Skip to main content

Table 1 Chitotriosidase activity in control groups and cystinotic patients

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
  1. *WBC cystine in nmol ½ cystine/mg protein; ESRD, end stage renal disease.