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Systemic amyloidosis in cystic fibrosis


American Journal of Diseases of Children 131(8): 886-888
Systemic amyloidosis in cystic fibrosis
We report two siblings with cystic fibrosis and systemic amyloidosis. The major clinical problem in both cases was recurrent respiratory infection with pulmonary fibrosis and bronchiectasis prior to death at ages 20 and 22 years. Findings from postmortem examinations disclosed diffuse amyloidosis. In addition, amyloid infiltration developed in both patients, with enlargement of the thyroid gland, and one required thyroidectomy. An autopsy review of 17 additional cases of cystic fibrosis failed to disclose any other instances of systemic amyloidosis.


Accession: 006574909

PMID: 888804

DOI: 10.1001/archpedi.1977.02120210064013



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