Profile of skills of development in children with Holoprosencephaly and Holoprosencephaly-like
Perfil de habilidades do desenvolvimento em crianças com Holoprosencefalia e Holoprosencefalia like
Tatiana Vialogo Cassab; Dionísia Aparecida Cusin Lamônica; Neivo Luiz Zorzetto; Célia Maria Giacheti
Abstract
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.
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Resumo
Objetivo: investigar e comparar o desempenho nas habilidades relacionadas ao desenvolvimento motor, cognitivo, linguístico, de socialização e autocuidados de indivíduos com holoprosencefalia e com holoprosencefalia-like.
Método: participaram deste estudo 20 indivíduos com diagnóstico de holoprosencefalia, na faixa etária de 18 a 72 meses, de ambos os sexos, divididos em 2 grupos. O grupo 1 (G1) composto por 12 indivíduos com sinais clínicos do espectro da holoprosencefalia, e o grupo 2 (G2) com holoprosencefalia-like composto por 8 indivíduos com sinais clínicos do espectro da holoprosencefalia-like. A coleta de dados foi realizada por meio da aplicação do Inventário Portage Operacionalizado que avalia as áreas alvos deste estudo. Para a análise estatística utilizou-se análise descritiva da mediana e dos valores mínimos e máximos e foi aplicado o teste estatístico de Mann Whitney (≤ 0,05% para significância).
Resultados: os grupos 1 e 2 apresentaram alterações em todas as áreas do desenvolvimento avaliadas. Entretanto, os indivíduos do G1, com holoprosencefalia apresentaram maiores comprometimentos nas habilidades: motora, cognitiva, de linguagem, de socialização e autocuidados, quanto comparados aos indivíduos do G2, com holoprosencefalia-like.
Conclusão: o desempenho nas áreas motoras, cognitivas, de linguagem, de socialização e autocuidados de indivíduos com holoprosencefalia e holoprosencefalia-like foi aquém do esperado, principalmente naqueles indivíduos com holoprosencefalia, que se justifica pelo maior comprometimento no sistema nervoso central. A natureza destas alterações pode estar associada ao universo de alterações neurológicas e craniofaciais descritas nestes quadros clínicos e também à influência do ambiente social.
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Referencias
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Submitted date:
04/11/2010
Accepted date:
26/04/2011
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