title: AA-amyloidosis in autosomal dominant polycystic kidney disease caused by chronic cyst infections lasting for 30 years.
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.
authors.affiliation: Nephrology Center, Toranomon Hospital, Japan. yoshiki1209@gmail.com
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Tsuchiya
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Ubara
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suwabe
authors.forename: Kazufumi
authors.initials: K
authors.lastname: Nomura
authors.forename: Keiichi
authors.initials: K
authors.lastname: Sumida
authors.forename: Rikako
authors.initials: R
authors.lastname: Hiramatsu
authors.forename: Junichi
authors.initials: J
authors.lastname: Hoshino
authors.forename: Eiko
authors.initials: E
authors.lastname: Hasegawa
authors.forename: Masayuki
authors.initials: M
authors.lastname: Yamanouchi
authors.forename: Noriko
authors.initials: N
authors.lastname: Hayami
authors.forename: Naoki
authors.initials: N
authors.lastname: Sawa
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
authors.forename: Kenichi
authors.initials: K
authors.lastname: Oohashi
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis*; D003560:Cysts / Q000150:complications / Q000175:diagnosis; D005260:Female; D006801:Humans; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000175:diagnosis*; D000685:Serum Amyloid A Protein / Q000032:analysis*; D013997:Time Factors
chemical_list: D000685:Serum Amyloid A Protein