| Autor: |
Nakayama Y; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Sawa N; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Suwabe T; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Yamanouchi M; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Ikuma D; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Mizuno H; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Hasegawa E; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Hoshino J; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Sekine A; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Oba Y; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan., Kono K; Department of Pathology, Toranomon Hospital Kajigaya, Japan., Kinowaki K; Department of Pathology, Toranomon Hospital Kajigaya, Japan., Ohashi K; Department of Pathology, Toranomon Hospital Kajigaya, Japan.; Department of Human Pathology, Tokyo Medical Dental University, Japan., Yamaguchi Y; Yamaguchi's Pathology Laboratory, Japan., Nozu K; Department of Pediatrics, Kobe University Graduate School of Medicine, Japan., Ubara Y; Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan. |
| Abstrakt: |
We evaluated kidney histology in a 43-year-old woman with autosomal dominant tubulointerstitial kidney disease subtype hepatocyte nuclear factor 1β. Magnetic resonance imaging showed multiple cysts in the renal medullary area, and computed tomography showed hypoplasia of the pancreatic body and tail. A kidney biopsy showed thinning of the cortex, size reduction of glomerular tuft area, proximal tubule clustering, fibrosis around the tubules, loss of peritubular capillaries, and multilayered epithelial cells of cortical collecting ducts; this last finding was consistent with so-called medullary dysplasia specific to congenital disease, in which the renal pelvic epithelial cells enter the collecting duct. |
