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High resolution chest computed tomography responses for a cohort of adult with pulmonary Langerhans cell histiocytosis.
Ann Hematol
January 2025
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, People's Republic of China.
Langerhans cell histiocytosis (LCH) is a heterogeneous histiocytosis with various pulmonary manifestations and imaging. We aim to evaluate the pulmonary response of LCH by high resolution chest computed tomography (HRCT) through continuous follow-up.We conducted a retrospective analysis of 73 adult LCH patients with pulmonary involvement.
View Article and Find Full Text PDFErdheim-Chester disease: Comprehensive insights from genetic mutations to clinical manifestations and therapeutic advances.
Dis Mon
January 2025
Department of Pharmaceutical Sciences, Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow-226025, (U.P.), India. Electronic address:
Erdheim-Chester disease (ECD) is an extremely rare non-Langerhans cell disorder that is believed to include both inflammatory and neoplastic characteristics. It is caused due to genetic mutations in proto-oncogenes like BRAF and MEK, while immunological pathways have an essential role in the onset and progression of the disease. Despite its rarity, ECD poses significant diagnostic and therapeutic challenges due to its heterogeneous clinical presentation and limited understanding of its underlying pathophysiology.
View Article and Find Full Text PDFAllogeneic haematopoietic stem-cell transplantation for children with refractory systemic juvenile idiopathic arthritis and associated lung disease: outcomes from an international, retrospective cohort study.
Lancet Rheumatol
December 2024
Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA; Pharming Healthcare, Warren, NJ, USA. Electronic address:
Background: Systemic juvenile idiopathic arthritis-related lung disease (sJIA-LD) is a severe complication in patients with treatment-refractory systemic juvenile idiopathic arthritis (sJIA). The objective of this study was to evaluate the effect of allogeneic haematopoietic stem-cell transplantation (HSCT) in a cohort of children with sJIA-LD.
Methods: This international, retrospective cohort study was performed in nine hospitals across the USA and Europe in children with sJIA-LD who had received allogeneic HSCT.
A case of adult-onset X-linked lymphoproliferative disease mimicking pulmonary infection.
BMC Infect Dis
December 2024
National Center for Respiratory Medicine, China-Japan Friendship Hospital, Beijing, 100029, China.
X-linked lymphoproliferative disease (XLP) is a rare primary immunodeficiency with susceptibility and vulnerability to Epstein-Barr virus (EBV) infection. Most patients were diagnosed in early childhood and do not survive into adulthood. Here we reported an adult-onset XLP patient presenting with fever, dyspnea, and pulmonary nodules, mimicking respiratory infection at disease onset.
View Article and Find Full Text PDFA case of Erdheim-Chester disease-a mimicker of IgG4-related disease and large vessel vasculitis.
Mod Rheumatol Case Rep
December 2024
The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan.
The patient was a 57-year-old man who developed bilateral thigh pain and chest tightness one year ago. Chest CT scan showed reticular shadows, thickened interlobular septa in both lung fields, and pericardial effusion. Three months ago, his symptoms worsened.
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