2,252 results match your criteria Alveolar Proteinosis


Increased Efficacy of Whole Lung Lavage Treatment in Alveolar Proteinosis Using a New Modified Lavage Technique.

J Bronchology Interv Pulmonol 2021 Jul;28(3):215-220

ILD Center of Excellence, Departments of Pulmonary Diseases.

Background: Autoimmune pulmonary alveolar proteinosis is an ultra-rare pulmonary disease. Whole lung lavage (WLL) is considered the gold standard therapy. We report a protocol for a new modified lavage technique (nMLT) in which controlled repetitive manual hyperinflation (MH) and intermittent chest percussion are used to enhance WLL efficacy. Read More

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Heterozygous gain-of-function variants cause an autoinflammatory immunodeficiency.

Sci Immunol 2021 Jun;6(60)

Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität München, Munich, Germany.

Analysis of autoinflammatory and immunodeficiency disorders elucidates human immunity and fosters the development of targeted therapies. Oligoadenylate synthetase 1 is a type I interferon-induced, intracellular double-stranded RNA (dsRNA) sensor that generates 2'-5'-oligoadenylate to activate ribonuclease L (RNase L) as a means of antiviral defense. We identified four de novo heterozygous gain-of-function variants in six patients with a polymorphic autoinflammatory immunodeficiency characterized by recurrent fever, dermatitis, inflammatory bowel disease, pulmonary alveolar proteinosis, and hypogammaglobulinemia. Read More

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Pulmonary alveolar proteinosis complicated with tuberculosis: A case report.

World J Clin Cases 2021 Jun;9(17):4400-4407

Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.

Background: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of phospholipoproteinaceous material in the alveoli. Cases of PAP complicated with tuberculosis are much more complex and have rarely been well recorded.

Case Summary: We describe a 21-year-old Han Chinese patient with suspicious lung infection associated with mild restrictive ventilatory dysfunction and diffusion reduction. Read More

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Pulmonary Manifestations of GATA2 Deficiency.

Chest 2021 Jun 3. Epub 2021 Jun 3.

Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Cancer Institute, National Institutes of Health, Bethesda, MD.

Background: GATA2 deficiency is a genetic disorder of hematopoiesis, lymphatics, and immunity caused by autosomal dominant or sporadic mutations in GATA2. The disease has a broad phenotype encompassing immunodeficiency, myelodysplasia, leukemia, and vascular or lymphatic dysfunction as well as prominent pulmonary manifestations.

Research Question: What are the pulmonary manifestations of GATA2 deficiency?

Study Design And Methods: A retrospective review was conducted of clinical medical records, diagnostic imaging, pulmonary pathologic specimens, and tests of pulmonary function. Read More

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Disseminated nontuberculous mycobacteriosis and fungemia after second delivery in a patient with MonoMAC syndrome/GATA2 mutation: a case report.

BMC Infect Dis 2021 May 29;21(1):502. Epub 2021 May 29.

Department of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.

Background: Heterozygous mutations in the transcription factor GATA2 result in a wide spectrum of clinical phenotypes, including monocytopenia and Mycobacterium avium complex (MAC) infection (MonoMAC) syndrome. Patients with MonoMAC syndrome typically are infected by disseminated nontuberculous mycobacteria, fungi, and human papillomavirus, exhibit pulmonary alveolar proteinosis during late adolescence or early adulthood, and manifest with decreased content of dendritic cells (DCs), monocytes, and B and natural killer (NK) cells.

Case Presentation: A 39-year-old woman was diagnosed with MonoMAC syndrome postmortem. Read More

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Development and Functions of Alveolar Macrophages.

Mol Cells 2021 May;44(5):292-300

Department of Pathology, Seoul National University College of Medicine, Seoul 03080, Korea.

Macrophages residing in various tissue types are unique in terms of their anatomical locations, ontogenies, developmental pathways, gene expression patterns, and immunological functions. Alveolar macrophages (AMs) reside in the alveolar lumen of the lungs and serve as the first line of defense for the respiratory tract. The immunological functions of AMs are implicated in the pathogenesis of various pulmonary diseases such as allergic asthma, chronic obstructive pulmonary disorder (COPD), pulmonary alveolar proteinosis (PAP), viral infection, and bacterial infection. Read More

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Pulmonary alveolar proteinosis complicated with nocardiosis: A case report and review of the literature.

World J Clin Cases 2021 Apr;9(12):2874-2883

Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou 325015, Zhejiang Province, China.

Background: Pulmonary alveolar proteinosis (PAP) is a pulmonary syndrome wherein large volumes of phospholipid and protein-rich surfactants accumulate within the alveoli. PAP forms include primary (auto-immune PAP), secondary, and congenital. Nocardiosis is a form of suppurative disease induced upon infection with bacteria of the genus. Read More

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Diagnostic yield and safety of bronchofiberscopy for pulmonary alveolar proteinosis.

Respir Investig 2021 May 6. Epub 2021 May 6.

Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai City, Osaka 591-8555, Japan. Electronic address:

Background: Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the abnormal accumulation of surfactant-like material within the alveolar spaces and distal bronchioles. If high-resolution computed tomography (HRCT) indicates the presence of PAP, a definitive diagnosis of PAP is established when consistent pathological findings are obtained. Herein, we retrospectively studied the yield and safety of bronchofiberscopy in the diagnosis of PAP. Read More

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Long-term follow-up and successful treatment of pulmonary alveolar proteinosis without hypercholesterolemia with statin therapy: a case report.

J Int Med Res 2021 Apr;49(4):3000605211010046

Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Beijing, China.

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of excessive surfactant lipids and proteins in alveolar macrophages and alveoli. Oral statin therapy is a novel treatment for PAP with hypercholesterolemia. However, this treatment has never been described in a patient without hypercholesterolemia. Read More

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Can YKL-40 be used as a biomarker for interstitial lung disease?: A systematic review and meta-analysis.

Medicine (Baltimore) 2021 Apr;100(17):e25631

Department of Respiratory Medicine and Critical Care Medicine, West China Hospital/West China School of Medicine, Sichuan University.

Background: Interstitial lung disease (ILD) has a poor prognosis and lacks specific biomarkers for early diagnosis, assessment of disease severity, and prognosis. YKL-40 levels were found to be elevated in patients with ILD, but these results are inconsistent. Therefore, we conducted a systematic review and meta-analysis to accurately study the relation between YKL-40 and ILD. Read More

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[Pulmonary alveolar proteinosis].

Medicina (B Aires) 2021 ;81(2):301-303

Servicio de Clínica Médica, Instituto de Investigaciones Médicas Alfredo Lanari, Buenos Aires, Argentina.

Pulmonary alveolar proteinosis (PAP) is a rare, diffuse pulmonary disease due to abnormal surfactant homeostasis. We present the case of a 69-year-old woman who was admitted to the hospital for progressive dyspnea with marked limitation in activity, and non-productive cough, of three months of evolution. Type I respiratory failure was confirmed. Read More

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Recommended Reading from the University of Florida Fellows.

Am J Respir Crit Care Med 2021 Apr 23. Epub 2021 Apr 23.

University of Florida, 3463, Pulmonary, Critical Care and Sleep Medicine, Gainesville, Florida, United States;

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Mild dyspnea presenting as 'crazy-paving' on chest computed tomography.

Authors:
M Oudah D Slack

J Community Hosp Intern Med Perspect 2021 Mar 23;11(2):273-276. Epub 2021 Mar 23.

Department of Pulmonology and Critical Care Medicine, Greater Baltimore Medical Center, Towson, Maryland, USA.

Pulmonary alveolar proteinoisis (PAP) is a rare disease characterized by accumulation of proteinaceous material in the alveolar spaces. Here, we report a case of mild dyspnea with incidental 'crazy-paving' pattern on chest computed tomography (CT). Further evaluation and bronchoscopy found to have PAP. Read More

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Bilateral whole lung lavage by lung isolation in a child with pulmonary alveolar proteinosis: A new technique.

Ann Card Anaesth 2021 Apr-Jun;24(2):266-268

Department of Cardiac Anaesthesia, Cardiothoracic Centre, AIIMS, New Delhi, India.

We present two rare cases of children who presented with progressive exertional breathlessness and dry cough. They also had history of bluish discoloration of mucous membranes, hands and feet on exertion. Both were diagnosed to have pulmonary alveolar proteinosis after a high-resolution computed tomography and bronchoalveolar lavage. Read More

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A lung mass: Not the usual suspect, but not above suspicion.

Respir Med Case Rep 2021 18;33:101389. Epub 2021 Mar 18.

Department of Respiratory and Critical Care Medicine, Changi General Hospital, Singapore.

Lipoid pneumonia is an entity that is not commonly thought of when faced with opacities on the chest radiograph. The radiological changes of lipoid pneumonia can mimic pneumonia or malignancy. Due to this mimicry, it is commonly missed and alternative diagnoses should always be considered and ruled out when suspecting lipoid pneumonia. Read More

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A case of progressive pulmonary alveolar proteinosis with lymphadenopathy revealed on F-FDG PET/CT.

Hell J Nucl Med 2021 Jan-Apr;24(1):94-95. Epub 2021 Apr 20.

Department of Nuclear Medicine, West China Hospital, Sichuan University, No. 37. Guoxue Alley, 610041 Chengdu, P.R. China. zhang_

Pulmonary alveolar proteinosis (PAP) is a rare lung disease, which may cause repeating infections. A 36-year-old man had repetitive admissions to our hospital, beginning two years ago, due to episodes of severe dyspnea. Serial computed tomography (CT) scans revealed extensive ground-glass opacities with interlobular/intralobular septal thickening, diffuse consolidations in both lungs and enlarged lower paratracheal lymph nodes. Read More

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Expression profiles and potential functions of long noncoding RNAs and mRNAs in autoimmune pulmonary alveolar proteinosis patients.

Aging (Albany NY) 2021 04 4;13(7):10535-10554. Epub 2021 Apr 4.

Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

Autoimmune pulmonary alveolar proteinosis (APAP) is a rare lung disease that may be associated with surfactant overaccumulation. To assess the function of long noncoding RNAs (lncRNAs) in APAP, we performed microarray analyses to identify differentially expressed (DE) lncRNAs and mRNAs between peripheral blood samples from five APAP patients and five healthy volunteers. In total, 12459 DE lncRNAs and 9331 DE mRNAs were identified in APAP patient samples. Read More

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ABCA3 deficiency dramatically improved by azithromycin administration.

Pediatr Int 2021 May 5;63(5):602-604. Epub 2021 Apr 5.

Aichi Children's Health and Medical Center, Obu, Aichi, Japan.

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Correlation of Lipid Ratios With the Severity of Pulmonary Alveolar Proteinosis: A Cross-Sectional Study.

Front Nutr 2021 18;8:610765. Epub 2021 Mar 18.

Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, China.

Lipids are known to accumulate abnormally in the alveoli and circulate during pulmonary alveolar proteinosis (PAP). However, the relationship between lipid ratios and PAP is not clear. In this study, we investigated the lipid ratios in PAP patients and explored the relationships between lipid ratios and the severity of PAP. Read More

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Autoimmune pulmonary alveolar proteinosis and idiopathic pulmonary haemosiderosis: a dual pathology.

BMJ Case Rep 2021 Apr 2;14(4). Epub 2021 Apr 2.

Department of Respiratory Medicine, Cork University Hospital, Cork, Ireland

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary condition which leads to excessive accumulation of proteinaceous material within the alveoli. Idiopathic pulmonary haemosiderosis (IPH) is another orphan lung disease and results in recurrent alveolar haemorrhage. This case study describes a case of these two rare pathologies occurring together. Read More

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Beyond "Big Eaters": The Versatile Role of Alveolar Macrophages in Health and Disease.

Int J Mol Sci 2021 Mar 24;22(7). Epub 2021 Mar 24.

REBIRTH Research Center for Translational and Regenerative Medicine, Hannover Medical School, 30625 Hannover, Germany.

Macrophages act as immune scavengers and are important cell types in the homeostasis of various tissues. Given the multiple roles of macrophages, these cells can also be found as tissue resident macrophages tightly integrated into a variety of tissues in which they fulfill crucial and organ-specific functions. The lung harbors at least two macrophage populations: interstitial and alveolar macrophages, which occupy different niches and functions. Read More

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Systemic Pulmonary Events Associated with Myelodysplastic Syndromes: A Retrospective Multicentre Study.

J Clin Med 2021 Mar 10;10(6). Epub 2021 Mar 10.

Department of Internal Medicine and Clinical Immunology, National Reference Centre for Rare Systemic Autoimmune Disease North and North-West of France, University of Lille, CHU Lille, F-59000 Lille, France.

Although pulmonary events are considered to be frequently associated with malignant haemopathies, they have been sparsely studied in the specific context of myelodysplastic syndromes (MDS). We aimed to describe their different types, their relative proportions and their relative effects on overall survival (OS). We conducted a multicentre retrospective cohort study. Read More

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Alveolar Proteinosis, Infectious Complications and Monocytopenia Associated with GATA2 Deficiency.

Neuro Endocrinol Lett 2021 Jan;41(6):290-295

Department of Clinical hematology and Onco-hematology, Medical Faculty P.J. Safarik University and L. Pasteur University Hospital, Kosice, Slovakia.

The authors describe a case of a rare disorder associated with GATA 2 deficiency, which is an important hematopoietic transcription factor for the development of monocytes. The clinical hallmarks of GATA2 deficiency include monocytopenia, cellular immunodeficiency with a resultant marked susceptibility to infections (mycobacterial, fungal, and viral), predisposition to myelodysplasia, alveolar proteinosis of the lungs and congenital lymphoedema. These features overlap with other genetic and acquired syndromes. Read More

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January 2021

Usefulness and safety of transbronchial biopsy with large forceps during flexible bronchoscopy.

Proc (Bayl Univ Med Cent) 2020 Oct 28;34(2):232-236. Epub 2020 Oct 28.

Division of Pulmonary and Critical Care, Cooper Medical School at Rowan University, Camden, New Jersey.

Transbronchial lung biopsy (TBLB) using forceps is one of the most common procedures used to obtain lung tissue. The procedure's usefulness remains limited when diagnosing interstitial lung diseases. This retrospective descriptive study analyzed the feasibility and safety of using large forceps for TBLB in all patients who underwent TBLB from 2014 to 2018 for diffuse lung disease where the diagnosis could not be made by high-resolution chest computed tomography. Read More

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October 2020

Clinical characteristics of pulmonary alveolar proteinosis.

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2021 Feb;46(2):156-161

Department of Respiratory and Critical Care Medicine, Second Xiangya Hospital; Research Unit of Respiratory Disease, Central South University; Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha 410011, China.

Objectives: Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP.

Methods: We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University. Read More

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February 2021

B cell-activating factors in autoimmune pulmonary alveolar proteinosis.

Orphanet J Rare Dis 2021 03 2;16(1):115. Epub 2021 Mar 2.

Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-Cho, Kita-Ku, Sakai City, Osaka, 591-8555, Japan.

Background: Autoimmune pulmonary alveolar proteinosis (APAP) results from the suppression of granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling by a neutralizing autoantibody against GM-CSF. B cell-activating factor (BAFF) and a proliferation-inducing ligand (APRIL) are involved in immunoglobulin G production and are overproduced in various autoimmune disorders. We hypothesized that BAFF and/or APRIL levels would be elevated in serum and bronchoalveolar lavage fluid (BALF) and serum and BALF levels of BAFF and APRIL respond to the treatments (whole lung lavage (WLL) or inhalation of recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF)) in patients with APAP. Read More

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Chronic myelomonocytic leukemia-associated pulmonary alveolar proteinosis: A case report and review of literature.

World J Clin Cases 2021 Feb;9(5):1156-1167

Department of Hematology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China.

Background: Pulmonary alveolar proteinosis (PAP) is a rare condition that can cause progressive symptoms including dyspnea, cough and respiratory insufficiency. Secondary PAP is generally associated with hematological malignancies including chronic myelomonocytic leukemia (CMML). To the best of our knowledge, this is the first reported case of PAP occurring secondary to CMML. Read More

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February 2021

Hereditary pulmonary alveolar proteinosis as collateral damage from a large chromosomal deletion.

Pediatr Pulmonol 2021 Jun 8;56(6):1687-1689. Epub 2021 Mar 8.

Department of Pediatric Respiratory Medicine, Royal Brompton Hospital, London, UK.

A girl with a known chromosomal deletion at Xp22.33, learning difficulties and short stature presented with dyspnea and dry cough and an abnormal chest X-ray. Computed tomography was typical for pulmonary alveolar proteinosis (PAP), and the diagnosis was confirmed invasively. Read More

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