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Table 5 Behaviour-related domain scores (10–15 year olds)

From: An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III

 

MPS III median ( N, range)

ID median ( N, range)

p value

ECBI

   

 Intensity score

76 (8, 36–174)

155 (7, 74–201)

0.028

 Problem score

1 (7, 0–21)

14.5 (6, 1–29)

0.037

ABC

   

 Irritability

2 (9, 0–24)

23 (7, 1–40)

0.043

 Lethargy

7 (9, 2–28)

7 (7, 4–27)

0.915

 Stereotypy

2 (9, 0–14)

5 (7, 8)

0.183

 Hyperactivity

7 (9, 2–28)

23 (7, 1–41)

0.152

 Inappropriate speech

0 (9,0- 5)

4 (7, 0–12)

0.054

 ABC total score

26 (9, 6–80)

54 (7, 11–106)

0.081

SBRS

   

 Current understanding

10 (10, 2–38)

33 (7, 14–41)

0.019

 Past understanding

28 (9, 16–42)

42 (2, 4–20)

0.056

 Current expression

2 (10, 0–12)

11 (7, 5–23)

0.004

 Past expression

12 (9, 6–24)

12 (2, 4–20)

0.813

 Orality

20 (10, 2–36)

15 (7, 0–26)

0.243

 Body movements

10 (10, 4–30)

10 (7, 0–23)

0.590

 Interactions with objects

11 (10, 3–19)

8 (7, 0–15)

0.240

 Activity and routines

14 (10, 4–30)

20 (7, 6–26)

0.845

 Emotional function

6 (10, 0–13)

5 (7, 2–14)

0.883

 Safety consciousness

12 (10, 0–18)

8 (7, 3–14)

0.352

 Fearfulness

28 (10, 12–35)

16 (7, 8–36)

0.405

 Social interaction

13.5 (10, 2–22)

17 (7, 8–25)

0.282

 Eye contact

3 (10, 0–8)

6 (7, 0–10)

0.258

 Emotional engagement

7.5 (10, 4–14)

6 (7, 2–13)

0.257

 Comfort seeking

10 (10, 8–14)

11 (7, 0–20)

0.428

 Attention

12 (10, 6–18)

11 (7, 3–18)

0.883

 Self-control/compliance

6 (10, 0–16)

10 (7, 4–18)

0.281

 Mood, anger and aggression

5.5 (10, 0–42)

19 (7, 2–33)

0.117

 Self-gratification

0 (10, 0–2)

2 (7, 0–5)

0.217