title: Rapidly Progressive Renal Dysfunction in Two Elderly Patients with Renal Enlargement and Medullary Cystic Kidney Disease-like Acute Tubulointerstitial Injury.
abstract: Medullary cystic kidney disease (MCKD) is a hereditary disease associated with bilateral medullary polycysts and interstitial fibrosis. MCKD is typically associated with slowly progressive renal dysfunction. We herein report two rare elderly cases with enlarged kidneys and rapidly progressive renal dysfunction without myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), PR3-ANCA, or anti-glomerular basement membrane (GBM) antibodies. Renal biopsies revealed extensive tubular dilatation and atrophy with interstitial fibrosis consistent with MCKD. Both patients began hemodialysis therapy a few months later. Our cases suggest a MCKD subgroup among elderly patients with an undefined genetic background, rapidly progressive renal dysfunction, and enlarged kidneys.
authors.affiliation: Department of Nephrology, Dokkyo Medical University Koshigaya Hospital, Japan.
authors.forename: Shinya
authors.initials: S
authors.lastname: Kawamoto
authors.forename: Ryo
authors.initials: R
authors.lastname: Koda
authors.forename: Atsunori
authors.initials: A
authors.lastname: Yoshino
authors.forename: Tetsuro
authors.initials: T
authors.lastname: Takeda
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Ueda
mesh_terms: D058186:Acute Kidney Injury / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D007684:Kidney Tubules / Q000473:pathology*; D006435:Renal Dialysis