July 1985

Amyloidosis as a Complication of Cystic Fibrosis

Author Affiliations

From the Department of Pediatrics and Pathology, The Children's Hospital, Boston (Drs Castile, Shwachman, Travis, and Hadley); the Department of Pediatrics, University of Minnesota Hospitals, Minneapolis (Dr Warwick); and the Department of Medicine, Marienkrankenhaus, Hamburg, West Germany (Dr Missmahl).

Am J Dis Child. 1985;139(7):728-732. doi:10.1001/archpedi.1985.02140090090040

•Amyloidosis appears to be a rare complication of cystic fibrosis. We discuss three patients with amyloidosis complicating cystic fibrosis to add to the six patients previously recorded. The presenting problem was proteinuria in five patients, thyromegaly in three patients, and hepatosplenomegaly in one patient. The progression of proteinuria to nephrotic syndrome and edema occurred in eight of nine patients and octended a very poor prognosis. The kidneys, adrenal glands, spleen, thyroid gland, liver, heart, and bowel were most frequently involved. Renal involvement is a frequent and devastating complication of amyloidosis in patients with cystic fibrosis.

(AJDC 1985;139:728-732)