doaj   +3 more sources

Intestinal Behçet’s disease complicated by myelodysplastic syndrome and secondary pulmonary alveolar proteinosis: a case report

BMC Gastroenterology, 2021
Background Gastrointestinal lesions, which sometimes develop in Behçet’s disease (BD), are referred to as intestinal BD. Although rare, intestinal BD can be accompanied by myelodysplastic syndrome (MDS) with abnormal karyotype trisomy 8, which is ...
Hiroshi Shimizu, Shuzo Sato, Tomohiro Suzuki, Tomomi Sasajima, Yosuke Takahata, Nobuhiko Shinohara, Kosuke Hideshima, Yuko Yokokawa, Nobuo Matsuhashi, Osamu Ichii, Mayumi Tai, Yutaka Ejiri, Kiori Yano, Takayuki Ikezoe, Hiromasa Ohira, Kiyoshi Migita   +15 more
doaj   +2 more sources

Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis

Therapeutics and Clinical Risk Management, 2021
Hira Iftikhar,1 Girish B Nair,1 Anupam Kumar2 1Division of Pulmonary and Critical Care, Beaumont Health, OUWB School of Medicine, Royal Oak, MI, USA; 2Division of Pulmonary and Critical Care, Baylor College of Medicine, Houston, TX, USACorrespondence ...
Iftikhar H, Nair GB, Kumar A
doaj   +2 more sources

Therapeutic Whole Lung Lavage for Pulmonary Alveolar Proteinosis: Technique With Continuous Video‐Enabled Double Lumen Endotracheal Tube [PDF]

Respirology Case Reports
Therapeutic whole lung lavage with a continuous video‐enabled double‐lumen endotracheal tube improves the safety of therapeutic whole lung lavage in autoimmune pulmonary alveolar proteinosis.
Kevin Davidson
doaj   +2 more sources

Autoimmune pulmonary alveolar proteinosis exacerbated by steroid therapy due to misdiagnosis as anti-aminoacyl-tRNA synthetase (ARS) antibody positive- interstitial pneumonia: a case report

BMC Pulmonary Medicine, 2022
Background Anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies are myositis-specific autoantibodies that have been identified in a subset of patients with interstitial pneumonia who do not present with dermatomyositis or polymyositis.
Hiroshi Ishimoto, Noriho Sakamoto, Hirokazu Yura, Atsuko Hara, Takashi Kido, Hiroyuki Yamaguchi, Kazuko Yamamoto, Yasushi Obase, Yuji Ishimatsu, Minoru Satoh, Hiroshi Mukae   +10 more
doaj   +2 more sources