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Autoimmune Enteropathy
Autoimmune enteropathy is a rare autoimmune disorder characterized by weight loss from malabsorption, severe and protracted diarrhea, and autoimmune damage to the intestinal mucosa. Autoimmune enteropathy typically occurs in infants and younger children however, adult cases have been reported in literature. Autoimmune enteropathy was first described by Walker-Smith et al. in 1982. The mechanisms of autoimmune enteropathy isn't well known but dysfunction or deficiency of CD25+CD4+ regulatory T cells may play a role. Numerous other illnesses and syndromes are linked to autoimmune enteropathy, the most prominent being Autoimmune polyendocrine syndrome type 1 and immune dysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome. Clinical symptoms, laboratory results, and the histological characteristics of a small bowel biopsy are used to make the diagnosis. These patients typically don't respond to diet modification and often require immune-suppressants and sometimes re ...
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Histology
Histology, also known as microscopic anatomy or microanatomy, is the branch of biology that studies the microscopic anatomy of biological tissue (biology), tissues. Histology is the microscopic counterpart to gross anatomy, which looks at larger structures visible without a microscope. Although one may divide microscopic anatomy into ''organology'', the study of organs, ''histology'', the study of tissues, and ''cytology'', the study of cell (biology), cells, modern usage places all of these topics under the field of histology. In medicine, histopathology is the branch of histology that includes the microscopic identification and study of diseased tissue. In the field of paleontology, the term paleohistology refers to the histology of fossil organisms. Biological tissues Animal tissue classification There are four basic types of animal tissues: muscle tissue, nervous tissue, connective tissue, and epithelial tissue. All animal tissues are considered to be subtypes of these ...
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Corticosteroid
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involved in a wide range of physiological processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior. Some common naturally occurring steroid hormones are cortisol (), corticosterone (), cortisone () and aldosterone () (cortisone and aldosterone are isomers). The main corticosteroids produced by the adrenal cortex are cortisol and aldosterone. The etymology of the '' cortico-'' part of the name refers to the adrenal cortex, which makes these steroid hormones. Thus a corticosteroid is a "cortex steroid". Classes * Glucocorticoids such as cortisol affect carbohydrate, fat, and protein metabolism, and have anti ...
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Failure To Thrive
Failure to thrive (FTT), also known as weight faltering or faltering growth, indicates insufficient weight gain or absence of appropriate physical growth in children. FTT is usually defined in terms of weight, and can be evaluated either by a low weight for the child's age, or by a low rate of increase in the weight. The term "failure to thrive" has been used in different ways, as no single objective standard or universally accepted definition exists for when to diagnose FTT. One definition describes FTT as a fall in one or more weight centile spaces on a World Health Organization (WHO) growth chart depending on birth weight or when weight is below the 2nd percentile of weight for age irrespective of birth weight. Another definition of FTT is a weight for age that is consistently below the fifth percentile or weight for age that falls by at least two major percentile lines on a growth chart. While weight loss after birth is normal and most babies return to their birth weight by t ...
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Malnourishment
Malnutrition occurs when an organism gets too few or too many nutrients, resulting in health problems. Specifically, it is a deficiency, excess, or imbalance of energy, protein and other nutrients which adversely affects the body's tissues and form. Malnutrition is a category of diseases that includes undernutrition and overnutrition. Undernutrition is a lack of nutrients, which can result in stunted growth, wasting, and being underweight. A surplus of nutrients causes overnutrition, which can result in obesity or toxic levels of micronutrients. In some developing countries, overnutrition in the form of obesity is beginning to appear within the same communities as undernutrition. Most clinical studies use the term 'malnutrition' to refer to undernutrition. However, the use of 'malnutrition' instead of 'undernutrition' makes it impossible to distinguish between undernutrition and overnutrition, a less acknowledged form of malnutrition. Accordingly, a 2019 report by The Lance ...
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Intestinal Mucosal Barrier
The intestinal mucosal barrier, also referred to as intestinal barrier, refers to the property of the intestinal mucosa that ensures adequate containment of undesirable luminal contents within the intestine while preserving the ability to absorb nutrients. The separation it provides between the body and the gut prevents the uncontrolled translocation of luminal contents into the body proper. Its role in protecting the mucosal tissues and circulatory system from exposure to pro-inflammatory molecules, such as microorganisms, toxins, and antigens is vital for the maintenance of health and well-being. Intestinal mucosal barrier dysfunction has been implicated in numerous health conditions such as: food allergies, microbial infections, irritable bowel syndrome, inflammatory bowel disease, celiac disease, metabolic syndrome, non-alcoholic fatty liver disease, diabetes, and septic shock. Composition The intestinal mucosal barrier is a heterogeneous entity composed of physical, bioch ...
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Immunotherapy
Immunotherapy or biological therapy is the treatment of disease by activating or suppressing the immune system. Immunotherapies designed to elicit or amplify an immune response are classified as ''activation immunotherapies,'' while immunotherapies that reduce or suppress are classified as '' suppression immunotherapies''. Immunotherapy is under preliminary research for its potential to treat various forms of cancer. Cell-based immunotherapies are effective for some cancers. Immune effector cells such as lymphocytes, macrophages, dendritic cells, natural killer cells, and cytotoxic T lymphocytes work together to defend the body against cancer by targeting abnormal antigens expressed on the surface of tumor cells. Vaccine-induced immunity to COVID-19 relies mostly on an immunomodulatory T-cell response. Therapies such as granulocyte colony-stimulating factor (G-CSF), interferons, imiquimod and cellular membrane fractions from bacteria are licensed for medical use. Others in ...
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Immunosuppressants
Immunosuppressive drugs, also known as immunosuppressive agents, immunosuppressants and antirejection medications, are drugs that inhibit or prevent the activity of the immune system. Classification Immunosuppressive drugs can be classified into five groups: * glucocorticoids * cytostatics * antibodies * drugs acting on immunophilins * other drugs Glucocorticoids In pharmacologic (supraphysiologic) doses, glucocorticoids, such as prednisone, dexamethasone, and hydrocortisone are used to suppress various allergic, inflammatory, and autoimmune disorders. They are also administered as posttransplantory immunosuppressants to prevent the acute transplant rejection and graft-versus-host disease. Nevertheless, they do not prevent an infection and also inhibit later reparative processes. Immunosuppressive mechanism Glucocorticoids suppress cell-mediated immunity. They act by inhibiting gene expression of cytokines including Interleukin 1 (IL-1), IL-2, IL-3, IL-4, IL-5, I ...
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Small Bowel
The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through the pancreatic duct to aid in digestion. The small intestine is about long and folds many times to fit in the abdomen. Although it is longer than the large intestine, it is called the small intestine because it is narrower in diameter. The small intestine has three distinct regions – the duodenum, jejunum, and ileum. The duodenum, the shortest, is where preparation for absorption through small finger-like protrusions called intestinal villi begins. The jejunum is specialized for the absorption through its lining by enterocytes: small nutrient particles which have been previously digested by enzymes in the duodenum. The main function of the ileum is to absorb vitamin B12, bile salts, and whatever products of digestion that were not absorbed ...
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IPEX Syndrome
Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX syndrome) is a rare autoimmune disease. It is one of the autoimmune polyendocrine syndromes. Most often, IPEX presents with autoimmune enteropathy, dermatitis (eczema), and autoimmune endocrinopathy (most often Type 1 diabetes), but other presentations exist. IPEX is caused by mutations in the gene ''FOXP3'', which encodes transcription factor forkhead box P3 (FOXP3). FOXP3 is widely considered to be the master regulator of the regulatory T cell (Treg) lineage. ''FOXP3'' mutation can lead to the dysfunction of CD4+ Tregs. In healthy people, Tregs maintain immune homeostasis. When there is a deleterious ''FOXP3'' mutation, Tregs do not function properly and cause autoimmunity. IPEX onset usually happens in infancy. If left untreated, it is often fatal by the age of 2 or 3. A bone marrow transplant is generally considered the best treatment option. IPEX exclusively affects males and is inherited in an X-l ...
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Autoimmune Polyendocrine Syndrome Type 1
Autoimmune polyendocrine syndrome type 1 (APS-1), is a subtype of autoimmune polyendocrine syndrome (autoimmune polyglandular syndrome). It causes the dysfunction of multiple endocrine glands due to autoimmunity. It is a genetic disorder, inherited in autosomal recessive fashion due to a defect in the ''AIRE'' gene (autoimmune regulator), which is located on chromosome 21 and normally confers immune tolerance. Signs and symptoms APS-1 tends to cause severe symptoms. These are present from early in life, usually around 3.5 years of age. Common symptoms of APS-1 include: * Chronic mucocutaneous candidiasis. * Hypoparathyroidism. * Addison's disease. * Ectodermal dystrophy (skin, dental enamel, and nails). APS-1 may also cause: * Autoimmune hepatitis. * Hypogonadism. * Vitiligo. * Alopecia. * Malabsorption. * Pernicious anemia. * Cataract. * Cerebellar ataxia. Cause APS-1 is caused by a mutation in the ''AIRE'' gene, encoding a protein called autoimmune regulator. ...
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Regulatory T Cell
The regulatory T cells (Tregs or Treg cells), formerly known as suppressor T cells, are a subpopulation of T cells that modulate the immune system, maintain immune tolerance, tolerance to self-antigens, and prevent autoimmune disease. Treg cells are immunosuppression, immunosuppressive and generally suppress or downregulation and upregulation, downregulate induction and proliferation of effector T cells. Treg cells express the biomarkers CD4, FOXP3, and CD25 and are thought to be derived from the same cell lineage, lineage as naïve T helper cell, CD4+ cells. Because effector T cells also express CD4 and CD25, Treg cells are very difficult to effectively discern from effector CD4+, making them difficult to study. Research has found that the cytokine Transforming growth factor beta, transforming growth factor beta (TGF-β) is essential for Treg cells to differentiate from naïve CD4+ cells and is important in maintaining Treg cell homeostas ...
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IL2RA
The interleukin-2 receptor alpha chain (also called Tac antigen, P55, and mainly CD25) is a protein involved in the assembly of the high-affinity interleukin-2 receptor, consisting of alpha (''IL2RA''), beta ('' IL2RB'') and the common gamma chain (''IL2RG''). As the name indicates, this receptor interacts with interleukin-2, a pleiotropic cytokine which plays an important role in immune homeostasis. Genetics The human protein interleukin-2 receptor subunit alpha is encoded by a gene called ''IL2RA'' with a length around 51,6 kb. Alternative names for this protein coding gene are ''IL2R'', ''IDDM10'' and ''TCGFR''. Location of ''IL2RA'' in human genome is on the short arm of 10th chromosome (10p15.1). Several frequent point mutations, single nucleotide polymorphism (SNP), have been identified in or in close proximity to ''IL2RA'' gene in the population. These SNPs have been linked mainly to susceptibility to immune dysregulation disorders, with majority found in research on ...
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