Kupfermangel als seltene Ursache von Panzytopenie, Arthralgien und Gangstörungen

Bartner, Rupert; Will, Matthias Georg; Conrad, Joachim; Engelhardt, A.; Schwarz-Eywill, M

doi:10.1007/s00063-005-1072-7

Cited by 10 publications

(10 citation statements)

References 15 publications

(19 reference statements)

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“…A total of 55 cases were found in the literature [1, 2, 4, 12, 13, 16, 18, 19, 21, 24, 28–30, 38, 39, 41, 42, 44–47, 58, 63, 67, 71, 74, 77, 88, 89]. Age at presentation ranged from 30 to 82 years with a peak in the fifth and sixth decades (mean 54 years, median 53 years; Fig.…”

Section: Resultsmentioning

confidence: 99%

“…Doses were usually equivalent to 2 mg/day of elemental copper, though administration of up to 9 mg/day was reported (ignoring extreme outliers which are likely to reflect reporting errors [89]). A variety of copper salts were used, including copper acetate [63], copper chloride [88], copper citrate [28], copper gluconate [2, 12, 38, 44, 45, 74, 89], copper histidine [4] and copper sulphate [1, 12, 38, 41, 44, 47, 67, 71, 77]. No adverse effects were reported.…”

Section: Resultsmentioning

confidence: 99%

“…An additional 30 cases of myelopathy or myeloneuropathy have now been reported [1, 2, 4, 12, 13, 16, 18, 19, 21, 24, 28–30, 58, 63, 67, 71, 74, 77, 88, 89]. Here we review the published cases with regards to their demographics, aetiology, laboratory indices, spinal imaging, treatment and outcome.…”

Section: Introductionmentioning

confidence: 99%

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Copper deficiency myelopathy

2010

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Acquired copper deficiency has been recognised as a rare cause of anaemia and neutropenia for over half a century. Copper deficiency myelopathy (CDM) was only described within the last decade, and represents a treatable cause of non-compressive myelopathy which closely mimics subacute combined degeneration due to vitamin B12 deficiency. Here, 55 case reports from the literature are reviewed regarding their demographics, aetiology, haematological and biochemical parameters, spinal imaging, treatment and outcome. The pathophysiology of disorders of copper metabolism is discussed. CDM most frequently presented in the fifth and sixth decades and was more common in women (F:M = 3.6:1). Risk factors included previous upper gastrointestinal surgery, zinc overload and malabsorption syndromes, all of which impair copper absorption in the upper gastrointestinal tract. No aetiology was established in 20% of cases. High zinc levels were detected in some cases not considered to have primary zinc overload, and in this situation the contribution of zinc to the copper deficiency state remained unclear. Cytopenias were found in 78%, particularly anaemia, and a myelodysplastic syndrome may have been falsely diagnosed in the past. Spinal MRI was abnormal in 47% and usually showed high T2 signal in the posterior cervical and thoracic cord. In a clinically compatible case, CDM may be suggested by the presence of one or more risk factors and/or cytopenias. Low serum copper and caeruloplasmin levels confirmed the diagnosis and, in contrast to Wilson’s disease, urinary copper levels were typically low. Treatment comprised copper supplementation and modification of any risk factors, and led to haematological normalisation and neurological improvement or stabilisation. Since any neurological recovery was partial and case numbers of CDM will continue to rise with the growing use of bariatric gastrointestinal surgery, clinical vigilance will remain the key to minimising neurological sequelae. Recommendations for treatment and prevention are made.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Copper deficiency myelopathy

2010

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“…1,2,8,11 Prevention is therefore essential and dentists have an important role in educating patients about denture adhesive use. Dentists are well placed to identify patients who may be using zinccontaining denture adhesives to excess and who have anaemia or neurological symptoms affecting balance and walking.…”

Section: How Does Zinc Excess Results In Copper Deficiency?mentioning

confidence: 99%

“…1,5,6 These have highlighted the critical role of copper in a wide range of physiological processes from ATP production and cell maintenance to erythropoeisis and myelin synthesis. 1,3,4,7 Neutropenia, anaemia and neurological symptoms seem to be the most common manifestations of copper deficiency 1,8,9 and perhaps reflect the higher metabolic activity of the nervous system and bone marrow.…”

Section: The Role Of Copper In Humansmentioning

confidence: 99%