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Table 2 Results

From: Worldwide view of nephropathic cystinosis: results from a survey from 30 countries

Variables Developing nations Developed nations p
  N Median [min-max]/N (%) N Median [min-max]/N (%)  
Demographic features 109   104   
 Sex ratio (M/F) 84 42/42 (50.0/50.0) 103 52/51 (50.5/49.5) 0.94c
 Age at diagnosis (yrs) 108 1.5 [0.0–33.0] 104 1.3 [0–9.7] 0.04a
 Follow-up until diagnosis 108 4.55 [0.0–26.2] 104 5 [0.75–33.6] 0.02a
 Age at last visit (yrs) 109 8.0 [0.5–35.0] 103 13 [1.2–38.6] 0.001a
 Diagnosis before 2 yrs. of age 108 63 (58.3) 103 68 (66.0) 0.25
 SD weight 77 −3.6 [−9.3–2.3] 101 −0.8 [−6.4–4.2] <0.0001b
 SD height 75 −3.5 [−11.7–1.7] 100 −1.58 [−7.5–1.8] <0.0001a
CTNS mutation analysis 102 23 (22.6) 97 68 (70.1) <0.0001c
 Distance centre – home (km) 68 60 [1–1000] 91 50 [0–20,000] 0.11a
 Number of visit 99 5 [4–7] 97 4 [4–6] 0.74a
Cysteamine Treatment
 Patient treated 106 57 (53.8) 101 101 (100.0) <0.0001c
 Age at treatment (yrs) 57 2.0 [0.0–33.0] 100 1.5 [0.0–12.0] 0.02a
 Patients treated before 2.5 years of age 57 29 (50.9) 100 73 (73.0) 0.005c
 Median dose (mg/m2 per day) 53 1050 [100–1800] 98 1265 [157–2769] 0.0002a
Other treatments
 Indomethacin 95 13 (13.7) 100 58 (58.0) <0.0001c
 rhGH 95 10 (10.5) 100 56 (56.0) <0.0001c
 Tube feeding 95 2 (2.1) 102 27 (26.5) <0.0001d
 Cysteamine eye drops 105 22 (20.9) 98 87 (88.8) <0.0001c
 Number of drops per eye per day 22 6 [1–14] 91 4 [0–20] 0.05a
Renal follow up
 ESRD 109 58 (53.2) 103 39 (37.9) 0.03c
 Age at ESRD 58 8.0 [0.5–17.5] 35 10·0 [4.0–19.5] 0.0008a
 Transplanted patients 56 34 (60.7) 38 31 (81.6) 0.86c
 Delay between ESRD and graft (yrs) 34 2 [0–14] 28 1 [0–11] 0.25a
 Patients on dialysis (PD/HD) 28 22/6 20 9/11 0.02
  1. SD standard deviation, ESRD end stage renal disease, PD peritoneal dialysis, HD hemodialysis
  2. aWilcoxon test
  3. bStudent test
  4. cChi-square test
  5. dFisher test