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)

PSC is an unusual disease of unknown etiology. In fact, sclerosis of the bile ducts may be the result of multiple factors, including autoimmune, bacterial, congenital, drug, or viral agents. The most commonly associated diseases are ulcerative colitis and chronic pancreatitis. Except for the earliest stages of the disease, liver histology is not specific. Most patients present with jaundice, pain, and pruritus, although an increasing number of asymptomatic patients with inflammatory bowel disease and abnormal liver function are being diagnosed. Cholangiography is the key to the diagnosis and is usually pathognomonic except in the unusual case where PSC is confused with cholangiocarcinoma. Multiple forms of medical therapy have been tried, including steroids, azothiaprine, D-penicillamine, colchicine, cholestyramine, and antibiotics. To date, however, none of these medications has altered the course of this disease. In recent years, balloon dilation of biliary strictures has been accomplished via endoscopic and percutaneous transhepatic approaches. However, in patients with PSC these nonoperative manipulations must be done repeatedly, may require multiple general anesthetics, and are difficult to perform. A direct surgical approach to the biliary tree with prolonged transhepatic stenting is indicated in patients with severe hilar or extrahepatic stricturing, persistent jaundice and/or recurrent cholangitis, and no evidence of cirrhosis. Hepatic transplantation should be reserved for patients with PSC who have well-established cirrhosis and in whom other therapeutic options have failed.
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PMID:Sclerosing cholangitis. 331 16

We describe a patient presenting with painless jaundice, anorexia and pruritus. The gall bladder was found to be lying above and behind a hypoplastic right lobe of liver. There was no evidence of cholangitis or biliary obstruction. The patient subsequently developed a bronchobiliary fistula with severe wheeze, cough and bile-stained sputum. Emergency percutaneous drainage of the gall bladder led to immediate cessation of bronchospasm and biloptysis, rendering the patient fit for definitive surgery.
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PMID:Bronchobiliary fistula due to acute cholecystitis in a suprahepatic gall bladder. 368 37

Nonoperative methods for decompressing the biliary tree obstructed by tumor offer viable alternatives to the use of surgery alone to palliate jaundice, pruritus, and impending cholangitis. Published data indicate that biliary drainage through endoscopic means may be superior to the percutaneous route, both being superior to surgical palliation in patients with unresectable pancreatic adenocarcinoma. We have inserted endoprostheses in 277 patients with carcinoma of the pancreas with a success rate of 89%. Eighteen percent of the patients were deceased in 30 days, however, there was no procedure related surgery or death. These results were achieved with a complication rate of only 21% (major = 4%). The occlusion rate was found to be 30% in 3 months. Patients were hospitalized for a mean of 3.5 days with a mean survival of 129 days. The advantages of endoscopic biliary drainage are as follows: low procedure related mortality, low incidence of major complications, and short hospital stay. In conclusion, given the short mean survival of patients with unresectable malignant biliary obstruction, nonsurgical decompression should be considered as primary treatment.
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PMID:The significance of endoscopically placed prostheses in the management of biliary obstruction due to carcinoma of the pancreas: results of nonoperative decompression in 277 patients. 374 22

Primary sclerosing cholangitis and primary biliary cirrhosis are chronic cholestatic syndromes that may be difficult to differentiate clinically. Destructive cholangitis occurs in both diseases and leads to similar clinical and biochemical abnormalities. Therefore, we compared the clinical, biochemical, immunologic, radiologic, and hepatic histologic features of these syndromes in two large groups of patients prospectively selected by predefined criteria. Primary biliary cirrhosis (n = 258) occurred predominantly in middle-aged women who were usually symptomatic with fatigue and pruritus, commonly had keratoconjunctivitis sicca, and often were hyperpigmented. Tests for antimitochondrial antibodies were always positive, usually in very high titer. Although the extrahepatic bile ducts were normal radiographically, smooth tapering and narrowing of the intrahepatic bile ducts was occasionally noted. Hepatic histology was diagnostic when a florid duct lesion was present. In contrast, primary sclerosing cholangitis (n = 60) occurred primarily in young men who were usually symptomatic with fatigue and pruritus and frequently had chronic ulcerative colitis. Tests for antimitochondrial antibodies were nearly always negative and cholangiography demonstrated abnormalities of the extrahepatic and intrahepatic bile ducts in all cases. Although hepatic histology was often compatible with the diagnosis, it was usually not diagnostic, and considerable overlap existed with the abnormalities seen in primary biliary cirrhosis. Likewise, biochemical tests of copper metabolism were similar in both syndromes. These results call attention to the differences and similarities in the clinicopathologic features of these two cholestatic syndromes and provide a basis for a rational diagnostic strategy.
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PMID:Comparison of the clinicopathologic features of primary sclerosing cholangitis and primary biliary cirrhosis. 388 May 53

Primary sclerosing cholangitis is a chronic, cholestatic disease affecting the biliary tree. Recent data suggest an autoimmune etiology. Clinical findings, roentgenographic characteristics, and compatible liver histology will help in establishing the diagnosis. There is no known treatment for cure, though relief of symptoms may be accomplished with certain drugs, such as antibiotics for cholangitis and cholestyramine for pruritus. Death usually ensues within five to seven years after diagnosis, as a consequence of liver failure, cholangitis, and cholangiocarcinoma.
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PMID:Primary sclerosing cholangitis. 388 8

Primary biliary cirrhosis (PBC) is a chronic nonsuppurative, destructive cholangitis, whose etiology is unknown. Morbidity arises early from pruritus and later from hypercholesterolemia with xanthoma formation. Therapy is supportive and directed at the complications of cholestasis. Plasmapheresis has been reported to benefit patients with hyperlipidemia and PBC; thus a pilot study of plasmapheresis utilizing the Haemonetics Model 30 with replacement by albumin and saline was conducted. Five patients (four female and one male) with a mean age of 43 (range 29-58) and a mean duration of illness of 9.5 years (range 6-21) with marked jaundice, xanthomas, xanthelasma, hepatomegaly, fatigability, anorexia, and pruritus, as well as mild nausea were studied. Peripheral neuropathy was present in two patients. Two patients had splenomegaly. Two patients had an associated Sjogren syndrome. All patients had high serum bilirubin, alkaline phosphatase, and cholesterol levels and mild elevations in aspartate amino transferase and alanine amino transferase activities. Immune complexes measured in four patients were present. Antimitochondrial antibody titers were significant in all patients. Patients underwent a mean of 63 plasmapheresis procedures over a mean of 112 weeks removing a mean of 94.7 liters of plasma. No serious toxicity was seen. All patients showed a reduction in pruritus, xanthomas, xanthelasmas, and serum cholesterol values. The two patients who had evidence of Sjogren syndrome noted subjective improvement. All patients who had fatigue, anorexia and nausea also noted moderate improvement. There was no change in hepatomegaly or splenomegaly in patients demonstrating such organomegaly. Liver function did not change significantly. Overall, four patients had improvement in their condition and one patient achieved stability.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The clinical effectiveness and safety of chronic plasmapheresis in patients with primary biliary cirrhosis. 403 Jul 9

A number of different chronic diseases affect the intrahepatic bile radicles or cholangioles. They include primary and secondary sclerosing cholangitis, primary biliary cirrhosis, chronic cholestatic drug jaundice, atresia, and carcinoma. Aetiological factors include infection, immunological changes, hormones, and congenital defects.Patients with chronic cholestasis have decreased bile salts in the intestinal contents and suffer from a bile salt deficiency syndrome. Failure to absorb dietary fat is managed by a low-fat diet and by medium-chain trigly-cerides which are absorbed in the absence of intestinal bile salts. Fat-soluble vitamin deficiencies are prevented by parenteral vitamins A, D, and K(1). Calcium absorption is defective, and improvement may follow intramuscular vitamin D, medium-chain triglycerides, a low-fat diet, and oral calcium supplements.In partial intestinal bile salt deficiency the anionic bile-salt-chelating resin cholestyramine controls pruritus though steatorrhoea increases. Pruritus associated with total lack of intestinal bile salts is managed by methyl-testosterone or norethandrolone, though the jaundice increases.
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PMID:Chronic cholangitides: aetiology, diagnosis, and treatment. 497 Oct 54

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

Three patients who had undergone orthrotopic liver transplantation for primary biliary cirrhosis and were being maintained on immunosuppressive therapy were investigated 31/2 to 41/2 years later because of the redevelopment of pruritus and mild jaundice. In one patient pigmentation was again evident, and all three had a rise in the titer of serum mitochondrial antibody after an initial fall. Liver histology showed features of primary biliary cirrhosis with non-suppurative destructive cholangitis, lymphoid aggregates, and increased deposition of copper-binding protein in the absence of cholestasis. None of these features was found in patients who had received grafts for other conditions and had lived for comparable periods, nor were they found in patients who had had rejection with bile-duct abnormalities. The overall findings indicate a recurrence of primary biliary cirrhosis in the donor organ.
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PMID:Recurrence of primary biliary cirrhosis after liver transplantation. 703 71

Twenty-seven patients underwent percutaneous antegrade biliary drainage for obstructive jaundice. The serum bilirubin levels was elevated in all patients. Generalized pruritus was a major complaint. Twenty of the patients had had a laparotomy for malignant disease. Of the 24 patients in whom this method of drainage was successful, the obstructing lesion was found at the porta hepatis in 12 and in the extrahepatic bile ducts in 12. Metastatic disease was the commonest cause of obstruction. Following drainage the serum bilirubin level fell from a mean of 21.4 mg/dl (366 mumol/l) to a mean of 4.1 mg/dl (70.1 mumol/l) within a week. Pruritus was relieved. The major complications were transient cholangitis in five patients and inadvertent dislodgement of the catheter in four. In three of these patients another catheter was reinserted with ease. There was no peritonitis or uncontrolled bleeding. Twenty-one patients were able to leave hospital. Their mean survival time was 7.3 months. A multiperforated catheter manipulated through the obstruction has the advantage of permitting bile flow into the duodenum (antegrade) in contrast to external drainage (retrograde) by T- or U-tubes. Although the mean survival time with this method is similar to that with insertion of drainage tubes at the time of laparotomy, morbidity and mortality are reduced; this is important in view of the poor prognosis of bile duct obstruction due to malignant disease.
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PMID:Percutaneous antegrade biliary drainage: a nonoperative approach to biliary obstruction. 732 20


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