Unmeasurable serum alkaline phosphatase activity in wilson's disease associated with fulminant hepatic failure and hemolysis

Willson, Richard A.; Clayson, Kathleen J.; Leon, Samuel H.

doi:10.1002/hep.1840070347

Cited by 28 publications

(8 citation statements)

References 4 publications

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“…Certain families may also have increased levels of serum alkaline phosphatase that are genetic in origin [42]. Finally, extremely low levels of alkaline phosphatase can be present in patients with fulminant Wilson disease complicated by hemolysis [49,50].…”

Section: Alkaline Phosphatasementioning

confidence: 96%

Laboratory Tests

Herlong

Mitchell

2011

Schiff's Diseases of the Liver

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Key conceptsr Biochemical tests can be used to detect acute injury to the liver and chronic diseases of the liver before development of symptoms.r Elevations in serum levels of aminotransferases (aspartate aminotransferase and alanine aminotransferase) reflect hepatocellular injury and/or permeability of the liver cell membrane, but do not correlate with the extent of fibrosis or measures of metabolic functions of the liver.r An elevation of serum alkaline phosphatase occurs in response to intrahepatic cholestasis, extrahepatic obstruction of bile ducts, or infiltration of the liver with granulomatous inflammation, amyloidosis, or venous congestion.r The rate-limiting step in metabolism and disposition of bilirubin is the transport of conjugated bilirubin from the hepatocyte into the bile.An elevation in the conjugated (direct-reacting) fraction of bilirubin is evidence of intrahepatic cholestasis or extrahepatic obstruction of the bile ducts.r Serum levels of albumin may be low in patients with acute or chronic liver disease, but very often drop in response to the "acute phase response," making it difficult to evaluate hepatic metabolic function in clinical situations such as infection.r Although single laboratory tests are generally not useful in evaluating the severity of acute or chronic liver diseases, the use of mathematical equations combining tests may be effective as surrogate markers for the extent of fibrosis, steatosis, and inflammation as well as to predict the short-term prognosis in patients with liver disease.The use of laboratory tests is an important component of the assessment of patients with liver disease. With increasing frequency, elevations in the hepatic enzymes detected on routine screening tests provide the first evidence of liver disease in otherwise asymptomatic patients. In those patients with previously recognized hepatic dysfunction, laboratory tests can help establish the etiology of liver disease and provide valuable prognostic information. Rarely does a single test provide sufficient information to establish a diagnosis or assess severity of liver disease. A combination of tests such as serum bilirubin, albumin, aminotransferases, and alkaline phosphatase is sometimes referred to "liver function tests" or a "liver panel." These tests, in combination with the prothrombin time can provide an initial characterization of the etiology and severity of liver disease. However, one should recognize that the aminotransferases and alkaline phosphatase are tests that reflect hepatic injury not function. Traditionally liver diseases have been characterized as primarily hepatocellular or cholestatic based on the predominance of elevated aminotransferases or alkaline phosphatase. While an individual laboratory test may not provide a specific diagnosis, the pattern of abnormalities can suggest a general category of hepatic dysfunction. Although this distinction helps direct the initial evaluation there is often considerable overlap so that aggregating the diseases is less useful, as in the case...

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Section: Alkaline Phosphatasementioning

confidence: 96%

Laboratory Tests

Herlong

Mitchell

2011

Schiff's Diseases of the Liver

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“…Recently simple biochemical indexes, derived by dividing the serum alkaline phosphatase (or AST/ALT) level by the total serum bilirubin concentration, have been proposed as a method of differentiating FWD from other causes of FLF (8). This process is based on an intriguing initial observation of low serum alkaline phosphatase activity in patients with FWD (11,12). We investigated the diagnostic specificity of these indexes and the role of diagnostic copper studies in a large cohort of patients with FLF.…”

mentioning

confidence: 99%

Failure of simple biochemical indexes to reliably differentiate fulminant wilson's disease from other causes of fulminant liver failure

Sallie¹,

Katsiyiannakis²,

Baldwin³

et al. 1992

Hepatology

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Serum, urine and tissue biochemical findings were studied in 21 cases of fulminant Wilson's disease with respect to the value of a recently described biochemical index based on serum alkaline phosphatase and total serum bilirubin levels, and these cases were compared with 193 other cases of fulminant liver failure. Serum bilirubin, alkaline phosphatase and AST levels found in fulminant Wilson's disease were significantly different from those found in other cases of fulminant liver failure, but differentiation from other causes of fulminant liver failure on the basis of these biochemical parameters was not possible. The alkaline phosphatase/bilirubin and aspartate AST/bilirubin ratios derived from the above parameters were also significantly lower in fulminant Wilson's disease than in other categories of fulminant liver failure, but distinction between diagnostic categories on this basis was not possible. When ratios that correctly identified all cases of fulminant Wilson's disease were selected, 59/190 (31%) and 84/190 (44%) cases of non-Wilsonian fulminant liver failure would erroneously be assigned a diagnosis of fulminant Wilson's disease, by alkaline phosphatase/bilirubin and AST/bilirubin ratios, respectively. A low alkaline phosphatase-to-bilirubin ratio (< 0.57) in any category of fulminant liver failure suggested a significantly worse prognosis than in cases with higher ratios (chi 2, Yates' corrected = 5.37, p = 0.02). In the Wilson's disease group, serum and hepatic copper and ceruloplasmin concentrations were normal in 4/21, 2/15 and 2/19, respectively, whereas urinary copper level was elevated in 18/18 and was the most valuable test in diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

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“…According to the reported method [54], the binding constant was calculated to be 1.263 × 10 5 M −1 . To further confirm the formation of the complexes, we directly synthesized CuCl 2 L complex by heating and stirring the dichloromethane/methanol solution of L with CuCl 2 (1:1.2) at 50 °C for 3 h [55]. Transmission electron microscopy (TEM) images revealed the nanorod structure of CuCl 2 L with an average diameter of 65.3 ± 3.0 nm (ESM Fig.…”

Section: Resultsmentioning

confidence: 99%

Smart probe for simultaneous detection of copper ion, pyrophosphate, and alkaline phosphatase in vitro and in clinical samples

2019

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Wilson’s disease (WD), which might lead to acute liver failure, is an inherited disorder characterized by accumulation of copper (Cu 2+ ) in the brain, the liver, and other vital organs. In the clinic, decreased serum alkaline phosphatase (ALP) concentration is used for WD diagnosis. But to the best of our knowledge, using a fluorescent probe to simultaneously detect multiple factors in WD (e.g., Cu 2+ , pyrophosphate (PPi), and ALP) has not been reported. Herein, we rationally designed a fluorescent switch (E)-8-((4-methylbenzylidene)amino)napthalen-1-amine ( L ) and successfully applied it for sequential and selective detections of Cu 2+ , PPi, and ALP in vitro, in living cells and synovial fluid samples with “Off,” “On,” and “Off” fluorescence signals, respectively. Considering the obvious correlations among Cu 2+ , PPi, and ALP in WD, we envision that our fluorescent probe L could be applied to in vitro diagnosing WD in the near future. Electronic supplementary material The online version of this article (10.1007/s00216-019-02027-2) contains supplementary material, which is available to authorized users.

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Unmeasurable serum alkaline phosphatase activity in wilson's disease associated with fulminant hepatic failure and hemolysis

Cited by 28 publications

References 4 publications

Laboratory Tests

Laboratory Tests

Failure of simple biochemical indexes to reliably differentiate fulminant wilson's disease from other causes of fulminant liver failure

Smart probe for simultaneous detection of copper ion, pyrophosphate, and alkaline phosphatase in vitro and in clinical samples

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