Failure of simple biochemical indexes to reliably differentiate fulminant wilson's disease from other causes of fulminant liver failure
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 ful...
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| Veröffentlicht in: | Hepatology (Baltimore, Md.) Md.), 1992-11, Vol.16 (5), p.1206-1211 |
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| creator | Sallie, Richard Katsiyiannakis, Leah Baldwin, Dianne Davies, Sue O'Grady, John Mowat, Alex Mieli‐Vergani, Giorgina Williams, Roger |
| description | 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 ( |
| doi_str_mv | 10.1002/hep.1840160517 |
| format | Article |
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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 (χ2, 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. Massively elevated hepatic copper stores with normal serum copper concentrations were seen in some patients; in others tissue copper concentration from different regions of the same liver varied up to 7‐fold. Treatment with D‐penicillamine in five patients had no observable beneficial effect. (HEPATOLOGY 1992;16:1206–1211.)</description><identifier>ISSN: 0270-9139</identifier><identifier>EISSN: 1527-3350</identifier><identifier>DOI: 10.1002/hep.1840160517</identifier><identifier>PMID: 1427659</identifier><identifier>CODEN: HPTLD9</identifier><language>eng</language><publisher>Philadelphia, PA: W.B. Saunders</publisher><subject>Adolescent ; Adult ; Alkaline Phosphatase - blood ; Aspartate Aminotransferases - blood ; Bilirubin - blood ; Biological and medical sciences ; Ceruloplasmin - metabolism ; Child ; Copper - blood ; Copper - metabolism ; Diagnosis, Differential ; Female ; Gastroenterology. Liver. Pancreas. Abdomen ; Hepatic Encephalopathy - blood ; Hepatic Encephalopathy - diagnosis ; Hepatolenticular Degeneration - blood ; Hepatolenticular Degeneration - diagnosis ; Humans ; Liver - metabolism ; Liver. Biliary tract. Portal circulation. Exocrine pancreas ; Male ; Medical sciences ; Other diseases. Semiology</subject><ispartof>Hepatology (Baltimore, Md.), 1992-11, Vol.16 (5), p.1206-1211</ispartof><rights>Copyright © 1992 American Association for the Study of Liver Diseases</rights><rights>1993 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4097-62e49c46d0b444814b011d347b9eb7c101fa2b4bf4b64115a593c19ac993d30b3</citedby><cites>FETCH-LOGICAL-c4097-62e49c46d0b444814b011d347b9eb7c101fa2b4bf4b64115a593c19ac993d30b3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fhep.1840160517$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fhep.1840160517$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=4431087$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/1427659$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sallie, Richard</creatorcontrib><creatorcontrib>Katsiyiannakis, Leah</creatorcontrib><creatorcontrib>Baldwin, Dianne</creatorcontrib><creatorcontrib>Davies, Sue</creatorcontrib><creatorcontrib>O'Grady, John</creatorcontrib><creatorcontrib>Mowat, Alex</creatorcontrib><creatorcontrib>Mieli‐Vergani, Giorgina</creatorcontrib><creatorcontrib>Williams, Roger</creatorcontrib><title>Failure of simple biochemical indexes to reliably differentiate fulminant wilson's disease from other causes of fulminant liver failure</title><title>Hepatology (Baltimore, Md.)</title><addtitle>Hepatology</addtitle><description>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 (χ2, 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. Massively elevated hepatic copper stores with normal serum copper concentrations were seen in some patients; in others tissue copper concentration from different regions of the same liver varied up to 7‐fold. Treatment with D‐penicillamine in five patients had no observable beneficial effect. (HEPATOLOGY 1992;16:1206–1211.)</description><subject>Adolescent</subject><subject>Adult</subject><subject>Alkaline Phosphatase - blood</subject><subject>Aspartate Aminotransferases - blood</subject><subject>Bilirubin - blood</subject><subject>Biological and medical sciences</subject><subject>Ceruloplasmin - metabolism</subject><subject>Child</subject><subject>Copper - blood</subject><subject>Copper - metabolism</subject><subject>Diagnosis, Differential</subject><subject>Female</subject><subject>Gastroenterology. Liver. Pancreas. Abdomen</subject><subject>Hepatic Encephalopathy - blood</subject><subject>Hepatic Encephalopathy - diagnosis</subject><subject>Hepatolenticular Degeneration - blood</subject><subject>Hepatolenticular Degeneration - diagnosis</subject><subject>Humans</subject><subject>Liver - metabolism</subject><subject>Liver. Biliary tract. Portal circulation. Exocrine pancreas</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Other diseases. Semiology</subject><issn>0270-9139</issn><issn>1527-3350</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1992</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkE1v1DAURS0EKtPClh2SF6hdZXgvdj68RFVLkSrRRVlHtvOsMXKSwU6A-QX927jKiGHHyot73r3WYewdwhYByo872m-xlYA1VNi8YBusyqYQooKXbANlA4VCoV6z85S-A4CSZXvGzlCWTV2pDXu61T4skfjkePLDPhA3frI7GrzVgfuxp9-U-DzxSMFrEw68985RpHH2eibuljD4UY8z_-VDmsarlIFEOuUoTgOf5h1FbvWSck0eOfHB_8yJW_ffsFdOh0Rvj-8F-3Z783h9V9x__fzl-tN9YSWopqhLksrKugcjpWxRGkDshWyMItNYBHS6NNI4aWqJWOlKCYtKW6VEL8CIC3a59u7j9GOhNHeDT5ZC0CNNS-oaUSpsa5XB7QraOKUUyXX76AcdDx1C92y-y-a7k_l88P7YvJiB-hO-qs75h2OuU1broh6tT38xKQVC-1yjVizrpMN_Rru7m4d_vvAHUIGegg</recordid><startdate>199211</startdate><enddate>199211</enddate><creator>Sallie, Richard</creator><creator>Katsiyiannakis, Leah</creator><creator>Baldwin, Dianne</creator><creator>Davies, Sue</creator><creator>O'Grady, John</creator><creator>Mowat, Alex</creator><creator>Mieli‐Vergani, Giorgina</creator><creator>Williams, Roger</creator><general>W.B. 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Liver. Pancreas. Abdomen</topic><topic>Hepatic Encephalopathy - blood</topic><topic>Hepatic Encephalopathy - diagnosis</topic><topic>Hepatolenticular Degeneration - blood</topic><topic>Hepatolenticular Degeneration - diagnosis</topic><topic>Humans</topic><topic>Liver - metabolism</topic><topic>Liver. Biliary tract. Portal circulation. Exocrine pancreas</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Other diseases. Semiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sallie, Richard</creatorcontrib><creatorcontrib>Katsiyiannakis, Leah</creatorcontrib><creatorcontrib>Baldwin, Dianne</creatorcontrib><creatorcontrib>Davies, Sue</creatorcontrib><creatorcontrib>O'Grady, John</creatorcontrib><creatorcontrib>Mowat, Alex</creatorcontrib><creatorcontrib>Mieli‐Vergani, Giorgina</creatorcontrib><creatorcontrib>Williams, Roger</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Hepatology (Baltimore, Md.)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sallie, Richard</au><au>Katsiyiannakis, Leah</au><au>Baldwin, Dianne</au><au>Davies, Sue</au><au>O'Grady, John</au><au>Mowat, Alex</au><au>Mieli‐Vergani, Giorgina</au><au>Williams, Roger</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Failure of simple biochemical indexes to reliably differentiate fulminant wilson's disease from other causes of fulminant liver failure</atitle><jtitle>Hepatology (Baltimore, Md.)</jtitle><addtitle>Hepatology</addtitle><date>1992-11</date><risdate>1992</risdate><volume>16</volume><issue>5</issue><spage>1206</spage><epage>1211</epage><pages>1206-1211</pages><issn>0270-9139</issn><eissn>1527-3350</eissn><coden>HPTLD9</coden><abstract>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 (χ2, 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. Massively elevated hepatic copper stores with normal serum copper concentrations were seen in some patients; in others tissue copper concentration from different regions of the same liver varied up to 7‐fold. Treatment with D‐penicillamine in five patients had no observable beneficial effect. (HEPATOLOGY 1992;16:1206–1211.)</abstract><cop>Philadelphia, PA</cop><pub>W.B. Saunders</pub><pmid>1427659</pmid><doi>10.1002/hep.1840160517</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record> |
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| identifier | ISSN: 0270-9139 |
| ispartof | Hepatology (Baltimore, Md.), 1992-11, Vol.16 (5), p.1206-1211 |
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| source | Wiley Online Library - AutoHoldings Journals; MEDLINE |
| subjects | Adolescent Adult Alkaline Phosphatase - blood Aspartate Aminotransferases - blood Bilirubin - blood Biological and medical sciences Ceruloplasmin - metabolism Child Copper - blood Copper - metabolism Diagnosis, Differential Female Gastroenterology. Liver. Pancreas. Abdomen Hepatic Encephalopathy - blood Hepatic Encephalopathy - diagnosis Hepatolenticular Degeneration - blood Hepatolenticular Degeneration - diagnosis Humans Liver - metabolism Liver. Biliary tract. Portal circulation. Exocrine pancreas Male Medical sciences Other diseases. Semiology |
| title | Failure of simple biochemical indexes to reliably differentiate fulminant wilson's disease from other causes of fulminant liver failure |
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