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Lung Transplantation for Familial Diffuse Pulmonary Ossification.
Ann Thorac Surg Short Rep
September 2024
Department of Thoracic Surgery, Kyoto University Hospital, Kyoto, Japan.
We report 3 cases of extremely rare familial idiopathic diffuse pulmonary ossification, 2 of 3 received lung transplantation and the other is listed for lung transplantation. The clinical courses of family members varied greatly, and rapid deterioration could occur; therefore, early and close examination is recommended for transplant registration. During transplantation, the lungs appeared and felt exactly like a "pumice stone" and could not collapse, and good visual field was not easily obtained.
View Article and Find Full Text PDFLung ultrasound for assessing disease progression in UIP and NSIP: a comparative study with HRCT and PFT/DLCO.
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.
Comparing multi-texture fibrosis analysis versus binary opacity-based abnormality detection for quantitative assessment of idiopathic pulmonary fibrosis.
Sci 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 PDFIdiopathic infantile hypercalcemia with a variant triggered by vitamin D supplementation in fortified milk: A case report.
Clin Pediatr Endocrinol
January 2025
Department of Pediatrics, Tohoku University Graduate School of Medicine, Sendai, Japan.
Idiopathic infantile hypercalcemia (IIH) is characterized by hypercalcemia, nephrocalcinosis, vomiting, dehydration, and failure to thrive. It is caused by the presence of biallelic loss-of-function variants in the locus. Although hypercalcemia has been linked to the consumption of vitamin D-fortified milk, no reports have documented its role in triggering IIH in patients with variants.
View Article and Find Full Text PDFExercise-Induced Oxygen Desaturation and Outcomes After Nintedanib Therapy for Fibrosing Interstitial Lung Disease in Patients Without Dyspnea.
J Clin Med
December 2024
Division of Respirology, Neurology and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, Asahi-Machi 67, Kurume 830-0011, Japan.
The degree of exercise-induced oxygen desaturation and outcomes following antifibrotic drug therapy in asymptomatic patients with fibrosing interstitial lung disease (FILD) remain unclear. We compared clinical data, incidence of annual FILD progression, overall survival, and tolerability after initiating nintedanib between 58 patients with dyspnea and 18 patients without. Annual FILD progression was defined as >10% decrease in forced vital capacity (FVC), >15% decrease in diffusing capacity of the lungs for carbon monoxide (D), developing acute exacerbations, or FILD-related death within 1 year of starting nintedanib.
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