Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
AA-amyloidosis in Autosomal Dominant Polycystic Kidney Disease Caused by Chronic Cyst Infections Lasting for 30 years
Yoshiki TsuchiyaYoshifumi UbaraTatsuya SuwabeKazufumi NomuraKeiichi SumidaRikako HiramatsuJunichi HoshinoEiko HasegawaMasayuki YamanouchiNoriko HayamiNaoki SawaKenmei TakaichiKenichi Oohashi
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JOURNAL OPEN ACCESS

2013 Volume 52 Issue 7 Pages 791-794

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Abstract

We herein report the case of a 66-year-old Japanese woman who was admitted to our hospital due to diarrhea and malaise. She had been diagnosed with autosomal dominant polycystic kidney disease (ADPKD) at 35 years of age and had suffered from recurrent cyst infections since that time. Antibiotic therapy combined with hepatic cyst drainage and cyst sclerosing therapy led to transient improvements each time. At 66 years of age, watery diarrhea occurred. The patient's serum albumin level declined to 1.8 g/dL, and her C-reactive protein level was 4.5 mg/dL. An endoscopic biopsy of the descending colon revealed amorphous deposits in the small arteries and tissues of the submucosal layer. The deposits were positive for Congo Red staining and amyloid A staining. Therefore, AA-amyloidosis was diagnosed. An endoscopic biopsy of the stomach and duodenum also showed AA-amyloid deposits. If an ADPKD patient with a long history of cyst infection develops diarrhea and malaise, AA-amyloidosis should be considered as a possible complication.

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© 2013 by The Japanese Society of Internal Medicine
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