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The Deadly Secret of Immune System Disorders: What is IPEX Syndrome?
When exploring the complexity of the immune system, one of the worrisome symptoms is immune system dysregulation. Such imbalances may not only lead to autoimmune diseases, but may also cause other health problems. IPEX syndrome, or immune dysregulation, polyglandular endocrine disorder, enteropathy, X-linked syndrome, is one of the rarer but deadly conditions. This disease is mainly caused by gene mutations, and its symptoms often appear early in the patient's life, making it difficult to ignore.
IPEX syndrome is caused by mutations in the FOXP3 gene, a master transcription factor for regulatory T cells (Tregs).
People with the syndrome usually develop diabetes, recurrent diarrhea, developmental delays, eczema, and hemolytic anemia within the first few months of life. These conditions indicate that the patient's immune system has lost the ability to self-regulate its own tissues, resulting in an uncontrolled immune response.
More genetic syndromes of immune disordersIn addition to IPEX syndrome, there are a variety of genetic syndromes associated with immune disorders, such as:
APECED syndromeAutoimmune polyglandular disease-candidiasis-endodermal dysplasia (APECED) is caused by mutations in the AIRE gene and usually presents as mucocutaneous candidal infections and various endocrine autoimmune diseases.
Omenn SyndromeThis syndrome is similar to graft-versus-host disease (GVHD), with immune dysregulation due to overproduction of IgE. Omenn syndrome-related gene mutations usually occur in genes such as RAG1 and RAG2.
Wiskott-Aldrich syndromeWiskott-Aldrich syndrome is caused by mutations in the WAS gene and is characterized by increased susceptibility to infection, eczema, and autoimmune hemolytic anemia.
The common feature of these genetic diseases is that the patients' immune systems show varying degrees of disorder and overreaction.
Immune disorders related to stress
Chronic stress in an individual's life may also lead to persistent dysregulation of the immune system. Studies have shown that children who are abused or neglected during childhood have a significantly increased risk of developing cardiovascular disease, type 2 diabetes and other immune disorders when they grow up.
Aging immune system
As we age, our immune system gradually declines, a phenomenon known as immunosenescence. Immunosenescence reduces the ability of T cells and B cells to respond to vaccines or infections, making patients more susceptible to infections and autoimmune diseases.
Toxin-induced immune disorders
The immune system may also become dysregulated due to exposure to toxins. For example, certain pesticides in the environment may cause damage to the immune system, and the extent of these effects is closely related to the individual's age, dose, and duration of exposure.
Mechanisms of allergic reactions
Allergy is an erroneous response of the immune system to common substances in the environment. This response involves the action of multiple immune cells and cytokines, the purpose of which is to expel allergens to protect the body.
Studies have shown that the mechanism of allergic reactions may be closely related to the interaction of genetic and environmental factors, especially the view put forward by the hygiene hypothesis that children living in developed countries are more susceptible to allergies due to lack of pathogen exposure.
What is worrying is that these disorders of the immune system do not exist in isolation, but interact in complex ways with each other. As we face these serious health challenges, it is necessary to think deeply: In future medical advances, can we find effective ways to address these immune system disorders?
