From: Clinical utility of chitotriosidase enzyme activity in nephropathic cystinosis
Group | N | Age in years range (median) | Chitotriosidase activity nmol/ml plasma/h range (median) | P values |
---|---|---|---|---|
All normal controls | 87 | 1-48 (12) | 0-90 (18) | |
Pediatric normal controls | 54 | 1-16 (4.8) | 0-72 (14) | |
Adult normal controls | 33 | 22-48 (36) | 0-90 (31) | <0.001 with pediatric normal controls |
All renal controls | 54 | 2-58 (22) | 0-321 (52) | <0.001 with all normal controls |
Pediatric renal controls | 24 | 2-12 (4.6) | 2-144 (39) | <0.001 with pediatric normal controls |
Fanconi syndrome | 12 | 2-5 (3) | 5-44 (19) | 0.60 with pediatric normal controls |
ESRD | 12 | 2-12 (6) | 2-144 (67) | <0.001 with pediatric normal controls |
<0.001 with pediatric Fanconi syndrome | ||||
Adult renal controls | 30 | 19-58 (38) | 0-321 (73) | <0.001 with adult normal controls |
Microalbuminuria | 15 | 26-46 (32) | 8-143 (60) | 0.008 with adult normal controls |
ESRD | 15 | 19-58 (41) | 0-321 (87) | <0.001 with adult normal controls |
0.40 with diabetic nephropathy | ||||
All controls with ESRD | 27 | 2-58 (22) | 0-321 (75) | |
All controls below 5 years | 39 | 0.5- 4.5 (2.2) | 0-49 (15) | |
Cystinosis patients | 45 | 0.8-49 (13) | 0-3880 (163) | <0.001 with all normal controls |
<0.001 with all renal controls | ||||
All pediatric patients | 25 | 0.8-16 (3.8) | 0-3880 (268) | <0.001 with pediatric normal controls |
<0.001 with pediatric renal controls | ||||
All adult patients | 20 | 18-49 (28) | 0-1487 (78) | <0.001 with adult normal controls |
0.44 with adult renal controls | ||||
Cysteamine treated patients | ||||
Cystine* <1 | 11 | 17-49 (34) | 18-342 (52) | 0.71 with all renal controls |
Cystine* >1 and <4 | 13 | 2-38 (24) | 0-843 (245) | 0.006 with all renal controls |
Cystine* >4 | 12 | 3.5-36 (10.5) | 84-1487 (788) | <0.001 with all renal controls |
Cystinosis patients with ESRD | 5 | 8-24 (12) | 899-3880 (1346) | 0.002 with all controls with ESRD |
Newly diagnosed patients | 9 | 0.8-2.7 (1.6) | 0-288 (122) | <0.001 with all controls <5 years |