Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four patients with acquired immunodeficiency syndrome (AIDS) (CDC group IV) were investigated for biliary disease because of the presence of both severe upper abdominal pain and raised levels of serum alkaline phosphatase. None was clinically jaundiced. Upper abdominal ultrasound was abnormal in three. All had endoscopic retrograde cholangiographic evidence of both an intrahepatic sclerosing cholangitis suggestive of primary sclerosing cholangitis and an irregular suprapapillary common bile duct dilation suggestive of papillary stenosis. Three had evidence of gastrointestinal cryptosporidiosis and two of disseminated cytomegalovirus infection. Endoscopic sphincterotomy, performed in two patients, gave good pain relief. We propose the name 'AIDS sclerosing cholangitis' for this form of secondary cholangitis. The cause of this disorder remains unclear. Recent evidence is discussed which suggests that it is not due to HIV itself but to an opportunistic infection. Cryptosporidium appears to be the most likely candidate.
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PMID:Sclerosing cholangitis in acquired immunodeficiency syndrome. Case reports and review of the literature. 307 60

We report 12 cases of primary sclerosing cholangitis (confirmed by cholangiography) in which the serum alkaline phosphatase activity was normal. The enzyme activity remained normal during follow-up in 7 cases and fluctuated in 5 cases (it returned to normal in 4). The presence of advanced histologic stage (fibrosis/cirrhosis) with marked cholangiographic changes in 4 patients establishes that cirrhotic-stage primary sclerosing cholangitis can occur without a concomitant increase in serum alkaline phosphatase activity. Therefore, primary sclerosing cholangitis may exist in an occult state without symptoms or increase in serum alkaline phosphatase activity. Our findings suggest that primary sclerosing cholangitis may be more prevalent than realized, especially in patients who have inflammatory bowel disease. A normal value for serum alkaline phosphatase activity should not preclude further investigation for primary sclerosing cholangitis in patients with inflammatory bowel disease when symptoms or signs suggest liver disease.
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PMID:Primary sclerosing cholangitis with normal serum alkaline phosphatase activity. 316 3

The diagnosis of sclerosing cholangitis is usually not considered in the absence of an elevation of serum alkaline phosphatase. The purpose of this paper is to report two symptomatic cases of primary sclerosing cholangitis (PSC) which presented with a normal alkaline phosphatase, and to review the literature regarding alkaline phosphatase in PSC. A total of 172 patients with PSC were identified in the literature; six of these patients (3%) presented with a normal alkaline phosphatase. Patients with diseases known to be associated with primary sclerosing cholangitis and having a compatible clinical presentation should be considered for evaluation with endoscopic retrograde cholangiography, even in the presence of a normal level of serum alkaline phosphatase.
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PMID:Symptomatic sclerosing cholangitis in patients with a normal alkaline phosphatase: two case reports and a review of the literature. 327 97

Serum levels of secretory IgA (SIgA) and secretory IgM (SIgM) were quantified by an enzyme-linked immunosorbent assay in 97 patients with various chronic liver diseases and 17 patients with uncomplicated ulcerative colitis. The values obtained were compared with 89 matched controls and related to other serum variables. All types of liver disease had elevated median levels of serum SIg. Patients with primary biliary cirrhosis (PBC) had the highest SIg levels, particularly SIgM, but increased total serum IgM was slightly more specific for PBC. Thus, the SIg levels did not add more discriminative information than several other variables. Elevated levels of circulating SIgA correlated mainly with variables that indicate reduced liver function. The difference observed between patients with PBC and primary sclerosing cholangitis (PSC) in the alkaline phosphatase (ALP)-to-SIg ratio is discussed.
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PMID:Circulating secretory immunoglobulins of the A and M isotypes in chronic liver disease. 358 32

Primary sclerosing cholangitis (PSC) in eight children, five males and three females between the ages of 4 and 13 years, presented with minimal clinical symptoms and few signs of liver disease. Diagnosis was made by a highly characteristic histology in all and additional endoscopic retrograde cholangiography (ERC) in four patients. Six children suffered from concomitant inflammatory bowel disease (IBD). Laboratory abnormalities consisted of mild elevation of transaminases and alkaline phosphatase, with marked elevation of immunoglobulin G in seven and detection of anti-nuclear antibodies in four of the eight children. In all cases, needle liver biopsy specimens revealed portal tracts considerably expanded by edema and chronic inflammation diagnostic of nonsuppurative cholangitis and nonsuppurative fibrosing pericholangitis. ERC showed decreased arborization in the whole biliary tree. In two patients, changes were confined to the intrahepatic portion of the biliary tract. No specific drug therapy was given to our patients. Those with concomitant IBD were treated with salazosulfapyridine (SASP). The clinical course has been mild in all patients up to now. It is concluded that PSC may be much more frequent in childhood than was considered before, especially in children with IBD.
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PMID:Primary sclerosing cholangitis in children. 369 43

A 15-yr-old girl presented with complaints of right upper quadrant pain and jaundice. Elevation of serum alkaline phosphatase, signs of protal hypertension, and computed tomographic scan findings suggested a diagnosis of primary sclerosing cholangitis. However, cultures of the bile and of the common bile duct specimen obtained during a surgical procedure grew Cryptococcus neoformans. Treatment with amphotericin B was begun. An episode of upper gastrointestinal bleeding, however, led to the hepatorenal syndrome, and the patient died before antifungal therapy was completed. At autopsy, active sclerosing cholangitis associated with cryptococci involved the common bile duct. We suggest that opportunistic infection of the biliary tree should be considered in pediatric patients with presumed primary sclerosing cholangitis.
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PMID:Cholangitis associated with Cryptococcus neoformans. 397 25

Primary sclerosing cholangitis is a progressive, ultimately fatal, chronic hepatobiliary disorder for which no effective medical or surgical therapy now exists. The syndrome occurs most commonly in young men and is characterized by a chronic cholestatic syndrome, frequent association with chronic ulcerative colitis, hepatic copper overload, a paucity of serologic markers, and characteristic abnormalities in some liver-biopsy specimens and most cholangiograms. The natural history of the syndrome is unclear; the disease is likely to progress slowly and relentlessly, over a decade or longer, from an asymptomatic stage to a condition characterized by symptoms of cholestasis and complicated by cirrhosis, portal hypertension, and possibly carcinoma of the bile ducts. Screening techniques based on automated biochemical analyses are likely to lead to a diagnosis of primary sclerosing cholangitis in increasing numbers of patients, perhaps in the early, preicteric stage. An increased level of serum alkaline phosphatase in a young man, particularly if he has chronic ulcerative colitis, should strongly suggest the presence of this syndrome and the need for additional diagnostic studies. Endoscopic retrograde cholangiography and liver biopsy should be considered under these circumstances.
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PMID:Current concepts. Primary sclerosing cholangitis. 636 57

Patients with inflammatory bowel disease and serum alkaline phosphatase persistently raised to more than twice the normal level were investigated to assess the frequency of primary sclerosing cholangitis (PSC) in the Gastro-intestinal Clinic from 1975 to 1981. Twelve patients had a persistently raised alkaline phosphatase level of hepatic origin, 9 out of 250 with ulcerative colitis and 3 out of 164 with Crohn's disease. PSC was demonstrated in 8(3%) of the patients with ulcerative colitis, and carcinoma of the pancreas in the remaining 1. Three of the patients with PSC also had gallstones. The colitis antedated the biliary symptoms and signs in all but 1 patient. There was no correlation between the duration, extent and activity of the colitis and the development and outcome of the liver involvement. Investigations in the 3 patients with Crohn's disease revealed the presence of PSC in 2 (1,2%) and chronic active hepatitis in the 3rd. Of the 2 with PSC, one had cholelithiasis and has had recurrent episodes of cholangitis. The other has had only mild symptoms.
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PMID:Primary sclerosing cholangitis associated with inflammatory bowel disease in Cape Town, 1975 - 1981. 682 39

Recent investigations have clarified the clinical, radiologic, and pathologic features of primary sclerosing cholangitis, a chronic fibrosing disorder affecting the biliary tree. This progressive, cholestatic biliary disease is diagnosed when an elevated serum alkaline phosphatase level and stricturing and beading of bile ducts on cholangiography are found in a patient with jaundice, bouts of cholangitis, and, frequently, ulcerative colitis. The disease results in a spectrum of hepatic changes characterized by periductal and portal fibrosis, bile ductular proliferation, diminished numbers of normal bile ducts, piecemeal necrosis, and copper accumulation. The clinicopathologic features of primary sclerosing cholangitis, hypotheses on its pathogenesis, and possible forms of therapy are examined.
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PMID:Primary sclerosing cholangitis. 709 26

Three patients, two males and one female, with asymptomatic primary sclerosing cholangitis (PSC) are described. The diagnosis was made in each case by endoscopic retrograde cholangiography after investigation of persistent elevation of the serum alkaline phosphatase. All three have remained completely well without any medical or surgical treatment for 3, 7, and 15 years, respectively, despite extensive involvement of the biliary tree. Follow-up liver biopsies in two have shown no histological evidence of progression to secondary biliary cirrhosis. PSC may occur more frequently and may follow a less severe clinical course than previously recognized.
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PMID:Long-standing asymptomatic primary sclerosing cholangitis: report of three cases. 728 44


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