Early human development · 2009
Pilot use of the early motor repertoire in infants with inborn errors of metabolism: outcomes in early and middle childhood.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Bruggink J L M, van Spronsen F J, Wijnberg-Williams B J, Bos A F
Аннотация
BACKGROUND: Predicting later outcome in neonates presenting with severe inborn errors of metabolism (IEM) is difficult. The assessment of the early motor repertoire is a reliable method of evaluating the integrity of the central nervous system in young infants. This method is based on an age-specific qualitative assessment of general movements (GMs, 0-8 weeks of age), fidgety movements (FMs) and the concurrent motor repertoire (9-20 weeks of age).
AIM: To determine the quality of the early motor repertoire (at 0-20 weeks post term age) in relation to later neurological outcome in infants with severe IEM.
STUDY DESIGN: Prospective cohort study. The quality of the motor repertoire was assessed from serial videotape recordings.
SUBJECTS: Five infants with IEM. Four presented with a severe IEM in the neonatal period: an undefined gluconeogenesis defect, propionic acidemia, arginosuccinate synthetase and arginosuccinate lyase deficiency. One neonate was antenatally diagnosed with arginosuccinate synthetase deficiency.
OUTCOME MEASURES: Outcome at the age of at least 18 m was determined by neurological examination and developmental tests.
RESULTS: All infants initially had abnormal GMs: hypokinesia, followed by GMs of a poor repertoire. The quality of the early motor repertoire normalised in 3 infants, and remained abnormal in 2. The more severe and persistent abnormalities of the motor repertoire were considered with the more abnormal neurological and developmental scores, later on.
CONCLUSIONS: The quality of the early motor repertoire might be related to later neurological outcome in infants with inborn errors of metabolism.
AIM: To determine the quality of the early motor repertoire (at 0-20 weeks post term age) in relation to later neurological outcome in infants with severe IEM.
STUDY DESIGN: Prospective cohort study. The quality of the motor repertoire was assessed from serial videotape recordings.
SUBJECTS: Five infants with IEM. Four presented with a severe IEM in the neonatal period: an undefined gluconeogenesis defect, propionic acidemia, arginosuccinate synthetase and arginosuccinate lyase deficiency. One neonate was antenatally diagnosed with arginosuccinate synthetase deficiency.
OUTCOME MEASURES: Outcome at the age of at least 18 m was determined by neurological examination and developmental tests.
RESULTS: All infants initially had abnormal GMs: hypokinesia, followed by GMs of a poor repertoire. The quality of the early motor repertoire normalised in 3 infants, and remained abnormal in 2. The more severe and persistent abnormalities of the motor repertoire were considered with the more abnormal neurological and developmental scores, later on.
CONCLUSIONS: The quality of the early motor repertoire might be related to later neurological outcome in infants with inborn errors of metabolism.