Purple glove syndrome

Introduction: Purple glove syndrome is an atypical and adverse reaction to intravenous phenytoin, which is characterized by oedema, pain and a dark purple-bluish discolouration, typically located on an upper extremity. The clinical manifestation of this syndrome occurs in three stages: appearance, progression and resolution of symptoms. PGS develops up to 12 hours after administration of intravenous phenytoin and it disappears in a few weeks or months. Objective: The aim of this article is to summarize the current state of knowledge about purple glove syndrome: the pathophysiology, risk factors, the diagnosis and the current treatment. Brief descriptions of the state of knowledge: Despite many years PGS is still unexplained phenomenon. It is claimed that vascular tearing, micro-extravasation, alkaline pH of the solution or unidentified procoagulant mechanism can cause tissue impairment. The treatment depends on a limb elevation, physiotherapy, intravenous heparin administration, pain control, nitroglycerine application and a nerve blocks. To prevent PGS it is recommended to use oral phenytoin whenever possible, slow infusion rate of phenytoin less than 25mg/min, large cannula (20G or larger) in a large calibre vein and suitable, small doses. What is important fosphenytoin, a pro-drug of phenytoin, can also cause PGS, even though it was thought to be a safe drug, and a purple glove syndrome induced by fosphenytoin has never been described before. Summary: Despite the existence of many clinical trials, long term observations and scientific speculations, PGS can still be challenging for clinicians. There is a need for further scientific research to explain this phenomenon and to increase the awareness of this problem in general medical practice

Parkes Weber Syndrome

Introduction: Parkes Weber Syndrome (PWS) is a traditional eponymous denomination of a certain type of angiodysplasia. It is a congenital vascular disease which consists of capillary malformation (CM), venous malformation (VM), lymphatic malformation (LM), congenital arteriovenous malformation (AVM) and multiple arteriovenous fistulas (AVFs). There is a soft-tissue and skeletal hypertrophy of the affected extremity (usually a lower extremity). Moreover the affected limb is warmer and longer than the other side. Objective: The aim of this article is to summarize the current state of knowledge about Parkes Weber Syndrome: the pathophysiology, genetic inheritance, the main symptoms, the diagnosis especially differential diagnosis and the current treatment. Brief descriptions of the state of knowledge: Despite many years, physicians still have difficulties with diagnosing PWS correctly. Although the aetiology is unknown, it is claimed that PWS is caused by mutations of the RASA1, gene located on chromosome 5q13.1, which are inherited in an autosomal dominant manner. This gene is responsible for mediating cellular growth, differentiation and proliferation. No efficacious pharmacological treatment has been found. Nowadays Tranexamic Acid, Sirolimus, Everolimus and Miconazole are used in medical practice. The most frequently utilized invasive treatment methods are amputation, surgical AVM resection and occasionally stent-graft implantation. Furthermore it is thought that embolization, alone or combined with surgical resection leads to clinical improvement. Summary: Despite the existence of many clinical trials, long term observations and scientific speculations, PWS can still be challenging for clinicians. There is a need for further scientific, molecular and genetic research to diagnose this phenomenon correctly, because despite fact, that its symptoms are similar to other syndromes or entities, therapeutic strategies differ significantly. It is important to increase the awareness of inheritance in an autosomal dominant manner in generation of patients with PWS

The Important Role of Endothelium and Extracellular Vesicles in the Cellular Mechanism of Aortic Aneurysm Formation

Homeostasis is a fundamental property of biological systems consisting of the ability to maintain a dynamic balance of the environment of biochemical processes. The action of endogenous and exogenous factors can lead to internal balance disorder, which results in the activation of the immune system and the development of inflammatory response. Inflammation determines the disturbances in the structure of the vessel wall, connected with the change in their diameter. These disorders consist of accumulation in the space between the endothelium and the muscle cells of low-density lipoproteins (LDL), resulting in the formation of fatty streaks narrowing the lumen and restricting the blood flow in the area behind the structure. The effect of inflammation may also be pathological dilatation of the vessel wall associated with the development of aneurysms. Described disease entities strongly correlate with the increased migration of immune cells. Recent scientific research indicates the secretion of specific vesicular structures during migration activated by the inflammation. The review focuses on the link between endothelial dysfunction and the inflammatory response and the impact of these processes on the development of disease entities potentially related to the secretion of extracellular vesicles (EVs)