Gorham disease, also known as massive osteolysis, is characterized by unregulated proliferation of lymphatic or vascular capillaries within bone, resulting in destruction and replacement with angiomatous tissue. This disease can lead to chylothorax that can be lethal. extract from European mistletoe is a complementary anti-cancer drug which is commonly prescribed in many European countries and is considered as a new generation of chemical agent for pleurodesis. A 14-year-old girl presented with dyspnea and chest pain. She was diagnosed as Gorham disease who was definitively treated with extract for chylothorax that was refractory to standard conservative management.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6849062 | PMC |
| http://dx.doi.org/10.4266/acc.2016.00164 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Complex lymphatic anomalies.
Pediatr Radiol
January 2025
Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Complex lymphatic anomalies are unique diseases marked by abnormal lymphatic vessel development and growth. Imaging is crucial in the evaluation and management of complex lymphatic anomalies, with dynamic contrast-enhanced MR lymphangiography emerging as a valuable modality for visualizing abnormal lymphatic structures and informing treatment decisions. This article gives an overview of complex lymphatic anomalies and their management strategies, focusing specifically on generalized lymphatic anomaly, Gorham-Stout disease, Kaposiform lymphangiomatosis, and central conducting lymphatic anomalies.
View Article and Find Full Text PDFContribution of hypoxia-inducible factor 1alpha to pathogenesis of sarcomeric hypertrophic cardiomyopathy.
Sci Rep
January 2025
Department of Congenital Heart Defects and Pediatric Cardiology, German Heart Center Munich, TUM University Hospital, School of Medicine & Health, Technical University of Munich, Munich, Germany.
Hypertrophic cardiomyopathy (HCM) caused by autosomal-dominant mutations in genes coding for structural sarcomeric proteins, is the most common inherited heart disease. HCM is associated with myocardial hypertrophy, fibrosis and ventricular dysfunction. Hypoxia-inducible transcription factor-1α (Hif-1α) is the central master regulators of cellular hypoxia response and associated with HCM.
View Article and Find Full Text PDFSuccessful Management of Chronic Chylothorax Secondary to Gorham-Stout Disease.
Ann Thorac Surg Short Rep
December 2024
Thoracic Surgery Department, Nottingham University Hospitals NHS Trust, UK.
Gorham-Scout disease (GSD) is a rare skeletal disorder of unknown etiology characterized by progressive osteolysis and excessive lymphovascular proliferation. Chylothorax is a life-threatening complication. A teenager presented with a left pleural effusion on a background of chronic flank collection secondary to lymphovascular malformation.
View Article and Find Full Text PDFCardiac fibroblast BAG3 regulates TGFBR2 signaling and fibrosis in dilated cardiomyopathy.
J Clin Invest
January 2025
Department of Biomedical Engineering, Columbia University, New York, New York, USA.
Loss of Bcl2-associated athanogene 3 (BAG3) is associated with dilated cardiomyopathy (DCM). BAG3 regulates sarcomere protein turnover in cardiomyocytes; however, the function of BAG3 in other cardiac cell types is understudied. In this study, we used an isogenic pair of BAG3-knockout and wild-type human induced pluripotent stem cells (hiPSCs) to interrogate the role of BAG3 in hiPSC-derived cardiac fibroblasts (CFs).
View Article and Find Full Text PDFSurgical treatment of Gorham-Stout disease combined with scoliosis: a case report and literature review.
BMC Musculoskelet Disord
December 2024
Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing, People's Republic of China.
Background: Gorham-Stout disease (GSD) is a rare disease characterized by osteolysis and lymphatic malformations. GSD involving the spine is exceptionally rare and lacks a standard cure. The aim of this article was to report a case of GSD with scoliosis treated via corrective surgery and medication.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!