Table 4: Studies reporting level of developmental delay in individuals with Cornelia de Lange syndrome
Per i genitori, gli assistenti, i fornitori di assistenza sanitaria e gli insegnanti, sorgono spesso preoccupazioni e domande riguardanti la cura e il benessere degli individui con sindrome di Cornelia de Lange.
Table 4. Studies reporting level of developmental delay in individuals with Cornelia de Lange syndrome 1
Study | Participant characteristics | Assessment | Individuals scoring within categories of developmental delay n (%) | ||||||||
N | Age in years Mean (SD) range | % Male | Molecular confirmation | Profound | Severe | Moderate | Mild | Borderline | Normal | ||
Bhuiyan et al. (2006) | 36 | N/A | N/A | Yes | VABS | 19 (53%) | 6 (16.5%) | 6 (16.5%) | 4 (11%) | 1 (3%) | --- |
Marchisio et al. (2008) | 50 | Median: 6.5 1-18 | 46.0 | No | Categorised based on language and cognition | --- | 20 (40%) | 24 (48%) | 6 (12%) | --- | --- |
Moss et al. (2008) | 34 | 12.4 (3.8) 5-18.96 | 47.2 | No | VABS | 9 (26%) | 16 (47%) | 6 (18%) | 3 (9%) | 0 (0%) | --- |
Nakanishi et al. (2013) | 66 | 14.7 4 – 44 | 47.0 | No | VABS | 9 (14%) | 3 (4%) | 9 (14%) | 35 (53%) | 10 (15%) | --- |
Oliver et al. (2009) | 54 | 13.9 (9.0) | 46 | No | VABS | 27 (50%) | 13 (24%) | 8 (15%) | 6 (11%) | --- | --- |
Richards et al. (2009) | 12 | 11.0 (5.2) 5.0-18.0 | 33.3 | No | VABS | --- | 4 (33%) | 5 (42%) | 3 (25%) | --- | --- |
Selicorni et al. (2007) | 62 | 12.0 0.5-48.0 | 61.3 | Yes | Unknown | 16 (26%) | 0 (0%) | 36 (58%) | 5 (8%) | --- | --- |
Wulffaert et al. (2009) | 37 | 18.1 (13.0) 1.4 – 46.2 | 56.8 | Yes | VABS | 19 (51%) | 6 (16%) | 6 (16%) | 5 (14%) | 1 (3%) | --- |
Yan et al. (2006) | 28 | 10.9 3–27 | 53.6 | Yes | Based on criteria from Gillis et al. (2004) | --- | 15 (54%) | 9 (32%) | 4 (14%) | --- | --- |
|
|
|
|
|
|
Mean (SD); range | |||||
Basile et al. (2007) |
56 |
10.6 (8.5) 1-31 |
51.8 |
No |
VABS |
AE (years) = 3.5 (2.9) 1.5–11
| |||||
Crawford et al. (2015) | 15 | 18.4 (9.8) 6.7-33.4 | 53.3 | No | VABS | SS = 60 (25); 21–121 | |||||
Kline et al. (1993) | 36 | 3.2 – 19.0 | N/A | No | VABS | SS = 48; 20–87 | |||||
Moss et al. (2005) | 8 | 9.8 4.3-14.3 | 62.5 | No | VABS | AE (months) = 25; 6–47 | |||||
Oliver et al. (2006) | 16 | 7.6 1.7-16.1 | 56.3 | No | VABS | AE (months) = 11.6 (4.9); 4–23 | |||||
Srivastava et al. (2014) | 41 | 11.4 (3.8) 5–18 | 43.9 | No | VABS | SS = 38.3 (23.1) | |||||
AE = Age Equivalence; SS = Standard Score; VABS= Vineland Adaptive Behavior Scales
- ^ Inclusion criteria for studies varied and may explain in part differences in scoring results
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