Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Initially liver morphology of chronic destructive non-suppurative cholangitis (CDNC) is rather atypical. Therefore, early morphological diagnosis is difficult. First symptoms are severe pruritus and an increase of IgM, AP and gamma-GT. Own investigation of 101 CDNC patients showed that antimitochondrial antibodies (AMA) are generally later present than the increase of the a/m enzymes. Also remarkable is the fact that among 101 patients are 13 men generally observed during the last 3 years. The most difficult problem is the treatment of CDNC. Here we have to differentiate between symptomatic basic treatment and so-called specific treatment. As basic treatment, ammonia-reducing amino acids, phenobarbital and finally cholestyramine are administered in order to diminish the severe pruritus. The diet must be rich on pectine. Lactulose and bifidum milk improve the diminished detoxication function of the liver. As specific treatment prednisolone and/or azathioprin have disappointed. D-penicillamine can influence CDNC at least temporarily. Because of the frequent side-effects D-penicillamine should be administered only in low doses (100-200 mg daily together with 300 mg vitaminee B6). Until not it is uncertain if the extremely bad prognosis of CDNC can be improved by medical treatment of its early stages.
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PMID:[Chronic destructive non-suppurating cholangitis]. 612 61

The authors described two cases of airborne contact dermatitis caused by 2-amino-2-methyl-1-propanol (AMP 100) in two subjects with periorbital erythema and itching skin. The AMP 100 has been used to replace ammonia as a hair dye component in the cosmetic industry with the purpose to eliminate its smell. Patch tests proved positive only to dilutions of 10% and 20% in the two described patients, as well as in other six asymptomatic subjects operating in the same working environment. The authors have diagnosed an irritative airborne contact dermatitis by AMP 100.
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PMID:Airborne contact dermatitis from 2-amino-2-methyl-1-propanol in a cosmetic company. 943 32

The treatment of children with end-stage liver disease involves the coordinated management of nutritional deficiencies, ascites, pruritus, encephalopathy, and portal hypertension. The implementation of management strategies depends upon a parent or guardian to administer the plan in the context of a child at different stages of developmental, physiologic, emotional, and physical maturity. Fat-soluble vitamins (A, D, E, and K) and micronutrient levels should be monitored routinely and supplemented if deficient. In some patients, supplemental nutrition to provide additional energy and protein is needed to ensure optimal growth and development. Ascites often respond to spironolactone and sodium restriction, but may require the addition of a loop diuretic or even abdominal paracentesis. Pruritus significantly impairs the quality of life of patients and is typically treated with ursodeoxycholic acid, rifampin, or an antihistamine. Partial biliary diversion, or liver transplant in some instances, is necessary for patients with self-mutilating pruritus that results from intrahepatic cholestasis. Hepatic encephalopathy is poorly defined in infants and small children. Elevated serum ammonia serves as a surrogate marker for encephalopathy, which is treated with dietary protein restriction and lactulose. The usefulness of medical prophylaxis for esophageal varices has been noted in adults, though such studies have not been performed in children. If variceal bleeding becomes problematic, treatment with endoscopic variceal banding or sclerotherapy is indicated. A surgical shunt to reduce portal pressure is needed in some cases. Orthotopic liver transplant ultimately may be necessary to overcome the unrelenting consequences of end-stage liver disease.
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PMID:End-stage Liver Disease in Children. 1156 Jul 88

The molecular adsorbent recirculating system (MARS) method removes from the blood catabolites either free in the plasma water such as uremic toxins and ammonia, taking advantage of dialysis or free albumin bound ones, like hepatic toxins, transferring them from the albumin in the blood to the albumin circulating in a closed loop where toxins are removed by adsorbtion on resins (charcoal and ion exchange resin). The efficacy of the method in removing the hepatic toxins either in the acute or in the acute on chronic liver failure is demonstrated in numerous studies. Based on these findings, 10 patients affected by acute on chronic liver failure were treated. The results demonstrated that the method, powerfully removing ammonia, bilirubin and bile acids (taken as method efficacy markers), reduced the blood concentrations of these molecules remarkably; allowing the elimination of the refractory pruritus (due to the lowering of plasma bile acid levels), an almost constant symptom in chronic liver diseases, especially with cholestasis, and improves other parameters (cholinesterase, alkaline phosphatase and prothrombin activity). These results agree with those reported in the literature concerning the efficacy of MARS in the replacement of the liver detoxifying function.
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PMID:[Molecular Adsorbent Recirculating System in liver function replacement therapy]. 1578 94

Prometheus is a new extracorporeal liver support device which facilitates the combined removal of both albumin-bound and water-soluble toxins based upon the method of fractionated plasma separation and adsorption (FPSA). The pilot trial included 11 patients with acute-on-chronic liver failure and concomitant renal failure. Prometheus therapy was found to be safe except for a reversible decrease of blood pressure. In three patients, clotting of the secondary system occurred. Prometheus treatment significantly improved blood levels of protein-bound (conjugated bilirubin, bile acids, ammonia) and water-soluble (creatinine, urea) substances. Thus, Prometheus might be a new therapeutic option in patients with severe hepatorenal syndrome. Furthermore, there is some preliminary experience with Prometheus in the treatment of refractory cholestatic pruritus and in successful bridging to liver transplantation. In order to compare extraction capacities of Prometheus and the molecular adsorbent recirculating system (MARS), five patients were crossover-treated with both systems. Prometheus resulted in significantly higher reduction ratios of bilirubin, ammonia and urea. Another study closely monitored whether the device causes an unselective removal. Neither important cytokines nor coagulation factors were found to be removed. In conclusion, Prometheus seems to be a new therapeutic option in artificial liver support. A significant improvement of the biochemical milieu was already observed after two treatments. The potential to remove protein-bound and water-soluble substances has been shown without signs of a significant unselective removal.
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PMID:Review article: clinical experience with Prometheus. 1668 14

We assessed the safety and clinical efficacy of the Molecular Adsorbent Recirculating System (MARS) in liver failure patients admitted to our intensive care unit (ICU) from May 2000 to February 2006. Of 28 adult patients with bilirubin >15 mg/dL and hepatic encephalopathy (HE) grade > or =2 or hepato-renal syndrome, 22 patients were included in the study, because 6 patients were older than 65 years of age or showed recent alcohol abuse or extrahepatic malignancy. Patients were assigned to 2 groups according to whether MARS therapy was associated with a transplantation procedure: 11 patients received MARS therapy and liver transplantation (OLT group) and 11 patients received MARS therapy alone (non-OLT group). Five of 11 patients in the OLT group were listed for transplantation and 6 patients with graft failure for retransplantation. The patients in the OLT and non-OLT groups were similar in MELD, SOFA, and SAPS scores. All patients were stable and free from complications. MARS significantly reduced bilirubin, bile acids, and blood urea nitrogen (BUN) levels in both groups (P < .05), whereas a significant decrease in ammonia level was observed in the OLT group. Patient survival rates at 3 and 6 months in the OLT group were 91% and 73%, respectively, and in the non-OLT group, 9% and 9%, respectively (P < .001). MARS was safe and well tolerated, improving biochemical parameters, neurological function, and pruritus. In terms of survival, the use of MARS alone was not effective due to the high rate of multiple organ failure. Nevertheless, the association of MARS with a transplant/retransplantation procedure was highly effective.
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PMID:Molecular adsorbent recirculating system in liver transplantation: Safety and efficacy. 1717 27

The Molecular Adsorbent Recirculating System (MARS) clears the blood from catabolites that either occur free in the plasma water (through dialysis), such as uremic toxins and ammonia, or are bound by albumin, such as hepatic toxins. The latter are transferred from the albumin in the blood to the albumin circulating in a closed loop where toxins are removed by adsorption on resins (charcoal and ion-exchange resin). The efficacy of this extracorporeal blood purification method in the treatment of acute or acute-on-chronic liver failure (also associated with renal failure) has been demonstrated in numerous studies. Fifty-one patients, 5 affected by acute liver failure and 46 by acute-onchronic liver failure (8 of them with additional renal failure) were treated with MARS. The results demonstrated that the method, which effectively removes ammonia, bilirubin, bile acids and uremic toxins, reduces the blood concentration of these molecules. It thereby improves the patient's clinical condition and biochemical parameters including cholinesterase, alkaline phosphatase and prothrombin activity, eliminating, in addition, the drug-refractory pruritus that is a very frequent symptom in cholestatic liver disease. These results agree with those reported in the literature concerning the efficacy of MARS in the replacement of the detoxifying function of kidneys and liver.
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PMID:[Development of extracorporeal blood purification methods: Molecular Adsorbent Recirculating System (MARS) for hepatic and renal function replacement]. 1792 57

Liver dysfunction is an important cause of morbidity and mortality after orthotopic liver transplantation (OLT). The Molecular Adsorbent Recirculating System (MARS) is an albumin-based dialysis system designed to enhance the excretory function of a failing liver. MARS has been successfully used in patients affected by advanced liver disease and presenting with severe cholestasis. The aim of this study was to evaluate the safety and clinical efficacy of MARS in patients with liver dysfunction after OLT. Seven patients (primary nonfunction, 2 patients; graft dysfunction, 5 patients) fulfilled the inclusion criteria of serum bilirubin level >15 mg/dL and least 1 of the following clinical signs: hepatic encephalopathy (HE) > or = grade II, hepatorenal syndrome (HRS), and intractable pruritus. Graft and patient survival rates at 6 months were 42.8% and 57.1%, respectively. All patients tolerated MARS treatment, with no adverse event. In all patients, a decrease in serum bilirubin (P < .05), bile acids (P < .05), serum creatinine, and ammonia levels was observed after treatment with MARS. A considerable improvement of HE, as well as renal and synthetic liver functions, was observed in 4 of 5 patients with graft dysfunction, but not among those with primary nonfunction. The patients with intractable pruritus showed significant improvement of this symptom after MARS therapy. Thus, MARS is a safe, therapeutic option for the treatment of liver dysfunction after OLT. Further studies are necessary to confirm whether this treatment is able to improve both graft and patient survival.
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PMID:Molecular adsorbent recirculating system (Mars) in patients with primary nonfunction and other causes of graft dysfunction after liver transplantation in the era of extended criteria donor organs. 1924 28

A 3-year old male presented with complaints of pruritus, abdominal pain for 3 weeks and jaundice. Stools were acholic. There was jaundice, liver palpable 3 cm below right costal margin, no ascites or palpable masses. Serology revealed albumin 2.9 g/dl; ammonia of 31 mmol/l; elevated conjugated bilirubin, GGT, ALT, AST and alkaline phosphatase; alpha fetoprotein 1.3 ngm/ml; BhCG 9.1 IU/; PT 12.3 secs, INR 0.9; negative hepatitis A,B,C serology. CT scan showed a non-calcified heterogeneously enhancing mass centered at the liver hilum. MRCP showed a large heterogeneously enhancing, partially solid mass in the region of the porta hepatic. Liver biopsy revealed patternless proliferation of polymorphic oval to spindled shaped neoplastic cells. There was bile ducts distortion. Immunohistochemistry revealed positivity for vimentin, desmin.These findings were diagnostic for biliary rhabdomyosarcoma.There was no evidence of metastasis. Chemotherapy was initiated. Repeat imaging 6 months after initiation of treatment showed improvement in the degree of intrahepatic ductal dilatation and decrease in tumor bulk size. Rhabdomyosarcoma is the most common malignant tumor of the biliary tree in childhood. It is difficult to diagnose and delayed diagnosis influences the prognosis.
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PMID:Biliary rhabdomyoscarcoma mimicking choledochal cyst. 1933 43

Previous studies have reported increased occurrence of respiratory and sensory irritation symptoms among residents living close to biodegradable waste sites. However, few studies have been able to quantify direct and annoyance-mediated effects based on individual-specific assessments of chemical exposures. We examined associations between residential exposures to a proxy gas (ammonia, NH3) from biodegradable wastes (mainly from farming, animal and agricultural activities) and odor annoyance and six respiratory and sensory irritation symptoms (self-reported), using adjusted logistic regression models and mediation analyses. Individual-specific NH3 exposures (n=454) in residential environments during 2005-2010 were calculated by the Danish Eulerian long-range transport model and the local-scale transport deposition model. Residential NH3 exposure was associated with increased frequency of four symptoms, including "eyes itching, dryness or irritation" and "cough" (ORadj=1.69; 95% CI: 1.09-2.61 and ORadj=1.75; 95% CI: 1.12-2.74, for each unit increase in loge(NH3 exposure)). Odor annoyance mediated the effect of exposure on cough and three sensory irritation symptoms. Mediation was either full (indirect-only effects) or partial (direct and indirect effects). This study provides support for the existence of indirect associations between residential exposures to low-to-moderate air pollution from wastes and symptoms, as well as direct dose-response associations for some of the symptoms.
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PMID:Respiratory and sensory irritation symptoms among residents exposed to low-to-moderate air pollution from biodegradable wastes. 2473 1


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