ABSTRACT
INTRODUCTION@#The diagnosis of Wilson disease (WD) is plagued by biochemical and clinical uncertainties. Thus, calculated parameters have been proposed. This study aimed to: (a) compare the diagnostic values of non-caeruloplasmin copper (NCC), NCC percentage (NCC%), copper-caeruloplasmin ratio (CCR) and adjusted copper in WD; and (b) derive and evaluate a discriminant function in diagnosing WD.@*METHODS@#A total of 213 subjects across all ages who were investigated for WD were recruited. WD was confirmed in 55 patients, and the rest were WD free. Based on serum copper and caeruloplasmin values, NCC, NCC%, CCR and adjusted copper were calculated for each subject. A function was derived using discriminant analysis, and the cut-off value was determined through receiver operating characteristic analysis. Classification accuracy was found by cross-tabulation.@*RESULTS@#Caeruloplasmin, total copper, NCC, NCC%, CCR, adjusted copper and discriminant function were significantly lower in WD compared to non-WD. Discriminant function showed the best diagnostic specificity (99.4%), sensitivity (98.2%) and classification accuracy (99.1%). Caeruloplasmin levels <0.14 g/L showed higher accuracy than the recommended 0.20 g/L cut-off value (97.7% vs. 87.8%). Similarly, molar NCC below the European cut-off of 1.6 umol/L showed higher accuracy than the American cut-off of 3.9 umol/L (80.3% vs. 59.6%) (P < 0.001). NCC%, mass NCC, CCR and adjusted copper showed poorer performances.@*CONCLUSION@#Discriminant function differentiates WD from non-WD with excellent specificity, sensitivity and accuracy. Performance of serum caeruloplasmin <0.14 g/L was better than that of <0.20 g/L. NCC, NCC%, CCR and adjusted copper are not helpful in diagnosing WD.
Subject(s)
Humans , Hepatolenticular Degeneration/diagnosis , Copper/analysis , Ceruloplasmin/metabolism , Repressor ProteinsABSTRACT
@#Kounis syndrome (KS) is defined as acute coronary syndrome (ACS) induced by coronary vasospasm associated with an anaphylactic reaction. This condition was described in 1991 by Kounis, detailing potential pathophysiological pathways for coronary spasm. We report a case of Type 2 KS – a patient with stented coronary artery disease who presented with ST-elevation myocardial infarction (STEMI) following intramuscular Diclofenac Sodium.
ABSTRACT
@#Introduction: Ionised calcium is a good prognostic and diagnostic tool as opposed to total calcium in critical patients but is not available in most central laboratories and non-intensive care units. To date, four equations to calculate ionised calcium in critical patients have been published. Objectives: (1) Evaluate the four published equations’ performance in estimating ionised calcium; (2) Determine the accuracy of calculated ionised and adjusted total calcium in classifying patients according to calcium states; and (3) Identify factors associated with hypocalcaemia in the critically ill population. Materials and methods: This is a cross-sectional study involving 281 critically ill patients aged 18-80 years of both genders in a Malaysian tertiary intensive care unit. Performance of the four equations was analysed using Bland-Altman difference plot and Passing Bablok regression analysis. Crosstabulation was conducted to assess classification accuracy. Mann-Whitney U or Pearson Chi-Square tests were performed to identify variables associated with hypocalcaemia. Results: Calculated ionised calcium using all four equations significantly overestimated ionised calcium. Calculated ionised and adjusted total calcium had poor accuracies in classifying hypocalcaemic patients. pH was significantly higher in hypocalcaemics. Conclusion: Calculated ionised and adjusted total calcium significantly overestimate ionised calcium in the critically ill. In this specific population, calcium status should only be confirmed with ionised calcium measured by direct ion-selective electrode (ISE).