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Progressive diaphyseal dysplasia mimicking childhood myopathy: Clinical and biochemical response to prednisolone
Author(s) -
LOW L. C. K.,
STEPHENSON J. B. P.,
STUARTSMITH D. A.
Publication year - 1985
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1111/j.1440-1754.1985.tb02133.x
Subject(s) - medicine , prednisolone , myopathy , disease , pediatrics , bone remodeling , dysplasia , pathology
Two siblings presented with symptoms suggestive of myopethy: the true diagnosis of Engelmaann's disease was delayed until X‐rays revealed the characteristic sclerosis and widening of long bone diaphyses. Dominant inheritance from their father had not been suspected because of his lack of symptoms. Biochemical studies of bone metabolism did not elucidate the underlying mechanism. Prednisolone dramatically improved the clinical condition, particularly in the more severely affected child. While research into the biochemical basis must continue, paediatricians should remeber to X‐ray the long bones of ‘myopathic’ children, and offer steroids when the clinical condition warrants.