Background: Among patients awaiting cadaveric lung transplantation, patients with idiopathic interstitial pneumonia (IIP) have been demonstrated to have the highest mortality rate. Contraindications to cadaveric lung transplantation include current high-dose systemic corticosteroid therapy because it may increase airway complications and various types of infection.
Study Objectives: To analyze the effect of living-donor lobar lung transplantation (LDLLT) for patients with advanced IIP including those receiving high-dose systemic corticosteroids.
Design: Retrospective analysis.
Setting: Okayama University Hospital and Okayama Medical Center.
Patients: We report on the first nine patients (seven female and two male; age range, 13 to 55 years) with advanced IIP receiving LDLLT. All nine patients had a very limited life expectancy, and eight patients were dependent on systemic corticosteroid therapy as high as 50 mg/d of prednisone. LDLLT was performed under cardiopulmonary bypass using two lower lobes donated by two healthy relatives.
Results: There were no airway complications in the 18 bronchial anastomoses. There was one early death (11%) due to severe acute rejection. Eight patients (89%) are currently alive with a follow-up period of 10 to 48 months. Their vital capacity reached 2.03 +/- 0.20 L (mean +/- SEM), 71.4% of predicted at 1 year. All 18 donors have returned to their previous lifestyles. Excised lungs were pathologically diagnosed as usual interstitial pneumonia (UIP) in six cases and fibrotic nonspecific interstitial pneumonia (NSIP) in three cases.
Conclusions: These early follow-up data support the option of LDLLT in patients with advanced IIP, including UIP and fibrotic NSIP, who would die soon otherwise. Current high-dose systemic corticosteroid therapy is not a contraindication in LDLLT.
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http://dx.doi.org/10.1378/chest.128.3.1364 | DOI Listing |
BMC Pulm Med
January 2025
Tehran Lung Research and Developmental Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Background: This study aims to compare Lung Ultrasound (LUS) findings with High-Resolution Computerized Tomography (HRCT) and Pulmonary Function Tests (PFTs) to detect the severity of lung involvement in patients with Usual Interstitial Pneumonia (UIP) and Non-Specific Interstitial Pneumonia (NSIP).
Methods: A cross-sectional study was conducted on 35 UIP and 30 NSIP patients at a referral hospital. All patients underwent LUS, HRCT, and PFT.
BMC Pediatr
January 2025
Department of Pediatrics II (Neonatology), Medical University of Innsbruck, Innsbruck, Austria.
Preterm infants are at high risk of developing respiratory distress syndrome (RDS). Mutations in the genes encoding for surfactant proteins B and C or the ATP-binding cassette transporter A3 (ABCA3) are rare but known to be associated with severe RDS and interstitial lung diseases. The exact prevalence of these mutations in the general population is difficult to determine, as they are usually studied in connection with clinical symptoms.
View Article and Find Full Text PDFLung
January 2025
National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University Lyon 1, UMR 754, ERN-LUNG, Lyon, France.
Purpose: In the INBUILD trial in patients with progressive pulmonary fibrosis (PPF), nintedanib slowed the decline in forced vital capacity (FVC) versus placebo, with a safety profile characterised mainly by gastrointestinal events. INBUILD-ON, the open-label extension of INBUILD, assessed the safety of nintedanib during longer-term treatment. Data on FVC were collected.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Diagnostic and Interventional Radiology, University Hospital Bonn, Venusberg-Campus 1, 53127, Bonn, Germany.
Automated tools for quantification of idiopathic pulmonary fibrosis (IPF) can aid in ensuring reproducibility, however their complexity and costs can differ substantially. In this retrospective study, two automated tools were compared in 45 patients with biopsy proven (12/45) and imaging-based (33/45) IPF diagnosis (mean age 74 ± 9 years, 37 male) for quantification of pulmonary fibrosis in CT. First, a tool that identifies multiple characteristic lung texture features was applied to measure multi-texture fibrotic lung (MTFL) by combining the amount of ground glass, reticulation, and honeycombing.
View Article and Find Full Text PDFAm J Med Sci
January 2025
Department of Pulmonary and Critical Care Medicine, Dongying People's Hospital, Dongying, Shandong, China. Electronic address:
Background: Patients with combined pulmonary fibrosis and emphysema (CPFE) may experience emphysema or fibrosis progression on chest computed tomography (CT). This study aimed to investigate the relationship and prognosis in CPFE patients with emphysema or fibrosis progression.
Methods: A total of 188 CPFE patients were included in our retrospective cohort study.
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