Fabry disease in focus

Management of Fabry disease

The information on this website is intended only to provide knowledge of Fabry disease health topics. This information should not be used in place of advice from your GP or other healthcare professional.

The Focus on Fabry website is intended for residents of the United Kingdom only. Focus on Fabry has been produced by Shire HGT, a business within the Shire group of companies. The website has been developed in accordance with industry and legal standards to provide information for the general public, patients and their families, and also healthcare professionals about Fabry disease health topics. Shire HGT makes every reasonable effort to include accurate and current information. However, the information provided on the website is not exhaustive. References for published sources of information cited on the Focus on Fabry website have been provided on the relevant pages. Please note that external links to other websites have been restricted to comply with UK pharmaceutical industry guidelines.

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Patient glossary

ACE inhibitor

Angiotensin-converting enzyme inhibitor - a type of drug used to treat high blood pressure


Acroparesthesia

Tingling sensations or numbness in the extremities (e.g. hands, fingers, toes)


a-Gal A

Alpha-galactosidase A - an enzyme that helps to break down globotriaosylceramide (Gb3)


Alpha-galactosidase A

An enzyme that helps to break down globotriaosylceramide (Gb3)


Anderson-Fabry disease

A lysosomal storage disorder (LSD) named after the doctors who first identified the disease; it is also known as Fabry disease


Angiokeratoma

A skin rash, which appears as small, raised, dark-red spots


Anhidrosis

An inability to sweat


Anti-blood clotting drugs

Drugs that prevent blood from thickening, which maintains the flow of blood around the body


Cardiac

Relating to the heart


Cardiologist

A doctor who specializes in problems of the heart


Central nervous system

The body's control network - consisting of the brain and the spinal cord


Chromosome

The structure in each cell of the body that contains a person’s DNA; humans have 46 chromosomes in each cell


Cornea

The transparent front part of the eye that helps the eye to focus


Cornea verticillata

A whorl-like opaque area on the cornea, which can be seen during an eye examination when using special equipment


Dermatologist

A doctor who specializes in problems of the skin


Dialysis

The process of removing waste products from the blood using a special machine


DNA

Deoxyribonucleic acid - the substance that contains the genetic instructions for the development of life in all living organisms


Enzyme

A protein that helps to speed up chemical reactions in living organisms


Enzyme replacement therapy (ERT)

A type of treatment which replaces a deficient enzyme in the body


Fabry disease

A lysosomal storage disorder (LSD); it is also known as Anderson-Fabry disease


Foetus

An unborn baby


Gastrointestinal

Relating to the digestive system (e.g. stomach, large and small bowel)


Gaucher disease

A lysosomal storage disorder (LSD) that is caused by the deficiency of an enzyme called 'glucocerebrosidase'


Gb3 (also known as GL3 or CTH)

Globotriaosylceramide - a fatty substance that is normally broken down by alpha-galactosidase A, and builds up in the body in patients with Fabry disease


Gene

A sequence of DNA that codes for a particular hereditary characteristic


Genetics

The science of inheritance


Globotriaosylceramide

A fatty substance that is normally broken down by alpha-galactosidase A, which builds up in the body in patients with Fabry disease


Haemorrhagic stroke

A type of stroke caused by a blood vessel bursting, which interrupts the flow of blood to the brain


Hereditary

The transfer of characteristics from a parent to a child


Hunter syndrome

A lysosomal storage disorder (LSD) that is caused by the deficiency of an enzyme called 'iduronate-2-sulfatase'


Hurler disease

A lysosomal storage disorder (LSD) that is caused by the deficiency of an enzyme called 'alpha-L iduronidase'


Hyperhidrosis

An abnormal increase in sweating


Hypohidrosis

An abnormal decrease in sweating


Infusion

The process of introducing a fluid into the body, usually through a vein (intravenous infusion)


Irritable bowel syndrome

A chronic disorder of the digestive system, which causes pain and discomfort


Ischaemic stroke

A type of stroke caused by a blockage that stops blood from reaching the brain


Left ventricular enlargement

An abnormal increase in size of one of the chambers of the heart


Lysosomal storage disorders (LSDs)

A group of rare genetic medical disorders caused by defects in the lysosomes; also known as lysosomal storage diseases


Lysosome

A subunit of human cells that contains enzymes to help break down waste products


Metabolic disease

A medical disorder that affects the production of energy in human cells


Molecule

The smallest unit of a substance


Mucopolysaccharide diseases

A group of genetic diseases caused by defects in lysosomes


Mutation

A change that takes place in a gene while a cell is dividing, e.g. in the production of eggs or sperm


Nausea

Feeling sick


Nephrologist

A doctor who specializes in problems of the kidneys


Neurologist

A doctor who specializes in problems of the nervous system (including the brain, spinal cord and nerves)


Neurovascular

Relating to the interaction between the nervous system and blood vessels


Ophthalmologist

A doctor who specializes in problems with the eyes


Opiate-based pain relief

A medicine that is given to reduce severe pain, and which is related to morphine


Pacemaker

A medical device that helps to regulate the heartbeat


Protein

A large molecule that forms an essential part of all living cells; all enzymes are proteins


Proteinuria

An abnormal increase in protein in the urine


Renal

Relating to the kidneys


Sign

A change caused by a disorder that is seen by a physician but is not always noticed by the patient


Stroke

A loss of brain function caused by the interruption of the blood supply to the brain


Symptom

A change caused by a disorder that is noticed by the patient


Tinnitus

A ringing sound in the ear


Vertigo

A sensation of dizziness or disorder of balance


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Page last updated: 10-Feb-2010