Adjusting copper concentrations for caeruloplasmin levels in routine clinical practice

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

doi:10.1136/jcp.2005.034876

Cited by 21 publications

(21 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Every child underwent blinded blood assays as follows:

Complete blood count: white blood cells and differential, red blood cells with indices, and platelets.

Quantitative immunoglobulins: IgG, IgM, IgA, and IgE and IgG subclasses (IgG-1, IgG-2, IgG-3, and IgG-4).

Biochemical markers: copper, ceruloplasmin, zinc, iron, transferrin, and ferritin. Additionally, equations of copper and ceruloplasmin were calculated (Twomey et al 2006), as follows: non-ceruloplasmin-bound copper level = total copper (mcg/dL) − 3 × ceruloplasmin (mg/dL); percentage non-ceruloplasmin copper = non-ceruplasmin copper/total copper (mcg/dL) × 100; and adjusted copper (copper index) = total copper (umol/L) − 0.052 × ceruloplasmin (mg/L) + 17.5.

…”

Section: Methodsmentioning

confidence: 99%

Involvement of immunologic and biochemical mechanisms in the pathogenesis of Tourette’s syndrome

et al. 2011

View full text Add to dashboard Cite

Tourette's syndrome is a neurodevelopmental disorder clinically characterized by multiple motor and phonic tics. It is likely that a neurobiological susceptibility to the disorder is established during development by the interaction of genetic, biochemical, immunological, and environmental factors. This study sought to investigate the possible correlation of several immunological and biochemical markers with Tourette's syndrome. Children with Tourette's syndrome attending a tertiary pediatric medical center from May 2008 to April 2010, and healthy age-matched control subjects underwent a comprehensive biochemical and immunological work-up. Demographic data were abstracted from the medical records. Findings were compared between the groups and analyzed statistically. Sixty-eight children with Tourette's syndrome (58 males, 85.3%) and 36 healthy children (25 males, 69.4%) were recruited. Compared with the control group, the Tourette's syndrome group had significantly higher levels of ferritin (p = 0.01) and hemoglobin (p = 0.02), a lower level of zinc (p = 0.05), and a lower percentage of non-ceruloplasmin copper (p = 0.01). Analysis of the immunological markers revealed no significant between-group differences in IgA, IgM or IgG; however, IgE and IgG-4 levels were significantly higher in the Tourette's syndrome group (p = 0.04 and p = 0.02, respectively). Children with Tourette's syndrome have high levels of biochemical indices of oxidative stress and the quantitative immunoglobulins. These findings add to the still-limited knowledge on the pathogenesis of Tourette's syndrome and may have implications for the development of novel therapeutic modalities.

show abstract

“…Every child underwent blinded blood assays as follows:

Complete blood count: white blood cells and differential, red blood cells with indices, and platelets.

Quantitative immunoglobulins: IgG, IgM, IgA, and IgE and IgG subclasses (IgG-1, IgG-2, IgG-3, and IgG-4).

…”

Section: Methodsmentioning

confidence: 99%

Involvement of immunologic and biochemical mechanisms in the pathogenesis of Tourette’s syndrome

et al. 2011

View full text Add to dashboard Cite

show abstract

“…Serum free-copper concentration can be estimated by calculation from serum ceruloplasmin concentration or measured directly. 168,172,173 The non-ceruloplasmin bound copper (µmol/l) 174 can be calculated as follows:…”

Section: Serum Free Copper and Non-ceruloplasmin-bound Copper Concentmentioning

confidence: 99%

“…168,174,175 Twomey et al proposed a new formula, adjusting copper concentrations for ceruloplasmin levels. 172 The authors claimed that this calculation overcame negative values and age and sex differences. However, the problem of standardization limits its application across a wide range of analytical systems.…”

Section: Serum Free Copper and Non-ceruloplasmin-bound Copper Concentmentioning

confidence: 99%

Diagnosis of Wilson's Disease: A Comprehensive Review

Mak

Lam

2008

Critical Reviews in Clinical Laboratory Sciences

122

View full text Add to dashboard Cite

2 Wilson's disease is an autosomal recessive disorder of copper metabolism. The culprit gene is ATP7B. The worldwide prevalence is about 1 in 30,000, which may vary by population. Higher prevalence rates were reported using more sensitive screening techniques and pilot population screening. Typical presentations include neuropsychiatric and hepatic dysfunction, whereas atypical presentations are protean. Diagnosis relies on a high clinical suspicion, typical neurological symptoms, presence of Kayser-Fleischer rings, and reduced serum ceruloplasmin concentration. The conventional value of <0.20 g/l is not a universal diagnostic value. Age of the subjects and analytical variations should be considered when interpreting these levels. Patients with inconclusive findings require further investigations such as 24 h urinary free-copper excretion, penicillamine challenge test, liver copper measurement, and detection of gene mutations. Direct molecular diagnosis remains the most decisive tool. Other tests such as non-ceruloplasmin-bound copper are unreliable. Potential pitfalls and limitations of these diagnostic markers are critically reviewed in this paper. The mainstays of therapy are trientine, penicillamine, and/or zinc. Liver transplantation is lifesaving for those with advanced disease. Ceruloplasmin oxidase activity and serum free-copper concentration should be monitored in patients on long-term de-coppering therapy to prevent iatrogenic copper deficiency.

show abstract

“…Furthermore, any index for the study of copper status should ideally have the potential to be used in the investigation of excess and deficiency copper states. Accordingly, new indices have been proposed, such as copper corrected for caeruloplasmin 13 and the copper:caeruloplasmin ratio. 14 The first study included only normal patients, whereas the second included patients with Wilson's disease who predominantly presented with neurological symptoms.…”

mentioning

confidence: 99%

Copper:caeruloplasmin ratio

et al. 2007

View full text Add to dashboard Cite

Investigation of copper status can be a diagnostic challenge. The non-caeruloplasmin-bound copper (NCC) has deficiencies; accordingly, the copper:caeruloplasmin ratio has been suggested as an alternative index of copper status. A reference interval for this index was derived. In addition to making the interpretation of copper easier, the copper:caeruloplasmin ratio should also enable adjustment for relatively high caeruloplasmin concentrations without recourse to producing gender- and age-derived intervals. The copper:caeruloplasmin ratio has weaknesses similar to those identified for NCC in that immunological methods used for caeruloplasmin can cross react with apocaeruloplasmin and there is no standardised method for caeruloplasmin. Caeruloplasmin assays also have uncertainty from precision, bias and specificity and, accordingly, method-related differences may have a large effect on the copper:caeruloplasmin ratio in a manner similar to the NCC.

show abstract

Adjusting copper concentrations for caeruloplasmin levels in routine clinical practice

Cited by 21 publications

References 21 publications

Involvement of immunologic and biochemical mechanisms in the pathogenesis of Tourette’s syndrome

Involvement of immunologic and biochemical mechanisms in the pathogenesis of Tourette’s syndrome

Diagnosis of Wilson's Disease: A Comprehensive Review

Copper:caeruloplasmin ratio

Contact Info

Product

Resources

About