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Table 3 Associations between DAS-II GCA and VABS-II ABC standard scores and HS-FOCUS items

From: Analysis of cognitive ability and adaptive behavior assessment tools used in an observational study of patients with mucopolysaccharidosis II

HS-FOCUS item

DAS-II GCA score coefficient (standard error)

VABS-II ABC score coefficient (standard error)

Activities (domain score)

 −0.00714 (0.00364)

 −0.01233* (0.00520)

 Go out with friends and/or family

 −0.00823* (0.00396)

 −0.01214* (0.00583)

 Take part in physical activities for 1 h

 −0.00780 (0.00439)

 −0.01337* (0.00614)

Breathing (domain score)

0.00170 (0.00424)

0.00689 (0.00606)

 Do daily activities without losing breath

 −0.00427 (0.00472)

0.00692 (0.00682)

 Sleep without snoring

0.00635 (0.00612)

0.01870* (0.00857)

 Talk to someone else without losing breath

0.00171 (0.00421)

 −0.00049 (0.00623)

 Breathe without making noise at rest

0.00369 (0.00538)

0.00747 (0.00781)

Grip/reach (domain score)

 −0.00775** (0.00277)

 −0.01074** (0.00411)

 Place palm and fingers flat on a table

 −0.01190 (0.00611)

 −0.00676 (0.00862)

 Throw/catch a medium-sized ball

 −0.00927* (0.00390)

 −0.01107 (0.00557)

 Pour a drink into a cup

 −0.00688 (0.00559)

 −0.01340 (0.00788)

 Reach all parts of his body

 −0.01549* (0.00702)

 −0.00972 (0.00961)

 Button a shirt

 −0.00832 (0.00672)

 −0.01516 (0.00915)

 Put on shoes while sitting in a chair

0.00344 (0.00627)

 −0.01324 (0.00923)

 Touch the top of his head

 −0.00838 (0.00530)

 −0.00844 (0.00775)

 Turn pages in a book

 −0.00662* (0.00306)

 −0.00616 (0.00443)

School/work (domain score)

 −0.01221*** (0.00304)

 −0.00853 (0.00452)

 Finish all classroom assignments

 −0.01551** (0.00495)

 −0.00842 (0.00724)

 Attend a full day of school or work

 −0.00488 (0.00353)

 −0.00522 (0.00491)

Walking/standing (domain score)

 −0.00163 (0.00230)

 −0.00230 (0.00325)

 Walk up a flight of stairs

0.00267 (0.00243)

0.00151 (0.00345)

 Stand without getting tired for 10 min

0.00055 (0.00337)

0.00067 (0.00489)

 Step on a stool

 −0.00695 (0.00339)

 −0.00116 (0.00493)

 Walk without getting tired for 15–20 min

0.00468 (0.00430)

0.00340 (0.00604)

 Walk for 20 m (yards) without stopping

0.00531 (0.00507)

 −0.00842 (0.00722)

  1. Bold font indicates statistically significant associations and asterisks denote p values (*p < 0.05, **p < 0.01, ***p < 0.001). A negative association indicated that increasing cognitive function/adaptive behavior (assessed using DAS-II GCA/VABS-II ABC scores) was associated with improved functional performance (assessed using the HS-FOCUS)
  2. ABC Adaptive Behavior Composite; DAS-II Differential Ability Scales, second edition; GCA General Conceptual Ability; HS-FOCUS Hunter Syndrome-Functional Outcomes for Clinical Understanding Scale; VABS-II Vineland Adaptive Behavior Scales, second edition