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Literature DB >> 1275538

Menkes's steely hair syndrome.

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.

Entities: Chemical Disease Species

Mesh：

Substances：
Copper

Year: 1976 PMID： 1275538 PMCID： PMC1545947 DOI： 10.1136/adc.51.4.269

Source DB: PubMed Journal: Arch Dis Child ISSN： 0003-9888 Impact factor: 3.791

10 in total

1. A sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration.

Authors: J H MENKES; M ALTER; G K STEIGLEDER; D R WEAKLEY; J H SUNG
Journal: Pediatrics Date: 1962-05 Impact factor: 7.124

2. Menkes' kinky hair disease: further definition of the defect in copper transport.

Authors: D M Danks; E Cartwright; B J Stevens; R R Townley
Journal: Science Date: 1973-03-16 Impact factor: 47.728

3. Menkes' kinky-hair syndrome.

Authors: D M Danks; P E Campbell; J Walker-Smith; B J Stevens; J M Gillespie; J Blomfield; B Turner
Journal: Lancet Date: 1972-05-20 Impact factor: 79.321

4. Kinky hair syndrome: response to copper therapy.

Authors: W E Bucknall; R H Haslam; N A Holtzman
Journal: Pediatrics Date: 1973-11 Impact factor: 7.124

5. Menkes's kinky hair syndrome. An inherited defect in copper absorption with widespread effects.

Authors: D M Danks; P E Campbell; B J Stevens; V Mayne; E Cartwright
Journal: Pediatrics Date: 1972-08 Impact factor: 7.124

6. Letter: Copper and steely hair.

Authors: N Horn; M Mikkelsen; K Heydorn; E Damsgaard; I Tygstrup
Journal: Lancet Date: 1975-05-31 Impact factor: 79.321

7. Therapeutic implications of copper deficiency in Menkes's steely-hair syndrome.

Authors: J A Walker-Smith; B Turner; J Blomfield; G Wise
Journal: Arch Dis Child Date: 1973-12 Impact factor: 3.791

8. Letter: Menkes' kinky hair disease treated with subcutaneous copper sulphate.

Authors: A S Dekaban; J K Steusing
Journal: Lancet Date: 1974-12-21 Impact factor: 79.321

9. Kinky hair disease. I. Clinical and pathological features.

Authors: M J Aguilar; D L Chadwick; K Okuyama; S Kamoshita
Journal: J Neuropathol Exp Neurol Date: 1966-10 Impact factor: 3.685

10. Copper infusion therapy in trichopoliodystrophy.

Authors: W D Grover; M C Scrutton
Journal: J Pediatr Date: 1975-02 Impact factor: 4.406

10 in total

12 in total

1. Menkes' disease: neurophysiological aspects.

Authors: E Friedman; A Harden; M Koivikko; G Pampiglione
Journal: J Neurol Neurosurg Psychiatry Date: 1978-06 Impact factor: 10.154

2. Bone marrow from blotchy mice is dispensable to regulate blood copper and aortic pathologies but required for inflammatory mediator production in LDLR-deficient mice during chronic angiotensin II infusion.

Authors: Devon Harris; Yuanyuan Liang; Cang Chen; Senlin Li; Om Patel; Zhenyu Qin
Journal: Ann Vasc Surg Date: 2014-10-29 Impact factor: 1.466

Menkes's steely hair syndrome.

1. A sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration.

2. Menkes' kinky hair disease: further definition of the defect in copper transport.

3. Menkes' kinky-hair syndrome.

4. Kinky hair syndrome: response to copper therapy.

5. Menkes's kinky hair syndrome. An inherited defect in copper absorption with widespread effects.

6. Letter: Copper and steely hair.

7. Therapeutic implications of copper deficiency in Menkes's steely-hair syndrome.

8. Letter: Menkes' kinky hair disease treated with subcutaneous copper sulphate.

9. Kinky hair disease. I. Clinical and pathological features.

10. Copper infusion therapy in trichopoliodystrophy.

1. Menkes' disease: neurophysiological aspects.

2. Bone marrow from blotchy mice is dispensable to regulate blood copper and aortic pathologies but required for inflammatory mediator production in LDLR-deficient mice during chronic angiotensin II infusion.

Review 3. The Role of Copper and Zinc Toxicity in Innate Immune Defense against Bacterial Pathogens.

4. Copper Is a Host Effector Mobilized to Urine during Urinary Tract Infection To Impair Bacterial Colonization.

5. Menkes's syndrome. Report of a patient treated from 21 days of age with parenteral copper.

6. Clinical and biochemical consequences of copper-histidine therapy in Menkes disease.

Review 7. [Trichopoliodystrophy or Menkes disease].

Review 8. Diverticula of the bladder in children.

9. Early osseous abnormalities in Menkes' kinky hair syndrome.

Review 10. Copper Homeostatic Mechanisms and Their Role in the Virulence of Escherichia coli and Salmonella enterica.