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)

Clinically apparent pancreatic disease due to sarcoidosis has not been described in typical sarcoid patients. A few cases have been described at exploratory laparotomy and pancreatic involvement in autopsied cases is more common than generally appreciated. We report a patient not previously known to have sarcoidosis whose presentation with pruritus, eosinophilia and cholestatic serum chemistry suggested a reaction to phenothiazines. The correct diagnosis was made during surgery for an asymptomatic gallstone. No other evidence of pancreatic disease has become apparent during follow-ups of two years.
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PMID:Granuloma (sarcoid?) of the pancreas. A case report. 66 51

The development of the syndrome of chronic intrahepatic cholestasis in five young, black men who had systemic granulomatous disease and clinical features consistent with those of sarcoidosis is described. Clinical and biochemical aspects, similar to those of primary biliary cirrhosis, included pruritus, jaundice, hepatomegaly and striking elevations of serum levels of alkaline phosphatase and cholesterol. (One patient had skin xanthomas.) Mitochondrial antibodies were not found; and survival of the patients (7 to 18 years) exceeded the usual survival of patients with primary biliary cirrhosis. The histologic abnormalities included noncaseating granulomas, chronic intrahepatic cholestasis, increased copper in hepatocytes, progressive diminution in number of interiobular bile ducts, periportal fibrosis and the eventual development of a micronodular "biliary" cirrhosis. The histologic evolution of the disease suggests a slow, progressive destruction of the bile ducts by granulomas. Although the end stage of this syndrome resembles primary biliary cirrhosis, the characteristic nonsuppurative, destructive cholangitis of primary biliary cirrhosis was not present.
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PMID:Chronic intrahepatic cholestasis of sarcoidosis. 116 46

The 2-oxo-acid dehydrogenase family of enzymes have been identified as the major mitochondrial autoantigens of primary biliary cirrhosis. Using immunoblotting, enzyme-linked immunosorbent assay and enzyme inhibition with both purified mitochondrial proteins and recombinant autoantigens, we have studied family members and spouses of patients with primary biliary cirrhosis for the presence of antimitochondrial antibodies. Antimitochondrial antibodies and other common autoantigens were also tested for by indirect immunofluorescence. This study included 27 index patients with primary biliary cirrhosis, 15 spouses and 48 first- and second-degree relatives. Overall, 7 relatives (11%) were positive for autoantibodies to nuclear and cytoplasmic antigens by indirect immunofluorescence against mouse liver and stomach sections. However, with immunofluorescence, the reactivity strictly paralleled that of antimitochondrial antibodies in only one of these (1:640)--a sibling with mild pruritus and a liver biopsy specimen diagnostic of primary biliary cirrhosis despite normal levels of serum alkaline phosphatase. In addition, one of the mothers, who had a history of sarcoidosis, was positive by immunoblotting for antibodies to the E2 subunit of the pyruvate dehydrogenase complex and protein X. All other relatives were negative for all of the assays. Antibodies to neither the 2-oxo-acid dehydrogenase enzymes nor the recently proposed family of naturally occurring mitochondrial antibodies were found in spouses or healthy relatives. Three other first-degree relatives suffered from liver disease: two died (one from primary biliary cirrhosis and the other from an unknown type of liver disease) and one (a sibling with primary biliary cirrhosis) was unavailable for testing. Our results are consistent with a familial predisposition to primary biliary cirrhosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Antimitochondrial antibodies in kindreds of patients with primary biliary cirrhosis: antimitochondrial antibodies are unique to clinical disease and are absent in asymptomatic family members. 139 96

A 62-year-old woman presented with uveitis and abnormal chest X-ray (bilateral hilar adenopathy). Skin biopsy in 1983 had revealed non-caseating epithelioid cell granuloma consistent with sarcoidosis. Her serum biochemical investigations and exploratory laparoscopy suggested nodular liver cirrhosis, but biopsy was not performed. Both blood urea nitrogen (BUN) and serum creatinine values were within normal limits. She received prednisolone therapy of 15 mg daily initially, and later a maintenance dose of 5 mg daily. In 1985, she complained of skin itching and her laboratory data revealed severe renal insufficiency (BUN 97 mg/dl, serum creatinine 12.2 mg/dl) and hypercalcemia (corrected serum calcium level: 11.5 mg/dl). Prednisolone treatment (40 mg daily) resulted in a dramatic improvement of renal function as well as other clinical abnormalities due to sarcoidosis, without any significant changes in liver function. She died of cerebral infarction in 1989. Autopsy showed interstitial nephritis with tubular calcinosis and hyalinized glomeruli. It is postulated that hypercalcemia due to sarcoidosis contributed to the renal failure in this patient. This case suggests that renal damage due to sarcoidosis may be reversible with appropriate corticosteroid therapy.
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PMID:[An autopsy case of sarcoidosis associated with renal failure]. 140 82

Initial symptoms in a hitherto healthy 23-year-old man were jaundice (bilirubin 21.7 mg/dl) and pruritus, but extensive radiological, endoscopical, microbiological and laboratory investigations failed to reveal the cause. Stool culture positive for Salmonella agona suggested intrahepatic cholestasis resulting from a Salmonella cholangitis. However, antibiotic treatment was not successful. As he was in generally good health the patient declined further investigations. He returned two years later because of fatigue, lack of appetite and weight loss. Further tests now revealed lymphogranulomatosis in stage IVb of the nodular sclerosing type. The case demonstrates that cholestasis as an isolated early symptom of Hodgkin's disease can precede by years any further signs of the disease.
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PMID:[Icteric cholestasis as an early symptom in Hodgkin's disease]. 169 16

Twenty-five patients with primary biliary cirrhosis (PBC) in different stages were investigated with respect to pulmonary function abnormalities. The results were compared with a reference sample of 17 sex- and age-matched healthy subjects. A high prevalence of lung function impairment was found in the PBC patients (14/25 [56%]). Bronchial asthma was present in three patients, and severe lung emphysema in one. These four patients had an abnormal lung function, mainly of obstructive type. There was a statistically significant difference between the remaining 21 PBC patients without chronic obstructive lung disease and the reference subjects with respect to diffusion capacity. Almost all abnormal lung function data were found in the symptomatic PBC patients (i.e. symptoms of pruritus, xanthoma, xanthelasmata, jaundice, hyperpigmentation, hepatosplenomegaly), 13 out of 18 (72%), whereas only one out of seven asymptomatic patients was affected. Nine patients (36%) had reduced diffusion capacity compared with none of the reference subjects. The lung function abnormalities in PBC patients are similar to those found in sarcoidosis, another granulomatous disease.
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PMID:Lung function abnormalities in patients with primary biliary cirrhosis. 338 7

Sarcoidosis carries a low prevalence in Israel, and acute pleural involvement in sarcoidosis is uncommon throughout the world. We report a case of a young Israeli male of Yemenite origin who presented with atypical manifestations of sarcoidosis: pleuritic pain, hemoptysis, pruritus and alcohol-induced pain. The differential diagnosis from Hodgkin's disease was involved. Various aspects of diagnosis are discussed.
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PMID:Sarcoidosis presenting with acute pleurisy, hemoptysis, pruritus and eosinophilia. 365 75

A 73 year old woman presented with redness, itching and painless proptosis of the left eye. Clinical examination and computed tomographic (CT) scan of the orbit revealed a postero-ocular mass, the biopsy of which showed a noncaseating, giant cellular, epithelioid granuloma compatible with sarcoidosis. The patient had no respiratory complaints, but the thoracic CT scan showed multiple bilateral hilar and mediastinal adenopathies. Gallium scan showed increased uptake in both hilar regions, in the left orbit and in the right parotid gland. Multiple bronchial biopsies revealed sarcoid granulomas, with a bronchoalveolar lavage (BAL) cytological pattern of active sarcoidosis. The evolution was favourable with prednisolone given at a dose of 0.5 mg.kg-1 daily. It is suggested that in an unusual presentation of sarcoidosis, a thorough search should be made for localization at other sites, lungs in particular, even in the absence of respiratory complaints.
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PMID:Systemic sarcoidosis initially presenting as an orbital tumour. 778 99

Twenty-six persons from five families comprising 34 members residing in different areas of Saptari district of the eastern region of Nepal developed symptoms of epidemic dropsy over 6-8 weeks. Seventeen patients were studied during July-August 1996. The age of affected individuals varied from 3 to 75 years. Members who had not consumed food cooked in mustard oil or who were not residing with the family were spared. Mustard oil, which was used for cooking, was found to be contaminated with oil of Argemone mexicana seeds. Sanguinarine was detected in all mustard oil samples collected from the homes of affected families. Gastrointestinal symptoms were present in 82 per cent of cases a week or so prior to the onset of pedal oedema. Pitting oedema of the lower limbs, fever, and darkening of the skin were the most consistent features, found in all cases. Other prominent features such as local erythema (82 per cent) and tenderness (88 per cent) of the lower limbs were present in most cases. Two striking features not previously noted were perianal itching (100 per cent) and severe carditis (35 per cent) with congestive cardiac failure (29 per cent). Other unique features noted were 'sarcoid' skin changes (18 per cent), bilateral pleural effusion, and Roth's spots and subhyloid haemorrhages in the fundus in one patient. Other important findings were anaemia (88 per cent), hepatomegaly (41 per cent), pneumonia (35 per cent) and ascites (12 per cent). There were no deaths due to epidemic dropsy. In the majority of cases, oedema, cutaneous changes, and carditis showed a marked improvement in 2-3 weeks and patients were well after 6-8 weeks of follow-up.
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PMID:Epidemic dropsy in the eastern region of Nepal. 1019 86

Intrahepatic cholestasis is characterized by a decrease in bile flow in the absence of overt bile duct obstruction, resulting in the accumulation of bile constituents in the liver and blood. Various etiological factors have been incriminated including drugs, total parenteral nutrition, sepsis, pregnancy, graft-versus-host disease and systemic disorders such as sarcoidosis, amyloidosis and Hodgkin's disease. The pathogenesis of cholestasis is unclear and several mechanisms have been hypothesized, without convincing evidence that any of these play a role in clinical cholestasis. Despite the uncertainty about the pathophysiology of intrahepatic cholestasis, several forms of therapy have been employed. Ursodeoxycholic acid may relieve pruritus and lethargy, and in some cases may modify disease progression. If cholestasis persists, supportive therapy is important to maintain optimal physical and nutritional well-being. In patients with advanced liver disease associated with hepatocellular failure, liver transplantation is the only viable option.
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PMID:Intrahepatic cholestatic syndromes: pathogenesis, clinical features and management. 1043 57


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