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.
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.
A DNA-binding protein that interacts with a 17-base pair sequence known as the CENP-B box motif. The protein is localized constitutively to the CENTROMERE and plays an important role in its maintenance.
An individual that contains cell populations derived from different zygotes.
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.
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.
The genetic process of crossbreeding between genetically dissimilar parents to produce a hybrid.
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.
Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.
Passages within the liver for the conveyance of bile. Includes right and left hepatic ducts even though these may join outside the liver to form the common hepatic duct.
A genetically heterogeneous group of heritable disorders resulting from defects in protein N-glycosylation.
The channels that collect and transport the bile secretion from the BILE CANALICULI, the smallest branch of the BILIARY TRACT in the LIVER, through the bile ductules, the bile ducts out the liver, and to the GALLBLADDER for storage.
Diseases in any part of the ductal system of the BILIARY TRACT from the smallest BILE CANALICULI to the largest COMMON BILE DUCT.
Tumors or cancer of the BILE DUCTS.

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 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.

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 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 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.

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 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 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 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, 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 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) 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 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 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, 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.

... 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, ...
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 ...
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 ...
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 ...
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 ...
... 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 ...
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. ...
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). ...
She suffered from Caroli disease, and documented her experiences in The Chronic Illness Experience: Embracing the Imperfect ...
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 ...
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 ...
They suspect Caroli disease to be the underlying cause of the liver failure, but fail to confirm it, leaving the case still ...
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: ...
... 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 ...
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 ( ...
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 ...
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 ...
... heart disease and defect Cor Caroli and Cor Hydrae, stars Côr Cymru, Welsh choir competition Cor Scorpii, Norwegian metal band ...
... 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 ...
... 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. ...
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 - ...
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( ...
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 ...
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 ...
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 ...
... 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 ...
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 ...
... 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 ...
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, ...
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, ...
... 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, ...
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 ...
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 ...
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 ...
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 ...
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 ...
Notes: Abstract We describe a case of Caroli syndrome (Carolis disease and congenital hepatic fibrosis) in a 10-year-old boy ... Keywords: Key words Caroli syndrome ; Carolis disease ; Congenital hepatic fibrosis ; Endoscopic retrograde cholangiography ; ...
Caroli disease. Crigler-Najjar syndrome Hemochromatosis Hepatitis E. Porphyria Primary Biliary Cholangitis (PBC) Primary ... Formerly called veno-occlusive disease.). • OTHER LIVER VASCULAR DISEASES. Arterio-Portal fistula. Hepatic arteriovenous ... Rare Liver Diseases covered:. - Acute Liver Failure (ALF). - Autoimmune Hepatitis (AIH). - Biliary Atresia (BA);. - IgG4- ... Diseases covered:. Acute Liver Failure (ALF). Alpha-1-Antitrypsin Deficiency (A1ATD). Autoimmune Hepatitis (AIH). Biliary ...
Caroli disease- Jaundice. Polycystic liver disease- Hepatomegaly. Question :97 All are true about splenic artery aneurysm ... Non-alcoholic fatty liver disease Obstructive sleep apnoea Gastro-oesophageal reflux disease. All of the above. ... Incidence of small bowel lymphoma is High in Crohns Disease and AIDS Hodgkins lymphoma doesnt affect the small bowel at all ... There is no symptom or stigmata of liver disease. Most appropriate approach:. Reassure the patient. Repeat titre every 3 years ...
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 ...
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 ...
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 Disease Whats New Last Posted: Mar 08, 2023 * Cholangiolocellular Carcinoma With Ductal Plate Malformation Pattern ... Selected Rare Diseases. Browse full list of rare diseases A-Z *Alpha-1 Antitrypsin Deficiency ... Inclusion in the update does not necessarily represent the views of the Centers for Disease Control and Prevention nor does it ...
Addisons disease Alders-Schonberg disease Alexanders disease Alzheimers disease Balls disease Barlo... ... A list of medical diseases with eponymous names: ... Bylers disease *Caisson disease *Canavan disease *Carolis ... Alzheimers disease *Balls disease *Barlows disease *Bazins disease *Bechterews disease *Behçets disease *Bergers disease ... Creutzfeldt-Jakob disease. Bornholm Disease. Barlows disease. Tay-Sachs Disease. Raynauds Disease. disease. Wernickes ...
mh:Caroli Disease (1) Order by. Year (decreasing). Relevance. Year (increasing). Show: 20 , 50 , 100 ... Maladie de Caroli à révélation précoce. A propos dun cas au service de néonatologie du Centre Hospitalier Abass Ndao de Dakar ... Léchographie abdominale et lIRM ont permis de confirmer le diagnostic de maladie de Caroli en montrant une dilatation ... Introduction : La maladie de Caroli est une maladie hépato-biliaire dorigine génétique rarement diagnostiquée en pédiatrie. ...
Other frequently associated conditions include Caroli syndrome and polycystic kidney disease. CHF is also a known accompaniment ... Switching to normal diet (ND) is the regular therapy for high-fat diet (HFD)-induced nonalcoholic fatty liver disease (NAFLD). ... Lipotoxicity in nonalcoholic fatty liver disease is mediated in part by the activation of the stress kinase JNK, but whether ... LESSONS: CHF is an AR disease resulting in portal hypertension and often associated with renal malformations. CHF is also ...
Tissue Diseases Skin Cancer Skin Diseases Skin Diseases Bacterial Skin Diseases Fungal Skin Diseases Infectious Skin Diseases ... Diseases of Camels and Camelids Diseases of Insectivores Diseases of Invertebrates Diseases of Leporidae and Rodents Diseases ... Pagets Disease Mammary Pagets Disease of Bone Pagets Disease of Breast Pain Pain Disorder Pain Insensitivity with Anhidrosis ... Disease Nickel Poisoning NIDDM Niemann-Pick Disease Niemann-Pick Diseases Night Terror Ninth Cranial Nerve Diseases Nipah Virus ...
Pathology Pearls: The 3 Cs - Congenital Hepatic Fibrosis, Caroli disease & Caroli Syndrome ... This is a classic case of polycystic liver disease in a patient with associated autosomal dominant polycystic kidney disease ( ... Cholestatic Liver Disease Drug-Induced Liver Disease Inherited Disorders Liver Anatomy, Development, and Physiology MASLD/MASH ... Neoplasms of the Liver Pathology Pediatrics Pregnancy-Related Liver Disease Symptoms and Clinical Presentation of Liver Disease ...
Caroli disease * Ascending colon * Oesophagus * Transjugular intrahepatic portosystemic shunt * Hepatopulmonary syndrome * ...
Caroli disease: This rare, autosomal recessive disorder is characterized by segmental cystic dilation of intrahepatic bile ... hydatid disease) or Echinococcus multilocularis (alveolar disease). Symptoms... read more : This disease is caused by the cyst- ... The rare congenital polycystic liver is commonly associated with polycystic disease of the kidneys Autosomal Dominant ... Polycystic Kidney Disease (ADPKD) Polycystic kidney disease (PKD) is a hereditary disorder of renal cyst formation causing ...
Caroli disease) Drug-induced liver disease Fatty liver disease Nonalcoholic fatty liver disease Nonalcoholic steatohepatitis ... Liver cancer Inherited diseases, such as hemochromatosis and Wilson disease Symptoms of liver disease can vary, but they often ... Congenital hepatic fibrosis and Carolis disease closely resemble each other pathophysiologically, in that both occur as a ... Liver cirrhosis; Chronic liver disease; End-stage liver disease; Liver failure - cirrhosis; Ascites - cirrhosis Cirrhosis is ...
Carolis disease and syndrome: long-term results of the French Association of Surgery Multicenter Study. Ann Surg, 258(5), 713- ... Patterns of initial disease recurrence after resection of gallbladder carcinoma and hilar cholangiocarcinoma: implications for ... Loved ones obtain various information about the progression of the patients cancer disease which is important for their ... Bile levels of carcino-embryonic antigen in patients with hepatopancreatobiliary disease. Eur J Gastroenterol Hepatol, 8(2), ...
I know this doctor is supposed to be one of the best, he diagnosed my sister with Caroli disease after she had been to numerous ... Have you had a colonoscopy-do you have IBS or is it UC-UC is a related disease to the PSC-I started with UC in 1987 and found I ... Have you had a colonoscopy-do you have IBS or is it UC-UC is a related disease to the PSC-I started with UC in 1987 and found I ... Yes, have had the colonoscopy they havent mentioned IBS, there was lots of diverticular disease showed up on cat-scan. I have ...
Suba South Member of Parliament Caroli Omondi said the project was instrumental in the fight against communicable diseases in ... Omondi said the project will protect residents from contracting waterborne diseases and save them the long treks searching for ...
Carolis disease, normal and fetal livers. In ADPKD and control livers Ang-1 and Ang-2 gene expression was studied by real-time ... Carolis disease, normal and fetal livers. In ADPKD and control livers Ang-1 and Ang-2 gene expression was studied by real-time ... Effects of angiogenic factor overexpression by cholangiocytes in polycystic liver diseases. FABRIS, LUCA;CADAMURO M;FIOROTTO R; ... Liver involvement in autosomal dominant polycystic kidney disease (ADPKD) is characterized by altered remodeling of the ...
Disease tracking markers for Alzheimers disease at the prodromal (MCI) stage. V Drago, C Babiloni, D Bartr s-Faz, A Caroli, B ... Longitudinal evaluation of cerebral morphological changes in Parkinsons disease with and without dementia. B Ram rez-Ruiz, MJ ... Modulation of verbal fluency networks by transcranial direct current stimulation (tDCS) in Parkinsons disease. JB Pereira, C ... mild cognitive impairment and Alzheimers disease. C Sol -Padull s, D Bartr s-Faz, C Junqu , P Vendrell, L Rami, ... ...
Betty Caroli, First Ladies: From Martha Washington to Michelle Obama, (Oxford, Oxford University Press, 2010), 56-58. ... In the absence of available sources, the timeline of Elizas contraction with the disease, her symptoms and the exact nature of ... Sometime during the 1850s and possibly during her last pregnancy, Eliza developed tuberculosis, a contagious disease that ...
Adult cystic fibrosis, Babers syndrome, Brachmann-de lange syndrome, Caroli disease, celiac disease, chester porphyria, ... autoimmune thyroid disease, Behcets disease, celiac disease, chronic fatigue syndrome, chronic necrotizing vasculitis, Churg- ... Crohns disease, desmoid disease, hereditary, Gilberts syndrome, Marfan syndrome, periodic fever syndrome, pyruvate ... Infectious Disease African horse sickness, amoebiasis, ancylostoma duodenale, angiostrongyliasis, anguillulosis, anisakis, ...
Caroli disease. *Biliary stents. *Pancreas *Normal pancreas. *Acute pancreatitis. *Chronic pancreatitis. *Pancreatic tumor> * ...
  • PKHD1, the gene linked to ARPKD, has been found mutated in patients with Caroli syndrome. (wikipedia.org)
  • The disease affects about one in 1,000,000 people, with more reported cases of Caroli syndrome than of Caroli disease. (wikipedia.org)
  • The number of cases of Caroli syndrome caused by PKHD1 mutations is not known. (medscape.com)
  • 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)
  • 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)
  • [ 1 ] As with congenital hepatic fibrosis , Caroli syndrome is often associated with autosomal recessive polycystic kidney disease (ARPKD) . (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)
  • type II, or complex Caroli disease, is also known as Caroli's syndrome. (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)
  • Patients with Caroli disease or Caroli syndrome may have a history of intermittent abdominal pain, which reflects episodes of bile stasis or the passage of bile stones. (medscape.com)
  • In Caroli syndrome, portal hypertension may result in hematemesis or melena secondary to bleeding varices. (medscape.com)
  • Because Caroli syndrome is associated with autosomal recessive polycystic kidney disease (ARPKD) and is inherited in an autosomal recessive manner, the patient may have a family history of kidney or liver disease. (medscape.com)
  • Mutations in PKHD1 on chromosome 6p21, which is the gene linked to ARPKD, have been identified in patients with Caroli syndrome. (medscape.com)
  • Caroli syndrome is inherited in an autosomal recessive manner. (medscape.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)
  • Zollinger-Ellison Syndrome (ZES) is related to the pancreatic disease in which islet cells starts the overproduction of gastric acid in the stomach which ultimately leads to peptic ulcers. (edwiserinternational.com)
  • 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)
  • 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)
  • To investigate the possible role of angiogenic mechanisms, we have studied the immunohistochemical expression of vascular endothelial growth factor (VEGF), angiopoietin-1 (Ang-1), angiopoietin-2 (Ang-2) and their receptors (VEGFR-1, VEGFR-2, Tie-2) in ADPKD, Caroli's disease, normal and fetal livers. (unipd.it)
  • Centers for Disease Control and Prevention. (cdc.gov)
  • Inclusion in the update does not necessarily represent the views of the Centers for Disease Control and Prevention nor does it imply endorsement of the article's methods or findings. (cdc.gov)
  • In a carefully conducted study, a major research laboratory at the Centers for Disease Control and Prevention (CDC) reported the determination of 28 elements in hair from non-occupationally exposed U.S. populations. (cdc.gov)
  • 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)
  • 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)
  • A rare association with autosomal dominant polycystic kidney disease (ADPKD) has also been reported. (medscape.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)
  • Liver involvement in autosomal dominant polycystic kidney disease (ADPKD) is characterized by altered remodeling of the embryonic ductal plate (DP) with presence of biliary cysts and aberrant portal vasculature. (unipd.it)
  • Cholangiocytes were strongly positive for VEGF, VEGFR-1, VEGFR-2 and Ang-2 in ADPKD and Caroli, and also for Ang-1 and Tie-2 in ADPKD, similar to fetal ductal plate cells. (unipd.it)
  • 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)
  • Family history may include kidney and liver disease due to the link between Caroli disease and ARPKD. (wikipedia.org)
  • 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)
  • L'échographie abdominale et l'IRM ont permis de confirmer le diagnostic de maladie de Caroli en montrant une dilatation kystique des voies biliaires intra-hépatiques. (bvsalud.org)
  • 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)
  • Granulomatous liver disease prompts a broad differential (see Pathology Pearls post ) that includes infection (generally associated with necrotizing granulomas, unlike the non-necrotizing granulomas seen in this instance), drug-induced liver injury (DILI), biliary disease (particularly primary biliary cholangitis), and sarcoidosis. (aasld.org)
  • 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)
  • In this instance, the presence of non-necrotizing hepatic granulomas and elevated alkaline phosphatase in a patient with a history of pulmonary disease and hilar lymphadenopathy is strongly suggestive of sarcoidosis . (aasld.org)
  • Introduction : La maladie de Caroli est une maladie hépato-biliaire d'origine génétique rarement diagnostiquée en pédiatrie. (bvsalud.org)
  • People with Caroli disease are 100 times more at risk for cholangiocarcinoma than the general population. (wikipedia.org)
  • 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)
  • Caroli disease, bilateral diffuse cystic renal dysplasia, situs inversus, postaxial polydactyly, and preauricular fistulas: a ciliopathy caused by a homozygous NPHP3 mutation. (medscape.com)
  • Caroli disease is commonly diagnosed after this "central dot sign" is detected on a CT scan or ultrasound. (wikipedia.org)
  • In conclusion, consistent with the immature phenotype of the cystic epithelium, expression of VEGF, VEGFRs, Ang-1 and Tie-2 is strongly upregulated in cholangiocytes from polycystic liver diseases. (unipd.it)
  • However, cholangiography is the best, and final, approach to show the enlarged bile ducts as a result of Caroli disease. (wikipedia.org)
  • Caroli disease is also associated with liver failure and polycystic kidney disease. (wikipedia.org)
  • Recent research supports a mechanistic link between ciliary dysfunction and polycystic kidney disease, although this remains controversial. (medscape.com)
  • Combined cystic disease of the liver and kidney. (medscape.com)
  • Ibraghimov-Beskrovnaya O, Bukanov N. Polycystic kidney diseases: from molecular discoveries to targeted therapeutic strategies. (medscape.com)
  • After recognizing symptoms of related diseases, Caroli disease can be diagnosed. (wikipedia.org)
  • Wirblich C , Thiel HJ , Meyers G . Genetic map of the calicivirus rabbit hemorrhagic disease virus as deduced from in vitro translation studies. (cdc.gov)
  • Le Gall-Reculé G , Zwingelstein F , Laurent S , de Boisséson C , Portejoie Y , Rasschaert D . Phylogenetic analysis of rabbit haemorrhagic disease virus in France between 1993 and 2000, and the characterisation of RHDV antigenic variants. (cdc.gov)
  • Get vaccinated for diseases such as influenza, hepatitis A and B, and pneumococcal pneumonia . (symptoma.com)
  • In a 10-year period between 1995 and 2005, only 10 patients were surgically treated for Caroli disease, with an average patient age of 45.8 years. (wikipedia.org)
  • Schirrmeier H , Reimann I , Köllner B , Granzow H . Pathogenic, antigenic and molecular properties of rabbit haemorrhagic disease virus (RHDV) isolated from vaccinated rabbits: detection and characterization of antigenic variants. (cdc.gov)
  • The disease had lasted for 1 year and there was history of indolent courses of resolution and subsequent recurrence. (who.int)
  • 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)
  • O'Hara P . The illegal introduction of rabbit haemorrhagic disease virus in New Zealand. (cdc.gov)
  • Factor in and metabolic-of-life diseases in men common sildenafil correction. (doxyline.top)
  • Switching to normal diet (ND) is the regular therapy for high-fat diet (HFD)-induced nonalcoholic fatty liver disease (NAFLD). (bvsalud.org)

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