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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Orthotopic liver transplantation for primary biliary cirrhosis and primary sclerosing cholangitis is a well-accepted therapy for complications of end-stage liver disease and is associated with an excellent outcome in the majority of cases. However, transplant centers are striving to improve on these outcomes by studying ways to optimize the timing of transplantation. Several natural history and prognostic models for both primary biliary cirrhosis and primary sclerosing cholangitis have been derived from the study of large populations of patients in an attempt to predict long-term rates of survival. In addition, models exist to predict resource utilization after liver transplantation. Other factors besides complications of end-stage liver disease may also be indications for transplantation, including refractory pruritus, recurrent bacterial cholangitis in patients with primary sclerosing cholangitis, hepatic osteodystrophy, and a poor quality of life.
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PMID:Liver transplantation for cholestatic liver disease: screening and assessment of risk factors. 974 88

Acquired perforating dermatosis (APD) is a very rare disorder which has been described in association with systemic diseases such as diabetes mellitus, HIV infection or lymphoma. In this report we describe a patient with APD associated with sclerosing cholangitis and diabetes mellitus who was successfully treated with rifampicin. A 33-year-old Indian woman with a history of extensive pancreatic surgery, sclerosing cholangitis and insulin dependent diabetes mellitus was referred to our unit with intractable pruritus. She was treated with cholestyramine, ursodeoxycholic acid, several analgesics, UVB therapy, topical steroids, sedative antihistamines and plasmapheresis without significant improvement. Increasingly severe itching was associated with papular skin changes limited initially to the lower limbs but which later involved her entire body. Biopsy of a representative lesion showed the changes of APD. She was subsequently treated with rifampicin which produced a dramatic resolution of pruritus within 3 weeks and the skin changes progressively resolved over subsequent months. In this newly described association of APD with sclerosing cholangitis, rifampicin treatment appeared to be efficient in ameliorating pruritus and the papular skin changes typical of APD.
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PMID:Successful treatment of acquired perforating dermatosis with rifampicin in an Asian patient with sclerosing cholangitis. 1022 Jul 47

We describe the case of a 18-year-old male patient who first presented with decompensated cirrhosis, fever and generalized lymphadenopathy. He had abnormal results for liver biochemical tests, with a hepatitic-like picture and high titre of antinuclear antibodies. According to the scoring system proposed by the International Autoimmune Hepatitis Group he had 'definite' autoimmune hepatitis and responded well to immunosuppressive treatment. One year later he developed pyoderma gangrenosum which was successfully treated with cyclosporine. Two years later he experienced bloody diarrhoea as a first presentation of ulcerative colitis. At that time both the cholestatic biochemical picture and the cholangiographic appearances of the biliary tree were consistent with primary sclerosing cholangitis. Despite the addition of azathioprine and ursodeoxycholic acid to his treatment regime he developed recurrent episodes of cholangitis and intractable pruritus for which he underwent successful liver transplantation.
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PMID:Overlapping syndrome of autoimmune hepatitis and primary sclerosing cholangitis associated with pyoderma gangrenosum and ulcerative colitis. 1065 5

Primary autoimmune liver diseases can be hepatitic or cholestatic in nature. Autoimmune hepatitis, more often diagnosed in women, is characterized by biochemical and histological activity, with polyclonal hypergammaglobulinemia as a frequent feature. Antinuclear and anti-smooth muscle antibodies are the serological hallmarks of type 1 autoimmune hepatitis, whereas liver-kidney microsomal antibody type 1 and liver cytosol antibody type 1 designate the type 2 form. Response to immunosuppression is usually excellent. The most frequent cholestatic autoimmune disease is primary biliary cirrhosis, characterized by anti-mitochondrial antibody positivity and typical bile duct lesions observed on liver biopsy. Treatment with biliary acids improves the biochemical picture, may alleviate pruritus, and delays the development of end-stage liver disease. Primary sclerosing cholangitis occurs more frequently in men and affects both the intra- and extrahepatic biliary trees, determining the typical "beading" appearance. Associated inflammatory bowel diseases are often observed. To date, no medical therapy is able to modify the course of this disease. Autoimmune cholangitis is an anti-mitochondrial antibody-negative cholestatic disease with most of the features of primary biliary cirrhosis. "Overlap" syndromes where autoimmune hepatitic and cholestatic features coexist in the same patient, have also been reported. Autoimmune phenomena secondary to hepatitis C virus-related liver disease such as the occurrence of antinuclear, anti-smooth muscle antibodies and liver-kidney microsomal antibody type 1 are often observed.
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PMID:[Primary and secondary autoimmunity in hepatology]. 1084 92

We describe a patient who suffered from intestinal perforation after abdominal trauma. Perioperatively, he was treated with a single dose of piperacillin and 9 doses of imipenem/cilastatin over 3 days. The patient was discharged 5 days after surgery in good clinical condition and with normal liver values except for a marginal elevation of alanine aminotransferase. Two weeks after discharge, he developed fatigue, fever and pruritus, necessitating rehospitalization. He was jaundiced and had elevated alkaline phosphatase and transaminases. After exclusion of an intra-abdominal fluid collection, a vascular problem, and infectious or autoimmune liver disease, a liver biopsy was performed. The biopsy revealed centrizonal bilirubinostasis, a portal infiltrate rich in eosinophils and cholangitis. Lymphocyte transformation tests for piperacillin and imipenem/cilastatin were positive, suggesting an immunological mechanism for the observed hepatopathy. Cholestasis gradually decreased but was detectable for several weeks. The patient had a full clinical and biochemical recovery after 3 months. We conclude that short-term therapy with piperacillin, imipenem/cilastatin or the combination of these drugs can lead to the same type of hepatopathy as described for amoxycillin/clavulanic acid or antistaphylococcal penicillins. Liver biopsy and positive lymphocyte transformation are compatible with an immunological mechanism.
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PMID:Cholangiopathy after short-term administration of piperacillin and imipenem/cilastatin. 1142 85

Majority of patients with advanced carcinoma gall bladder have irresectable disease and require palliation for jaundice, pruritus and cholangitis. These cases presenting with jaundice are usually high risk cases to undergo any major surgical procedure like segment-III hepaticodochojejunostomy. So intra-operative tube stenting was attempted in 6 patients of carcinoma gall bladder but technically, it was possible in 4 cases only. In 2 cases because of massive locoregional disease it was not possible to cannulate the proximal part of the bile duct. The distal end of the tube was passed either in the duodenum or proximal part of the jejunum. Patients were followed up, showed good recovery from jaundice and pruritus.
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PMID:Intra-operative tube stenting, palliation for jaundice in carcinoma gall bladder. 1201 44

There is an association between celiac disease (CD) and primary biliary cirrhosis, but there is little information regarding the association between CD and autoimmune cholangitis (antimitochondrial antibody-negative primary biliary cirrhosis). We describe a case of a 60-yr-old woman with chronic serum liver biochemistry elevations, recent onset of pruritus, and unexplained iron deficiency anemia. Liver biopsy was suggestive of stage 1 primary biliary cirrhosis, but serum antimitochondrial antibody testing was negative. Subsequent evaluation revealed CD based on markedly elevated antiendomysial antibody titers and characteristic histological features on mucosal biopsies. Initiation of a gluten-free diet led to resolution of iron deficiency anemia, pruritus, and elevated serum liver biochemistries. This suggests that CD may play a direct role in the development of autoimmune cholangitis. Additionally, normalization of hepatic biochemistries may be achieved without the use of immunosuppressive agents in some patients. CD should be considered in all patients diagnosed with autoimmune cholangitis as a gluten-free diet may avoid the need for immunosuppressive therapy in affected patients.
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PMID:Celiac disease-associated autoimmune cholangitis. 1249 11

Liver transplantation is an effective form of therapy for patients with end-stage cholestatic disease that improves both survival and quality of life. Liver transplantation is very effective for the treatment of intractable pruritus but less effective for the treatment of lethargy. Survival rates are good (more than 70% at 5 years); these patients are at greater risk of developing acute and chronic rejection and are more likely to require long-term immunosuppression. Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) recur in the graft. Recurrent PSC may be difficult to differentiate from secondary sclerosing cholangitis, but it recurs in up to 60% of patients at 5 years and may reduce graft survival. PBC recurrence, noted in up to 40% of patients at 10 years, has little effect on graft survival with respect to cancers. Patients with PSC are at greater risk of both colonic cancer (which may be reduced by ursodeoxycholic acid) and cholangiocarcinoma. Diagnosis of cholangiocarcinoma before transplantation usually contraindicates transplantation. The main challenges facing liver transplantation are the need to expand the donor pool and the need to find immunosuppressive regimens with fewer long-term toxicities.
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PMID:Liver Transplantation for Cholestatic Liver Disease. 1262 70

While hemorrhagic complications of portal cavernoma are frequent, compression of the bile ducts by portal cavernoma is uncommon and treatment is still a matter for debate. We report here six new cases in order to describe: (a) the clinical, biological, and morphological features of this condition, and (b) the long-term results of a combined endoscopic and surgical treatment. The median age of patients at the time of diagnosis was 36.5 years. The circumstances of diagnosis were acute cholangitis (n=3), asymptomatic biological cholestasis (n=1), pruritus, jaundice and asthenia (n=1) and jaundice alone (n=1). Portal cavernoma and bile duct dilatation were confirmed by abdominal ultrasonography with pulsed color doppler and endoscopic retrograde cholangiography (ERC). Gallstones were found in four patients. Following stenting of the bile duct, there was a good outcome in two patients. In four patients, after failure of prolonged endoscopic treatment, second-line surgical portal-systemic shunting allowed removal of the biliary stent, and no recurrence of disease. In conclusion, biliary involvement in portal cavernoma is now a well-recognized entity, and our results suggest that combined endoscopic and surgical treatment could be required.
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PMID:Diagnosis and treatment of biliary obstruction caused by portal cavernoma. 1270 Oct 19

Cryptosporidium parvum is a well-known cause of chronic diarrhea. In human immunodeficiency virus (HIV)-infected patients as well as in other immunocompromised patients it has also been shown to cause sclerosing cholangitis. We report a case of reversible C. parvum-induced sclerosing cholangitis in a renal transplant patient. This 40-year-old female received a renal transplant 9 years prior to presentation. She had no history of liver disease and was doing well on tacrolimus, prednisone, and azathioprine. She developed diarrhea and was found to have C. parvum present in the stool. Shortly after, she developed clinical, biochemical, radiologic, and histologic features of SC. After accidental reduction in her immunesuppression secondary to starting her on rifampin to treat her itching, she cleared C. parvum from her stool and had a marked improvement in her diarrhea, jaundice, and general health. Her liver enzymes normalized and magnetic resonance cholangiography showed complete resolution of biliary abnormalities. To our knowledge, this is the first case of C. parvum-induced sclerosing cholangitis in a renal transplant patient and one of a few in non-HIV patients. It is also the first to document resolution of sclerosing cholangitis after eradication of C. parvum in a non-HIV patient.
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PMID:Reversible sclerosing cholangitis secondary to cryptosporidiosis in a renal transplant patient. 1271 84


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