Related Experiment Video
Updated: Sep 1, 2025
11:39
The Goeckerman Regimen for the Treatment of Moderate to Severe Psoriasis
Published on: July 11, 2013
39.0K
Peripheral Interventions in Patients with Pseudoxanthoma Elasticum (PXE).
Maarten C Verwer1, Constantijn E V B Hazenberg1, Wilko Spiering2
1Department of Vascular Surgery, Division of Surgical Specialties, University Medical Centre Utrecht, Utrecht University, Utrecht, the Netherlands.
Summary
Peripheral artery disease (PAD) affects half of patients with pseudoxanthoma elasticum (PXE). Interventions for PAD in PXE patients show a low rate but unfavorable re-intervention outcomes within a year.
Area of Science:
- Vascular Medicine
- Genetics
- Metabolic Disorders
Background:
- Pseudoxanthoma elasticum (PXE) is an autosomal recessive disorder.
- PXE may be linked to peripheral artery disease (PAD), but evidence is limited.
- Outcomes of peripheral arterial interventions (PAI) in PXE patients are poorly documented.
Purpose of the Study:
- To determine the prevalence of PAD in a large cohort of PXE patients.
- To assess the occurrence and outcomes of PAI in PXE patients.
Main Methods:
- Retrospective review of prospectively collected data from a Dutch PXE database.
- Analysis of clinical data from 285 diagnosed PXE patients.
- PAD defined by ankle-brachial index < 0.9; secondary endpoint included PAI and revascularization data.
Main Results:
- 50.9% of PXE patients (n=145) had PAD.
- 17 patients underwent PAI, primarily for intermittent claudication, at a median age of 51.
- 58 interventions were recorded, with 35 target lesion revascularizations; 21 revascularizations occurred within a year, often due to acute occlusion.
Conclusions:
- PAD is highly prevalent in PXE patients (1 in 2).
- The rate of peripheral interventions in this cohort was low.
- Re-intervention rates after PAI were unfavorable, with over half occurring within one year.
Related Concept Videos
01:27Peripheral Artery Disease III: Interprofessional Care
28
Peripheral Artery Disease (PAD) is characterized by narrowed arteries that diminish blood flow to the extremities. Effective management of PAD requires an interprofessional approach involving various healthcare professionals. The critical aspects of interprofessional care for PAD patients focus on risk factor modification, drug therapy, exercise therapy, nutrition therapy, critical limb ischemia care, and interventional radiology and surgical procedures.The primary treatment goal for PAD...
28
01:26Peripheral Artery Disease IV: Nursing Management
32
The nursing management of a patient with peripheral artery disease (PAD) begins with a thorough assessment of the patient’s health history and clinical manifestations.AssessmentHealth History: Evaluate the patient’s history of hypertension, hyperlipidemia, family history of cardiovascular issues, and lifestyle factors such as dietary patterns, smoking, and physical activity.Physical Examination:Assess the affected extremity for decreased or absent peripheral pulses,...
32
01:23Peripheral Artery Disease V: Postoperative Nursing Management
19
During the postoperative period, it is crucial to focus on maintaining circulation, identifying and managing potential complications, and planning for discharge.Nursing AssessmentVital signs monitoring: Regularly monitor vital signs, including blood pressure, heart rate, respiratory rate, and temperature, to detect early signs of complications such as bleeding and infection.Circulation assessment: Monitor pulses, perform Doppler assessments, and check capillary refill, color, temperature, and...
19
01:21Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation
28
Clinical manifestationsPeripheral Arterial Disease (PAD) manifests through a range of symptoms, from the characteristic intermittent claudication to atypical presentations and severe complications in advanced stages. Intermittent claudication, a hallmark symptom of PAD, presents as exercise-induced muscle pain that typically resolves within minutes of rest. This pain is reproducible and stems from inadequate blood flow, leading to the accumulation of lactic acid produced during anaerobic...
28
01:26Varicose Veins II: Diagnostic Studies and Interprofessional Care
26
Varicose veins, or varicosities, develop when the valves in the veins, which control blood flow, weaken or damage. It causes blood to pool and the veins to enlarge. Understanding the clinical manifestations, diagnostic approaches, and management options for varicose veins is crucial for effective treatment and relief.Clinical manifestationsClinical manifestations of varicose veins include a heavy, achy feeling or pain after prolonged standing or sitting. This discomfort can often be relieved by...
26