Caroli Disease
Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.
Muridae
A family of the order Rodentia containing 250 genera including the two genera Mus (MICE) and Rattus (RATS), from which the laboratory inbred strains are developed. The fifteen subfamilies are SIGMODONTINAE (New World mice and rats), CRICETINAE, Spalacinae, Myospalacinae, Lophiomyinae, ARVICOLINAE, Platacanthomyinae, Nesomyinae, Otomyinae, Rhizomyinae, GERBILLINAE, Dendromurinae, Cricetomyinae, MURINAE (Old World mice and rats), and Hydromyinae.
Centromere Protein B
DNA, Satellite
Highly repetitive DNA sequences found in HETEROCHROMATIN, mainly near centromeres. They are composed of simple sequences (very short) (see MINISATELLITE REPEATS) repeated in tandem many times to form large blocks of sequence. Additionally, following the accumulation of mutations, these blocks of repeats have been repeated in tandem themselves. The degree of repetition is on the order of 1000 to 10 million at each locus. Loci are few, usually one or two per chromosome. They were called satellites since in density gradients, they often sediment as distinct, satellite bands separate from the bulk of genomic DNA owing to a distinct BASE COMPOSITION.
Species Specificity
The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species.
Hybridization, Genetic
Embryo Transfer
The transfer of mammalian embryos from an in vivo or in vitro environment to a suitable host to improve pregnancy or gestational outcome in human or animal. In human fertility treatment programs, preimplantation embryos ranging from the 4-cell stage to the blastocyst stage are transferred to the uterine cavity between 3-5 days after FERTILIZATION IN VITRO.
alpha 1-Antitrypsin
Bile Ducts, Intrahepatic
Congenital Disorders of Glycosylation
Bile Ducts
Bile Duct Diseases
Monolobar Caroli's Disease and cholangiocarcinoma. (1/34)
Caroli's Disease (CD) is a rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts. This report describes a patient with cholangiocarcinoma arising in the setting of monolobar CD. In spite of detailed investigations including biliary enteric bypass and endoscopic retrograde cholangiography, the diagnosis of mucinous cholangiocarcinoma (CCA) was not made for almost one year. The presentation, diagnosis and treatment of monolobar CD and the association between monolobar CD and biliary tract cancer are discussed. Hepatic resection is the treatment of choice for monolobar CD. (+info)Clonal chromosomal abnormalities in congenital bile duct dilatation (Caroli's disease). (2/34)
BACKGROUND: Caroli's disease is a rare congenital disorder characterised by cystic dilatation of the intrahepatic bile ducts and an increased risk of cholangiocellular carcinoma. The cause is unknown, but occasional familial clustering suggests that some cases are inherited, in particular when occurring in association with polycystic kidney disease and germline PKD1 gene mutations. To date, no gene responsible for familial isolated Caroli's disease has been identified, and no genetic investigations of liver tissue from patients with Caroli's disease have been reported. PATIENT/METHOD: A liver biopsy specimen from a patient with isolated Caroli's disease, without any signs of cholangiocellular carcinoma, was short term cultured and cytogenetically investigated after G banding with Wright's stain. RESULT: Cytogenetic analysis disclosed the karyotype 45-47,XX,der(3)t(3;8)(p23;q13), +2mar[cp6]/46,XX[18]. CONCLUSIONS: The finding of an unbalanced translocation between chromosomes 3 and 8 suggests that loss of distal 3p and/or gain of 8q is of pathogenetic importance in Caroli's disease. Alternatively, structural rearrangements of genes located in 3p23 and 8q13 may be of the essence. These chromosomal breakpoints may also pinpoint the location of genes involved in inherited forms of Caroli's disease not associated with polycystic kidney disease. (+info)Polycystic kidney rat is a novel animal model of Caroli's disease associated with congenital hepatic fibrosis. (3/34)
Caroli's disease (congenital intrahepatic biliary dilatation) associated with congenital hepatic fibrosis is an autosomal recessive polycystic kidney disease. Recently, the polycystic kidney (PCK) rat, a spontaneous mutant derived from a colony of CRJ:CD rats with polycystic lesions in the liver and an autosomal recessive mode of inheritance, was reported. In the present study, the pathology of the hepatobiliary system and the biliary cell-kinetics were evaluated in fetuses (day 18 to 21 of gestation) and neonates and adults (1 day to 4 months after delivery) of PCK rats. CRJ:CD rats were used as a control. Multiple segmental and saccular dilatations of intrahepatic bile ducts were first observed in fetuses at 19 days of gestation. The dilatation spread throughout the liver and the degree of dilatation increased with aging. Gross and histological features characterizing ductal plate malformation were common in the intrahepatic bile ducts. Overgrowth of portal connective tissue was evident and progressive after delivery. These features were very similar to those of Caroli's disease with congenital hepatic fibrosis. Proliferative activity in the biliary epithelial cells was greater in PCK rats than controls during the development. In contrast, the biliary epithelial apoptosis was less extensive in PCK rats than the controls until 1 week after delivery, but greater after 3 weeks, suggesting that the remodeling defect in immature bile ducts associated with the imbalance of cell kinetics plays a role in the occurrence of intrahepatic biliary anomalies in PCK rats. The PCK rat could be a useful and promising animal model of Caroli's disease with congenital hepatic fibrosis. (+info)An unusual case of biliary atresia. (4/34)
Intrahepatic biliary cysts are rarely seen in the patients with biliary atresia. We describe a ten-month-old child with biliary atresia in whom the abdominal imaging studies (ultrasonography, computed tomographic scan and magnetic resonance cholangiopancreatography) revealed multiple intrahepatic biliary cysts ('bile lakes'). The child also had intrapulmonary shunting of blood due to pulmonary arteriovenous fistulae, which were demonstrated on contrast-enhanced echocardiography. Both these findings, 'bile lakes' and pulmonary arteriovenous fistulae occur rarely in biliary atresia. (+info)Bile duct dysplasia and congenital hepatic fibrosis associated with polycystic kidney (Caroli syndrome) in a rat. (5/34)
Hepatic fibrosis with bile duct ectasia and hyperplasia associated with polycystic kidney disease, analogous to Caroli syndrome in humans, was observed in a rat used as a control in a subchronic toxicity study. Light microscopy of liver sections showed multiple cystic and segmental saccular dilatations and hyperplasia of the intrahepatic bile ducts associated with overgrowth of portal connective tissue; the kidneys had diffuse cystic dilatation of cortical renal tubules. The lesions resembled those of human cases of the fibropolycystic disease termed as Caroli syndrome, which is thought to be the result of a pathologic developmental process known as ductal plate malformation. Recently, an animal model of Caroli syndrome has been described in mutant rats from a colony that constantly showed renal and hepatic cysts and an autosomal recessive mode of inheritance. The finding in our case of identical hepatorenal lesions suggests that the same mutation has occurred incidentally in a standard colony. (+info)Caroli's disease: prenatal diagnosis, postnatal outcome and genetic analysis. (6/34)
Caroli's disease is a rare autosomal recessive condition characterized by cystic dilatation of the intrahepatic bile ducts and infantile polycystic kidney disease. We report a case with Caroli's disease detected prenatally at 33 weeks' gestation with fetal ultrasound findings of a cystic liver mass and echogenic kidneys. Postnatal investigation confirmed enlarged and echogenic kidneys with dilatation of the intrahepatic bile ducts consistent with the diagnosis of Caroli's disease. Genetic analysis of the gene, PKHD1, associated with autosomal recessive polycystic kidney disease (ARPKD) showed that the patient had compound heterozygous mutations, confirming that this early onset Caroli's disease was part of the spectrum of ARPKD. To our knowledge this is the third case of Caroli's disease detected prenatally and the first in which the infant survived. (+info)Prenatal sonographic appearance of congenital bile duct dilatation associated with renal-hepatic-pancreatic dysplasia. (7/34)
We report the prenatal sonographic features of congenital bile duct dilatation associated with renal-hepatic-pancreatic dysplasia. The condition was seen at 22 weeks of gestation and led to termination of pregnancy. This is the first description of congenital bile duct dilatation using prenatal sonography. It is also the first report of a case in which the features of dysplasia were evident in all three of the organs which may be affected, the kidneys, liver and pancreas. (+info)Caroli's disease. (8/34)
Carolis disease is a rare communicating segmental or diffuse dilatation of the intrahepatic biliary tree. Cholangitis, liver cirrhosis and cholangiocarcinoma are its potential complications. A case of Carolis disease in a boy of 6 years with bilobal involvement presenting with intermittent abdominal pain, fever and hepatomegaly is reported here. (+info)Caroli Disease is a rare congenital anomaly characterized by abnormal dilatation of the intrahepatic bile ducts, often associated with hepatic fibrosis and an increased risk of cholangiocarcinoma.
Caroli disease is a rare genetic disorder that affects the liver and bile ducts. It is characterized by abnormal dilations or sac-like structures in the intrahepatic bile ducts, which are the ducts that carry bile from the liver to the gallbladder and small intestine. These dilations can lead to recurrent cholangitis (inflammation of the bile ducts), stone formation, and liver damage.
Caroli disease is usually diagnosed in childhood or early adulthood, and it can be associated with other congenital anomalies such as polycystic kidney disease. The exact cause of Caroli disease is not fully understood, but it is believed to be inherited in an autosomal recessive manner, meaning that an individual must inherit two copies of the abnormal gene, one from each parent, to develop the condition.
Treatment for Caroli disease may include antibiotics to manage cholangitis, endoscopic procedures to remove stones or dilate strictures, and surgery to bypass or remove affected bile ducts. In severe cases, liver transplantation may be necessary. Regular monitoring of liver function and surveillance for complications are essential in the management of this condition.
Muridae is a large family of rodents, primarily found in Eurasia and Africa, which includes hamsters, rats, mice, gerbils, and many other related species, characterized by their small to medium size, slender bodies, and long tails.
I apologize for any confusion, but "Muridae" is not a medical term. It is a taxonomic category in biology, specifically it is a family of rodents that includes mice, rats, hamsters, gerbils, and many other species. If you have any questions about medical terminology or concepts, I would be happy to help with those.
Centromere Protein B (CENP-B) is a specific protein that binds to the repetitive DNA sequences of the human centromere region, playing a crucial role in the assembly and maintenance of the kinetochore structure during chromosome segregation in cell division.
Centromere Protein B (CENP-B) is a protein that plays a crucial role in the organization and function of centromeres, which are specialized regions of chromosomes where the spindle fibers attach during cell division. CENP-B is one of the proteins that make up the constitutive centromere-associated network (CCAN), which is a complex of proteins that forms the foundation of the kinetochore, the structure that connects the chromosome to the spindle fibers.
CENP-B has a unique ability to recognize and bind to specific DNA sequences within the centromere region called CENP-B boxes. This binding helps to establish and maintain the structural integrity of the centromere, ensuring that it functions correctly during cell division. Mutations in the CENP-B gene can lead to chromosomal instability and may contribute to the development of certain genetic disorders.
It's worth noting that while CENP-B is an important protein involved in centromere function, it is not present in all centromeres, and its absence does not necessarily mean that a centromere will be nonfunctional. Other proteins can compensate for the lack of CENP-B and help maintain centromere function.
A Chimera, in the context of medicine and genetics, is an individual with cells of different genetic makeup derived from two or more distinct zygotes, which can result in tissue mosaicism and may occur naturally in some cases like twin gestations or as a result of transplantation procedures.
A chimera, in the context of medicine and biology, is a single organism that is composed of cells with different genetics. This can occur naturally in some situations, such as when fraternal twins do not fully separate in utero and end up sharing some organs or tissues. The term "chimera" can also refer to an organism that contains cells from two different species, which can happen in certain types of genetic research or medical treatments. For example, a patient's cells might be genetically modified in a lab and then introduced into their body to treat a disease; if some of these modified cells mix with the patient's original cells, the result could be a chimera.
It's worth noting that the term "chimera" comes from Greek mythology, where it referred to a fire-breathing monster that was part lion, part goat, and part snake. In modern scientific usage, the term has a specific technical meaning related to genetics and organisms, but it may still evoke images of fantastical creatures for some people.
'DNA satellite' refers to the minor fraction of DNA in a genome that is composed of short, repetitive sequences which can form distinct bands on density gradient centrifugation due to their different buoyant densities.
Satellite DNA is a type of DNA sequence that is repeated in a tandem arrangement in the genome. These repeats are usually relatively short, ranging from 2 to 10 base pairs, and are often present in thousands to millions of copies arranged in head-to-tail fashion. Satellite DNA can be found in centromeric and pericentromeric regions of chromosomes, as well as at telomeres and other heterochromatic regions of the genome.
Due to their repetitive nature, satellite DNAs are often excluded from the main part of the genome during DNA sequencing projects, and therefore have been referred to as "satellite" DNA. However, recent studies suggest that satellite DNA may play important roles in chromosome structure, function, and evolution.
It's worth noting that not all repetitive DNA sequences are considered satellite DNA. For example, microsatellites and minisatellites are also repetitive DNA sequences, but they have different repeat lengths and arrangements than satellite DNA.
Species specificity in medicine refers to the characteristic of a disease, pathogen, or immune response that is unique to a particular species, making it incapable of affecting other species or only affecting them under specific conditions.
Species specificity is a term used in the field of biology, including medicine, to refer to the characteristic of a biological entity (such as a virus, bacterium, or other microorganism) that allows it to interact exclusively or preferentially with a particular species. This means that the biological entity has a strong affinity for, or is only able to infect, a specific host species.
For example, HIV is specifically adapted to infect human cells and does not typically infect other animal species. Similarly, some bacterial toxins are species-specific and can only affect certain types of animals or humans. This concept is important in understanding the transmission dynamics and host range of various pathogens, as well as in developing targeted therapies and vaccines.
Genetic hybridization is the process of combining genetic material from two different populations or species to create a new hybrid population or individual with unique genetic traits and characteristics.
Genetic hybridization is a biological process that involves the crossing of two individuals from different populations or species, which can lead to the creation of offspring with new combinations of genetic material. This occurs when the gametes (sex cells) from each parent combine during fertilization, resulting in a zygote with a unique genetic makeup.
In genetics, hybridization can also refer to the process of introducing new genetic material into an organism through various means, such as genetic engineering or selective breeding. This type of hybridization is often used in agriculture and biotechnology to create crops or animals with desirable traits, such as increased disease resistance or higher yields.
It's important to note that the term "hybrid" can refer to both crosses between different populations within a single species (intraspecific hybrids) and crosses between different species (interspecific hybrids). The latter is often more challenging, as significant genetic differences between the two parental species can lead to various reproductive barriers, making it difficult for the hybrid offspring to produce viable offspring of their own.
Embryo transfer is a medical procedure whereby an embryo, created through in vitro fertilization (IVF), is transferred to a uterus with the intent of establishing a pregnancy.
Embryo transfer is a medical procedure that involves the transfer of an embryo, which is typically created through in vitro fertilization (IVF), into the uterus of a woman with the aim of establishing a pregnancy. The embryo may be created using the intended parent's own sperm and eggs or those from donors. After fertilization and early cell division, the resulting embryo is transferred into the uterus of the recipient mother through a thin catheter that is inserted through the cervix. This procedure is typically performed under ultrasound guidance to ensure proper placement of the embryo. Embryo transfer is a key step in assisted reproductive technology (ART) and is often used as a treatment for infertility.
Alpha 1-Antitrypsin (AAT) is a protein produced mainly in the liver, functioning as an important antiprotease to inhibit neutrophil elastase and protect tissue integrity, primarily in the lungs. (27 words)
Alpha 1-antitrypsin (AAT, or α1-antiproteinase, A1AP) is a protein that is primarily produced by the liver and released into the bloodstream. It belongs to a group of proteins called serine protease inhibitors, which help regulate inflammation and protect tissues from damage caused by enzymes involved in the immune response.
Alpha 1-antitrypsin is particularly important for protecting the lungs from damage caused by neutrophil elastase, an enzyme released by white blood cells called neutrophils during inflammation. In the lungs, AAT binds to and inhibits neutrophil elastase, preventing it from degrading the extracellular matrix and damaging lung tissue.
Deficiency in alpha 1-antitrypsin can lead to chronic obstructive pulmonary disease (COPD) and liver disease. The most common cause of AAT deficiency is a genetic mutation that results in abnormal folding and accumulation of the protein within liver cells, leading to reduced levels of functional AAT in the bloodstream. This condition is called alpha 1-antitrypsin deficiency (AATD) and can be inherited in an autosomal codominant manner. Individuals with severe AATD may require augmentation therapy with intravenous infusions of purified human AAT to help prevent lung damage.
Intrahepatic bile ducts are the small tubular structures within the liver that collect bile from the liver cells and carry it to the common hepatic duct.
Intrahepatic bile ducts are the small tubular structures inside the liver that collect bile from the liver cells (hepatocytes). Bile is a digestive fluid produced by the liver that helps in the absorption of fats and fat-soluble vitamins from food. The intrahepatic bile ducts merge to form larger ducts, which eventually exit the liver and join with the cystic duct from the gallbladder to form the common bile duct. The common bile duct then empties into the duodenum, the first part of the small intestine, where bile aids in digestion. Intrahepatic bile ducts can become obstructed or damaged due to various conditions such as gallstones, tumors, or inflammation, leading to complications like jaundice, liver damage, and infection.
Congenital Disorders of Glycosylation (CDG) refer to a group of inherited metabolic disorders caused by defects in the glycosylation process, which is essential for the proper functioning and stability of proteins and lipids, leading to multisystemic clinical manifestations affecting various organs and tissues.
Congenital Disorders of Glycosylation (CDG) are a group of genetic disorders that affect the body's ability to add sugar molecules (glycans) to proteins and lipids. This process, known as glycosylation, is essential for the proper functioning of many cellular processes, including protein folding, trafficking, and signaling.
CDG can be caused by mutations in genes that are involved in the synthesis or transport of glycans. These genetic defects can lead to abnormal glycosylation patterns, which can result in a wide range of clinical manifestations, including developmental delay, intellectual disability, seizures, movement disorders, hypotonia, coagulation abnormalities, and multi-organ involvement.
CDG are typically classified into two main types: type I CDG, which involves defects in the synthesis of the lipid-linked oligosaccharide precursor used for N-glycosylation, and type II CDG, which involves defects in the processing and transfer of glycans to proteins.
The diagnosis of CDG is often based on clinical features, laboratory tests, and genetic analysis. Treatment is typically supportive and multidisciplinary, focusing on addressing specific symptoms and improving quality of life. In some cases, dietary modifications or supplementation with mannose or other sugars may be beneficial.
'Bile ducts' are tubular structures that transport bile from the liver and gallbladder into the small intestine, facilitating fat digestion and absorption.
Bile ducts are tubular structures that carry bile from the liver to the gallbladder for storage or directly to the small intestine to aid in digestion. There are two types of bile ducts: intrahepatic and extrahepatic. Intrahepatic bile ducts are located within the liver and drain bile from liver cells, while extrahepatic bile ducts are outside the liver and include the common hepatic duct, cystic duct, and common bile duct. These ducts can become obstructed or inflamed, leading to various medical conditions such as cholestasis, cholecystitis, and gallstones.
'Bile duct diseases' refer to a range of medical conditions affecting the biliary system, including the extrahepatic and intrahepatic bile ducts, characterized by impaired bile flow, inflammation, infection, obstruction, or structural abnormalities, which can lead to cholestasis, liver damage, and potential complications such as cirrhosis and liver failure.
Bile duct diseases refer to a group of medical conditions that affect the bile ducts, which are tiny tubes that carry bile from the liver to the gallbladder and small intestine. Bile is a digestive juice produced by the liver that helps break down fats in food.
There are several types of bile duct diseases, including:
1. Choledocholithiasis: This occurs when stones form in the common bile duct, causing blockage and leading to symptoms such as abdominal pain, jaundice, and fever.
2. Cholangitis: This is an infection of the bile ducts that can cause inflammation, pain, and fever. It can occur due to obstruction of the bile ducts or as a complication of other medical procedures.
3. Primary Biliary Cirrhosis (PBC): This is a chronic autoimmune disease that affects the bile ducts in the liver, causing inflammation and scarring that can lead to cirrhosis and liver failure.
4. Primary Sclerosing Cholangitis (PSC): This is another autoimmune disease that causes inflammation and scarring of the bile ducts, leading to liver damage and potential liver failure.
5. Bile Duct Cancer: Also known as cholangiocarcinoma, this is a rare form of cancer that affects the bile ducts and can cause jaundice, abdominal pain, and weight loss.
6. Benign Strictures: These are narrowing of the bile ducts that can occur due to injury, inflammation, or surgery, leading to blockage and potential infection.
Symptoms of bile duct diseases may include jaundice, abdominal pain, fever, itching, dark urine, and light-colored stools. Treatment depends on the specific condition and may involve medication, surgery, or other medical interventions.
Bile Duct Neoplasms are abnormal growths that can be benign or malignant, occurring in the bile ducts (both intrahepatic and extrahepatic), which can obstruct bile flow, cause cholestasis, and potentially metastasize.
Bile duct neoplasms, also known as cholangiocarcinomas, refer to a group of malignancies that arise from the bile ducts. These are the tubes that carry bile from the liver to the gallbladder and small intestine. Bile duct neoplasms can be further classified based on their location as intrahepatic (within the liver), perihilar (at the junction of the left and right hepatic ducts), or distal (in the common bile duct).
These tumors are relatively rare, but their incidence has been increasing in recent years. They can cause a variety of symptoms, including jaundice, abdominal pain, weight loss, and fever. The diagnosis of bile duct neoplasms typically involves imaging studies such as CT or MRI scans, as well as blood tests to assess liver function. In some cases, a biopsy may be necessary to confirm the diagnosis.
Treatment options for bile duct neoplasms depend on several factors, including the location and stage of the tumor, as well as the patient's overall health. Surgical resection is the preferred treatment for early-stage tumors, while chemotherapy and radiation therapy may be used in more advanced cases. For patients who are not candidates for surgery, palliative treatments such as stenting or bypass procedures may be recommended to relieve symptoms and improve quality of life.
Caroli disease
... with more reported cases of Caroli syndrome than of Caroli disease. Caroli disease is distinct from other diseases that cause ... Friedman JR: Caroli Disease at eMedicine Choi BI, Yeon KM, Kim SH, Han MC (January 1990). "Caroli disease: central dot sign in ... Caroli disease is also associated with liver failure and polycystic kidney disease. The disease affects about one in 1,000,000 ... Caroli disease usually occurs in the presence of other diseases, such as autosomal recessive polycystic kidney disease, ...
Klatskin tumor
Congenital biliary cystic disease, such as choledochal cysts or Caroli's disease, has also been associated with malignant ... Falco E, Nardini A, Celoria G, Briglia R, Stefani R, Gadducci G, Belloni E (1993). "[Caroli's disease associated with ... Faría G, de Aretxabala X, Sierralta A, Flores P, Burgos L (2001). "[Primary cholangiocarcinoma associated with Caroli disease ... Totkas S, Hohenberger P (2000). "Cholangiocellular carcinoma associated with segmental Caroli's disease". European Journal of ...
Cholangiocarcinoma
Congenital liver abnormalities, such as Caroli disease (a specific type of five recognized choledochal cysts), have been ... Dayton MT, Longmire WP, Tompkins RK (January 1983). "Caroli's Disease: a premalignant condition?". American Journal of Surgery ... The disease is confirmed by examination of cells from the tumor under a microscope. It is typically an adenocarcinoma (a cancer ... Certain parasitic liver diseases may be risk factors as well. Colonization with the liver flukes Opisthorchis viverrini (found ...
Medullary sponge kidney
A rare, autosomal recessive form is associated with Caroli disease. Classically, MSK is seen as hyperechoic papillae with ... The disease is bilateral in 70% of cases.[citation needed] "Medullary Sponge Kidney". Archived from the original on 7 August ... Polycystic Kidney Disease and Other Inherited Tubular Disorders". Harrison's Principles of Internal Medicine (18th ed.). New ... This explains why so many tubular defects coexist in this disease, and particularly a distal tubular acidification defect of ...
Liver cancer
Choledochal cysts, Caroli's disease, and congenital hepatic fibrosis are associated with cholangiocarcinoma development. ... In these high disease burden areas, evidence indicates the majority of the HBC and HCV infections occur via perinatal ... Dooley JS, Lok AS, Garcia-Tsao G, Pinzani M (8 June 2018). Sherlock's Diseases of the Liver and Biliary System. John Wiley & ... Other causes include aflatoxin, non-alcoholic fatty liver disease and liver flukes. The most common types are HCC, which makes ...
Caroli
... may refer tà Caroli (surname) Caroli disease of bile ducts Caroli Group, a company based in Monaco Caroli Church, Malmö ... in Sweden Caroli church, Borås in Sweden USS Cor Caroli (AK-91), American cargo ship This disambiguation page lists articles ... associated with the title Caroli. If an internal link led you here, you may wish to change the link to point directly to the ...
Choledochal cysts
The presence of multiple saccular or cystic dilations of the intrahepatic ducts is known as Caroli's disease. Type VI: An ... Type V: Cystic dilatation of intrahepatic biliary ducts without extrahepatic duct disease. ...
Cheri Register
She suffered from Caroli disease, and documented her experiences in The Chronic Illness Experience: Embracing the Imperfect ...
Psittacine beak and feather disease
Circella, E.; Legretto, M.; Pugliese, N.; Caroli, A.; Bozzo, G.; Accogli, G.; Lavazza, A.; Camarda, A. (2014). "Psittacine Beak ... Psittacine beak and feather disease (PBFD) is a viral disease affecting all Old World and New World parrots. The causative ... "Beak and feather disease virus: biology and resultant disease" (PDF). WikiJournal of Science. 3 (1): 7. doi:10.15347/WJS/ ... "psittacine beak and feather disease syndrome" (PBFDS). This soon became known as psittacine beak and feather disease (PBFD). ...
Recurrent pyogenic cholangitis
Caroli, J. (1973-01-01). "Diseases of the intrahepatic biliary tree". Clinics in Gastroenterology. 2 (1): 147-161. ISSN 0300- ... Caroli, J. (1973-01-01). "Diseases of the intrahepatic biliary tree". Clinics in Gastroenterology. 2 (1): 147-161. ISSN 0300- ... Retrospective study". Digestive Diseases and Sciences. 31 (1): 21-6. doi:10.1007/BF01347905. PMID 3940820. ONG, GB (February ... Recurrent pyogenic cholangitis (RPC), also known as Hong Kong disease, Oriental cholangitis, and Oriental infestational ...
Congenital hepatic fibrosis
Caroli disease Polycystic kidney disease Von Meyenburg complex Biliary hamartomas "eMedicine - Congenital Hepatic Fibrosis : ... Diseases of liver, All stub articles, Disease stubs). ... bleeding/varices and treatment of associated renal disease if ... It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most ... Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts ...
Unplanned Parenthood (House)
They suspect Caroli disease to be the underlying cause of the liver failure, but fail to confirm it, leaving the case still ...
Alzheimer's Disease Neuroimaging Initiative
PMID 24886908.{{cite journal}}: CS1 maint: multiple names: authors list (link) Caroli, Anna; Prestia, Annapaola; Wade, Sara; ... Alzheimer's disease related Worldwide ADNI : documents disease progression in a diverse range of racial and ethnic groups in ... Other diseases Parkinson's Progressive Markers Initiative: aims to identify biomarkers for Parkinson's disease progression and ... Rafii, Michael S. (2014-01-01). "Preclinical Alzheimer's disease therapeutics". Journal of Alzheimer's Disease. 42 Suppl 4: ...
List of diseases (C)
... transporter deficiency Carnitine-acylcarnitine translocase deficiency Carnosinase deficiency Carnosinemia Caroli disease ... Marie-Tooth disease type 1A Charcot-Marie-Tooth disease type 1B Charcot-Marie-Tooth disease type 1C Charcot-Marie-Tooth disease ... Marie-Tooth disease type 2C Charcot-Marie-Tooth disease type 2D Charcot-Marie-Tooth disease type 4A Charcot-Marie-Tooth disease ... Tooth disease Charcot-Marie-Tooth disease Charcot-Marie-Tooth disease deafness dominant type Charcot-Marie-Tooth disease ...
Fever of unknown origin
Caroli disease Cholesterol emboli Complex partial status epilepticus Cyclic neutropenia Drug fever Erdheim-Chester disease ... Thromboembolic disease (i.e. pulmonary embolism, deep venous thrombosis) occasionally shows fever. Although infrequent, its ... Rheumatoid arthritis or adult-onset Still's disease have mixed features and are common causes of FUO. Although most neoplasms ... Extrinsic allergic alveolitis Factitious disease Fire-eater's lung Fraudulent fever Gaucher's disease Hamman-Rich syndrome ( ...
List of syndromes
Cardiac syndrome X Cardiofaciocutaneous syndrome Cardiorenal syndrome Cardiovascular syndrome Carney complex Caroli disease ... Town Lawrence-Seip syndrome Lazarus syndrome Leaky gut syndrome Legg-Calvé-Perthes disease Legius syndrome Leiner's disease ... VIP syndrome VIPoma Visual looming syndrome Vitreous touch syndrome Vogt-Koyanagi-Harada disease Von Hippel-Lindau disease ... disease MEDNIK syndrome Megavitamin-B6 syndrome Meige's syndrome Meigs' syndrome MELAS syndrome Melkersson-Rosenthal syndrome ...
Ciliopathy
Caroli disease and liver cirrhosis due to ciliopathy has been described. Phenotypes sometimes associated with ciliopathies can ... cystic renal disease, cilia-related genes and proteins have been identified to have causal effect in polycystic kidney disease ... Additional studies of how ciliary dysfunction can lead to such severe disease and developmental pathologies is still a subject ... Cilia have recently been implicated in a wide variety of human genetic diseases by "the discovery that numerous proteins ...
Cor
... heart disease and defect Cor Caroli and Cor Hydrae, stars Côr Cymru, Welsh choir competition Cor Scorpii, Norwegian metal band ...
Peliosis hepatis
... liver disease Solitary congenital cysts Congenital hepatic fibrosis Hydatid cyst Von Meyenburg complexes Caroli disease (type V ... Infections: HIV, bacillary peliosis (caused by genus Bartonella, bacteria responsible for cat-scratch disease which are ... Sleisenger, Marvin (2006). Sleisenger and Fordtran's Gastrointestinal and Liver Disease. Philadelphia: W.B. Saunders Company. ... Hodgkin disease, malignant histiocytosis, seminoma, hepatocellular adenoma, and hepatocarcinoma Kidney transplants: It can be ...
List of MeSH codes (C06)
... caroli disease MeSH C06.267.250.725 - tracheoesophageal fistula MeSH C06.267.550.600 - rectal fistula MeSH C06.267.550.600.650 ... caroli disease MeSH C06.130.120.135 - cholestasis MeSH C06.130.120.135.150 - cholestasis, extrahepatic MeSH C06.130.120.135.250 ... rectal diseases MeSH C06.405.469.860.101 - anus diseases MeSH C06.405.469.860.101.163 - anus neoplasms MeSH C06.405.469.860. ... hirschsprung disease MeSH C06.405.469.158.701.591 - megacolon, toxic MeSH C06.405.469.158.850 - sigmoid diseases MeSH C06.405. ...
List of MeSH codes (C16)
Caroli disease MeSH C16.131.314.244 - diaphragmatic eventration MeSH C16.131.314.330 - esophageal atresia MeSH C16.131.314.439 ... Tay-Sachs disease MeSH C16.320.565.150.435.825.300.300.920 - Tay-Sachs disease, AB variant MeSH C16.320.565.150.435.825.300.400 ... glycogen storage disease type I MeSH C16.320.565.202.449.500 - glycogen storage disease type II MeSH C16.320.565.202.449.510 - ... glycogen storage disease type IV MeSH C16.320.565.202.449.560 - glycogen storage disease type V MeSH C16.320.565.202.449.580 - ...
Vanishing bile duct syndrome
Extrahepatic bile duct atresia Autosomal recessive polycystic kidney disease Congential hepatic fibrosis Caroli's disease Von ... Vanishing bile duct syndrome is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ... Rossini, M. S.; Lorand-Metze, I; Oliveira, G. B.; Souza, C. A. (2000). "Vanishing bile duct syndrome in Hodgkin's disease: Case ... Primary biliary cirrhosis Primary sclerosing cholangitis Hodgkin's lymphoma Chronic graft-versus-host disease Drugs( ...
List of eponymous diseases
Stratakis Caroli syndrome - Jacques Caroli Carrion disease - Daniel Alcides Carrión Castleman disease - Benjamin Castleman ... Begbie disease, Flajan disease, Flajani-Basedow syndrome, Graves disease, Graves-Basedow disease, Marsh disease, Morbus Basedow ... Disease naming structures which reference place names (such as Bornholm disease, Lyme disease, and Ebola virus disease) are ... examples being Lou Gehrig disease, Hartnup disease, and Mortimer disease. In one instance, Machado-Joseph disease, the eponym ...
Coffea canephora
Penarredonda, Jose Luis (6 November 2017). "The disease that could change how we drink coffee". BBC: In depth, Food. Retrieved ... cite journal}}: Cite journal requires ,journal= (help) von, Linné, Carl; Lars, Salvius (1753). "Caroli Linnaei ... Species ... As it is less susceptible to pests and disease, robusta needs much less herbicide and pesticide than arabica. C. canephora ... and is less susceptible to disease than arabica coffea. It represents 43% of global coffee production, with arabica ...
Helophilus seelandicus
Despite being native to New Zealand, this species is commonly reported to the Ministry of Primary Industries Pest and Disease ... Linné, Carl von; Gmelin, Johann Friedrich (1788-93). Caroli a Linné. Systema naturae per regna tria naturae : secundum classes ...
Ringtail (disambiguation)
... disease), a rodent disease Ringtail (sail), an extra sail, usually set in light winds, extending abaft the leech of a fore and ... Pseudochirulus caroli Ring-tailed pigeon Patagioenas caribaea, a bird Ring-tails (harrier), an informal term for the juveniles ...
Biliary atresia
Caroli disease, choledochal cyst, cholestasis, congenital cytomegalovirus disease, congenital herpes simplex virus infection, ... The influence of age differs according to the disease etiology-i.e., whether biliary atresia is isolated, cystic (CBA), or ... The biliary atresia phenotype caused by congenital aflatoxicosis in GST M1 deficient neonates is named Kotb disease. Syndromic ... Mack, Cara (2007). "The Pathogenesis of Biliary Atresia: Evidence for a Virus-Induced Autoimmune Disease". Seminars in Liver ...
Deaths in November 2020
... neurological disease. Benjamin LaGuer, 57, American convicted rapist, liver disease. Naomi Long Madgett, 97, American poet. ... Angelo Caroli, 83, Italian footballer (Juventus, Catania, Lucchese) and journalist. Malcolm CasSelle, 50, American businessman ... Carol Arthur, 85, American actress (Blazing Saddles, The Sunshine Boys, Making It), Alzheimer's disease. Julio Bécquer, 88, ... Tom Metzger, 82, American white supremacist, founder of White Aryan Resistance, Parkinson's disease. John Meyer, 78, American ...
Systemic-onset juvenile idiopathic arthritis
Connective tissue diseases, Inflammatory polyarthropathies, Idiopathic diseases, Rare diseases). ... Caroli, Francesco; Buoncompagni, Antonella; Viola, Stefania; Loy, Anna; Sironi, Marina; Vecchi, Annunciata; Ravelli, Angelo; ... "juvenile-onset Still's disease" to distinguish it from adult-onset Still's disease. However, there is some evidence that the ... "Still's Disease". MedicineNet. Retrieved 8 June 2017. Vastert SJ, Jamilloux Y, Quartier P, Ohlman S, Osterling Koskinen L, ...
Mallory-Weiss syndrome
Caroli A, Follador R, Gobbi V, Breda P, Ricci G (1989). "[Mallory-Weiss syndrome. Personal experience and review of the ... In rare instances some chronic disorders like Ménière's disease that cause long term nausea and vomiting could be a factor. The ... National Digestive Diseases Information Clearinghouse (November 2004). "Upper Endoscopy". National Institutes of Health. ... Diseases of the Esophagus. 12 (1): 65-67. doi:10.1046/j.1442-2050.1999.00006.x. ISSN 1120-8694. PMID 10941865. Kitagawa, ...
Caroli disease - Wikipedia
... with more reported cases of Caroli syndrome than of Caroli disease. Caroli disease is distinct from other diseases that cause ... Friedman JR: Caroli Disease at eMedicine Choi BI, Yeon KM, Kim SH, Han MC (January 1990). "Caroli disease: central dot sign in ... Caroli disease is also associated with liver failure and polycystic kidney disease. The disease affects about one in 1,000,000 ... Caroli disease usually occurs in the presence of other diseases, such as autosomal recessive polycystic kidney disease, ...
Pediatric Caroli Disease: Background, Pathophysiology, Epidemiology
Caroli disease and Caroli syndrome are rare congenital disorders of the intrahepatic bile ducts. They are both characterized by ... Another classification of Caroli disease is type I, or simple Caroli disease, which consists of pure cystic dilatations of the ... encoded search term (Pediatric Caroli Disease) and Pediatric Caroli Disease What to Read Next on Medscape ... Caroli disease is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner. [1] As with ...
Researchers interested in Caroli Disease | Internal Medicine
Pediatric Caroli Disease: Background, Pathophysiology, Epidemiology
Caroli disease and Caroli syndrome are rare congenital disorders of the intrahepatic bile ducts. They are both characterized by ... Another classification of Caroli disease is type I, or simple Caroli disease, which consists of pure cystic dilatations of the ... encoded search term (Pediatric Caroli Disease) and Pediatric Caroli Disease What to Read Next on Medscape ... Caroli disease is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner. [1] As with ...
English (en)
Caroli syndrome associated with atrial septal defect and polydactyly » Latest news ... There are two types of Caroli: the first type is the simple type, Caroli disease, which includes only cystic dilatation of the ... The second is called Caroli syndrome, which consists of Caroli disease and congenital hepatic fibrosis and might lead to portal ... Caroli disease is multifocal segmental dilatation of the large intrahepatic bile ducts that connect to the main duct. It is ...
Imaging findings in Caroli's disease. | Read by QxMD
... often life-threatening hepatobiliary disease. The disease now most often referred to as Carolis disease is a rare condition ... Imaging findings in Carolis disease.. W J Miller, A G Sechtin, W L Campbell, P C Pieters. AJR. American Journal of ... Carolis disease usually is manifested in childhood and is thought to be congenital and probably inherited. Associated ... This pictorial essay illustrates the broad spectrum of imaging findings in Carolis disease. ...
Caroli Disease
... had been diagnosed in a 29-year-old man 5 years earlier based on MRI findings of multiple sacculated, dilated ... Caroli syndrome, which is associated with kidney disease, occurs in up to 60% of patients with Caroli disease; the renal ... This extrahepatic manifestation of Caroli disease is a component of Caroli syndrome. All other laboratory values were normal.. ... Caroli disease had been diagnosed in a 29-year-old man 5 years earlier based on MRI findings of multiple sacculated, dilated ...
Caroli Disease; Caroli's Disease
Pediatric Caroli Disease: Background, Pathophysiology, Epidemiology
Caroli disease and Caroli syndrome are rare congenital disorders of the intrahepatic bile ducts. They are both characterized by ... encoded search term (Pediatric Caroli Disease) and Pediatric Caroli Disease What to Read Next on Medscape ... Both Caroli disease and Caroli syndrome are associated with a risk of cholangiocarcinoma at a rate of 100 times that of the ... Caroli disease is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner. [1] As with ...
Pediatric Caroli Disease Clinical Presentation: History, Physical, Causes
Caroli disease and Caroli syndrome are rare congenital disorders of the intrahepatic bile ducts. They are both characterized by ... encoded search term (Pediatric Caroli Disease) and Pediatric Caroli Disease What to Read Next on Medscape ... Patients with Caroli disease or Caroli syndrome may have a history of intermittent abdominal pain, which reflects episodes of ... Carolis disease in congenital hepatic fibrosis and infantile polycystic disease. Liver. 1982 Dec. 2(4):346-54. [QxMD MEDLINE ...
Hepatic resection in localized Caroli disease<...
keywords = "Caroli disease, Hepatectomy, Lithiasis, Caroli disease, Hepatectomy, Lithiasis",. author = "{Espinoza G}, Ricardo ... title = "Hepatic resection in localized Caroli disease",. abstract = "Caroli disease is a cystic congenital malformation of the ... Espinoza G R, San Martin R S, Court A F, Vera P E, Ferreira S R, Croxatto A H. Hepatic resection in localized Caroli disease. ... Hepatic resection in localized Caroli disease. / Espinoza G, Ricardo; San Martin R, Sergio; Court A, Felipe et al. En: Revista ...
Congenital hypothyroidism in association with Caroli's disease and autosomal recessive polycystic kidney disease: Patient...
JCM | Free Full-Text | Management of Intrahepatic Cholangiocarcinoma
These modalities can improve overall disease-free and progression-free survival chances. In this review, we will discuss the ... and in very advanced and metastatic disease, external radiation are the available options. ... If diagnosed early these can be resected, but most cases of intrahepatic cholangiocarcinoma present late in the disease course ... Carolis disease, hepatobiliary flukes, and cirrhosis of the liver irrespective of the etiology are the risk factors associated ...
Pathological classification of intrahepatic cholangiocarcinoma based on a new concept
Liver Care Center
More than 900 children and teens with a wide range of liver diseases, from acute hepatitis to a chronic liver disease that may ... Treating pediatric liver disease. The Liver Care Center is designed to provide a medical home for children dealing with highly ... He was diagnosed with Wilsons disease, a rare inherited condition. A liver transplant at Childrens Mercy Kansas City has ... not just the disease. We work directly with other specialists throughout Childrens Mercy whenever needed in order to give ...
NA15210
Together at home: your stories of 'life in lockdown' - British Liver Trust
Biophysical Characterization of Flagellar Motor Functions | Protocol (Translated to Turkish)
32 Biliary Flashcards
David Carr-Locke Endoscopy Clinic - Index
Flashcards - ABSITE ch 43 pediatric surg.txt
Mario Spaggiari | Chicago Medicine
Diagnostic Pathology Nonneoplastic Pediatrics, 2nd edition - Angelica R. Putnam - 1020 - ELSEVIER HEALTH SCIENCES -...
Abhd10 Mouse Gene Details | abhydrolase domain containing 10 | International Mouse Phenotyping Consortium
Human diseases predicted to be associated with Abhd10 (382 diseases) No human diseases associated to this gene by orthology or ... Human diseases caused by Abhd10 mutations The analysis uses data from IMPC, along with published data on other mouse mutants, ... The table below shows human diseases predicted to be associated to Abhd10 by phenotypic similarity. ... Phenotype comparisons summarize the similarity of mouse phenotypes with human disease phenotypes. ...
Table 2 - Detection and Circulation of a Novel Rabbit Hemorrhagic Disease Virus in Australia - Volume 24, Number 1-January 2018...
Camarda A, Pugliese N, Cavadini P, Circella E, Capucci L, Caroli A, et al. Detection of the new emerging rabbit haemorrhagic ... Rabbit haemorrhagic disease (RHD) and rabbit haemorrhagic disease virus (RHDV): a review. Vet Res (Faisalabad). 2012;43:12. DOI ... Wang X, Hao H, Qiu L, Dang R, Du E, Zhang S, et al. Phylogenetic analysis of rabbit hemorrhagic disease virus in China and the ... Oem JK, Lee KN, Roh IS, Lee KK, Kim SH, Kim HR, et al. Identification and characterization of rabbit hemorrhagic disease virus ...
Vol. 5 No. 11 (2018): November 2018
| International Surgery Journal
Find Research outputs
- Universidad de los Andes
Hepatic resection in localized Caroli disease. Espinoza G, R., San Martin R, S., Court A, F., Vera P, E., Ferreira S, R. & ... Tumor necrosis factor alpha genetic polymorphism as a risk factor in disease. Aguillón G, J. C., Cruzat C, A., Cuenca M, J. & ... Restriction in the usage of variable β regions in T-cells infiltrating valvular tissue from rheumatic heart disease patients. ...
How is Hepatitis E Transmitted?
Glycogen Storage Disease Type 1 (GSD 1) or Von Gierkes Disease: Definition, Causes, Symptoms, Diagnosis, Treatment. ... Fulminant hepatitis is also more common in patients with malnutrition or with underlying liver disease ... it is usually accompanied by progressive disease and cases of evolution to cirrhosis have been described. Patients with chronic ...
DeCS 2004 - Changed terms
C06 - DIGESTIVE SYSTEM DISEASES. Bile Duct Obstruction, Extrahepatic. Cholestasis, Extrahepatic. Carolis Disease. Caroli ... C14 - CARDIOVASCULAR DISEASES. Raynauds Disease. Raynaud Disease. C16 - CONGENITAL, HEREDITARY, AND NEONATAL DISEASES AND ... C08 - RESPIRATORY TRACT DISEASES. Respiratory Distress Syndrome. Respiratory Distress Syndrome, Newborn. ... C17 - SKIN AND CONNECTIVE TISSUE DISEASES. Scleroderma, Circumscribed. Scleroderma, Localized. C18 - NUTRITIONAL AND METABOLIC ...
Caroli'sPolycystic kidneySimple Caroli diseaseBiliary treeSyndromeCholangitisEctasiaCirrhosisChronic liver dCholangiocarcinomaHepaticDilatationLeft lobeDigestive DiseasesARPKDSymptomsGeneticCholelithiasisPKHD1MalignancyPatients with severeDiffuseGallstonesDiagnosisSporadicCommonlyDuctsAlzheimer'sOccurAbdominalDuctalLiver DiseasesInfectious DiseasesCongenital heart dVariantClinicalDementia
Caroli's30
- Nakanuma Y, Harada K, Sato Y, Ikeda H. Recent progress in the etiopathogenesis of pediatric biliary disease, particularly Caroli's disease with congenital hepatic fibrosis and biliary atresia. (medscape.com)
- Clinical classification of Caroli's disease: an analysis of 30 patients. (medscape.com)
- Sato Y, Ren XS, Nakanuma Y. Caroli's Disease: Current Knowledge of Its Biliary Pathogenesis Obtained from an Orthologous Rat Model. (medscape.com)
- Lai Q, Lerut J. Proposal for an algorithm for liver transplantation in Caroli's disease and syndrome: putting an uncommon effort into a common task. (medscape.com)
- Caroli's disease and outcomes after liver transplantation. (medscape.com)
- Orthotopic liver transplantation for patients with Caroli's disease. (medscape.com)
- The role of surgery in Caroli's disease. (medscape.com)
- Caroli's disease: magnetic resonance imaging features. (medscape.com)
- Caroli's disease and orthotopic liver transplantation. (medscape.com)
- Caroli's disease associated with congenital hepatic fibrosis and renal tubular ectasia. (medscape.com)
- Imaging findings in Caroli's disease. (medscape.com)
- Nakanuma Y, Terada T, Ohta G, Kurachi M, Matsubara F. Caroli's disease in congenital hepatic fibrosis and infantile polycystic disease. (medscape.com)
- type II, or complex Caroli disease, is also known as Caroli's syndrome. (medscape.com)
- How can I prevent caroli's disease? (klarity.health)
- How common is caroli's disease? (klarity.health)
- Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. (e-cmh.org)
- Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. (e-cmh.org)
- Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. (e-cmh.org)
- Caroli's disease belongs to a group of hepatic fibropolycystic diseases. (e-cmh.org)
- Two types have been described: a pure form or Caroli's disease (type 1) and a complex form associated with congenital hepatic fibrosis or Caroli's syndrome (type 2). (e-cmh.org)
- 1 2 Clinical symptoms of Caroli's disease include right upper quadrant abdominal pain, jaundice, and recurrent cholangitis. (e-cmh.org)
- Caroli's disease limited to 1 liver segment is extremely rare, and there have been only a few cases of segmentectomy for Caroli's disease in Korea. (e-cmh.org)
- In this report, we present a case of Caroli's disease that was misdiagnosed as an intraductal papillary neoplasm of the bile duct (IPNB) and was treated with segmentectomy. (e-cmh.org)
- 8. Cholangiocarcinoma arising in Caroli's disease. (nih.gov)
- Catenin, A Novel Signaling Defect in Congenital Hepatic Fibrosis (CHF) and Caroli's Disease (CD). (nih.gov)
- Some biliary cystic diseases are congenital, such as Caroli's disease or choledochal cysts and almost 25% of Klatskin Tumor cases have an association with this condition. (cancerwall.com)
- Abstract We describe a case of Caroli syndrome (Caroli's disease and congenital hepatic fibrosis) in a 10-year-old boy with bilobar involvement and numerous renal cysts. (zib.de)
- article{RPC, author = {Urânia FERNANDES and Ricardo PEREIRA and Rita MARQUES and João PINTO-DE-SOUSA}, title = { CAROLI'S DISEASE IN ADULTHOOD - A CASE REPORT}, journal = {Revista Portuguesa de Cirurgia}, number = {55}, year = {2023}, keywords = {}, abstract = { Caroli's disease (CD) is a rare congenital disorder defined by multifocal segmental dilatation of intrahepatic bile ducts. (spcir.com)
- Investigation revealed infracentimetric left cystic dilatation of intrahepatic bile ducts suggestive of Caroli's disease. (spcir.com)
- Type V, or Caroli's disease, has cystic dilation of the intrahepatic ducts but relatively normal extrahepatic ducts. (mhmedical.com)
Polycystic kidney8
- Caroli disease is also associated with liver failure and polycystic kidney disease. (wikipedia.org)
- Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease. (medscape.com)
- [ 1 ] As with congenital hepatic fibrosis , Caroli syndrome is often associated with autosomal recessive polycystic kidney disease (ARPKD) . (medscape.com)
- A rare association with autosomal dominant polycystic kidney disease (ADPKD) has also been reported. (medscape.com)
- Caroli disease is associated with conditions such as polycystic kidney disease, cholangitis, gallstones, and liver cancer. (klarity.health)
- Caroli disease may be associated with mutations in the PKHD1 gene, which is also involved in autosomal recessive polycystic kidney disease . (klarity.health)
- In this review, we examine the clinical features and differential diagnoses of this group of syndromes, including autosomal recessive polycystic kidney disease (ARPKD), juvenile nephronophthisis (NPHP), Meckel-Gruber syndrome (MKS), Bardet-Biedl syndrome (BBS), and Jeune asphyxiating thoracic dystrophy (JATD). (bmj.com)
- Autosomal Dominant Polycystic Kidney Disease (ADPKD) Polycystic kidney disease (PKD) is a hereditary disorder of renal cyst formation causing gradual enlargement of both kidneys, sometimes with progression to renal failure. (msdmanuals.com)
Simple Caroli disease1
- There are two patterns of Caroli disease: focal or simple Caroli disease consists of abnormally widened bile ducts affecting an isolated portion of liver. (wikipedia.org)
Biliary tree3
- 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. (wikipedia.org)
- After reviewing 46 cases of Caroli disease before 1990, 21.7% of the cases were the result of an intrahepatic cyst or nonobstructive biliary tree dilation, 34.7% were linked with congenital hepatic fibrosis, 13% were isolated choledochal cystic dilation, and the remaining 24.6% had a combination of all three. (wikipedia.org)
- The Notch signaling pathway plays a key role in the morphogenesis of the biliary tree, but its involvement in cystic biliary diseases, such as Caroli disease (CD) and polycystic liver disease (PLD), has yet to be determined. (nih.gov)
Syndrome20
- The disease affects about one in 1,000,000 people, with more reported cases of Caroli syndrome than of Caroli disease. (wikipedia.org)
- PKHD1, the gene linked to ARPKD, has been found mutated in patients with Caroli syndrome. (wikipedia.org)
- The genetic basis for the difference between Caroli disease and Caroli syndrome has not been defined. (wikipedia.org)
- Caroli disease and Caroli syndrome are rare congenital disorders of the intrahepatic bile ducts. (medscape.com)
- This form is less common than Caroli syndrome, in which malformations of small bile ducts and congenital hepatic fibrosis are also present. (medscape.com)
- Caroli disease is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner. (medscape.com)
- Caroli syndrome belongs to a subcategory of diseases thought to originate from DP malformation. (medscape.com)
- In Caroli syndrome, DP malformation is present at the level of the smallest portal tracts and is associated with varying degrees of portal fibrosis. (medscape.com)
- therefore, Caroli syndrome is thought to belong in the same spectrum of disease as congenital hepatic fibrosis and ARPKD. (medscape.com)
- Caroli disease and Caroli syndrome are very rare, with an estimated incidence of less than 1 case per 100,000 population. (medscape.com)
- Caroli syndrome (ectasia of the large and small bile ducts with congenital hepatic fibrosis) is more common than Caroli disease (ectasia of only the large bile ducts). (medscape.com)
- Patients with Caroli disease or Caroli syndrome may have recurrent episodes of cholangitis and are also at risk for associated bacteremia and sepsis. (medscape.com)
- Patients with Caroli syndrome or Caroli disease may have cholangitis and may also have complications of portal hypertension as is observed in congenital hepatic fibrosis. (medscape.com)
- Caroli syndrome is associated with ARPKD, and patients may have various degrees of renal cysts, interstitial fibrosis, and renal failure. (medscape.com)
- Both Caroli disease and Caroli syndrome are associated with a risk of cholangiocarcinoma at a rate of 100 times that of the general population. (medscape.com)
- Symptoms of Caroli disease or syndrome are more common in female patients than in male patients. (medscape.com)
- In some cases, Caroli disease may be associated with Caroli syndrome, which involves congenital hepatic fibrosis and portal hypertension . (klarity.health)
- Caroli syndrome, which is a variant of Caroli disease with associated congenital hepatic fibrosis , is more common and has an estimated prevalence of one in 100,000 individuals. (klarity.health)
- Caroli syndrome. (cancer.net)
- More tracers are coming online and are being used on related diseases such as Down's syndrome. (alzforum.org)
Cholangitis1
- The tumor was unrelated to Caroli disease, primary sclerosing cholangitis, ulcerative colitis, or nonbiliary cirrhosis, as demonstrated by further clinical and histopathologic investigations, but probably was associated with the presence of multiple bile duct hamartomas. (nih.gov)
Ectasia3
- The term Caroli disease is applied if the disease is limited to ectasia or segmental dilatation of the larger intrahepatic ducts. (medscape.com)
- In Caroli disease, abnormalities of the bile duct occur at the level of the large intrahepatic ducts (ie, left and right hepatic ducts, segmental ducts), resulting in dilatation and ectasia. (medscape.com)
- Benign renal tubular ectasia is associated with both types of Caroli disease. (nih.gov)
Cirrhosis2
- It is important to identify the underlying etiology of chronic liver injury to reverse or slow the progression of fibrosis toward cirrhosis and end-stage liver disease. (logicalimages.com)
- Cirrhosis is liver disease that can cause scarring or long-lasting irritation. (cancer.net)
Chronic liver d1
- Review of published clinical and epidemiologic stud- eases of the exocrine pancreas, particularly pancreatic ies reveal that it is difficult to demonstrate that people cancer and chronic pancreatitis, and may also be in- with diabetes are at much higher risk of digestive creased by chronic liver disease. (nih.gov)
Cholangiocarcinoma2
- People with Caroli disease are 100 times more at risk for cholangiocarcinoma than the general population. (wikipedia.org)
- The rate of cholangiocarcinoma in Caroli Disease A German multicenter study. (uni-koeln.de)
Hepatic3
- When the disease is localized to one hepatic lobe, hepatectomy relieves symptoms and appears to remove the risk of malignancy. (wikipedia.org)
- Hepatic ultrasonogram of a neonate with Caroli disease. (medscape.com)
- and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension ( HYPERTENSION, PORTAL ). (nih.gov)
Dilatation3
- 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. (wikipedia.org)
- Caroli disease is a rare, inherited disorder that causes cystic dilatation of the bile ducts within the liver. (klarity.health)
- The diagnosis of Caroli disease is based on the detection of cystic dilatation of the bile ducts. (klarity.health)
Left lobe2
- Commonly, the disease is limited to the left lobe of the liver. (wikipedia.org)
- [ 1 ] . Because reports have described cases limited to the left lobe of the liver, some have described Caroli disease as either localized or diffuse. (medscape.com)
Digestive Diseases4
- SUMMARY: Findings of research misconduct have been made against Carlo Spirli, Ph.D. (Respondent), who was an Assistant Professor of Medicine, Department of Digestive Diseases, Yale University (YU). (nih.gov)
- Reaching conclusions regarding digestive diseases indicate that diabetic sub- relationships between diabetes and most digestive jects are more likely than the general U.S. population conditions is limited by inconsistent case definitions to report a number of digestive conditions, including and the cross-sectional nature of most studies. (nih.gov)
- A similar approach affected by diabetes, but the liver and exocrine pan- has been taken for the few digestive diseases that may creas may also have a modulating if not causal role in increase the risk of developing diabetes, such as liver the development of diabetes. (nih.gov)
- 1,412 also received the digestive diseases component. (nih.gov)
ARPKD1
- Family history may include kidney and liver disease due to the link between Caroli disease and ARPKD. (wikipedia.org)
Symptoms3
- After recognizing symptoms of related diseases, Caroli disease can be diagnosed. (wikipedia.org)
- This article will discuss the symptoms of Caroli disease and describe ways that can be used to diagnose and treat it. (klarity.health)
- NPH is suspected in the absence of congenital anomalies of the kidneys and/or urinary tract (CAKUT) and signs or symptoms of glomerular kidney disease. (nih.gov)
Genetic3
- The exact cause of Caroli disease is unknown but it is theorised that the genetic disorder affects the development of the bile ducts in the liver. (klarity.health)
- Wirblich C , Thiel HJ , Meyers G . Genetic map of the calicivirus rabbit hemorrhagic disease virus as deduced from in vitro translation studies. (cdc.gov)
- The potential brain functional and structural basis of reserve in Alzheimer's disease and other brain disorders are discussed, as well as relevant lifestyle and genetic factors assessed in both humans and animal models. (biomedcentral.com)
Cholelithiasis1
- Caroli disease may also cause cholelithiasis , which is the formation of gallstones in the bile ducts. (klarity.health)
PKHD11
- PKHD1 is expressed primarily in the kidneys with lower levels in the liver, pancreas, and lungs, a pattern consistent with phenotype of the disease, which primarily affects the liver and kidneys. (wikipedia.org)
Malignancy1
- Good evidence suggests that malignancy complicates Caroli disease in roughly 7% of cases. (wikipedia.org)
Patients with severe1
- In patients with severe polycystic liver disease (PLD), there is a need for new treatments. (biomedcentral.com)
Diffuse1
- In diffuse cases of Caroli disease, treatment options include conservative or endoscopic therapy, internal biliary bypass procedures, and liver transplantation in carefully selected cases. (wikipedia.org)
Gallstones1
- Although subject to a num- diabetes may have increased risk of liver disease and ber of biases, national hospitalization data suggest gallstones, although these relationships are entangled that diabetic patients may also be more prone than the with those of obesity and hyperinsulinemia. (nih.gov)
Diagnosis3
- This confirmed the diagnosis of Caroli disease. (medscape.com)
- In short, longitudinal AIBL data support new diagnostic criteria that no longer require dementia to make a diagnosis of Alzheimer's disease, Rowe said. (alzforum.org)
- Substantial advances in the diagnosis, treatment, and long-term care of children presenting paediatric liver disease mean that survival into adulthood is now the expectation rather than the exception. (rare-liver.eu)
Sporadic1
- In addition, the registry also contains a number of sporadic colorectal cancer patients in whom the disease is associated with FAP extracolonic manifestations, in particular congenital hypertrophy of the retinal pigment epithelium (CHRPE). (bmj.com)
Commonly1
- Caroli disease is commonly diagnosed after this "central dot sign" is detected on a CT scan or ultrasound. (wikipedia.org)
Ducts1
- However, cholangiography is the best, and final, approach to show the enlarged bile ducts as a result of Caroli disease. (wikipedia.org)
Alzheimer's4
- In a sign of how brain imaging is increasingly infiltrating clinical research on age-related neurodegeneration, the 11th AD/PD International Conference on Alzheimer's and Parkinson's Diseases hosted a day-long exchange of new imaging data and discussion of current challenges for the research community in these two disorders. (alzforum.org)
- Alzheimer's disease (AD) impacts men and women differently, but the effect of sex on predementia stages is unclear. (biomedcentral.com)
- Seven hundred forty-two participants with NC and participants with eMCI from the Alzheimer's Disease Neuroimaging Initiative (second cohort [ADNI2] and Grand Opportunity Cohort [ADNI-GO]) were included. (biomedcentral.com)
- Florbetapir positron emission tomography (PET) amyloid positivity (A + ) is a biomarker for fibrillar amyloid associated with a high likelihood of progression to Alzheimer's disease (AD) dementia [ 1 ]. (biomedcentral.com)
Occur2
- Caroli disease may occur sporadically or be inherited in an autosomal dominant or recessive manner . (klarity.health)
- The predominance of this disease mainly occur after six decades and that can extend up to eight decades of life. (cancerwall.com)
Abdominal1
- A 30-year-old man with no underlying diseases visited our hospital for further evaluation and treatment of a liver mass discovered on abdominal computed tomography (CT) scan at another hospital. (e-cmh.org)
Ductal1
- Although an obstructing lesion was not found on the images, we considered the duct obstructed with a mass or parasite proximal to the dilated ductal area because size of the dilated ductal site was very limited and could not find any evidence of other bile duct disease. (e-cmh.org)
Liver Diseases1
- PKA-Dependent p-SER675-b-Catenin Phosphorylation Increases Cholangiocyte Motility in Pkhd1del4/del4 Mouse, a Model of Fibropolycystic Liver Diseases Caused by Defective Fibrocystin Function. (nih.gov)
Infectious Diseases1
- Incidences of Infectious Events in a Renal Transplant Cohort of the German Center of Infectious Diseases (DZIF). (uni-koeln.de)
Congenital heart d1
- Ang mga kondisyon ng puso, na na-diagnose ng mga pediatric cardiologist, ay maaaring kabilang ang congenital heart disease at cardiomyopathy. (donatelife.net)
Variant2
- Delibes-Mateos M , Ferreira C , Carro F , Escudero MA , Gortázar C . Ecosystem effects of variant rabbit hemorrhagic disease virus, Iberian Peninsula. (cdc.gov)
- Capucci L , Fallacara F , Grazioli S , Lavazza A , Pacciarini ML , Brocchi E . A further step in the evolution of rabbit hemorrhagic disease virus: the appearance of the first consistent antigenic variant. (cdc.gov)
Clinical2
- He was awarded his MD by the University of Leeds in 1961, based on his clinical and laboratory studies of liver disease. (britishlivertrust.org.uk)
- The main objective of the present paper is to develop a translational perspective on putative mechanisms underlying reserve against neurodegenerative disease, combining evidence from epidemiological and clinical studies with knowledge from animal and basic research. (biomedcentral.com)
Dementia2
- There is a large amount of evidence on epidemiological risk and protective factors for neurodegenerative diseases and dementia, yet the biological mechanisms that underpin the protective effects of certain lifestyle and physiological variables remain poorly understood, limiting the development of more effective preventive and treatment strategies. (biomedcentral.com)
- Therefore, a cultural transformation of the dementia research field is urgently required to replicate successes in other disease areas [ 5 ]. (biomedcentral.com)
