Abstract
| Original language | English |
|---|---|
| Pages (from-to) | 2168-2176 |
| Number of pages | 9 |
| Journal | Clinical journal of the American Society of Nephrology : CJASN |
| Volume | 11 |
| Issue number | 12 |
| Publication status | Published - 2016 |
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Keywords
- Dent disease
- Disease Progression
- Glomerulosclerosis, Focal Segmental
- Humans
- Inflammation
- Kidney Glomerulus
- Male
- Molecular Weight
- Podocytes
- biopsy
- glomerular filtration rate
- glomerulosclerosis
- interstitial fibrosis
- kidney
- podocyte
- proteinuria
Cite this
Glomerular Pathology in Dent Disease and Its Association with Kidney Function. / Wang, X.; Anglani, F.; Beara-Lasic, Lada; Mehta, A. J.; Vaughan, Lisa E; Herrera Hernandez, Loren; Cogal, Andrea; Scheinman, Steven J.; Ariceta, Gema; Isom, Robert; Copelovitch, Lawrence; Enders, Felicity T; Del Prete, D.; Vezzoli, G.; Paglialonga, F.; Harris, Peter C.; Lieske, John C; Consortium, Investigators of the Rare Kidney Stone.
In: Clinical journal of the American Society of Nephrology : CJASN, Vol. 11, No. 12, 2016, p. 2168-2176.Research output: Contribution to journal › Article
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TY - JOUR
T1 - Glomerular Pathology in Dent Disease and Its Association with Kidney Function
AU - Wang, X.
AU - Anglani, F.
AU - Beara-Lasic, Lada
AU - Mehta, A. J.
AU - Vaughan, Lisa E
AU - Herrera Hernandez, Loren
AU - Cogal, Andrea
AU - Scheinman, Steven J.
AU - Ariceta, Gema
AU - Isom, Robert
AU - Copelovitch, Lawrence
AU - Enders, Felicity T
AU - Del Prete, D.
AU - Vezzoli, G.
AU - Paglialonga, F.
AU - Harris, Peter C.
AU - Lieske, John C
AU - Consortium, Investigators of the Rare Kidney Stone
N1 - LR: 20170220; CI: Copyright (c) 2016; JID: 101271570; OTO: NOTNLM; PMCR: 2017/12/07; 2016/04/01 [received]; 2016/08/16 [accepted]; ppublish M1 - Journal Article
PY - 2016
Y1 - 2016
N2 - BACKGROUND AND OBJECTIVES: Dent disease is a rare X-linked disorder characterized by low molecular weight proteinuria and often considered a renal tubular disease. However, glomerulosclerosis was recently reported in several patients. Thus, Dent disease renal histopathologic features were characterized and assessed, and their association with kidney function was assessed. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Clinical renal pathology reports and slides (where available) were collected from 30 boys and men in eight countries who had undergone clinical renal biopsy between 1995 and 2014. RESULTS: Median (25th, 75th percentiles) age at biopsy was 7.5 (5, 19) years with an eGFR of 69 (44, 94) ml/min per 1.73 m2 and a 24-hour urine protein of 2000 (1325, 2936) mg. A repeat biopsy for steroid-resistant proteinuria was performed in 13% (four of 30) of the patients. Prominent histologic findings included focal global glomerulosclerosis in 83% (25 of 30; affecting 16%+/-19% glomeruli), mild segmental foot process effacement in 57% (13 of 23), focal interstitial fibrosis in 60% (18 of 30), interstitial lymphocytic infiltration in 53% (16 of 30), and tubular damage in 70% (21 of 30). Higher percentages of globally sclerotic glomeruli, foot process effacement, and interstitial inflammation were associated with lower eGFR at biopsy, whereas foot process effacement was associated with steeper annual eGFR decline. CONCLUSIONS: These associations suggest a potential role for glomerular pathology, specifically involving the podocyte, in disease progression, which deserves further study. Furthermore, Dent disease should be suspected in boys and men who have unexplained proteinuria with focal global glomerulosclerosis and segmental foot process effacement on renal biopsy.
AB - BACKGROUND AND OBJECTIVES: Dent disease is a rare X-linked disorder characterized by low molecular weight proteinuria and often considered a renal tubular disease. However, glomerulosclerosis was recently reported in several patients. Thus, Dent disease renal histopathologic features were characterized and assessed, and their association with kidney function was assessed. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Clinical renal pathology reports and slides (where available) were collected from 30 boys and men in eight countries who had undergone clinical renal biopsy between 1995 and 2014. RESULTS: Median (25th, 75th percentiles) age at biopsy was 7.5 (5, 19) years with an eGFR of 69 (44, 94) ml/min per 1.73 m2 and a 24-hour urine protein of 2000 (1325, 2936) mg. A repeat biopsy for steroid-resistant proteinuria was performed in 13% (four of 30) of the patients. Prominent histologic findings included focal global glomerulosclerosis in 83% (25 of 30; affecting 16%+/-19% glomeruli), mild segmental foot process effacement in 57% (13 of 23), focal interstitial fibrosis in 60% (18 of 30), interstitial lymphocytic infiltration in 53% (16 of 30), and tubular damage in 70% (21 of 30). Higher percentages of globally sclerotic glomeruli, foot process effacement, and interstitial inflammation were associated with lower eGFR at biopsy, whereas foot process effacement was associated with steeper annual eGFR decline. CONCLUSIONS: These associations suggest a potential role for glomerular pathology, specifically involving the podocyte, in disease progression, which deserves further study. Furthermore, Dent disease should be suspected in boys and men who have unexplained proteinuria with focal global glomerulosclerosis and segmental foot process effacement on renal biopsy.
KW - Dent disease
KW - Disease Progression
KW - Glomerulosclerosis, Focal Segmental
KW - Humans
KW - Inflammation
KW - Kidney Glomerulus
KW - Male
KW - Molecular Weight
KW - Podocytes
KW - biopsy
KW - glomerular filtration rate
KW - glomerulosclerosis
KW - interstitial fibrosis
KW - kidney
KW - podocyte
KW - proteinuria
M3 - Article
VL - 11
SP - 2168
EP - 2176
JO - Clinical journal of the American Society of Nephrology : CJASN
JF - Clinical journal of the American Society of Nephrology : CJASN
SN - 1555-9041
IS - 12
ER -