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)

The association of prurigo nodularis (PN) and macular amyloidosis (MA) has not been reported before. Although pruritus related frictional trauma is a well-known cause of PN, its role in the development of MA has always been questioned. We herein report two cases with chronic liver disease and iron deficiency who concomitantly developed MA and PN lesions. Pruritus was the preceding factor and both lesions were confined to scratched areas. The association of two otherwise uncommon dermatoses in pruritic patients and their characteristic distribution might indicate an important role for pruritus-induced scratching in the pathogenesis of MA, too.
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PMID:The combined occurrence of macular amyloidosis and prurigo nodularis. 1084 58

Most of the care of liver disease in alpha(1)-antitrypsin (alpha(1)-AT) deficiency involves supportive management for complications of chronic liver disease including gastrointestinal bleeding, ascites, edema, encephalopathy, coagulation disturbances, spontaneous bacterial peritonitis, and hepatorenal syndrome. Some of these patients will have manifestations of cholestatic injury, including pruritus, hypercholesterolemia, and steatorrhea with fat-soluble vitamin deficiencies. The major challenge for the clinician taking care of these patients is the timing of referral for liver transplantation therapy. Timing of such referral is a relatively straightforward decision in alpha(1)-AT-deficient patients with progressive liver dysfunction. Some patients have nonprogressive or slowly progressing liver disease even after the development of cirrhosis or portal hypertension. Timing of liver transplantation in these patients should not be based simply on the presence of cirrhosis, portal hypertension or mild liver synthetic dysfunction, but rather on the basis of a subjective judgment by the hepatologist, patient, and family that manifestations of liver disease are interfering with overall life functioning.
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PMID:Alpha(1)-Antitrypsin Deficiency. 1109 5

Drug-induced liver disease can result from dosage-dependent hepatotoxicity or from adverse reactions to drugs used in therapeutic dosage. The latter idiosyncratic hepatotoxins can cause clinical syndromes that mimic all known liver diseases, so that drugs must be considered as the possible causal agent for all unexplained cases of liver disease. The only specific antidote for dosage-dependent hepatotoxicity is n-acetylcysteine (and some other sulfhydryl donors), which is highly effective for the prevention of significant hepatotoxicity after acetaminophen overdose. Early diagnosis and prompt withdrawal of the offending drug is the key to successful management of most drug-induced liver diseases. The mainstay of treatment is supportive care, with careful monitoring for signs of acute liver failure or progression to chronic liver disease. In cases of liver failure, close liaison with a liver transplant center is crucial; referral for liver transplantation should be considered if standard transplant criteria are fulfilled. Pruritus is a major symptom of drug-induced cholestasis; protracted cases may respond to ursodeoxycholic acid. Corticosteroids can be considered for cases of drug-induced hepatitis, especially those with evidence of immune hypersensitivity, if no improvement is seen in 8 to 12 weeks. Although there are no controlled trials, some patients may respond favorably.
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PMID:Drug-Induced Liver Disease. 1109 6

Alagille syndrome is characterized by a paucity of bile ducts in the liver. The syndrome is associated with some or all the features of chronic cholestasis, cardiac disease, skeletal abnormalities, ocular defects and a distinctive facial appearance. The most common finding is chronic cholestasis, which causes intractable pruritus, xanthoma, deficiency of certain metabolic nutrients and growth retardation. Cardiac abnormalities are the most common cause of death in these patients. It is unusual to see the clinical picture of hepatic failure resulting in cirrhosis and requiring transplantation, but liver transplantation is indicated in Alagille syndrome patients who have chronic cholestasis. If the disease is diagnosed in childhood, transplantation can improve significantly the patient's prognosis and the quality of life. In recent years, auxiliary liver transplantation has gained popularity for treating both acute and chronic liver disease. Heterotopic segmental liver transplantation is an alternative treatment modality for patients who do not require native liver removal. Individuals with Alagille syndrome are good candidates for this type of treatment. J Pediatr Surg 36:667-671.
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PMID:Successful heterotopic segmental liver transplantation from a live donor to a patient with Alagille syndrome. 1128 5

Altered central neurotransmission contributes to behavioural complications of chronic liver disease, such as pruritus and hepatic encephalopathy. Another behavioural complication of chronic liver disease, including chronic hepatitis C, is profound fatigue. Evidence that altered serotoninergic neurotransmission contributes to fatigue of central origin, and relief of profound fatigue in a patient with chronic hepatitis C associated with long-term ondansetron therapy, support the hypothesis that altered central serotoninergic neurotransmission contributes to fatigue complicating chronic hepatitis C. Drugs that specifically modulate serotoninergic neurotransmission may be effective in ameliorating fatigue in patients with chronic hepatitis C.
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PMID:Altered central serotoninergic neurotransmission: a potential mechanism for profound fatigue complicating chronic hepatitis C. 1146 Nov 59

Pruritus is often the most troublesome symptom in patients with chronic liver disease, particularly when cholestasis is a prominent feature. The exact pathogenesis is unknown, but empirical treatment, such as cholestyramine, based on a liver-based origin of pruritus, has been used for many years. Recently, evidence for a central mechanism for pruritus has been obtained and opioid antagonists have been tried clinically with some benefit, but their use is not widespread. In addition, the pruritus associated with intrahepatic cholestasis of pregnancy can now be alleviated in many cases by ursodeoxycholic acid. As it also improves foetal outcome, this should become first-line therapy. We review the pathogenesis and therapy of pruritus, highlighting practical aspects to help with patients with seemingly intractable pruritus.
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PMID:Review article: pruritus in cholestatic and other liver diseases. 1265 88

Albumin dialysis has been shown to improve the outcome in patients with cholestatic liver failure caused by chronic liver disease. This study reports 7 liver transplant recipients who were treated with albumin dialysis for intractable pruritus of different origin (ductopenic graft rejection, non-anastomotic strictures, and recurrence of hepatitis C). Treatment with histamine (H1) blockers, opioid antagonists, and cholestyramine had not been effective. The Molecular Adsorbent Recirculating System (MARS; Teraklin, Rostock, Germany) was used for albumin dialysis. All patients presented with numerous scratch marks, 6 of whom had a pronounced icterus. Six patients (86%) responded to 3 consecutive treatments with significant reduction of pruritus. The mean pruritus score, which was quantified by a visual analog scale (VAS), decreased from 9.7 +/- 0.5 to 3.7 +/- 0.8 (SD). The mean duration of 1 treatment was 15.6 hours. The procedure was well tolerated by all patients. The mean total serum bilirubin in patients who responded to therapy declined from 19.11 +/- 16.96 mg/dL (SD) before MARS therapy to 9.24 +/- 3.52 mg/dL after treatment. The mean serum concentration of 3 alpha-hydroxy bile acids decreased from 192.67 +/- 58.12 micromol/L (SD) to 42.33 +/- 31.58 micromol/L (SD). Follow-up in 3 cases showed sustained improvement of pruritus lasting for more than 3 months. In 3 patients, however, pruritus relapsed. One patient, who showed severe pruritus, without relevant elevation of serum bile acids before treatment, did not respond to albumin dialysis. Our data indicate that MARS is an effective therapeutic option for patients with intractable cholestatic pruritus.
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PMID:Treatment of refractory cholestatic pruritus after liver transplantation with albumin dialysis. 1475 86

Patients with ICP should be considered to have a high-risk pregnancy. Once the diagnosis of ICP is suspected, usually because of generalized pruritus, it should be confirmed by liver function tests, and other causes of cholestasis should be ruled out. Treatment with UDCA is effective in ameliorating the cholestasis and is especially useful in severe forms or when there is a history of sudden fetal death in a previous pregnancy. The understanding of the pathogenesis of ICP has recently progressed as the result of the discovery of several defects in the MDR3 gene in isolated affected patients. More studies of this and other genes that regulate bile flow, linked with careful clinical observations to rule out unsuspected chronic liver disease not related to pregnancy, should lead to the discovery of the pathogenesis of this enigmatic disorder.
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PMID:Intrahepatic cholestasis of pregnancy. 1506 99

The Alagille syndrome is one of the most common inherited disorders causing chronic liver disease during childhood. During the 1990s, 38 children with Alagille syndrome were evaluated at two pediatric centers in Buenos Aires, Argentina. Characteristic clinical, humoral, and cutaneous features were analyzed. The average age of diagnosis was 29 months old (range of between 2 months and 15 years). Cholestasis was evident in 92% of patients during the neonatal period. Family antecedents related to the syndrome were found in 18.5% of the patients. Peculiar facies developed in 85% of patients. Chronic cholestasis and pruritus were observed in all of the patients and jaundice was evident in 78%. Eighty-four percent of the patients had heart disease (pulmonary stenosis, intraauricular communication, intraventricular communication), 76% of them showed growth retardation, and vertebrae abnormalities were found in 63%. Embryotoxon appeared in 76% of patients, and renal disturbances in 21%. Eleven children (28%) had xanthomas, in the neck, elbows, palms, helixes, inguinal area, gluteus, and knees. The earliest findings appeared in the first months of life, and the latest at 5 years of age. The xanthomas located in the folds had a stony aspect. Cholesterol levels ranging from 220 to 1600 mg percentage (mg%) were demonstrated in all of the children with xanthomas. Liver transplantation was performed in seven of the patients (18.4%). Two of them died after this operation. The disappearance of xanthomas after transplantation was remarkable in all of the patients.
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PMID:Alagille syndrome: cutaneous manifestations in 38 children. 1566 Aug 89

In this study, we present a case of mild PBC that had anti-p97/VCP. A 53-year-old woman had been suspected of having chronic liver disease since 1983. In 1998, she visited the clinic, complaining of struma and pruritus. Laboratory findings on the first visit showed elevated levels of alkaline phosphatase (ALP) 454 IU/l(110-360), gammaGTP 250IU/l(-45) and IgM 671mg/dl(35-220). A screening of anti-mitochondrial antibody test was positive at a 1:80 dilution. A liver biopsy specimen revealed PBC at Scheuer stage 1. Following a treatment of ursodeoxycolic acid (UDCA) 300mg/day for 6 months, AMA and IgM were reduced to 1:20 and 220mg/dl, respectively. However, she was found to have low titer of anti-p97/VCP antibodies, determined by immunoprecipitation of radiolabeled recombinant protein produced by in vitro translation and transcription of the full length p97 cDNA. She has continued to be clinically stable following administration of UDCA 300mg. A PBC patient with anti-p97/VCP antibody showed a milder clinical course, suggesting some beneficial role of this antibody.
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PMID:[Case of primary biliary cirrhosis patient with anti-p97/VCP antibodies presenting a mild clinical course]. 1572 86


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