Primary intimal fibroplasia in a child with Down's syndrome
G. R. Fleisher, B. E. Buck and D. Cornfeld
A 14-year-old girl known to have trisomy 21 and who came to us with weight
loss followed by hypertension proved to have diffuse arterial dysplasia
involving the intimal layer (primary intimal fibroplasia). This is a rare
cause of elevated blood pressure in children and has not been previously
reported in a child with Down's syndrome. Unusual features of this case
also include the widespread distribution of the lesions limited to the
large muscular distributing arteries, the rapidly progressive course, and
the predominance of abdominal signs and symptoms.
