VEXAS syndrome is a disorder involving episodes of fever and abnormal inflammation. VEXAS is an acronym that stands for the technical terms of key descriptors of the condition. Normally, inflammation is an immune system response to injury or foreign invaders (such as bacteria). In people with VEXAS syndrome, part of the immune system called the innate immune response is turned on (activated) abnormally, when there is no injury or foreign invader, which causes fevers and inflammation-related damage to tissues and organs. Based on this process, VEXAS syndrome is classified as an autoinflammatory disease.
VEXAS syndrome typically affects older adults, primarily males, with signs and symptoms of the condition developing in a person’s fifties, sixties, or seventies. People with VEXAS syndrome often have inflammation of the joints (arthritis), skin (dermatitis), cartilage in the ear and nose (chondritis), or blood vessels (vasculitis). Inflammation can also develop in other tissues, including in the lungs and eyes. Affected individuals may also have enlarged lymph nodes.
Blood cell abnormalities are common in VEXAS syndrome. Most affected individuals develop a shortage of red blood cells (a condition called anemia), and the red blood cells that are present are abnormally large (macrocytic). People with VEXAS syndrome can also have a shortage of blood cells called platelets (a disorder known as thrombocytopenia); platelets are needed for normal blood clotting. Some affected individuals develop myelodyspastic syndrome, a condition in which immature blood cells fail to develop normally; this condition may progress to a form of blood cancer called leukemia.
Vibratory angioedema: A rare condition where the skin produces a hypersensitivity reaction to a vibrating stimulus.
Vibrio vulnificus: This organism causes wound infections, gastroenteritis, or a syndrome known as "primary septicemia." Vibrio vulnificus, a lactose-fermenting, halophilic,
Vibrio Infections: Infection by a bacteria which occurs naturally in seawater and in the stomach of many seawater animals. It is a serious infection and accounts for most seafood-related deaths. It causes severe gastrointestinal symptoms and can also cause wound infection.
Viljoen Kallis Voges syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Viljoen Kallis Voges syndrome, or a subtype of Viljoen Kallis Voges syndrome, affects less than 200,000 people in the US population.
Viljoen Smart syndrome: A type of x-linked mental retardation
discovered by Hamel.
A type of x-linked mental retardation
discovered by Gustavson.
A rare disorder caused by an increase in secretion of vasoactive intestinal peptide (VIP) by the pancreas. The syndrome is often caused by an islet-cell tumor (except for beta cells) in the pancreas.
The viral hemorrhagic fevers (VHFs) are a diverse group of animal and human illnesses that are caused by five distinct families of RNA viruses: the Arenaviridae, Filoviridae, Bunyaviridae, Togaviridae, and Flaviviridae. All types of VHF are characterized by fever and bleeding disorders and all can progress to high fever, shock and death in extreme cases. Some of the VHF agents cause relatively mild illnesses, such as the Scandinavian nephropathia epidemica, whilst others, such as the African Ebola virus, can cause severe, life-threatening disease.
An ovarian tumor made up of hormone secreting cells which results in excessive male hormone (androgen) production.
A fulminant condition characterised by viral infection causing multiple organ infiltrates of haemophagocytic histiocytes.
A very rare form of muscle wasting disease involving the eye and gastrointestinal muscles.
The deposition of small papules composing of granulomas in organs of the body.
The abnormal position of the atriums of the heart.
A morbid condition of the embryo caused by the consumption of excess Vitamin A during pregnancy.
Vitamin D-resistant rickets is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Vitamin D-resistant rickets, or a subtype of Vitamin D-resistant rickets, affects less than 200,000 people in the US population.
A condition that is characterised by a number of symptoms including mental retardation, facial dysmorphism and uremia.
A condition characterised by the degeneration of the vitreous and retina of the eye
A form of hereditary fundus dystrophy.
A rare condition that is characterised by cardiomyopathy, fatty liver, skeletal myopathy, pericardial effusions, ventricular arrhythmias
, and sudden death
A rare inherited disorder where the vocal cord closes while breathing in which causes breathing difficulty which can be severe in some cases. Mental retardation is also associated
A rare inherited disorder where the vocal cord closes while breathing in which causes breathing difficulty which can be severe in some cases. Mental retardation is also associated.
A rare condition where tight bands of thickened skin form around the base of fingers and toes and generally leads to self-amputation of the digit. Hearing loss is also associated with the condition.
An inherited metabolic disorder where a deficiency of the enzyme glucose-6-phosphatase prevents glycogen being turned into glucose leading to a buildup of glycogen in the liver and kidneys.
Von Hippel–Lindau syndrome (VHL, Von hippel-Lindau disease) is a disease which results from a mutation in the von Hippel–Lindau tumor suppressor gene on chromosome 3p25.3.
Von Willebrand disease is a bleeding disorder that slows the blood clotting process. People with this disease often experience bruising, nosebleeds, and prolonged bleeding or oozing following an injury, affer surgery, or having a tooth pulled. Affected women may have heavy menstrual bleeding. In severe cases, heavy bleeding occurs after minor injury or even in the absence of injury.
It is divided into three types. Type 1 is the mildest and most common, and type 3 is the most severe and rarest form. Type 2 (four subtypes) is intermediate in severity. Increased age, pregnancy, exercise, and stress may cause von Willebrand factor levels in the blood to rise, which can make bleeding symptoms less frequent. This disease is caused by a mutation in the VWF gene and can have different inheritance patterns. Treatment varies according to the severity of the disease and includes plasma-derived clotting factor concentrates and other medications.
A condition that is characterised by autosomal inheritance and is a disorder that is charaterised by prolonged bleeding time.
A condition that is characterised by autosomal inheritance and is a disorder that is charaterised by prolonged bleeding time.
A condition that is characterised by autosomal inheritance and is a disorder that is charaterised by prolonged bleeding time.
