Glomerular Pathology in Dent Disease and Its Association with Kidney Function
| Autor: | Andrea G. Cogal, Dorella Del Prete, Lawrence Copelovitch, Lisa E. Vaughan, Franca Anglani, Steven J. Scheinman, Fabio Paglialonga, Gema Ariceta, Giuseppe Vezzoli, John C. Lieske, Peter C. Harris, Loren P. Herrera Hernandez, Robert Isom, Anila J. Mehta, Lada Beara-Lasic, Xiangling Wang, Felicity Enders |
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| Přispěvatelé: | Wang, X, Anglani, F, Beara-Lasic, L, Mehta, Aj, Vaughan, Le, Herrera Hernandez, L, Cogal, A, Scheinman, Sj, Ariceta, G, Isom, R, Copelovitch, L, Enders, Ft, Del Prete, D, Vezzoli, G, Paglialonga, F, Harris, Pc, Lieske, Jc |
| Rok vydání: | 2016 |
| Předmět: |
Male
0301 basic medicine Pathology podocyte Epidemiology Biopsy Kidney Glomerulus 030232 urology & nephrology Dent Disease Critical Care and Intensive Care Medicine Glomerulosclerosis 0302 clinical medicine Lymphocytes Child Kidney Proteinuria medicine.diagnostic_test Podocytes Glomerulosclerosis Focal Segmental Middle Aged Kidney Tubules medicine.anatomical_structure Renal pathology Nephrology Child Preschool Disease Progression Renal biopsy medicine.symptom Glomerular Filtration Rate Adult kidney medicine.medical_specialty Adolescent Renal function Dent disease Humans Inflammation Molecular Weight biopsy glomerular filtration rate glomerulosclerosis interstitial fibrosis proteinuria Focal Segmental Young Adult 03 medical and health sciences medicine Transplantation business.industry Infant Original Articles medicine.disease Fibrosis 030104 developmental biology business |
| Zdroj: | Clinical Journal of the American Society of Nephrology. 11:2168-2176 |
| ISSN: | 1555-905X 1555-9041 |
| Popis: | 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 m 2 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. |
| Databáze: | OpenAIRE |
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