Analysis of fatal outcomes of patients with mucopolysaccharidosis type II according to the Russian mucopolysaccharidosis registry

Table 1 Clinical characteristics of 20 Russian mucopolysaccharidosis II patients who died between 2016-2022 years

ID	Height at birth, cm	1st-year psychomotor delay	Delayed mental and speech	Intellectual disability: 5-12 / > 12 year	Hydrocephalus	Epilepsy	Swallow disorders	Independent walking 5-12 / > 12 year	Hurler pheno-type	Cardio-myopathy	Myxomatous valve degeneration	Respiratory disorders	Tracheostomy	Carpal tunnel syndrome
46	49	No	Yes	No/no	Yes	No	No	Yes/yes	Yes	Yes	Yes	0	0	0
58	N/A	N/A	N/A	N/A/N/A	N/A	N/A	No	N/A/N/A	Yes	N/A	N/A	N/A	N/A	N/A
65	52	No	Yes	Mod/profound	Yes	Yes	Yes	Yes/no	Yes	Yes	Yes	Yes	0	Yes
71	57	Yes	No	No/no	N/A	N/A	N/A	Yes/yes	Yes	N/A	N/A	Yes	N/A	N/A
72	N/A	Yes	Yes	No/no	N/A	Yes	Yes	Yes/no	Yes	No	Yes	Yes	0	0
92	52	No	Yes	No/N/A	Yes	Yes	Yes	No/N/A	Yes	No	Yes	Yes	0	Yes
93	51	No	Yes	Profound/profound	No	Yes	Yes	No/no	Yes	No	Yes	Yes	Yes	Yes
96	52	No	Yes	Severe/severe	No	Yes	Yes	Yes/no	Yes	Yes	Yes	0	0	Yes
104	N/A	Yes	Yes	No/no	Yes	No	Yes	No/no	Yes	Yes	Yes	1	0	N/A
106	50	Yes	Yes	No/not significant	No	N/A	N/A	N/A/N/A	Yes	No	Yes	N/A	N/A	N/A
109	57	Yes	Yes	Moderate/severe	Yes	Yes	Yes	Yes/no	Yes	No	Yes	Yes	0	Yes
110	N/A	No	No	No/no	Yes	No	No	Yes/yes	Yes	No	Yes	Yes	0	Yes
111	57	Yes	Yes	Not significant/profound	Yes	Yes	Yes	Yes/no	Yes	Yes	Yes	Yes	0	Yes
113	51	N/A	N/A	N/A/N/A	N/A	N/A	N/A	N/A/N/A	Yes	N/A	N/A	N/A	N/A	N/A
116	55	Yes	Yes	Severe/N/A	Yes	N	Yes	N/A/N/A	Yes	Yes	Yes	1	0	1
120	N/A	No	No	No/no	N/A	N/A	N/A	Yes/no	N/A	N/A	N/A	N/A	N/A	N/A
124	56	Yes	Yes	No/severe	Yes	No	Yes	Yes/yes	Yes	Yes	Yes	Yes	0	0
144	N/A	No	Yes	No/N/A	Yes	No	No	Yes/N/A	Yes	N/A	N/A	Yes	0	Yes
147	54	No	No	No/no	No	No	No	Yes/yes	Yes	Yes	Yes	Yes	Yes	Yes
149	51	Yes	Yes	Moderate/profound	Yes	Yes	Yes	Yes/no	Yes	Yes	Yes	Yes	Yes	Yes