Kartagener syndrome complicated by immunoglobulin A nephropathy

Abstract

Kentaro Oka,1 Taro Sugase,1 Tetsu Akimoto,1,2 Takuya Murakami,1 Izumi Nagayama,1 Miwa Kaneko,1 Maki Asakura,1 Ken Ohara,1 Osamu Saito,1 Daisuke Nagata1 1Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Tochigi, Japan; 2Department of Chronic Kidney Disease Pathophysiology, Jichi Medical University, Tochigi, Japan Abstract: We herein report the case of a 36-year-old woman with Kartagener syndrome (KS), which is an autosomal recessive disorder defined by a triad of bronchiectasis, sinusitis, and situs inversus, with complications of asymptomatic microhematuria and proteinuria. She was finally diagnosed with biopsy-proven immunoglobulin (Ig) A nephropathy. KS constitutes a subgroup of primary ciliary dyskinesia (PCD) characterized by structural and/or functional ciliary abnormalities resulting in sinopulmonary involvement with varying severity. Our case does not allow us to corroborate the clinical impact of KS and/or PCD as a pathogenic basis for the IgA nephropathy, and each disease might develop independently. However, systematic studies on this topic are quite lacking, so we strongly recommend the accumulation of more cases similar to our own, which would allow us to clarify the nature of this disease state more precisely. Keywords: primary ciliary dyskinesia, Kartagener syndrome, situs inversus, IgA nephropathy, glomerulonephritis

Keywords

• primary ciliary dyskinesia
• Kartagener syndrome
• situs inversus
• IgA nephropathy
• glomerulonephritis