ISSN:
1432-198X
Keywords:
Key words: Alport syndrome
;
Renal scarring
;
Cortical interstitial volume fraction
;
Global glomerular sclerosis
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract. We investigated the onset of renal scarring in 62 males (aged 4 – 26 years) with Alport syndrome by measuring cortical interstitial volume fraction [Vv (interstitium/cortex)] and percentage global glomerular sclerosis in kidney biopsies. Male pediatric (n = 9) and adult (n = 7) donor kidneys served as controls. Creatinine clearance at the time of biopsy was available for 43 Alport patients. A statistically insignificant correlation between age and Vv (interstitium/cortex) was observed in normal subjects (r = +0.47, slope = 0.0009, P = 0.07). In the Alport patients, age was significantly correlated with Vv (interstitium/cortex (r = +0.49, slope = 0.01, P = 0.001) and global glomerular sclerosis (r = +0.41, P = 0.01), and inversely correlated with creatinine clearance (r = –0.33, P = 0.04). Creatinine clearance was inversely correlated with Vv (interstitium/cortex) (r = –0.78, P = 0.001) and global glomerular sclerosis (r = –0.74, P = 0.001). The correlation with creatinine clearance was especially strong for Vv (interstitium/cortex) values above the normal range, i. e., 〉0.2 (r = –0.82, P = 0.001), and was absent for Vv (interstitium/cortex) 〈0.2 (r = –0.119, P = 0.55). Creatinine clearance values less than 80 ml/min per 1.73 m2 occurred more frequently in patients with Vv (interstitium/cortex) values 〉0.2 (P 〈0.0001) and in patients with 〉10% globally sclerosed glomeruli (P 〈0.001). Patients ≤ or 〉10 years of age differed in Vv (interstitium/cortex) [0.13±0.09 (mean ±SD) vs. 0.24±0.026, P 〈0.001], the frequency of Vv (interstitium/cortex) 〉0.2 (3/32 vs. 15/31, P 〈0.0001), the frequency of 〉10% globally sclerosed glomeruli (3/33 vs. 11/30, P 〈0.05), mean creatinine clearance (113±7 vs. 84±10 ml/min per 1.73 m2, P = 0.057), and the frequency of creatinine clearance 〈80 ml/min per 1.73 m2 (1/20 vs. 11/23, P 〈0.01). Thus, reduced creatinine clearance in males with Alport syndrome is associated with Vv (interstitium/cortex) 〉0.2 and 〉10% globally sclerosed glomeruli. These are frequently detectable in the 2nd decade. We hypothesize that most Alport males will require intervention during the 1st decade for optimal preservation of kidney function.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s004670050451
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