TITLE:
A rare complication of a rare disease: Amyloidosis in situs inversus
AUTHORS:
Abdullah Ozkok, Mirac Vural Keskinler, Erhan Eken, Osman Kostek, Ozge Caklili, Alihan Oral, Kubra Aydin Bahat, Omer Celal Elcioglu, Ali Bakan, Ali Riza Odabas
KEYWORDS:
Situs Inversus; Amyloidosis; Bronchiectasis; Nephrotic Syndrome
JOURNAL NAME:
Open Journal of Internal Medicine,
Vol.3 No.2,
May
31,
2013
ABSTRACT: 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.