Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Portal hypertension (PH) is a complication that may occur in patients with inflammatory bowel disease (IBD). In these patients, the etiology of PH may not be alcoholic or viral cirrhosis (which cause 90% of cases in the general population). Consequently, etiologic study of PH in patients with IBD should always include a wide spectrum of possibilities. Moreover, the development of PH in IBD patients often requires a distinct therapeutic approach to IBD (both medical and surgical) as PH may be a contraindication for some drugs and is a risk factor for surgical morbidity and mortality. We present the cases of two patients with IBD who developed PH and review the most likely causes of PH in IBD, as well as preventive and therapeutic strategies.
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PMID:[Portal hypertension in patients with inflammatory bowel disease]. 2020 13

Sclerosing cholangitis is a rare progressive cholestatic liver disease affecting the biliary tract. It may be associated with other diseases including autoimmune hepatitis, immunodeficiencies, cystic fibrosis, and sickle cell disease. Sclerosing cholangitis not associated with other diseases is termed "primary sclerosing cholangitis," which has a strong association with male gender, Caucasian race, and inflammatory bowel disease. Diagnosis is based on typical biochemical, radiologic, and histologic features. Medical management is directed mainly at managing complications (pruritus, cholangitis, strictures, and nutritional deficiencies). Administration of ursodeoxycholic acid results in biochemical improvement, but has not been proven to prolong transplant-free survival. Patients with autoimmune overlap respond to immunosuppression. The disease is typically progressive and evolves to biliary cirrhosis and possibly cholangiocarcinoma. Orthotopic liver transplantation remains the only life-extending alternative for patients with sclerosing cholangitis, with good long-term patient and graft survival, and recurrent graft primary sclerosing cholangitis in about 10% of children.
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PMID:Sclerosing cholangitis: pediatric perspective. 2042 75

Inflammatory bowel disease (IBD), especially ulcerative colitis (UC), sometimes accompanies with primary sclerosing cirrhosis (PSC). Primary biliary cirrhosis (PBC) is also a chronic liver disease, but is attributed to an unknown immune abnormality. PBC frequently accompanies various autoimmune diseases, but rarely IBD. We report 2 rare cases of IBD which developed in the course of treatment for PBC. A 64-year-old man had blood in his stool 17 months after he was given a diagnosis of PBC. Colonoscopy revealed erosion and disappearance of the vascular network pattern in the rectum. UC (rectal type) was diagnosed and he received mesalazine, and following which his symptoms improved. A 40-year-old woman developed diarrhea, fever and arthralgia while receiving treatment for PBC. Colonoscopy revealed ileal erosion, a longitudinal ulcer in the ascending colon but only mild rectal inflammation. These findings were atypical of UC, and we therefore diagnosed indeterminate colitis. Her symptoms disappeared as a result of the administration of mesalazine.
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PMID:Two cases of inflammatory bowel disease diagnosed in the course of primary biliary cirrhosis. 2053 Sep 26

Primary sclerosing cholangitis is a cholestatic liver disease characterized by inflammation and fibrosis of intra-/extrahepatic bile ducts, leading to multifocal strictures. Primary sclerosing cholangitis exhibits a progressive course resulting in cirrhosis and the need for liver transplantation over a median period of 12 years. The disease is frequently associated with inflammatory bowel disease and carries an increased risk of colorectal cancer and cholangiocarcinoma. Despite extensive research, there is currently no effective medical treatment. Multiple drugs are shown to be ineffective in halting disease progression, including ursodeoxycholic acid, the most widely evaluated drug. High-dose ursodeoxycholic acid (28-30 mg/kg/day) was recently shown to increase the adverse events rate. Endoscopic or radiological dilatation of a 'dominant' stricture may lead to symptomatic and biochemical improvement. However, liver transplantation is the only life-prolonging treatment for patients with end-stage disease. Studies with promising drugs, such as antibiotics, antifibrotic agents and bile acid derivatives, are eagerly awaited.
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PMID:Treatment options for primary sclerosing cholangitis. 2067 20

Primary sclerosing cholangitis (PSC) is a chronic bile duct disease leading to fibrotic biliary strictures and liver cirrhosis. The patient population is heterogeneous with regard to disease progression and the presence of co-morbidities, complicating the practical handling of patients as well as studies of pathogenetic mechanisms. The aetiology of PSC is unknown, but the recent findings of several robust susceptibility genes emphasise the importance of genetic risk factors. There is no effective medical treatment available to delay the disease progression, but endoscopic therapy of biliary stenoses may be indicated. Follow-up of patients includes management of the inflammatory bowel disease that is found in the majority of cases along with investigations aimed at the early detection of cholangiocarcinoma and colorectal cancer, which also occur at increased frequencies. In the present review, we aim to summarise the present knowledge of PSC with a particular emphasis on the possible basis of disease variability.
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PMID:Primary sclerosing cholangitis. 2095 68

We developed a high-sensitivity C-reactive protein quantifiable chemiluminescent immunoassay (hs-CRP CLIA). The high-purity native CRP was purified from hepatic cirrhosis patient ascetic fluid by affinity and ion exchange chromatography and used as an immunogen to develop the monoclonal antibodies (mAbs) against CRP. Twenty-two mAbs were identified reactive with CRP in ELISA and 13 of them were reactive in the phosphorycholine ligand capture ELISA. The mAbs 10C5 and 10C11 were selected to develop the hs-CRP CLIA. The linearity and performance of the hs-CRP CLIA was characterized. It was showed not reactive when testing against other serum materials (IgG, hemoglobin and triglyceride). The reliable correlation (R2 > 0.993) was obtained between testing value (RLU/S) and the concentration of human serum CRP calibrator. The linearity fell in the range of 0.04-20.38 mg/L. The assay has good accuracy and reproducibility, the mean recovery was 99% and the precision of the intra- and inter assay was CVs (4.2%-5.8%) and (9.0%-11.5%), respectively. In testing of 90 human sera, this assay performed well and correlated comparably with a commercial hs-CRP ELISA kit. Thus, hs-CRP CLIA is an accurate, reliable, quantifiable assay for detection of high-sensitive C-reactive protein in serum, it may be useful to improve the risk assessment of cardiovascular disease and the prognosis of inflammatory bowel disease.
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PMID:[Chemiluminescent immunoassay for high-sensitivity C-reactive protein]. 2109 Jan 22

Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease characterized by the destruction of medium- to large-sized bile ducts and intense concentric fibrosis. Complications from PSC include bacterial cholangitis, cirrhosis, and cholangiocarcinoma and a therapy that might alter the natural history of the disease remains lacking. Our understanding of the pathogenesis of PSC also remains rudimentary but the strong association between PSC and inflammatory bowel disease suggest causal links between the diseases. The male predominance in PSC, lack of a defined, pathogenic auto-antigen, and the potential role of the innate immune system suggest that PSC may be due to dysregulation of immunity rather than a classic autoimmune disease. However, PSC shares several genetic susceptibility loci with other autoimmune diseases including the human leukocyte antigen DRB01*03 haplotype. The precise immune response of PSC is largely unknown but likely involves activation of the innate immune system by bacterial components delivered to the liver via the portal vein. Induction of adhesion molecules and chemokines leads to the recruitment of intestinal lymphocytes. Bile duct injury results from the sustained inflammation and production of inflammatory cytokines. Biliary strictures may cause further damage as a result of bile stasis and recurrent secondary bacterial cholangitis. Progress in our basic understanding of PSC is desperately needed in order to rationally design new therapeutic approaches to this disease.
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PMID:Cutting edge issues in primary sclerosing cholangitis. 2117 Jun 5

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are important causes of cholestatic liver disease. IgG4-associated cholangitis (IAC) also belongs to the same entity. Overlap syndromes combine characteristics of cholestatic liver diseases and autoimmune hepatitis. The diagnosis of PBC is based on the detection of anti-mitochondrial antibodies. PBC is frequently associated with other autoimmune disorders. The treatment of choice is ursodeoxycholic acid. PSC is frequently associated with inflammatory bowel disease (IBD). The cholangiography shows characteristic bile duct lesions. Bile duct strictures and bacterial cholangitis should be treated by dilatation and antibiotics, respectively. Cirrhosis may ultimately develop in PBC and PSC. In advanced PBC or PSC, liver transplantation might be indicated. The clinical course of IAC is similar to PSC. In contrast to PSC, however, there is no association with IBD.
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PMID:[Cholestatic liver diseases]. 2145 40

Cholangiocarcinoma (CC) is the second most common primary hepatic malignancy after hepatocellular cancer. CC accounts for approximately 10%-25% of all hepatobiliary malignancies. There are considerable geographic and demographic variations in the incidence of CC. There are several established risk factors for CC, including parasitic infections, primary sclerosing cholangitis, biliary-duct cysts, hepatolithiasis, and toxins. Other less-established potential risk factors include inflammatory bowel disease, hepatitis C virus, hepatitis B virus, cirrhosis, diabetes, obesity, alcohol drinking, tobacco smoking, and host genetic polymorphisms. In studies where the distinction between intra- and extrahepatic CC was used, some potential risk factors seem to have a differential effect on CC, depending on the site. Therefore, the consistent use of a more refined classification would allow a better understanding of risk factors for CC.
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PMID:Risk factors for cholangiocarcinoma. 2148 76

Epigenetics is defined as heritable changes in gene expression that are, unlike mutations, not attributable to alterations in the sequence of DNA. The predominant epigenetic mechanisms are DNA methylation, modifications to chromatin, loss of imprinting and non-coding RNA. Epigenetic regulation of gene expression appears to have long-term effects and wide-ranging effects on health. Diet and environmental exposures may potentially alter the level and scope of epigenetic regulation, thus interesting developments in the study of epigenetics might explain correlations that researchers have found between lifestyle and risk of disease. Aberrant epigenetic patterns have been linked to a number of digestive diseases including Barrett's esophagus, cirrhosis, inflammatory bowel disease, and numerous gastrointestinal malignancies. In fact, many exciting discoveries about epigenetics in general have been made by studying diseases of the gastrointestinal tract and hepatobiliary tree. Epigenetic modifications of DNA in cancer and precancerous lesions offer hope and the promise of novel biomarkers for early cancer detection, prediction, prognosis, and response to treatment. Furthermore, reversal of epigenetic changes represents a potential target of novel therapeutic strategies and medication design. In the future, it is anticipated that innovative diagnostic tests, treatment regimens, and even lifestyle modifications will be based on epigenetic mechanisms and be incorporated into the practice of medicine.
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PMID:Epigenetics: principles and practice. 2173 76


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