July 1978

Primary Intimal Fibroplasia in a Child With Down's Syndrome

Author Affiliations

From the Departments of Pediatrics (Drs Fleisher and Cornfeld) and Pathology (Dr Buck), University of Pennsylvania, and the Children's Hospital of Philadelphia.

Am J Dis Child. 1978;132(7):700-703. doi:10.1001/archpedi.1978.02120320060014

• 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.

(Am J Dis Child 132:700-703, 1978)