Table 2.

Clinical and biochemical findings of PH1 patients, subdivided according to the different phenotypic groups

Group PT S/A C.D ESRF GFR POX PGLY UOX UGLY %AGT GGT PyrR
PH1, Primary hyperoxaluria type 1; PT, patient; S/A, age at onset/present age (yr); ESRF, age at onset of end stage renal failure; CD, clinical details; NL, nephrolitiasis; NC, nephrocalcinosis; GFR, glomerular filtration rate (ml/min/1.73m2); TxK, kidney transplant; TxLK, kidney-liver transplant; HD, hemodialysis; POX, plasma oxalate (μmol/l); PGLY, plasma glycolate (μmol/l); UOX, urine oxalate μmol/mmol creatinine; UGLY, urine glycolate μmol/mmol creatinine; %AGT, normalized alanine:glyoxylate aminotransferase activity; GGT, gamma-glutamyl transferase activity (normal value, 0.7-1.2 μmol/h per mg protein); PyrR, response to pyridoxine; ND, not determined; Group A, onset before 10 yr and ESRF before 20 yr; Group B, onset after 10 yr and ESRF after 20 yr; Group C, early or late onset with maintained renal function over 20 yr; Group D, patients not included in the groups A, B, or C because of short follow-up.
Group A
1 6/16 NL 15 TxLK 116 597 ND ND 10 0.72 No
3 6/10 NC 10 TxLK 78 377 ND ND 8 0.77 No
5 8/19 NL 16 TxLK 253 420 ND ND 9 1 No
10 1/20 NL 9 TxLK 37 327 ND ND 0 0.65 No
11 1/6 NC 1 TxLK 260 297 ND ND 0 ND ND
16 1/15 NL 7 HD 222 388 ND ND 0 0.54 No
18 4/4 NC 4 HD 167 498 ND ND 0 0.21 No
20 1/10 NL 9 HD 169 201 ND ND 2 0.3 ND
Group B
2 16/52 NL 35 HD 165 41.3 ND ND 12 0.84 Yes
6 20/46 NL 30 TxK 16 43 ND ND 34 1.02 Yes
13 31/39 NL 31 HD 166 63 ND ND 7 0.9 ND
14 40/54 NL 45 TxK 63 145 ND ND 50 1.7 Yes
17 49/55 NL 50 HD 227 581 ND ND 4 0.84 Yes
19 22/50 NC 49 HD 147 163 ND ND 34 0.55 Yes
21 13/33 NL 29 TxK 80 177 365 195 10 0.4 Yes
Group C
4 4/20 NL 80 8 138 169 478 5 2.05 No
9 6/22 NL 40 8 103 240 258 10 0.6 Partial
12 20/30 NL 100 21 36 92 179 40 0.7 Yes
22 8/29 NC 77 22 56 161 32 3.6 0.72 Yes
23 12/22 NL 54 9.3 157 144 76 ND ND Yes
Group D
7 4/13 NL 80 15 159 319 320 3 0.69 No
8 1/5 NL 80 7 58 534 536 58 1.4 Yes
15 8/12 NL 70 15.3 186 161 458 15 1.24 Partial