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Congenital Hepatic Fibrosis
Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis that do not alter hepatic lobular architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension. Cause The condition is usually congenital, but sporadic cases have also been reported. It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation. Mechanism Embryogenically, congenital hepatic fibrosis is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive hepatocytes, which differentiate into cholangiocytes. Congenital hepatic fibrosis usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhoo ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Recessive Gene
In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and the second recessive. This state of having two different variants of the same gene on each chromosome is originally caused by a mutation in one of the genes, either new (''de novo'') or inherited. The terms autosomal dominant or autosomal recessive are used to describe gene variants on non-sex chromosomes ( autosomes) and their associated traits, while those on sex chromosomes (allosomes) are termed X-linked dominant, X-linked recessive or Y-linked; these have an inheritance and presentation pattern that depends on the sex of both the parent and the child (see Sex linkage). Since there is only one copy of the Y chromosome, Y-linked traits cannot be dominant or recessive. Additionally, there are other forms of dominance such as incomplete d ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hepatocytes
A hepatocyte is a cell of the main parenchymal tissue of the liver. Hepatocytes make up 80% of the liver's mass. These cells are involved in: * Protein synthesis * Protein storage * Transformation of carbohydrates * Synthesis of cholesterol, bile salts and phospholipids * Detoxification, modification, and excretion of exogenous and endogenous substances * Initiation of formation and secretion of bile Structure The typical hepatocyte is cubical with sides of 20-30 μm, (in comparison, a human hair has a diameter of 17 to 180 μm).The diameter of human hair ranges from 17 to 181 μm. The typical volume of a hepatocyte is 3.4 x 10−9 cm3. Smooth endoplasmic reticulum is abundant in hepatocytes, in contrast to most other cell types. Microanatomy Hepatocytes display an eosinophilic cytoplasm, reflecting numerous mitochondria, and basophilic stippling due to large amounts of smooth endoplasmic reticulum and free ribosomes. Brown lipofuscin granules are also observed ( ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Biliary Hamartomas
A bile duct hamartoma or biliary hamartoma, is a benign tumour-like malformation of the liver. They are classically associated with polycystic liver disease, as may be seen in the context of polycystic kidney disease, and represent a malformation of the liver plate. Cause It is a developmental anomaly in which abnormal tissues are present at normal site. Due to failure of regression of embryonic biliary duct. Diagnosis File:Histopathology of a bile duct hamartoma, high magnification.jpg, Histopathology of a bile duct hamartoma, high magnification, H&E stain. It shows typical features of bile duct hamartoma: Topic Completed: 23 November 2020. Minor changes: 23 November 2020 - Small to medium sized, irregularly shaped bile ducts lined by bland cuboidal epithelium (may also be flattened).- Prominent intervening collagenous stroma. - Bile ducts containing eosinophilic debris (may also contain inspissated bile) File:Von Meyenburg complex cropped.tif, von Meyenburg Complex in ultras ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Von Meyenburg Complex
A bile duct hamartoma or biliary hamartoma, is a benign tumour-like malformation of the liver. They are classically associated with polycystic liver disease, as may be seen in the context of polycystic kidney disease, and represent a malformation of the liver plate. Cause It is a developmental anomaly in which abnormal tissues are present at normal site. Due to failure of regression of embryonic biliary duct. Diagnosis File:Histopathology of a bile duct hamartoma, high magnification.jpg, Histopathology of a bile duct hamartoma, high magnification, H&E stain. It shows typical features of bile duct hamartoma: Topic Completed: 23 November 2020. Minor changes: 23 November 2020 - Small to medium sized, irregularly shaped bile ducts lined by bland cuboidal epithelium (may also be flattened).- Prominent intervening collagenous stroma. - Bile ducts containing eosinophilic debris (may also contain inspissated bile) File:Von Meyenburg complex cropped.tif, von Meyenburg Complex in ultras ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Polycystic Kidney Disease
Polycystic kidney disease (PKD or PCKD, also known as polycystic kidney syndrome) is a genetic disorder in which the renal tubules become structurally abnormal, resulting in the development and growth of multiple cysts within the kidney. These cysts may begin to develop in utero, in infancy, in childhood, or in adulthood. Cysts are non-functioning tubules filled with fluid pumped into them, which range in size from microscopic to enormous, crushing adjacent normal tubules and eventually rendering them non-functional as well. PKD is caused by abnormal genes that produce a specific abnormal protein; this protein has an adverse effect on tubule development. PKD is a general term for two types, each having their own pathology and genetic cause: autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). The abnormal gene exists in all cells in the body; as a result, cysts may occur in the liver, seminal vesicles, and pancreas. This ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Caroli Disease
Caroli disease (communicating cavernous ectasia, or congenital cystic dilatation of the intrahepatic biliary tree) is a rare inherited disorder characterized by cystic dilatation (or ectasia) of the bile ducts within the liver. There are two patterns of Caroli disease: focal or simple Caroli disease consists of abnormally widened bile ducts affecting an isolated portion of liver. The second form is more diffuse, and when associated with portal hypertension and congenital hepatic fibrosis, is often referred to as "Caroli syndrome". The underlying differences between the two types are not well understood. Caroli disease is also associated with liver failure and polycystic kidney disease. The disease affects about one in 1,000,000 people, with more reported cases of Caroli syndrome than of Caroli disease. Caroli disease is distinct from other diseases that cause ductal dilatation caused by obstruction, in that it is not one of the many choledochal cyst derivatives. Signs and symptom ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hepatomegaly
Hepatomegaly is the condition of having an enlarged liver. It is a non-specific medical sign having many causes, which can broadly be broken down into infection, hepatic tumours, or metabolic disorder. Often, hepatomegaly will present as an abdominal mass. Depending on the cause, it may sometimes present along with jaundice. Signs and symptoms The individual may experience many symptoms, including weight loss, poor appetite and lethargy (jaundice and bruising may also be present). Causes Among the causes of hepatomegaly are the following: Infective Mechanism The mechanism of hepatomegaly consists of vascular swelling, inflammation (due to the various causes that are infectious in origin) and deposition of (1) non-hepatic cells or (2) increased cell contents (such due to iron in hemochromatosis or hemosiderosis and fat in fatty liver disease). Diagnosis Suspicion of hepatomegaly indicates a thorough medical history and physical examination, wherein the latter typically incl ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cholangitis (other)
Cholangitis is any inflammation of the biliary tree, including: *Ascending cholangitis, a severe acute bacterial infection associated with gallstones in the common bile duct *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 ha ..., a chronic autoimmune disease leading to liver failure * Secondary sclerosing cholangitis, an umbrella term for other unrelated medical conditions that cause sclerosis of the bile ducts {{disambiguation ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cholangiocytes
Cholangiocytes are the epithelial cells of the bile duct. They are cuboidal epithelium in the small interlobular bile ducts, but become columnar and HCO3:-secreting in larger bile ducts approaching the porta hepatis and the extrahepatic ducts. They contribute to hepatocyte survival by transporting bile acids. Function In the healthy liver, cholangiocytes contribute to bile secretion via release of bicarbonate and water. Several hormones and locally acting mediators are known to contribute to cholangiocyte fluid/electrolyte secretion. These include secretin, acetylcholine, ATP, and bombesin. Cholangiocytes act through bile-acid independent bile flow, which is driven by the active transport of electrolytes. In contrast, hepatocytes secrete bile through bile-acid dependent bile flow, which is coupled to canalicular secretion of bile acids via ATP-driven transporters. This results in passive transcellular and paracellular secretion of fluid and electrolytes through an osmotic effect ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gestation
Gestation is the period of development during the carrying of an embryo, and later fetus, inside viviparous animals (the embryo develops within the parent). It is typical for mammals, but also occurs for some non-mammals. Mammals during pregnancy can have one or more gestations at the same time, for example in a multiple birth. The time interval of a gestation is called the '' gestation period''. In obstetrics, ''gestational age'' refers to the time since the onset of the last menses, which on average is fertilization age plus two weeks. Mammals In mammals, pregnancy begins when a zygote (fertilized ovum) implants in the female's uterus and ends once the fetus leaves the uterus during labor or an abortion (whether induced or spontaneous). Humans In humans, pregnancy can be defined clinically or biochemically. Clinically, pregnancy starts from first day of the mother's last period. Biochemically, pregnancy starts when a woman's human chorionic gonadotropin (hCG) levels ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gasteroenterology
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 includes 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, irritable bowel disease ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Duct Plate
The word duct is derived from the Latin word for ''led/leading''. It may refer to: * Duct (anatomy), various ducts in anatomy and physiology ** Tear duct, which carry tears to the eyes * Duct (HVAC), for transfer of air between spaces in a structure * Duct tape, a kind of adhesive tape * Ducted fan, motor for aircraft * Electrical bus duct, a metal enclosure for busbars * Duct (industrial exhaust), industrial exhaust duct system designed for low pressure-pneumatic convey of gas, fumes, dusts, shavings, and other pollutants from works space to atmosphere after cleaning and removal of contaminants * Atmospheric duct, a horizontal layer in the lower atmosphere in which the vertical refractive index gradients are such that radio signals (a) are guided or ducted, (b) tend to follow the curvature of the Earth, and (c) experience less attenuation in the ducts than they would if the ducts were not present ** Tropospheric ducting, a type of radio propagation in the troposphere that allows s ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
