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)

Alcohol-induced diseases of the liver, such as fatty liver, hepatitis and cirrhosis with the potential development of hepato-cellular carcinoma can cause many effects on the skin. Even though they are not caused by excessive alcohol alone, but also by other diseases of the liver or other diseases of internal organs, an experienced person will be able to carry out specific diagnostic procedures. Skin symptoms due to liver diseases include 1. Vascular changes, such as spider nevi, teleangiectasias and palmar erythema. 2. Nail changes, particularly white nails. 3. Changes of the mucous membranes, i.e. glossy tongue. 4. Changes due to altered hormones, particularly gyneco-mastia, female distribution of hair and testicular atrophy and 5. Changes in the color of the skin like icterus and melanosis cutis. Rarely pruritus and other diseases of the skin are seen, such as porphyria cutanea tarda, which is often caused by an altered liver function. In the final stages of alcoholism, the neglect of personal hygiene particularly of the skin is evident (cutis vagantium). Since the exact mechanism of the skin symptoms remains obscure, it is difficult to evaluate the significance. Most often they do not correlate with the severity of the liver disease.
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PMID:[Skin manifestations of alcoholic liver damage]. 1080 82

Progressive familial intrahepatic cholestasis (PFIC) is a congenital liver disease. First symptoms can frequently be seen shortly after birth. Quality and expectation of life are substantially reduced due to severe pruritus and the complications of progressive liver cirrhosis. PFIC is diagnosed on the basis of characteristic clinical and laboratory parameters and genetic analysis after exclusion of other liver diseases leading to intrahepatic cholestasis. Medical therapy is only effective in a proportion of children with PFIC. Partial biliary diversion (PBD) is nowadays considered the therapy of choice in patients with therapy-refractive pruritus. If performed in time, damage to the liver can be delayed or arrested, thus orthotopic liver transplantation (OLT) can be postponed or even avoided in at least some patients with PFIC. Besides providing a current overview of PFIC, we report on three patients who were successfully treated surgically. One patient was subjected to a new technique of PBD (cholecysto-appendicostomy), the other two had OLT.
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PMID:[Progressive familial intrahepatic cholestasis (Byler disease): current genetics and therapy]. 1081 55

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

Cholestasis of pregnancy is a liver disorder that occurs during the second half of pregnancy, causing pruritus and elevated serum bile acid levels. Its etiology remains unknown but probably involves vascular and humoral immune responses, mediated by bile acids. This disorder is associated with substantially increased fetal morbidity and mortality. The most satisfactory treatment consists in delivering the fetus as soon as pulmonary maturation has occurred.
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PMID:Fetal impact of cholestasis of pregnancy: experience at Tenon Hospital and literature review. 1086 78

Primary biliary cirrhosis is a cholestatic autoimmune disease of the liver. Its prevalence has significantly increased over the last seven years, therefore it can no longer be considered to be a rare liver disease. It is seen in 94/100,000 women older than 40 years. The treatment consists of administration of ursodeoxycholic acid, better therapy results seem to be achieved by combining UDCA with immunosuppressants. While UDCA-monotherapy proved to extend the time until liver transplantation and thus leads to life prolongation, this could not yet be shown for the combination therapies. Therapy of the often agonizing pruritus and of osteoporosis remains problematic. Frequently this can only be managed by the combination of several treatment measures.
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PMID:[Cholestatic hepatopathy: primary biliary cirrhosis]. 1090 60

Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterised by the destruction of small intrahepatic bile ducts. Although known for many years its etiology and pathogenesis still remains uncertain. Alterations of humoral and cellular immune functions suggests an autoimmune pathogenesis. Primary biliary cirrhosis typically affects middle-aged women who reports fatigue and itching. Diagnosis is usually based on abnormal biochemical tests of liver function, presence of antimitochodrial antibodies (especially anti-M2) and histologic evaluation of liver biopsy specimens. Relatively small number of diagnosed cases in Poland may be due to insufficient knowledge on this disease in our country.
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PMID:[Primary biliary cirrhosis]. 1096 8

Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are chronic progressive cholestatic diseases that frequently lead to biliary cirrhosis. The exact pathogenesis of these diseases remains elusive but is likely immunologically based. Complications range from fatigue and pruritus to end-stage liver disease. The risk of developing hepatocellular carcinoma is low for patients with PBC, whereas cholangiocarcinoma is common in PSC and carries an ominous prognosis. Although ursodeoxycholic acid is effective in slowing the progression of PBC, no effective medical therapy exists for PSC. Liver transplantation is the only option for patients with end-stage liver disease and yields excellent long-term survival in both groups.
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PMID:Biliary tract inflammatory disorders: primary sclerosing cholangitis and primary biliary cirrhosis. 1098 Sep 34

Use and efficacy of hyperbaric oxygen therapy (HOT) in liver disease has not been established. A prospective control study of HOT in liver diseases was undertaken. Sixty cases were selected for this study (30 with HOT and 30 with conventional therapy only). Almost equal number of Hepatitis B virus surface antigen (HbsAg) positive cases were included in both groups. All patients were male. Only cases with serum bilirubin over 10 mg/dl were included in this study. It was found that study cases recovered faster, gained appetite and had an earlier sense of well-being. There was faster disappearance of pruritus, earlier achievement of normal liver function, HbsAg negativity and overall shorter duration of hospital stay and convalescence. Short term adverse effects were unremarkable.
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PMID:Hyperbaric oxygen therapy in parenchymal liver disease. 1099 61

A 28-year-old man was admitted to our department for investigation in 1992. He presented with a red, scaly, centrifugally spreading eruption, which had appeared in 1990, beginning on the neck and thorax, and later extending to the trunk and limbs. The cutaneous lesions, located mainly on the trunk and proximal upper limbs, were arranged in rings, with a slightly raised prominent scaling edge (Fig. 1a). The characteristic feature was the presence of rings or waves within already existing rings, whereas the central part was flattened, with the texture of normal skin. The concentric figurate lesions resembled a wood grain pattern (Fig. 1b). The clinical picture was strikingly similar to tinea imbricata; there was, however, no itching, and repeated mycologic studies did not disclose Trichophyton concentricum. The histology was not characteristic. The epidermis, which was slightly edematous, was covered with a heavy crust. In the dermis, a sparse inflammatory infiltrate, somewhat more pronounced in the subpapillary areas, was composed of lymphocytes with some eosinophils. Periodic acid-Schiff (PAS) and other stains for mycotic infection were negative. The general condition was not affected and laboratory studies did not show any abnormalities, except for low serum protein (5.1 g/L) and decreased gamma globulins (10.5%). Cell-mediated immunity was preserved. Immunofluorescence studies (direct and indirect) were negative. In spite of repeatedly negative mycotic examinations and due to the striking similarity to tinea imbricata, we applied various antimycotic therapies (terbinafine, itraconazole), with no effect. The figurate pattern, with normal skin in between, altered from day to day, while new concentric rings appeared within the cleared skin. The migrating rate was about 2-3 cm per 2 weeks. The patient had undergone a thorough search for internal malignancy. During the follow-up period of 1992-98, cutaneous involvement slowly became almost generalized (1996), and the confluent lesions formed large plaques, but still with pronounced concentric rings. Transitional blood eosinophilia (27% in 1993 and 11% in 1996) regressed with no therapy. Since 1995, antibodies to HBs and HBc have been present with no clinical symptoms of liver disease. The blood proteins increased to 7.0 g/L, and gamma globulins to 17.2% (normal). The histology, studied repeatedly, started to display some signs of psoriasis from 1996 and, in 1998, was already consistent with the disease (Fig. 2). RE-PUVA (0.8 mg/kg acitretin and UVA 0.8 J/cm2 ) was applied for 2 weeks before the patient interrupted the therapy. In spite of this, there was further improvement and, in 1999, the patient was almost free of lesions with some abortive rings left. From time to time, single vesicles appeared within the elevated borders of the rings. The histology of such vesicles was consistent with abortive pustular psoriasis (Fig. 3).
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PMID:Erythema gyratum repens-like psoriasis. 1104 95

Clinical and epidemiological data from 232 patients with lichen planus is presented. Lichen planus constituted 0.38% of the total dermatology, outpatients diagnosed. The patient ages ranged from 8 to 76 years, most being in the age range from 20 to 49 years. Duration of disease varied from 1 month to 7 years. Both sexes were equally affected. The majority of the patients (47.4%) had classical lesions followed by hypertrophic and actinic lichen planus next in frequency. Itching was the predominant symptom in 79.3%. Limbs were the most frequent initial site of onset (55.6%). Mucosal involvement along with cutaneous lesions were observed in 16.8% and genital involvement in only 5.2%. Nail changes were observed in 15.1% of patients. A history of recurrence of the disease was obtained from 10.3% of patients. Liver disease was found to be associated in 2.2% of patients. No malignant changes were observed in any of the lesions of lichen planus.
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PMID:Lichen planus: a clinical and epidemiological study. 1105 33


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