A rare complication of a rare disease: Amyloidosis in situs inversus

• 作者: Abdullah Ozkok Ali Bakan Ali Riza Odabas Alihan Oral Erhan Eken Kubra Aydin Bahat Mirac Vural Keskinler Omer Celal Elcioglu Osman Kostek Ozge Caklili
• 来源期刊: 内科学期刊(英文)
• 学科: 医学
• 关键词: Situs Inversus AMYLOIDOSIS BRONCHIECTASIS Nephrotic SYNDROME
• 年，卷（期）: 2013,（2）
• 页码范围: 55-57
• 页数: 3页
• 分类号: R5

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.