Uromodulin storage disease

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Presentation transcript:

Uromodulin storage disease S.H. Nasr, J.P. Lucia, S.J. Galgano, G.S. Markowitz, V.D. D'Agati Kidney International Volume 73, Issue 8, Pages 971-976 (April 2008) DOI: 10.1038/sj.ki.5002679 Copyright © 2008 International Society of Nephrology Terms and Conditions

Figure 1 On low-power view, the cortex displays focal patchy tubular atrophy and interstitial fibrosis, with minimal interstitial inflammation. No tubulitis is seen. The glomerulus is unremarkable (hematoxylin and eosin, original magnification × 200). Kidney International 2008 73, 971-976DOI: (10.1038/sj.ki.5002679) Copyright © 2008 International Society of Nephrology Terms and Conditions

Figure 2 On high-power, distal tubular cells contain focal intracytoplasmic inclusions of lamellated trichrome blue material (arrows) that contrasts with the red staining cytoplasm (Masson trichrome, original magnification × 600). Kidney International 2008 73, 971-976DOI: (10.1038/sj.ki.5002679) Copyright © 2008 International Society of Nephrology Terms and Conditions

Figure 3 A representative distal tubular profile shows a cell at center containing dilated cisternae of the ER in which there is accumulation of granular, moderately electron dense storage material (arrow). The tubular basement membrane appears thickened and lamellated (electron micrograph, original magnification × 4000). Kidney International 2008 73, 971-976DOI: (10.1038/sj.ki.5002679) Copyright © 2008 International Society of Nephrology Terms and Conditions

Figure 4 On high power, distal tubular cells contain hyperplastic bundles of the ER (arrow) with few intervening mitochondria alternating with areas of dilated ER containing storage material (arrowhead) (electron micrograph, original magnification × 6000). Kidney International 2008 73, 971-976DOI: (10.1038/sj.ki.5002679) Copyright © 2008 International Society of Nephrology Terms and Conditions

Figure 5 Immunostain for UMOD in control kidney shows homogeneous diffuse staining of cytoplasm and apical membranes of TALH cells (original magnification × 600). Kidney International 2008 73, 971-976DOI: (10.1038/sj.ki.5002679) Copyright © 2008 International Society of Nephrology Terms and Conditions

Figure 6 Immunostain of the patient's biopsy shows focal coarse, dense clusters of UMOD within TALH cells, corresponding to the intracellular inclusions (original magnification × 600). Kidney International 2008 73, 971-976DOI: (10.1038/sj.ki.5002679) Copyright © 2008 International Society of Nephrology Terms and Conditions