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Journal List > Arch Dis Child > v.51(4); Apr 1976

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Arch Dis Child. 1976 April; 51(4): 269–274.
PMCID: PMC1545947
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Menkes's steely hair syndrome.
E M Wheeler and P F Roberts
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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.
  • Aguilar MJ, Chadwick DL, Okuyama K, Kamoshita S. Kinky hair disease. I. Clinical and pathological features. J Neuropathol Exp Neurol. 1966 Oct;25(4):507–522. [PubMed]
  • Bucknall WE, Haslam RH, Holtzman NA. Kinky hair syndrome: response to copper therapy. Pediatrics. 1973 Nov;52(5):653–657. [PubMed]
  • Danks DM, Campbell PE, Stevens BJ, Mayne V, Cartwright E. Menkes's kinky hair syndrome. An inherited defect in copper absorption with widespread effects. Pediatrics. 1972 Aug;50(2):188–201. [PubMed]
  • Danks DM, Campbell PE, Walker-Smith J, Stevens BJ, Gillespie JM, Blomfield J, Turner B. Menkes' kinky-hair syndrome. Lancet. 1972 May 20;1(7760):1100–1102. [PubMed]
  • Danks DM, Cartwright E, Stevens BJ, Townley RR. Menkes' kinky hair disease: further definition of the defect in copper transport. Science. 1973 Mar 16;179(78):1140–1142. [PubMed]
  • Dekaban AS, Steusing JK. Letter: Menkes' kinky hair disease treated with subcutaneous copper sulphate. Lancet. 1974 Dec 21;2(7895):1523. [PubMed]
  • Grover WD, Scrutton MC. Copper infusion therapy in trichopoliodystrophy. J Pediatr. 1975 Feb;86(2):216–220. [PubMed]
  • Horn N, Mikkelsen M, Heydorn K, Damsgaard E, Tygstrup I. Letter: Copper and steely hair. Lancet. 1975 May 31;1(7918):1236. [PubMed]
  • MENKES JH, ALTER M, STEIGLEDER GK, WEAKLEY DR, SUNG JH. A sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration. Pediatrics. 1962 May;29:764–779. [PubMed]
  • Walker-Smith JA, Turner B, Blomfield J, Wise G. Therapeutic implications of copper deficiency in Menkes's steely-hair syndrome. Arch Dis Child. 1973 Dec;48(12):958–962. [PubMed]
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