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Amyloidosis as a Complication of Cystic Fibrosis
Robert Castile, MD;
Harry Shwachman, MD;
William Travis, MD;
Cynthia A. Hadley, MD;
Warren Warwick, MD;
H. P. Missmahl, MD
Am J Dis Child. 1985;139(7):728-732.
Abstract
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)
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).
Footnotes
Reprint requests to Division of Respiratory Diseases, The Children's Hospital, 300 Longwood Ave, Boston, MA 02115 (Dr Castile).
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