Tubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases.
Tubular proteinuria defined by a study of Dent's ( CLCN5 mutation) and other tubular diseases. BACKGROUND: The term "tubular proteinuria" is often used interchangeably with "low molecular weight proteinuria" (LMWP), although the former implies a definite etiology. A specific...
| Main Authors: | , , , , , , , |
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| Format: | Conference item |
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2000
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| _version_ | 1797091690705584128 |
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| author | Norden, A Scheinman, S Deschodt-Lanckman, M Lapsley, M Nortier, J Thakker, R Unwin, R Wrong, O |
| author_facet | Norden, A Scheinman, S Deschodt-Lanckman, M Lapsley, M Nortier, J Thakker, R Unwin, R Wrong, O |
| author_sort | Norden, A |
| collection | OXFORD |
| description | Tubular proteinuria defined by a study of Dent's ( CLCN5 mutation) and other tubular diseases. BACKGROUND: The term "tubular proteinuria" is often used interchangeably with "low molecular weight proteinuria" (LMWP), although the former implies a definite etiology. A specific quantitative definition of tubular proteinuria is needed, and we address this by studying five different renal disorders. METHODS: Tubular proteinuria was assessed by measuring urinary retinol-binding protein (RBP), beta2-microglobulin (beta2M), alpha1-microglobulin (alpha1M), and albumin in 138 patients: 26 affected males and 24 female carriers of the X-linked syndrome "Dent's disease," 6 patients with other Fanconi syndromes, 17 with distal renal tubular acidosis (dRTA), 39 with glomerulonephritis (GN), and 26 with Chinese herbs nephropathy (CHN). RESULTS: RBP was better than beta2M or alpha1M in identifying the tubular proteinuria of Dent's disease. Median urinary RBP levels in mg/mmol creatinine were: affected male Dent's, 18.2, N = 26; carrier female Dent's, 0. 30, N = 24; dRTA, 0.027, N = 17; GN, 0.077, N = 39; and normal adults, 0.0079, N = 61. Elevated urinary RBP (>0.017) and albumin < (10 x RBP) + 2 identified all patients with the LMWP of Dent's disease and clearly distinguished their LMWP from that of dRTA and GN. This is a quantitative definition of tubular proteinuria. Consistent with this definition, 80% of those patients with CHN who had an elevated RBP had tubular proteinuria. Urinary RBP and albumin in carriers of Dent's disease were strikingly correlated over a 100-fold range (R = 0.933). CONCLUSION: The combination of elevated urinary RBP (>0.017) and albumin < (10 x RBP) + 2 (mg protein/mmol creatinine) is a quantitative definition of tubular proteinuria. Furthermore, our findings suggest that a shared defect in tubular RBP and albumin reuptake causes this form of proteinuria. |
| first_indexed | 2024-03-07T03:36:38Z |
| format | Conference item |
| id | oxford-uuid:bc837d51-8263-4565-99d3-31e8e587cfc2 |
| institution | University of Oxford |
| last_indexed | 2024-03-07T03:36:38Z |
| publishDate | 2000 |
| record_format | dspace |
| spelling | oxford-uuid:bc837d51-8263-4565-99d3-31e8e587cfc22022-03-27T05:24:56ZTubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases.Conference itemhttp://purl.org/coar/resource_type/c_5794uuid:bc837d51-8263-4565-99d3-31e8e587cfc2Symplectic Elements at Oxford2000Norden, AScheinman, SDeschodt-Lanckman, MLapsley, MNortier, JThakker, RUnwin, RWrong, OTubular proteinuria defined by a study of Dent's ( CLCN5 mutation) and other tubular diseases. BACKGROUND: The term "tubular proteinuria" is often used interchangeably with "low molecular weight proteinuria" (LMWP), although the former implies a definite etiology. A specific quantitative definition of tubular proteinuria is needed, and we address this by studying five different renal disorders. METHODS: Tubular proteinuria was assessed by measuring urinary retinol-binding protein (RBP), beta2-microglobulin (beta2M), alpha1-microglobulin (alpha1M), and albumin in 138 patients: 26 affected males and 24 female carriers of the X-linked syndrome "Dent's disease," 6 patients with other Fanconi syndromes, 17 with distal renal tubular acidosis (dRTA), 39 with glomerulonephritis (GN), and 26 with Chinese herbs nephropathy (CHN). RESULTS: RBP was better than beta2M or alpha1M in identifying the tubular proteinuria of Dent's disease. Median urinary RBP levels in mg/mmol creatinine were: affected male Dent's, 18.2, N = 26; carrier female Dent's, 0. 30, N = 24; dRTA, 0.027, N = 17; GN, 0.077, N = 39; and normal adults, 0.0079, N = 61. Elevated urinary RBP (>0.017) and albumin < (10 x RBP) + 2 identified all patients with the LMWP of Dent's disease and clearly distinguished their LMWP from that of dRTA and GN. This is a quantitative definition of tubular proteinuria. Consistent with this definition, 80% of those patients with CHN who had an elevated RBP had tubular proteinuria. Urinary RBP and albumin in carriers of Dent's disease were strikingly correlated over a 100-fold range (R = 0.933). CONCLUSION: The combination of elevated urinary RBP (>0.017) and albumin < (10 x RBP) + 2 (mg protein/mmol creatinine) is a quantitative definition of tubular proteinuria. Furthermore, our findings suggest that a shared defect in tubular RBP and albumin reuptake causes this form of proteinuria. |
| spellingShingle | Norden, A Scheinman, S Deschodt-Lanckman, M Lapsley, M Nortier, J Thakker, R Unwin, R Wrong, O Tubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases. |
| title | Tubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases. |
| title_full | Tubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases. |
| title_fullStr | Tubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases. |
| title_full_unstemmed | Tubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases. |
| title_short | Tubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases. |
| title_sort | tubular proteinuria defined by a study of dent s clcn5 mutation and other tubular diseases |
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