Skip to main content
. Author manuscript; available in PMC: 2013 Aug 27.
Published in final edited form as: Mol Genet Metab. 2011 Oct 7;105(1):34–43. doi: 10.1016/j.ymgme.2011.09.032

Table 5.

Outcome of 294 patients with PDC deficiency.

Deceased Alive
Subunit (No. Cases) Percent Mean Age in Years Range in Years Percent Mean Age in Years Range in Years
Total (294) 36.2 (108) 2.71 ± 4.53 (107) 0.1-35 63.8 (186) 7.1 ± 6.11 (167) 0-21
E1 (57) 42.1 (24) 0.98 ± 1.69 (24) 0-8 57.9 (33) 6.65 ± 5.17 (31) 0.1-22
E1a (163) 36.8 (60) 3.22 ± 5.22 (60) 0-21 63.2 (103) 7.5 ± 6.43 (92) 0.2-35
E1b (9) 44.4(4) 0.65 ± 0.48 (4) 0.1-1.1 55.5 (5) 9.14 ± 10.3 (5) 0.3-27
E2 (4) 0 100 (4) 7.5 ± 4.77 (3) 3.5-11
E3 (13) 50.0(9) 2.40 ± 2.16 (8) 0.3-5.5 50.0 (9) 4.01 ± 2.86 (8) 0.8-5
E3BP (22) 22.7 (5) 4.88 ± 6.67 (5) 0.1-16 77.2 (17) 7.8 ± 7.08 (17) 0.9-24
Unclassified (21) 28.6 (6) 4.5 ± 6.58 (6) 0-16 71.4 (15) 5.78 ± 4.72 (14) 0.8-15