Common Mutations in the Surfactant Protein-C Gene in Iranian Patients with Diffuse Parenchymal Lung Disease.

Background: Recently, genetic mutations in surfactant protein C (SFTPC) have been linked to diffuse parenchymal lung diseases (DPLD). The present study investigated mutations among Iranian patients with DPLD for the first time.

Materials And Methods: In this study, we examined 28 patients diagnosed with DPLD.

Rituximab Therapy in Pulmonary Alveolar Proteinosis: A Case Report.

Autoimmune pulmonary alveolar proteinosis (PAP) is a rare lung condition characterized by the accumulation of surfactant proteins within the alveoli, leading to respiratory distress. We describe a 49-year-old female homemaker with a history of passive smoking and exposure to wood smoke and pigeon droppings. She presented with a dry cough and progressive dyspnea, experiencing significant deterioration in her condition over one year.

Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study.

Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking.

Methods: In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium.

Autoantibodies targeting interferons and GM-CSF are associated with adverse outcome risk, comorbidities, and pathogen in community-acquired pneumonia.

Introduction: Cytokine autoantibodies (c-aAb) have been associated with pulmonary diseases, including severe novel coronavirus disease 2019 (COVID-19) and pulmonary alveolar proteinosis. This study aimed to determine c-aAb association with community-acquired pneumonia (CAP) etiology (SARS-CoV-2, influenza, or bacteria) and c-aAb associations with CAP-related clinical outcomes and pulmonary comorbidities.

Methods: In a cohort of 665 patients hospitalized with CAP, c-aAb targeting interferon α (IFNα), IFNβ, IFNγ, interleukin-1α (IL-1α), IL-6, IL-10, and granulocyte-macrophage colony-stimulating factor (GM-CSF) were measured in plasma samples.

Anesthesia Management of the Patient With Pulmonary Alveolar Proteinosis Undergoing Lung Lavage.

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder characterized by the accumulation of surfactant/lipoprotein material in the alveoli and subsequent hypoxemic respiratory failure. Whole lung lavage (WLL), a procedure used to physically remove the lipoprotein material from the alveoli, is the first-line treatment for this disease process. Anesthesia providers may infrequently encounter the management of the WLL procedure due to the rarity of the underlying disease process.

Usage of Foley's catheter in pediatric lung isolation, whole lung lavage: A case report.

This case report delves into pediatric lung isolation challenges and innovations in managing patients with pulmonary alveolar proteinosis undergoing whole lung lavage. The central focus is on a 5-year-old girl who initially encountered intraoperative complications, including bilateral pneumothorax and pulmonary edema. However, a subsequent attempt employing a Foley's catheter for lung isolation proved successful, with the patient displaying marked postoperative improvements.

Surfactant Protein A Inhibits Human Rhinovirus C Binding and Infection of Airway Epithelial Cells from Pediatric Asthma.

Rhinovirus C (RV-C) infection can trigger asthma exacerbations in children and adults, and RV-C-induced wheezing illnesses in preschool children correlate with the development of childhood asthma. Surfactant protein A (SP-A) plays a critical role in regulating pulmonary innate immunity by binding to numerous respiratory pathogens. Mature SP-A consists of multiple isoforms that form the hetero-oligomers of SP-A1 and SP-A2, organized in 18-mers.

Repeated inhalation of GM-CSF by nonhuman primates induces bronchus-associated lymphoid tissue along the lower respiratory tract.

Background: Repeated inhalation of granulocyte-macrophage colony-stimulating factor (GM-CSF) was recently approved in Japan as a treatment for autoimmune pulmonary alveolar proteinosis. However, the detailed physiological and pathological effects of repeated inhalation in the long term, especially at increasing doses, remain unclear.

Methods: In this chronic safety study, we administered 24 cynomolgus monkeys (Macaca fascicularis) aged 2-3 years with aerosolized sargramostim (a yeast-derived recombinant human GM-CSF [rhGM-CSF]) biweekly for 26 weeks across four dosing groups (0, 5, 100, and 500 µg/kg/day).

Pulmonary fibrosis in pulmonary alveolar proteinosis evaluated by transbronchial lung cryobiopsy: A single-center retrospective study.

Background: Approximately 20% of patients with pulmonary alveolar proteinosis (PAP) present with pulmonary fibrosis on high-resolution computed tomography (HRCT). Although transbronchial lung cryobiopsy (TBLC) has recently been used to diagnose fibrotic interstitial lung disease, no studies have investigated whether TBLC is useful for the histopathological detection of pulmonary fibrosis coexisting with PAP. Therefore, the present study aimed to investigate the utility of TBLC for evaluating pulmonary fibrosis in patients with PAP.

Napsin-A Immunohistochemistry in the Diagnosis of Pulmonary Alveolar Proteinosis.

Context.—: The diagnosis of pulmonary alveolar proteinosis (PAP) relies on a limited set of stains, namely hematoxylin-eosin and periodic acid-Schiff-diastase (PAS-D), demonstrating abundant alveolar material representing mostly surfactant. As cells harboring surfactant also express Napsin-A (pneumocytes and macrophages), we hypothesized that it would also be expressed within alveoli in PAP.