ISSN:
0736-0266
Keywords:
Hereditary sensory neuropathy
;
Bone resorption
;
Osteoclasts
;
Vascular reflex
;
Life and Medical Sciences
Source:
Wiley InterScience Backfile Collection 1832-2000
Topics:
Medicine
Notes:
This study concerns two children, from the same otherwise normal family, presenting progressive spontaneous amputations of the limbs, suggesting peripheral hereditary sensory neuropathy. Samples from the distal nerves and vessels and the distal tibial epiphyses were examined ultrastructurally. The nerves showed signs of degeneration, and a typical bone necrosis, without osteoclasts, was observed in the 2 cm margin of the samples. Simultaneous modifications of the vessels suggest a neurally initiated vascular reflex resulting in bone destruction. Theories that might explain the observations are discussed.
Additional Material:
10 Ill.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1002/jor.1100030213
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