Suppl. Table 6a. Chronic renal failure
Disease
Mode of
Total
No CRF
CRF
Diagnosis n
n (%)
n (%)
NBS
32
24 (75)
8 (25)
Selective
73
42 (58)
31 (43)
NBS
7
6 (86)
1 (14)
Selective
32
29 (91)
3 (9)
NBS
43
40 (93)
3 (7)
Selective
79
71 (90)
8 (10)
NBS
61
57 (93)
4 (7)
Selective
98
91 (93)
7 (7)
MMA-Cbl-
+
MMA-Cbl
PA
GA1
OR from logistic regression† with covariate age*
MoD: ORa=1.70, 95% CI [0.68;4.53], p=0.263
LR (df=1)=9.59, p=0.002
MoD: ORu=0.51, 95% CI [0.07;5.95], p=0.547
LR (df=1)=0.12, p=0.728
MoD: ORu=1.38, 95% CI [0.40;5.83], p=0.626
LR (df=1)=0.62, p=0.430
MoD: ORu=1.05, 95% CI [0.32;3.84], p=0.940
LR (df=1)=0.47, p=0.494
†According to Firth's bias reduced logistic regression approach with penalized profile likelihood based confidence
intervals for parameter estimates (Heinze et al. 2002; Heinze et al. 2013); *age at first time of CRF for those with
CRF and age at last regular visit for those without CRF; CI, confidence interval; CRF, chronic renal failure; LR,
penalized likelihood ratio test for comparison of models (Heinze et al. 2013); MoD, mode of diagnosis; NBS,
newborn screening; OR, odds ratio (reference group: NBS, reference category: no CRF); OR a, age-adjusted OR (if LR
was significant); ORu, unadjusted OR (if LR was not significant); Selective, selective screening. Age distribution is
specified in Suppl. Table 6b.
Suppl. Table 6b. Distribution of age at first time of chronic renal failure or at last regular visit
respectively by disease and mode of diagnosis
Disease
Mode of Diagnosis
Total n
NBS
32
Selective
73
MMA-Cbl-
Age*
Mean; median
(Q1;Q3)
[min;max] in years
6.72; 5.26
(2.18;10.80)
[0.05;20.39]
9.58; 8.32
(3.28;13.60)
[0.29;38.79]
7.22; 10.13
(1.94;11.70)
[0.28;12.88]
MMA-Cbl+
12.83; 9.40
Selective
32
(5.15;17.07)
[0.12;39.55]
7.92; 6.28
NBS
43
(2.62;13.27)
[0.12;21.10]
PA
11.54; 10.11
Selective
79
(5.28;16.96)
[0.05;28.83]
4.81; 4.00
NBS
61
(2.02;6.46)
[0.09;14.23]
GA1
13.36; 12.49
Selective
98
(6.33;17.26)
[1.64;48.94]
Data are shown as mean; median (Q1;Q3) [min;max]; *Age at first time of CRF for those with chronic renal failure
and age at last regular visit for those without chronic renal failure; NBS, newborn screening; Selective, selective
screening.
NBS
7