Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0239946 (liver fibrosis)
 8,268 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From 1976-1987 a total of 26 infants and children with polycystic kidney disease were treated at the Children's Hospital of the Medical School Hannover. 13 of them suffered from infantile recessive polycystic kidney disease (IRPKD), and 13 from adult dominant polycystic kidney disease (ADPKD). IRPKD was diagnosed at a median age of 0.33 years (range 1 day-13 years), ADPKD at 6.0 years (3 days-14 years). Of those with IRPKD two infants died from bacterial infection and two others developed terminal renal insufficiency at the age of 8 years, while the others are living and 1-20 years old. All those suffer from severe arterial hypertension and have reduced renal function, but only 5 developed signs of liver fibrosis. Of those with ADPKD one infant died from sepsis and renal insufficiency, while the others are well and now 2-17 years old. Only one child needs an antihypertensive treatment. The most important criteria to differentiate IRKPD and ADKPD in children are the genetic transmission, age of first manifestation, hypertension and renal function. The prognosis is much more severe in IRPKD than in ADPKD, but is not as infaust in IRPKD as often assumed.
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PMID:[Cystic kidneys in children]. 266 42

Schistosomiasis is a parasitic disease caused by blood flukes which live in the mesenteric and/or vesical veins of humans over a life span of several years. Cercariae are released by infected intermediate snail hosts into fresh water whose larvae then penetrate the skin of man when the individual contacts infected fresh water. Schistosomiasis is debilitating, setting in slowly and causing concern in its chronic stages. Chronic infection results in complications such as liver fibrosis and portal hypertension for Schistosoma mansoni and ureteric obstruction, bacterial infection, and cancer of the bladder for S. haematobium. In endemic areas, children have the highest prevalence and intensity of infection due to their more extensive contact with water relative to adults. Chemotherapy helps to control the disease, but population immigration, untreated pregnant women and very young children, and the selectiveness of control strategies make reinfection inevitable. This paper reports findings on the rate of reinfection with S. mansoni in Kirinyaga district, Kenya, between September 1983 and December 1988, after a prevention and control intervention. Schistosomiasis is endemic in that area of Kenya. Measures applied during the intervention included chemotherapy and community mobilization to effect change in water contact habits and faecal disposal. Individuals aged 5-19 years showed an increasing trend of reinfection compared to individuals aged 30-59 years, with more than 50% of subjects in the 5-19 year old age group being reinfected by twelve months of follow-up. The young age group also accounted for 91% of the egg-load and 83% of all infections at the end of the study period. The majority of the 5-19 year old age group comprises school children. An urgent need therefore exists to cover schistosomiasis-related issues in schools.
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PMID:Pattern of Schistosoma mansoni infection after intervention in Mwea irrigation scheme in Kenya. 779 62

Stellate cells are star-shaped cells located in the liver and mediate a multitude of primarily non-immunological functions. They play a pivotal role in the metabolism of vitamin A and store 80% of total body retinol. Upon activation, stellate cells differentiate to myofibroblasts for production of extracellular matrix, leading to liver fibrosis. Moreover, activated stellate cells regulate liver blood flow through vasoconstriction implicated in portal hypertension. Earlier work demonstrated stellate cell derived secretion of chemokines and cytokines such as transforming growth factor beta (TGF-beta), suggesting an association with immunological processes. Indeed, recent evidence indicated that hepatic stellate cells perform potent APC function for stimulation of NKT cells as well as CD8 and CD4 T cells. Additionally, stellate cell mediated antigen presentation induced protective immunity against bacterial infection. Current experiments reveal that the presenting ability of stellate cells is the key to antigen-dependent T cell instruction by vitamin A derived retinoic acid. Finally, future studies will show whether in the firmament of immunology stellate cells will represent fixed or falling stars.
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PMID:Starring stellate cells in liver immunology. 1806 43

Endogenous cannabinoids (EC) are ubiquitous lipid signalling molecules providing different central and peripheral effects that are mediated mostly by the specific receptors CB1 and CB2. The EC system is highly upregulated during chronic liver disease and consistent experimental and clinical findings indicate that it plays a role in the pathogenesis of liver fibrosis and fatty liver disease associated with obesity, alcohol abuse and hepatitis C. Furthermore, a considerable number of studies have shown that EC and their receptors contribute to the pathogenesis of the cardio-circulatory disturbances occurring in advanced cirrhosis, such as portal hypertension, hyperdynamic circulatory syndrome and cirrhotic cardiomyopathy. More recently, the EC system has been implicated in the development of ascites, hepatic encephalopathy and the inflammatory response related to bacterial infection. Rimonabant, a selective CB1 antagonist, was the first drug acting on the EC system approved for the treatment of obesity. Unfortunately, it has been withdrawn from the market because of its neuropsychiatric side effects. Compounds able to target selectively the peripheral CB1 receptors are under evaluation. In addition, molecules stimulating CB2 receptor or modulating the activity of enzymes implicated in EC metabolism are promising areas of pharmacological research. Liver cirrhosis and the related complications represent an important target for the clinical application of these compounds.
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PMID:The endocannabinoid system in advanced liver cirrhosis: pathophysiological implication and future perspectives. 2389 Feb 8