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Vanishing Bile Duct Syndrome
Vanishing bile duct syndrome is a loose collection of diseases leading to hepatic bile duct injury and eventual ductopenia. Signs and symptoms The presentation is dependent upon the underlying cause. The course can be rapid or chronic. * Fatigue * Anorexia * Abdominal pain * Weight loss * Pruritus * Hyperlipidemia * Malabsorption * Fat-soluble vitamin deficiencies * Elevated alkaline phosphatase * Elevated gamma-glutamyltransferase * Elevated conjugated bilirubin Cause Congenital In fetal and neonatal life, the ductal plates are remodeled. The malformations can be atretic or fibrocystic. Atretic causes * Intrahepatic bile duct atresia (Alagille syndrome) (ALGS2 MIM:610205 and ALGS1 MIM:118450) * Extrahepatic bile duct atresia Fibrocystic causes * Autosomal recessive polycystic kidney disease * Congential hepatic fibrosis * Caroli's disease * Von Meyenburg complex Chromosomal associations * Trisomy 17, 18 and 21 Genetic associations * Cystic fibrosis * Alpha 1 antit ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gastroenterology
Gastroenterology (from the Greek gastḗr- "belly", -énteron "intestine", and -logía "study of") is the branch of medicine focused on the digestive system and its disorders. The digestive system consists of the gastrointestinal tract, sometimes referred to as the ''GI tract,'' which includes the esophagus, stomach, small intestine and large intestine as well as the accessory organs of digestion which include the pancreas, gallbladder, and liver. The digestive system functions to move material through the GI tract via peristalsis, break down that material via digestion, absorb nutrients for use throughout the body, and remove waste from the body via defecation. Physicians who specialize in the medical specialty of gastroenterology are called gastroenterologists or sometimes ''GI doctors''. Some of the most common conditions managed by gastroenterologists include gastroesophageal reflux disease, gastrointestinal bleeding, irritable bowel syndrome, inflammatory bowel disease (IBD ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Trisomy 18
Trisomy 18, also known as Edwards syndrome, is a genetic disorder caused by the presence of a third copy of all or part of chromosome 18. Many parts of the body are affected. Babies are often born small and have heart defects. Other features include a small head, small jaw, clenched fists with overlapping fingers, and severe intellectual disability. Most cases of trisomy 18 occur due to problems during the formation of the reproductive cells or during early development. The chance of this condition occurring increases with the mother's age. Rarely, cases may be inherited. Occasionally, not all cells have the extra chromosome, known as mosaic trisomy, and symptoms in these cases may be less severe. An ultrasound during pregnancy can increase suspicion for the condition, which can be confirmed by amniocentesis. Treatment is supportive. After having one child with the condition, the risk of having a second is typically around one percent. It is the second-most common co ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Supportive Treatment
Symptomatic treatment, supportive care, supportive therapy, or palliative treatment is any medical therapy of a disease that only affects its symptoms, not the underlying cause. It is usually aimed at reducing the signs and symptoms for the comfort and well-being of the patient, but it also may be useful in reducing organic consequences and sequelae of these signs and symptoms of the disease. In many diseases, even in those whose etiologies are known (e.g., most viral diseases, such as influenza and Rift Valley fever), symptomatic treatment is the only treatment available so far. For more detail, see supportive therapy. For conditions like cancer, arthritis, neuropathy, tendinopathy, and injury, it can be useful to distinguish treatments that are supportive/palliative and cannot alter the natural history of the disease ( disease modifying treatments). Examples Examples of symptomatic treatments: * Analgesics, to reduce pain * Anti-inflammatory agents, for inflammation ca ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ischemia
Ischemia or ischaemia is a restriction in blood supply to any tissue, muscle group, or organ of the body, causing a shortage of oxygen that is needed for cellular metabolism (to keep tissue alive). Ischemia is generally caused by problems with blood vessels, with resultant damage to or dysfunction of tissue, i.e., hypoxia and microvascular dysfunction. It also implies local hypoxia in a part of a body resulting from constriction (such as vasoconstriction, thrombosis, or embolism). Ischemia causes not only insufficiency of oxygen but also reduced availability of nutrients and inadequate removal of metabolic wastes. Ischemia can be partial (poor perfusion) or total blockage. The inadequate delivery of oxygenated blood to the organs must be resolved either by treating the cause of the inadequate delivery or reducing the oxygen demand of the system that needs it. For example, patients with myocardial ischemia have a decreased blood flow to the heart and are prescribe ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Chlorpromazine
Chlorpromazine (CPZ), marketed under the brand names Thorazine and Largactil among others, is an antipsychotic medication. It is primarily used to treat psychotic disorders such as schizophrenia. Other uses include the treatment of bipolar disorder, severe behavioral problems in children including those with attention deficit hyperactivity disorder, nausea and vomiting, anxiety before surgery, and hiccups that do not improve following other measures. It can be given orally (by mouth), by intramuscular injection (injection into a muscle), or intravenously (injection into a vein). Chlorpromazine is in the typical antipsychotic class, and, chemically, is one of the phenothiazines. Its mechanism of action is not entirely clear but is believed to be related to its ability as a dopamine antagonist. It has antiserotonergic and antihistaminergic properties. Common side effects include movement problems, sleepiness, dry mouth, low blood pressure upon standing, and incr ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Graft-versus-host Disease
Graft-versus-host disease (GvHD) is a syndrome, characterized by inflammation in different organs. GvHD is commonly associated with bone marrow transplants and stem cell transplants. White blood cells of the donor's immune system which remain within the donated tissue (the graft) recognize the recipient (the host) as foreign (non-self). The white blood cells present within the transplanted tissue then attack the recipient's body's cells, which leads to GvHD. This should not be confused with a transplant rejection, which occurs when the immune system of the transplant recipient rejects the transplanted tissue; GvHD occurs when the donor's immune system's white blood cells reject the recipient. The underlying principle ( alloimmunity) is the same, but the details and course may differ. GvHD can also occur after a blood transfusion, known as ''Transfusion-associated graft-versus-host disease'' or TA-GvHD if the blood products used have not been gamma irradiated or treated ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hodgkin's Lymphoma
Hodgkin lymphoma (HL) is a type of lymphoma in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells (RS cells) are present in the lymph nodes. The condition was named after the English physician Thomas Hodgkin, who first described it in 1832. Symptoms may include fever, night sweats, and weight loss. Often, non-painful enlarged lymph nodes occur in the neck, under the arm, or in the groin. Persons affected may feel tired or be itchy. The two major types of Hodgkin lymphoma are classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. About half of cases of Hodgkin lymphoma are due to Epstein–Barr virus (EBV) and these are generally the classic form. Other risk factors include a family history of the condition and having HIV/AIDS. Diagnosis is conducted by confirming the presence of cancer and identifying Reed–Sternberg cells in lymph node biopsies. The virus-positive cases are ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Primary Sclerosing Cholangitis
Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts, which normally allow bile to drain from the gallbladder. Affected individuals may have no symptoms or may experience signs and symptoms of liver disease, such as jaundice, pruritus, itching, and abdominal pain. The bile duct scarring that occurs in PSC narrows the ducts of the Biliary tract, biliary tree and impedes the flow of bile to the duodenum. Eventually, it can lead to cirrhosis of the liver and liver failure. PSC increases the risk of various cancers, including hepatocellular carcinoma, liver cancer, gallbladder carcinoma, colorectal cancer, and cholangiocarcinoma. The underlying cause of PSC is unknown. Genetic susceptibility, autoimmunity, immune system dysfunction, and dysbiosis, abnormal composition of the gut flora may play a role. This is further suggested by the observation that around 75% of individual ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Primary Biliary Cirrhosis
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis. Further slow damage to the liver tissue can lead to scarring, fibrosis, and eventually cirrhosis. Common symptoms are tiredness, itching, and in more advanced cases, jaundice. In early cases, the only changes may be those seen in blood tests. PBC is a relatively rare disease, affecting up to one in 3,000–4,000 people. As with many other autoimmune diseases, it is much more common in women, with a sex ratio of at least 9:1 female to male. The reasons for this disparity are unclear, but may involve the expression of sex hormones such as estrogen, which impact immune system response. The condition has been recognised since at least 1851, and was named "primary biliary cirrhosis" i ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Antigen
In immunology, an antigen (Ag) is a molecule, moiety, foreign particulate matter, or an allergen, such as pollen, that can bind to a specific antibody or T-cell receptor. The presence of antigens in the body may trigger an immune response. Antigens can be proteins, peptides (amino acid chains), polysaccharides (chains of simple sugars), lipids, or nucleic acids. Antigens exist on normal cells, cancer cells, parasites, viruses, fungus, fungi, and bacteria. Antigens are recognized by antigen receptors, including antibodies and T-cell receptors. Diverse antigen receptors are made by cells of the immune system so that each cell has a specificity for a single antigen. Upon exposure to an antigen, only the lymphocytes that recognize that antigen are activated and expanded, a process known as clonal selection. In most cases, antibodies are ''antigen-specific'', meaning that an antibody can only react to and bind one specific antigen; in some instances, however, antibodies may cr ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
T Cell
T cells (also known as T lymphocytes) are an important part of the immune system and play a central role in the adaptive immune response. T cells can be distinguished from other lymphocytes by the presence of a T-cell receptor (TCR) on their cell surface receptor, cell surface. T cells are born from hematopoietic stem cells, found in the bone marrow. Developing T cells then migrate to the thymus gland to develop (or mature). T cells derive their name from the thymus. After migration to the thymus, the precursor cells mature into several distinct types of T cells. T cell differentiation also continues after they have left the thymus. Groups of specific, differentiated T cell subtypes have a variety of important functions in controlling and shaping the immune response. One of these functions is immune-mediated cell death, and it is carried out by two major subtypes: Cytotoxic T cell, CD8+ "killer" (cytotoxic) and T helper cell, CD4+ "helper" T cells. (These are named for the presen ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Byler's Disease
Progressive familial intrahepatic cholestasis (PFIC) is a group of familial cholestatic conditions caused by defects in biliary epithelial transporters. The clinical presentation usually occurs first in childhood with progressive cholestasis. This usually leads to failure to thrive, cirrhosis, and the need for liver transplantation. Types Types of progressive familial intrahepatic cholestasis are as follows: * Type 1 (OMIM #211600), also called Byler disease * Type 2 (OMIM #601847), also called ABCB11 deficiency or BSEP deficiency * Type 3 (OMIM #602347), also called ABCB4 deficiency or MDR3 deficiency * Type 4 (OMIM #615878), from mutation in '' TJP2'' Signs and symptoms The onset of the disease is usually before age 2, but patients have been diagnosed with PFIC even into adolescence. Of the three entities, PFIC-1 usually presents earliest. Patients usually present in early childhood with cholestasis, jaundice, and failure to thrive. Intense pruritus is characteristic; in p ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
