AI Article Synopsis

  • - Pseudoxanthoma elasticum (PXE) is a rare connective tissue disease that impacts the formation of elastic fibers, which can complicate surgical procedures like revascularization in affected patients.
  • - A notable case involved a 43-year-old man with PXE and coronary artery disease who had successful off-pump coronary artery bypass graft (CABG) surgery using the left internal mammary artery (LIMA) to the left anterior descending (LAD) artery.
  • - This case represents the first occurrence of off-pump surgery for PXE in the UK and the second recorded in medical literature, suggesting a need for further research to establish standardized surgical practices for PXE patients.

Article Abstract

Pseudoxanthoma elasticum is a rare connective tissue disease which affects the synthesis of the elastic fibres. It is an area of uncertainty for patients affected by this disease requiring surgical revascularization. We present a case report of a 43-year-old gentleman was known to have pseudoxanthoma elasticum syndrome (PXE) and coronary artery disease. He underwent successful off pump LIMA to LAD. Interestingly, this is the first case in UK and second one in the literature managed with an off-pump strategy. The predilection of the histopathological changes in PXE patients, should not prevent the routine use in of the left internal mammary artery as a standard conduit in CABG. Further studies are required to standardize the practice for surgical revascularization in the patients with PXE.

Download full-text PDF

Source
http://dx.doi.org/10.1177/02676591241288180DOI Listing

Publication Analysis

Top Keywords

pseudoxanthoma elasticum
12
surgical revascularization
8
elasticum unique
4
unique entity
4
entity coronary
4
coronary surgery
4
surgery pseudoxanthoma
4
elasticum rare
4
rare connective
4
connective tissue
4

Similar Publications

Background/aim: Pseudoxanthoma elasticum (PXE) is a genetic connective tissue disorder that affects the skin with limited treatment options. A recent technology employing particle-free polycaprolactone (PCL) has shown promising results in treating inner thighs and kness of a 27-year-old female patient. This article provides a case report along with our detailed treatment protocol based on the efficacy of PCL in reversing skin laxity that can be easily incorporated into the therapeutic approaches for patients with PXE.

View Article and Find Full Text PDF

Pseudoxanthoma elasticum-like papillary dermal elastolysis; A report of two cases and a literature review.

Dermatol Online J

October 2024

Division of Dermatology, Department of Medicine, School of Medicine, The University of Jordan, Amman, Jordan.

Pseudoxanthoma elasticum-like papillary dermal elastolysis is a rare, benign, acquired, gradually-developing chronic elastic tissue disorder that almost exclusively affects post-menopausal women. It is essential to recognize this disease as it mimics the inherited pseudoxanthoma clinically. The pathophysiology behind this disease is multifactorial; it includes intrinsic skin aging, ultraviolet radiation exposure, and genetic components.

View Article and Find Full Text PDF
Article Synopsis
  • Pseudoxanthoma elasticum (PXE) is a rare genetic disorder that leads to abnormal calcium deposits in elastic tissue, causing skin changes and other health issues.
  • The study focused on a 13-year-old patient with flare-ups in PXE lesions, leading to a closer examination of the inflammation involved through skin biopsies and various analyses.
  • Findings showed inflammation with T-cell infiltrates in the skin, particularly with a Th1 response, suggesting an inflammatory role in PXE disease, but the exact impact on the condition needs more research.
View Article and Find Full Text PDF

Arterial calcification volume is associated with a higher risk of cardiovascular events in pseudoxanthoma elasticum.

Atherosclerosis

January 2025

Department of Vascular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. Electronic address:

Background And Aims: Pseudoxanthoma elasticum (PXE) patients have more arterial calcification due to lower levels of inorganic pyrophosphate, caused by mutations in the ABCC6 gene, but the relation with vascular complications is poorly understood. Because of the slow progressing nature of arterial disease in PXE patients, there is a need for a valid and reliable intermediate endpoint to be used in future clinical trials. Arterial calcification measured on computed tomography (CT) is a promising candidate, if associated with future cardiovascular events.

View Article and Find Full Text PDF

Want 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!