FAILURE OF SIMPLE BIOCHEMICAL INDEXES TO RELIABLY DIFFERENTIATE FULMINANT WILSONS-DISEASE FROM OTHER CAUSES OF FULMINANT LIVER-FAILURE

被引:51
作者
SALLIE, R
KATSIYIANNAKIS, L
BALDWIN, D
DAVIES, S
OGRADY, J
MOWAT, A
MIELIVERGANI, G
WILLIAMS, R
机构
[1] UNIV LONDON KINGS COLL HOSP, DEPT CHILD HLTH, LONDON SE5 8RX, ENGLAND
[2] UNIV LONDON KINGS COLL HOSP, DEPT CHEM PATHOL, LONDON SE5 8RX, ENGLAND
[3] UNIV LONDON KINGS COLL HOSP, INST LIVER STUDIES, LONDON SE5 8RX, ENGLAND
关键词
D O I
10.1016/0270-9139(92)90016-3
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
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 (chi2, 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.
引用
收藏
页码:1206 / 1211
页数:6
相关论文
共 23 条
[21]   THE EFFECT OF ACETAMINOPHEN (AC) ON THE CHANGE OF INTRACELLULAR AND EXTRACELLULAR GLUTATHIONE IN ISOLATED HEPATOCYTES FROM FISCHER (F) AND SPRAGUE-DAWLEY (S-D) RATS [J].
WILLSON, RA ;
HART, JR ;
HALL, T .
HEPATOLOGY, 1987, 7 (05) :1086-1086
[22]  
Wilson SAK, 1911, BRAIN, V34, P295
[23]   INDICATIONS FOR LIVER-TRANSPLANTATION IN FULMINANT WILSONS-DISEASE [J].
ZILKER, TR ;
FELGENHAUER, N ;
HIBLER, A ;
VONCLARMANN, M .
DEUTSCHE MEDIZINISCHE WOCHENSCHRIFT, 1989, 114 (22) :871-875