A rare complication of a rare disease: Amyloidosis in situs inversus
A rare complication of a rare disease: Amyloidosis in situs inversus作者机构：Istanbul Medeniyet University Goztepe Training and Research Hospital Nephrology Clinic Istanbul Turkey
出 版 物：《Open Journal of Internal Medicine》 (内科学期刊（英文）)
年 卷 期：2013年第3卷第2期
页 面：55-57页
学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学]
主 题：Situs Inversus Amyloidosis Bronchiectasis Nephrotic Syndrome
摘 要：A 31-year-old female admitted with productive cough and progressively increasing edema in her legs. Four years ago she had the diagnosis of situs inversus (SI) totalis. She had6.5 g/day proteinuria in 24-hour urine analysis. High resolution computed tomography revealed bronchiectatic areas in the left lung. In renal biopsy, kongo and amyloid AA antibody positivity was detected in arteriolar walls and mesangial areas. The patient is currently being followed with ramipril and colchicine treatments. Primary ciliary dyskinesia (PCD) and resultant SI totalis are very rare anomalies characterized by the total inversion of all organs and chronic bronchiectasis. There are very few reports on the association of SI totalis with nephrotic syndrome. Patients with SI totalis and chronic bronchiectasis have increased risk of AA amyloidosis and possibly end stage renal disease and thus these patients should be regularly followed with proteinuria and serum creatinine levels.
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