Cardiac characteristics and natural progression in Taiwanese patients with mucopolysaccharidosis III

Table 7 Six patients with MPS IIIB who had follow-up echocardiographic examinations after 1.9–18.1 years of follow-up compared with the baseline data

MPS type	Gender	Age at baseline (years)	Age at follow-up (years)	Duration (years)	Severity score of MS		Change of severity score	Severity score of MR		Change of severity score	Severity score of AS		Change of severity score	Severity score of AR		Change of severity score
MPS type	Gender	Age at baseline (years)	Age at follow-up (years)	Duration (years)	Baseline	Follow-up	Change of severity score	Baseline	Follow-up	Change of severity score	Baseline	Follow-up	Change of severity score	Baseline	Follow-up	Change of severity score
IIIB	F	5.2	23.3	18.1	0	1	1	0	1.5	2	0	0	0	0	0	0
IIIB	F	5.2	23.3	18.1	0	1	1	1	1.5	0.5	0	0	0	0	0	0
IIIB	M	5.1	7.8	2.7	0	0	0	0	0	0	0	0	0	0	0	0
IIIB	M	5.1	9.3	4.2	0	1	1	0	0	0	0	1	1	0	0	0
IIIB	M	4.2	6.8	2.6	0	0	0	0	1	1	0	0	0	0	0	0
IIIB	M	1.8	3.7	1.9	0	0	0	0	0	0	0	0	0	0	0	0
Mean		4.4	12.4	8.0	0	0.5	0.5	0.2	0.7	0.5	0	0.2	0.2	0	0	0

MPS Mucopolysaccharidosis, MS Mitral stenosis, MR Mitral regurgitation, AS Aortic stenosis, AR Aortic regurgitation. Severity of valvular stenosis and regurgitation (MS, MR, AS, AR) were estimated and graded on the following scores: 0 (none), 1 (mild), 2 (moderate), and 3 (severe)

ISSN: 1750-1172