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. 1976 Apr;51(4):269–274. doi: 10.1136/adc.51.4.269

Menkes's steely hair syndrome.

E M Wheeler, P F Roberts
PMCID: PMC1545947  PMID: 1275538

Abstract

Three unrelated cases of Menkes's syndrome are described. In addition to the typical features these cases all showed abnormalities of the urinary tract characterized by hydronephrosis, hydroureter, and bladder diverticula. There was no organic obstruction in the urinary tract and the changes are presumed to be due to disturbed innervation. The 3 patients showed no overall clinical response to parenteral copper therapy administered for periods of 1 to 8 months. Necropsy studies showed structural changes in veins as well as arteries together with aneurysm formation in both, and one case showed a polypoid lesion in the stomach.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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