Benign notochordal cell tumor (BNCT) is a benign lesion derived from notochordal cells. Although it is relatively common in intraosseous lesion, pulmonary BNCT is extremely rare. We present a case of 54-year-old male with multiple pulmonary nodules, in which were considered to be metastatic chordomas initially. For 20 months follow-up without any therapy, most of the nodules had no remarkable change but some nodules showed cystic change. We consulted with pathologists specializing in chordoma and the final diagnosis of the nodules was considered as BNCT rather than chordoma. We herein report the case of multiple pulmonary BNCTs with cystic change, comparing with previous reports.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10319637 | PMC |
http://dx.doi.org/10.1016/j.radcr.2023.06.014 | DOI Listing |
Arch Dis Child Educ Pract Ed
January 2025
National Heart and Lung Institute, Imperial College London, London, UK.
Transformative changes in the health of children with cystic fibrosis (CF) mean that more sensitive outcome measures are needed to monitor paediatric CF lung disease. Multiple breath washout (MBW) and its primary readout lung clearance index are gaining increasing traction as an endpoint for clinical trials in the CF space and show promise as a clinical investigation. In this article, we use four clinically based questions to explore what MBW can and cannot (yet) do and highlight some of its strengths and weaknesses as an investigation.
View Article and Find Full Text PDFJ Cyst Fibros
January 2025
Department NEUROFARBA, University of Florence; Paediatric and Liver Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
Background: Elexacaftor-tezacaftor-ivacaftor (ETI) has significantly improved the clinical course of people with cystic fibrosis (pwCF) and eligible CFTR variants. In this study, we prospectively evaluated liver elastography, liver fibrosis indices and liver tests in children with CF aged 6-12 years started on ETI therapy.
Methods: Body mass index, sweat test, percent predicted forced expiratory volume in one second, serum markers of liver injury or portal hypertension, liver fibrosis indices, controlled attenuation parameter and liver stiffness were assessed before starting ETI and three and twelve months post-ETI, according to new international guidelines.
Introduction: The Lung Clearance Index (LCI) is an established research test, but its role in clinical decision-making is not well defined. This study estimated the proportion of treatment decisions that are changed or supported by the added information provided by LCI.
Methods: A mixed methods prospective observational study was conducted in North America.
Glycoconj J
January 2025
Department of Medical Biotechnology and Translational Medicine, University of Milano, Milan, Italy.
Cystic Fibrosis (CF) is a life-threatening hereditary disease resulting from mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene that encodes a chloride channel essential for ion transport in epithelial cells. Mutations in CFTR, notably the prevalent F508del mutation, impair chloride transport, severely affecting the respiratory system and leading to recurrent infections. Recent therapeutic advancements include CFTR modulators such as ETI, a combination of two correctors (Elexacaftor and Tezacaftor) and a potentiator (Ivacaftor), that can improve CFTR function in patients with the F508del mutation.
View Article and Find Full Text PDFDiagnostics (Basel)
December 2024
Department of Radiology, Jeonbuk National University Medical School and Hospital, 20 Geonji-ro, Deokjin-gu, Jeonju 54907, Jeonbuk, Republic of Korea.
Low-grade endometrial stromal sarcoma (LGESS) is a rare uterine malignancy that causes non-specific symptoms which presents more typically in younger women compared to other uterine sarcomas. Preoperative diagnosis of myometrial LGESS is challenging, as it is frequently mistaken for a benign uterine mass, such as a degenerating leiomyoma. Despite its rarity, the imaging findings of LGESS are highly variable, complicating the diagnostic process.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!