Castile R, Shwachman H, Travis W, Hadley C A, Warwick W, Missmahl H P
Am J Dis Child. 1985 Jul;139(7):728-32. 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 portended 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.
淀粉样变性似乎是囊性纤维化的一种罕见并发症。我们讨论了三例并发囊性纤维化的淀粉样变性患者，以补充之前记录的六例患者。五例患者的主要问题是蛋白尿，三例患者是甲状腺肿大，一例患者是肝脾肿大。9例患者中有8例出现蛋白尿进展为肾病综合征和水肿，预示预后极差。肾脏、肾上腺、脾脏、甲状腺、肝脏、心脏和肠道是最常受累的部位。肾脏受累是囊性纤维化患者淀粉样变性常见且具有破坏性的并发症。
