Observation on Cases of Pyle's Congenital Familial Bone Dysplasia
Billewicz, O.; Zaleska, M.
Polski Przeglad Radiologii i Medycyny Nuklearnej 27: 361-374
1963
ISSN/ISBN: 0137-7183 PMID: 14194565 Document Number: 9465
Three cases of the Pyle bone dysplasia are presented. The main characteristic of this syndrome is the dilatation of the metaphyseal regions of the long bones resulting from an developmental impairment. In the mentioned cases, impaired ossification of the cranial bones was also observed, and this finding permitted to classify them to the so called cranioepiphyseal dysplasia. The mentioned syndrome is congenital, the hereditary pattern is unstable and may be recessive as well as dominating. The observed disturbance in the bone formation is caused by a decrease of the normal functions of the osteoclasts. The article contains a literature review, and the pathologic process, leading to the mentioned deformations, is discussed.
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