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2016 ; 55
(20
): 3001-3007
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Rapidly Progressive Renal Dysfunction in Two Elderly Patients with Renal
Enlargement and Medullary Cystic Kidney Disease-like Acute Tubulointerstitial
Injury
#MMPMID27746439
Kawamoto S
; Koda R
; Yoshino A
; Takeda T
; Ueda Y
Intern Med
2016[]; 55
(20
): 3001-3007
PMID27746439
show ga
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.