Copper:caeruloplasmin ratio

Twomey, Patrick J; Viljoen, Adie; House, Ivan; Reynolds, Tim; Wierzbicki, Anthony S.

doi:10.1136/jcp.2006.041756

Cited by 27 publications

(23 citation statements)

References 10 publications

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“…The present study's copper:caeruloplasmin ratio (mean 8 SD, mol/g) of 6.99 8 0.84 ( table 3 ) is similar to 6.81 8 0.87 reported by Twomey et al [9] . Also, the Omani reference range of 5.46-8.14 mol/g for copper:caeruloplasmin ratio ( table 3 ) is close to 5.2-7.3 and 5.08-8.55 mol/g Beetham [8] and Twomey et al [9] reported, respectively.…”

Section: Discussionsupporting

confidence: 76%

“…Also, the Omani reference range of 5.46-8.14 mol/g for copper:caeruloplasmin ratio ( table 3 ) is close to 5.2-7.3 and 5.08-8.55 mol/g Beetham [8] and Twomey et al [9] reported, respectively. Furthermore, Omani reference ranges of 10.2-21.3 M (copper) and 0.215-0.427 g/l (caeruloplasmin) are similar to the 10-25 M (copper) and 0.200-0.500 g/l (caeruloplasmin) reported by Twomey et al [9] . However, the observation that the copper:caeruloplasmin ratio was markedly higher in women compared to men ( table 3 ) contrasted with the previous reports [8,9] .…”

Section: Discussionmentioning

confidence: 99%

“…Caeruloplasmin, an endogenous antioxidant and a major copper binder, is important in the interpretation of serum copper results [7] . Copper:caeruloplasmin ratio has recently been suggested as an alternative index of copper status [8,9] that enables adjustment for relatively high caeruloplasmin levels without recourse to producing gender-and age-derived reference intervals [9] .…”

Section: Introductionmentioning

confidence: 99%

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Serum Micronutrient and Micromineral Concentrations and Ratios in Healthy Omani Subjects

2008

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Objective: This study aimed to establish reference ranges of serum concentrations of copper, zinc, retinol, α-tocopherol, copper:caeruloplasmin and copper:zinc ratios in a group of healthy Omani men and women. Materials and Methods: Assay techniques employed were atomic absorption spectrophotometry (copper and zinc), reverse-phase high-pressure liquid chromatography with isocratic elution (retinol and α-tocopherol), immunonephelometry (caeruloplasmin) and spectrophotometry (albumin and cholesterol). Results: The mean ± SD (µM) obtained for copper, zinc, retinol, and α-tocopherol were 15.9 ± 3.0, 14.2 ± 2.0, 1.45 ± 0.39 and 16.9 ± 4.4, respectively. The mean ± SD for copper:zinc and copper:caeruloplasmin ratios were 1.15 ± 0.30 µmol/mmol and 6.99 ± 0.84 µmol/g, respectively. Significantly higher (p < 0.0001) copper and caeruloplasmin concentrations, copper:zinc and copper:caeruloplasmin ratios and lower zinc, retinol, α-tocopherol, cholesterol concentrations and α-tocopherol:cholesterol ratio were present in women compared to men. Age appeared to be associated with copper and retinol concentrations, and copper:caeruloplasmin ratios in women; in men, the association was mostly moderate with caeruloplasmin, α-tocopherol, cholesterol concentrations and α-tocopherol:cholesterol ratios. Smokers had decreased albumin (p = 0.002), zinc (p = 0.023) concentrations, and copper:caeruloplasmin ratios (p = 0.002), increased α-tocopherol concentrations (p = 0.016) and α-tocopherol:cholesterol ratios (p = 0.021) compared with non-smokers. Deficiency incidence was ≤5% for all investigated parameters. Conclusions: Reference ranges of micronutrient concentrations and micromineral ratios were established for Omani subjects. The mean values of several micronutrients were lower than those reported for other populations and some showed gender effects.

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Section: Discussionsupporting

confidence: 76%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Serum Micronutrient and Micromineral Concentrations and Ratios in Healthy Omani Subjects

2008

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“…A diagnostic scoring system has been developed that includes 7 parameters (neurological symptoms, KF rings, Coombs-negative hemolytic anemia, serum ceruloplasmin concentration, urinary copper excretion, liver copper measurement, and genetic mutations) (10 ). Other biochemical markers have been proposed such as serum free copper concentration (11 ) and copper:ceruloplasmin ratio (12 ), but these appear to be less reliable. False-positive and -negative rates are high among the traditional markers.…”

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confidence: 99%

Diagnostic Accuracy of Serum Ceruloplasmin in Wilson Disease: Determination of Sensitivity and Specificity by ROC Curve Analysis among ATP7B-Genotyped Subjects

2008

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Background: A serum ceruloplasmin concentration below 0.20 g/L is conventionally considered as one of the major diagnostic criteria for Wilson disease. This decision threshold has not been fully validated for its diagnostic characteristics, however. In this study, we evaluated various decision thresholds of serum ceruloplasmin concentration in the diagnosis of Wilson disease based on genotype-verified Wilson disease patients, carriers, and normal individuals. Methods: Serum ceruloplasmin concentration was measured by a nephelometric method in 57 Wilson disease patients and 71 family members (49 heterozygotes and 22 wild-type homozygotes), a validation group of 25 subjects clinically suspected of Wilson disease, and 690 normal individuals. We performed ROC analysis using Analyze-it software and confirmed the genotypes by direct DNA sequencing of ATP7B. Results: Serum ceruloplasmin concentrations <0.20, 0.14, and 0.10 g/L showed positive predictive values of 48.3%, 100%, and 100%, respectively, and negative predictive values of 98.7%, 97.1%, and 91.9%. In the validation group, a serum ceruloplasmin threshold of 0.14 g/L rendered 100% sensitivity and specificity. Forty of 690 healthy subjects had serum ceruloplasmin concentrations <0.20 g/L; however, these 40 individuals had normal genotypes by DNA sequencing, and none of the 40 had ceruloplasmin concentrations <0.14 g/L. Conclusions: The diagnostic accuracy for Wilson disease using a serum ceruloplasmin concentration of 0.14 g/L as the local decision threshold was better than that using a threshold of 0.20 g/L. We suggest that laboratories providing ceruloplasmin assays determine decision thresholds based on local populations.

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“…Normalization of NCC in blood reflects the effectiveness of anti-copper therapy (Desai and Kaler, 2008;EASL, 2012). Although direct measurement of free copper is possible, it is not currently routinely available, and most measurements of free copper are not standardized (Twomey et al, 2005(Twomey et al, , 2007El Balkhi et al, 2011). Twenty-four-hour urinary copper excretion is a very important parameter for the diagnosis of WD as well as for treatment monitoring, as it reflects the fraction of free copper in serum (EASL, 2012).…”

Section: Measurement Of Urinary Copper Excretion After 48-h D-penicilmentioning

confidence: 99%

Measurement of urinary copper excretion after 48-h d-penicillamine cessation as a compliance assessment in Wilson�s disease

Dzieżyc¹

2015

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SummaryTreatment of Wilson's disease (WD) with anti-copper agents is effective in most compliant patients. During long-term treatment with chelating agents, a two-day interruption of the treatment should result in normal urinary copper concentrations (<50 μg/dl). The aim of this study was to establish the usefulness of this method as a compliance assessment in these patients. We examined consecutive patients treated with d-penicillamine (DPA) undergoing routine follow-up studies at our center. We performed 24-h urinary copper excretion analysis 48 h after interruption of chelating therapy. Thirty-two patients were enrolled. After DPA cessation, normalization of copper excretion was observed in 91% of reportedly compliant patients. The specificity and sensitivity values of this test were 87% and 77%, respectively. Measurement of 24-h urinary copper excretion after a 48-h interruption of DPA therapy in patients with WD is a reliable method for confirming patients' compliance.

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Copper:caeruloplasmin ratio

Cited by 27 publications

References 10 publications

Serum Micronutrient and Micromineral Concentrations and Ratios in Healthy Omani Subjects

Serum Micronutrient and Micromineral Concentrations and Ratios in Healthy Omani Subjects

Diagnostic Accuracy of Serum Ceruloplasmin in Wilson Disease: Determination of Sensitivity and Specificity by ROC Curve Analysis among ATP7B-Genotyped Subjects

Measurement of urinary copper excretion after 48-h d-penicillamine cessation as a compliance assessment in Wilson�s disease

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