Use of a Stable Copper Isotope (65Cu) in the Differential Diagnosis of Wilson's Disease

Lyon, T. D. B.; Fell, Gordon S.; Gaffney, Dairena; McGaw, Brian A.; Russell, Robin; Park, R. H. R.; Beattie, A. D.; Curry, GW; Crofton, R. J.; Gunn, I R; Sturniolo, G; D’Incà, R.; Patriarca, Marina

doi:10.1042/cs0880727

Cited by 43 publications

(20 citation statements)

References 9 publications

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“…Models of Zn metabolism have enabled the effect of acute dietary Zn depletion on homeostasis to be investigated in young subjects (King et al 2001). Modelling of stable Cu isotope data has been useful in investigating the changes in Cu metabolism that occur in patients with Wilson's disease, as a tool to aid diagnosis (Lyon et al 1995). Application of this approach to investigate changes in mineral metabolism that occur due to ageing and in patients with osteoporosis will enable the identification of sites of altered mineral metabolism, and provide essential information on status, absorption and also endogenous excretion, so that supplementation regimens can be optimised.…”

Section: Discussionmentioning

confidence: 99%

Is there a potential therapeutic value of copper and zinc for osteoporosis?

Fraser²,

2002

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Osteoporosis is almost universal in very old age, and is a major cause of morbidity and mortality in the elderly of both sexes. Bone is lost at a rate of 0-2-0-5 %/year in both men and women after the age of 40-45 years. The causes of age-related changes in bone mass are multifactorial and include genetic predisposition, nutritional factors, endocrine changes, habitual exercise levels and body weight. Bone loss is accelerated to 2-5 % year immediately before and for up to 10 years post-menopause (Heaney, 1986). In women hormone-replacement therapy is effective in reducing the rate of bone loss caused by this peri-menopausal decrease in hormone levels (Smith & Studd, 1993); however, in men and older women (>10 years post-menopause) nutrition plays a key role in the rate of bone loss. One factor contributing to bone loss in the elderly may be a subclinical Zn and/or Cu deficiency, due to a reduced dietary intake of micronutrients and reduced absorption (Thomson & Keelan, 1986). Zn and Cu are essential cofactors for enzymes involved in the synthesis of various bone matrix constituents. Paradoxically, Ca supplementation may accentuate the problem of reduced Zn and Cu levels by impairing the absorption of simultaneously-ingested Zn and the retention of Cu (Snedeker et al. 1982;Grekas et al. 1988). The present paper will review the current literature on the potential benefits of Cu and Zn supplementation in reducing bone loss, and present new information on the effect of Ca supplementation on Zn and Cu status in post-menopausal women with osteoporosis.

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Section: Discussionmentioning

confidence: 99%

Is there a potential therapeutic value of copper and zinc for osteoporosis?

Fraser²,

2002

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“…This test is now rarely used because of the difficulty in obtaining isotope. An experimental alternative to using radiocopper is the use of a nonradioactive isotope for copper, 65 Cu, which can be detected by mass spectroscopic methods 77 ; however, this methodology has difficulty in distinguishing heterozygotes from patients and is not routinely available.…”

Section: Radiocopper Studymentioning

confidence: 99%

A practice guideline on Wilson disease

2003

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PreambleThis guideline is intended for use by physicians. It describes preferable up-to-date approaches to the diagnosis and treatment of patients with Wilson disease. As their purpose is to direct patient care, these guidelines should not be considered inflexible mandates. They have been developed in a manner consistent with the American Association for the Study of Liver Diseases Policy Statement on Development and Use of Practice Guidelines.These guidelines provide data-supported approaches to the diagnosis and management of patients with Wilson disease. They are based on, first, broad-based review of the published literature in pediatrics and medicine including Medline searches on hepatolenticular degeneration and related subjects; and second, 40 accumulated years of personal experience of the authors. In order to standardize recommendations as much as possible, each has been characterized with Roman numerals I through IV to indicate the quality of evidence on which the recommendation is based (Table 1). 1 A significant problem with the literature on Wilson disease is that patients are sufficiently rare to preclude large cohort studies or randomized controlled trials; moreover, most treatment modalities were developed at a time when conventions for drug assessment were less stringent than currently.

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“…Lyon et al described the application of the stable isotope 65 Cu to the differential diagnosis of WD (57,58). The stable isotope could be followed for 5 weeks after administration, thus allowing long term studies of Cu metabolism in heterozygous subjects.…”

Section: Contribution Of New Methodologies To the Diagnosis Of Trace mentioning

confidence: 98%