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Much confusion exists in the accurate identification and
classification of the many disorders in infancy which affect the
skeleton by producing sclerosing lesions throughout the entire
skeleton or in selected areas. A scholarly effort to bring order
into this confusing field must be welcomed. Professors Beighton and
Cremin have skillfully defined the problem and concisely yet
thoroughly have identified the various sclerosing dysplasias. They
bring to this task a wealth of clinical experience in their many
years in the Departments of Radiology at the University of Cape
Town Medical School and the Groote Schuur and Red Cross Children's
Hospitals in Cape Town, South Africa. The authors also have had
available data from the University of Cape Town Skeletal Dysplasia
Registry. Professors Beighton and Cremin have used as a basis for
their treatise the Paris Nomenclature (the modified version of
1977). This monograph is a felicitous combination of a working
atlas and a scholarly exposition of the various sclerosing bone
dysplasias, considering in detail eighteen major entities adopted
from the Paris classification. Other disorders which present with
osteosclerosis are also described. These include Caffey disease,
oculodento-osseous dysplasia, central osteosclerosis with bamboo
hair, fluorosis, osteosclerosis associated with renal disease and
mastocytosis.
The tremendous expansion of medical knowledge during the last few
decades, together with the introduction of many new diagnostic
techniques, has demanded such a degree of specialisation that no
single individual can be conversant with all the information
available. More and more emphasis, therefore, has been placed on
the importance of teamwork and close collaboration between
associated disciplines. The bone dysplasias of infancy represent a
classical example of this concept. Only a few years ago these
heritable conditions were divided into a relatively small number of
entities, for many of which "atypical variants" were accepted. More
recent studies have resulted in appreciation and early recognition
of a large number of these disorders, thanks to co-operation
between paediatricians, radiologists, geneticists and biochemists.
Not only maya reasonably accurate prognosis be offered for the
affected child in many instances, but, almost of greater value,
genetic counselling concerning the chance of subsequent offspring
being similarly affected has become available to parents. Most
radiologists have little opportunity of becoming familiar with this
rapidly widening field of diagnosis, so that the occasional case
which may be encountered is likely to engender diagnostic
difficulty. This Atlas should facilitate greatly the solution of
the problem. It has been prepared by Professor CREMIN, an
outstanding paediatric radiologist whose work has been known and
admired by me for many years, in close collaboration with his
colleague Professor BEIGHTON, a geneticist of great distinction.
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