Resumo

ROSSATO, Norma E. et al. Secuencia de hipokinesia fetal (Fenotipo de Pena-Shokeir tipo I): Sobrevida y calidad de vida. Enfoque multidisciplinario. Arch. argent. pediatr. [online]. 2005, vol.103, n.5, pp.430-434. ISSN 0325-0075.

Fetal akinesia deformation sequence was described as a syndrome characterized by multiple ankyloses, facial anomalies and pulmonary hypoplasia secondary to abnormal motor neurons. Indeed, this is a heterogeneous entity associated to decreased fetal activity (myogenic, neurogenic, functional, ischemic, anoxic). Most patients are stillborn and the majority of liveborn, die during the neonatal period with pulmonary hypoplasia complications. We report an infant with this pathology. Nowadays, the girl is 4 years and 5 months old, lives at her home with special cares. During the first year she required intensive care for severe pulmonary complications. After this, a multidisciplinary team, obtained the social and familiar reinsertion with an adequate quality of life.

Palavras-chave : Pulmonary hypoplasia; Arthrogryposis multiplex; Discapacity and quality of life; Multidisciplinary approach.

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