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Revista CEFAC

On-line version ISSN 1982-0216

Abstract

CASSAB, Tatiana Vialogo; LAMONICA, Dionísia Aparecida Cusin; ZORZETTO, Neivo Luiz  and  GIACHETI, Célia Maria. Profile of skills of development in children with Holoprosencephaly and Holoprosencephaly-like. Rev. CEFAC [online]. 2012, vol.14, n.3, pp. 423-429.  Epub Oct 14, 2011. ISSN 1982-0216.  http://dx.doi.org/10.1590/S1516-18462011005000109.

PURPOSE: to investigate and compare the performance skills related to motor development, cognition, language, socialization and self-care of subjects with holoprosencephaly, and holoprosencephaly-like. METHOD: the study included 20 subjects diagnosed with holoprosencephaly, aged 18 up to 72 months old of both genders, divided in 2 groups. Group 1 (G1) consisting of 12 subjects with clinical signs of the spectrum of holoprosencephaly and group 2 (G2) with holoprosencephaly-like composed of 8 subjects with clinical signs of the spectrum of holoprosencephaly-like. Data collection was performed, applying Operating Portage Inventory which assesses the areas targeted in this study. For statistical analysis, descriptive analysis of the median and the minimum and maximum test was applied to the Mann Whitney (< 0.05% for significance). RESULTS: groups 1 and 2 showed changes in all assessed areas of development. However, subjects of G1 with holoprosencephaly showed greater impairments in motor skills, cognitive, language, socialization and self-care, as compared with subjects of G2, with holoprosencephaly-like. CONCLUSION: the profile of motor skills, cognitive, language, socialization and self-care of subjects with holoprosencephaly, and holoprosencephaly-like, according to age groups was above the expected, especially for subjects with holoprosencephaly, which is justified by greater involvement in the nervous system central. The nature of these changes may be related to the universe of neurological and craniofacial described in these clinical pictures and also the influence of social environment.

Keywords : Cognition; Language; Motor Activity.

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