abb-entrelec XLP1 – 6BC – desconectador Fuse 3 Poles

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It is part of the herpes family of viruses and is passed on through contact with bodily fluids, including saliva. Blood tests are the main form of diagnosis and will be used to measure the levels of SAP present in the blood. If lymphoproliferation is severe, giving immunosuppressant medications to dampen down (suppress) the immune system may be helpful. The only definite ‘cure’ for XLP at present is a haematopoietic stem cell transplant (HSCT), where the bone marrow is replaced with donated stem cells that do not contain the overactive immune cells. The donor cells are given by intravenous infusion and make their way to the recipient bone marrow to provide a new immune system, curing the immunodeficiency.

biopsy surgical removal of a small sample of tissue for examination under a microscope for diagnostic purposes. Girls inherit one X chromosome from each parent, so have a normal one to compensate for the faulty one. If SAP is found to be absent or in low level, the presence of a gene mutation will also be checked through a blood sample.Some people affected by XLP1 are more likely to develop infections than normal and may benefit from regular (prophylactic) antibiotics and immunoglobulin replacement. Children often have an unremarkable childhood until primary-school age and exposure to the Epstein-Barr virus. The outlook for children with XLP1 is improving all the time, owing to better recognition of the condition and improved diagnostic testing so that treatment can be started as quickly as possible. There are two major forms of lymphocytes, B-cells and T-cells, which have distinct but related functions in generating an immune response. There are many conditions that can cause similar symptoms and it is important that careful evaluation is performed to exclude any of these.

If there are neurological symptoms, such as headache, vomiting, irritability, visual disturbances or seizures, a sample of cerebrospinal fluid may be taken by lumbar puncture. Children with XLP1 have a greatly increased risk of developing lymphoma (cancer of the immune system cells) compared with the general population. XLP1 is caused by a mutation (change) in a gene named SH2D1A, which is important for producing a protein that regulates cells of the immune system, known as lymphocytes. Instead of fighting off the infection, some white blood cells (T-cells and macrophages) become over-activated, causing severe inflammation and damage to tissues, such as the liver, spleen and bone marrow. Lymphoma causes symptoms such as fever, fatigue, weight loss, loss of appetite and/or sudden enlargement of one or a few lymph nodes.PID UK is affiliated to the International Patient Organisation for Primary Immunodeficiencies (IPOPI) ( www. immunosuppressants medications that suppress or ‘dampen down’ the immune system; used to suppress inflammation and autoimmunity.

In many cases, XLP1 is an inherited condition, meaning it is passed on in families in the same way that physical characteristics, such as eye colour, are passed from parent to child. There are two specialist centres in the UK that treat children with XLP1 – Great Ormond Street Hospital in London, and the Great North Children’s Hospital in Newcastle. white blood cells (leucocytes) a group of small, colourless blood cells that play a major role in the body’s immune system. A female who has a mistake on one of her two X chromosomes also has a normal X chromosome, which compensates for the abnormal one. New types of medicine are also being developed to treat HLH more effectively and with fewer side effects.In affected individuals, the body cannot properly regulate an important type of white blood cell (lymphocytes) in response to a viral infection, usually the Epstein-Barr virus that causes glandular fever. PID UK is the main support organisation in the UK for anyone affected by a primary immunodeficiency disease. Please note this is a generic GOSH information sheet so should not be used for the diagnosis or treatment of any medical condition.

Prenatal diagnosis is available for future pregnancies, provided that the faulty gene can be identified. haematopoietic stem cell transplant (HSCT) the transfer of stem cells from a donor – either related or unrelated – to a recipient. This appears to trigger the development of symptoms, although the number and severity of symptoms is variable. The most important step is recognising that there is a problem with the immune system and involving the right specialists to investigate and treat further. The aim is to give enough treatment to control the overactive lymphocytes without losing protection against infection, while avoiding other side effects.This page has been produced jointly between PID UK, Great Ormond Street Hospital (GOSH) and the Great North Children’s Hospital. lymphocytes small white blood cells, normally present in the blood and in lymphoid tissue, that carry out the functions of the immune system. Bone marrow is the spongy tissue inside bones and contains cells that produce white blood cells, red blood cells and platelets. A needle is inserted through the skin of the back into the space between the bones that make up the spine.