Neurodevelopmental outcomes at five years after early-onset fetal growth restriction: Analyses in a Dutch subgroup participating in a European management trial.

OBJECTIVE: The objective of this study is to explore developmental outcomes at five years after early-onset fetal growth restriction (FGR). STUDY DESIGN: Retrospective data analysis of prospective follow-up of patients of three Dutch centres, who participated in a twenty centre European randomized controlled trial on timing of delivery in early-onset FGR. Developmental outcome of very preterm infants born after extreme FGR is assessed at (corrected) age of five. RESULTS: Seventy-four very preterm FGR children underwent follow-up at the age of five. Mean gestational age at birth was 30 weeks and birth weight was 910 g, 7% had a Bayley score <85 at two years. Median five years' FSIQ was 97, 16% had a FSIQ < 85, and 35% had one or more IQ scores <85. Motor score ≤ 7 on movement ABC-II (M-ABC-II-NL) was seen in 38%. Absent or reversed end-diastolic flow, gestational age at delivery, birthweight and neonatal morbidity were related to an FSIQ < 85. Any abnormal IQ scale score was related to birthweight, male sex and severity of FGR, and abnormal motor score to male sex and bronchopulmonary dysplasia (BPD). CONCLUSIONS: Overall, median cognitive outcome at five years was within normal range, but 35% of the children had any abnormal IQ score at age five, depending on the IQ measure, and motor impairment was seen in 38% of the children. GA at delivery, birthweight, EDF prior to delivery and neonatal morbidity were the most important risk factors for cognitive outcomes.

Pilot use of the early motor repertoire in infants with inborn errors of metabolism: outcomes in early and middle childhood.

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.

Specificity of specific language impairment.

In children with specific language impairment (SLI) their problems are supposed to be specifically restricted to language. However, both on theoretical basis as well as on a practical basis it is often difficult to make a sharp distinction between specific and non-specific language disorders. In a three-step study we found in the first place that in a group of children with supposed SLI (n = 319), after multidisciplinary examination, only in 25% of the cases the diagnosis SLI was correct. Secondly we found that after a period of time children with correctly diagnosed SLI showed problems with attention, motor functioning and school achievement. Thirdly we found that the learning difficulties of the children were related to their previous language problems. We hypothesize that SLI is less specific than is generally assumed. It could be a part of a neuropsychological 'syndrome' in which neurological maturation is jeopardized.

[Children with a presumably isolated speech-language disorder often have medical or cognitive problems as well]. / Bij kinderen met een vermoedelijk geïsoleerde spraak-taalstoornis vaak tevens medische of cognitieve problemen.

OBJECTIVE: To assess the language problems of children who otherwise appear to develop normally. DESIGN: A clinical inventory. SETTING: University Hospital Groningen. METHOD: The children (n = 319) were examined according to a standard protocol consisting of: ENT examination, audiologic examination, paedogogic observation and logopaedic examination. Further psychological examination was carried out when needed according to the paedagogic observation. RESULTS: 25 per cent of the children had isolated language problems. For these children logopaedic therapy was indicated. In 75 per cent, a clear associated medical factor, or a retarded cognitive and (or) motor development, was found. Forty per cent of these children received medical treatment and 35 per cent specific paedagogic guidance. In some cases additional logopaedic therapy was given. CONCLUSION: It appears advisable to give children with language problems logopaedic therapy only after a diagnostic examination has been carried out according to a standard protocol as described.