Osteogenesis imperfecta tarda in a child with hyper-IgE syndrome
E. P. Brestel, W. G. Klingberg, R. W. Veltri and J. S. Dorn
A patient had recurrent pyogenic infections, chronic mucocutaneous
candidiasis, and repeated long-bone fractures. Her disorder was diagnosed
as hyper-IgE syndrome on the basis of clinical data, elevated levels of
serum IgE, increased levels of IgE specific for Staphylococcus aureus, and
impaired T-lymphocyte function. Roentgenograms confirmed the diagnosis of
osteogenesis imperfecta tarda. The estimated likelihood of both conditions
occurring in the same person is approximately one in 10 billion. The
coincident finding of bone dysplasia in a patient with impaired
T-lymphocyte function suggests a common mechanism for birth defects.
