Author(s)

Abdullah Ozkok, Mirac Vural Keskinler, Erhan Eken, Osman Kostek, Ozge Caklili, Alihan Oral, Kubra Aydin Bahat, Omer Celal Elcioglu, Ali Bakan, Ali Riza Odabas

Istanbul Medeniyet University, Goztepe Training and Research Hospital, Nephrology Clinic, Istanbul, Turkey.

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.

Situs Inversus; Amyloidosis; Bronchiectasis; Nephrotic Syndrome

Ozkok, A. , Keskinler, M. , Eken, E. , Kostek, O. , Caklili, O. , Oral, A. , Bahat, K. , Elcioglu, O. , Bakan, A. and Odabas, A. (2013) A rare complication of a rare disease: Amyloidosis in situs inversus. Open Journal of Internal Medicine, 3, 55-57. doi: 10.4236/ojim.2013.32013.