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

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.

DOI: 10.4236/ojim.2013.32013                

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.

Keywords

Situs Inversus; Amyloidosis; Bronchiectasis; Nephrotic Syndrome

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	Leigh, M.W., Pittman, J.E., Carson, J.L., et al. (2009) Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome. Genetics in Medicine, 11, 473z487. doi:10.1097/GIM.0b013e3181a53562
[2]	Osman, E.M., Abboud, O.I., Sulaiman, S.M., Musa, A.R., Beleil, O.M. and Sharfi, A.A. (1991) End-stage renal failure in Kartagener’s syndrome. Nephrology Dialysis Transplantation, 6, 747. doi:10.1093/ndt/6.10.747
[3]	Demir, M., Kutlucan, A. and Sezer, M.T. (2007) Renal amyloidosis in a patient with Kartagener syndrome. Renal Failure, 29, 117. doi:10.1080/08860220601039569
[4]	Vikrant, S., Kumar, S., Raina, R. and Sharma, A. (2008) Nephrotic syndrome in a patient with situs inversus totalis. Clinical and Experimental Nephrology, 12, 215-218. doi:10.1007/s10157-007-0027-0
[5]	Domański, M., Domański, L. and Ciechanowski, K. (2005) Nephrotic syndrome and situs inversus viscerum: Correlation or coincidence. Journal of Nephrology, 18, 623-625.
[6]	Katsuhara, K., Kawamoto, S., Wakabayashi, T. and Belsky, J.L. (1972) Situs inversus totalis and Kartagener’s syndrome in a Japanese population. Chest, 61, 56-61. doi:10.1378/chest.61.1.56
[7]	Noone, P.G., Leigh, M.W., Sannuti, A., et al. (2004) Primary ciliary dyskinesia: Diagnostic and phenotypic features. American Journal of Respiratory and Critical Care Medicine, 169, 459-467. doi:10.1164/rccm.200303-365OC
[8]	Kennedy, M.P., Noone, P.G., Leigh, M.W., et al. (2007) High-resolution CT of patients with primary ciliary dyskinesia. American Journal of Roentgenology, 188, 1232-1238. doi:10.2214/AJR.06.0965