Yang Eun Ae, Lee Dong Won, Hyun Myung Chul, Cho Min Hyun
Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea.
Korean J Pediatr. 2010 Jul;53(7):770-3. doi: 10.3345/kjp.2010.53.7.770. Epub 2010 Jul 31.
A 13-year-old girl was diagnosed with non-cystic fibrosis (CF)-related multifocal bronchiectasis accompanied by nephrotic-range proteinuria of unknown cause. On renal biopsy, there were many segmental homogeneous deposits of amyloid tissue with positive Congo red staining in the glomeruli and interstitium. On electron microscopy, relatively straight, non-branching, randomly arranged amyloid fibrils were showed in the mesangium of the glomeruli. These fibrils were approximately 10 nm in diameter, compatible with secondary amyloidosis. Her level of serum amyloid A was remarkably elevated. To our knowledge, this girl is the first case of secondary renal amyloidosis induced by bronchiectasis in Korean children.
一名13岁女孩被诊断为非囊性纤维化(CF)相关的多灶性支气管扩张,伴有原因不明的肾病范围蛋白尿。肾活检显示,肾小球和间质中有许多节段性均匀的淀粉样组织沉积,刚果红染色呈阳性。电子显微镜检查显示,肾小球系膜中有相对笔直、无分支、随机排列的淀粉样纤维。这些纤维直径约为10纳米,符合继发性淀粉样变。她的血清淀粉样蛋白A水平显著升高。据我们所知,这名女孩是韩国儿童中首例由支气管扩张引起的继发性肾淀粉样变病例。