Table 4.

Scoring of renal biopsy specimens (light microscopy and electron microscopy)

Patient No.Age (yr)/SexTime (yr)GSFSGSGlomerular HyalineInterstitial Fibrosis (%)aArteriopathyMesangial Cell InclusionGlomerular Endothelial Cell InclusionsSegmental Foot-Process EffacementDistal Tubule Epithelial Cell InclusionMesangium
17/M00/23+++++
50/11+(+)
211/M00/24++(+)+
30/26(+)+
50/16(+)+
311/F00/29+++++Matrix+
50/10+(+)
412/M00/23+++(+)+
50/12++
516/M−2
0b1/12+5++++Matrix+
11/7+(+)+
50/18(+)(+)+
616/M00/22++(+)+
30/15(+)(+)+
50/17(+)++
717/M−2
0b1/95+++
10/7+++
50/11+++
817/M00/17(+)++++++Cells +
10/33(+)+++
918/M01/215++++Cells +
10/12++(+)+Cells (+)
50/12++
1023/M00/18++++Matrix +
10/9+++Matrix +
50/13++++Matr ix+
1130/M01/18+5++++Matrix +
11/23+5(+)++Matrix +
50/12+5+(+)+
1233/M−3
0c1/155+++Matrix+
51/125+++Matrix +
  • The podocytes are scored separately in Table 5. Plus signs represent clear presence, plus signs in parentheses mean slight presence, and minus signs represent absence. Time, time of biopsy: 0 is baseline, numbers represent years after baseline; GS, global glomerular sclerosis (number of GS/total glomeruli); M, male; F, female.

  • a Estimated semiquantitively and scored to nearest 5%.

  • b Two years of ERT at baseline.

  • c Three years of ERT at baseline.