Table 3.

Clinical severity for cases with and without C-terminal deletions and by type and position of C-terminal deletion

Severity score	Factor	Univariate			Multivariate with age group and data source
		Mean	(95% CI)	p Value	Adjusted mean	(95% CI)	p Value
Kerr	No C-terminal deletion	15.68	(15.26 to 16.09)	0.237	15.7	(15.33 to 16.07)	0.069
	C-terminal deletion	14.91	(13.7 to 16.11)		14.91	(13.84 to 15.98)
	In-frame C-terminal deletion	15.55	(11.39 to 19.72)	0.753	15.55	(12.17 to 18.94)	0.548
	Frameshift C-terminal deletion	14.86	(13.49 to 16.24)		14.86	(13.75 to 15.98)
	Before L386	16.94	(13.5 to 20.39)	Baseline	16.94	(14.19 to 19.69)	Baseline
	L386 to P388	14.49	(12.68 to 16.3)	0.211	14.49	(13.06 to 15.93)	0.142
	P389 and after	14.38	(11.67 to 17.09)	0.248	14.38	(12.24 to 16.52)	0.192
Percy	No C-terminal deletion	21.38	(20.76 to 21.99)	0.188	21.43	(20.86 to 22)	0.065
	C-terminal deletion	20.12	(18.36 to 21.89)		20.12	(18.49 to 21.75)
	In-frame C-terminal deletion	21.24	(15.55 to 26.93)	0.694	21.24	(16.46 to 26.02)	0.21
	Frameshift C-terminal deletion	20.05	(18.19 to 21.92)		20.05	(18.49 to 21.62)
	Before L386	21.72	(17.02 to 26.42)	Baseline	21.72	(17.79 to 25.65)	Baseline
	L386 to P388	20.43	(17.95 to 22.91)	0.628	20.43	(18.36 to 22.5)	0.367
	P389 and after	18.11	(14.4 to 21.82)	0.233	18.11	(15.03 to 21.2)	0.212
Pineda	No C-terminal deletion	16.18	(15.83 to 16.52)	0.036	16.2	(15.86 to 16.54)	0.018
	C-terminal deletion	14.99	(13.94 to 16.05)		14.99	(13.96 to 16.02)
	In-frame C-terminal deletion	16.39	(13.21 to 19.58)	0.396	16.39	(13.52 to 19.27)	0.138
	Frameshift C-terminal deletion	14.96	(13.91 to 16)		14.96	(14.01 to 15.9)
	Before L386	16.51	(14.29 to 18.73)	Baseline	16.51	(14.49 to 18.54)	Baseline
	L386 to P388	15.19	(13.83 to 16.56)	0.318	15.19	(13.95 to 16.44)	0.389
	P389 and after	13.38	(11.43 to 15.32)	0.036	13.38	(11.59 to 15.16)	0.032