One-year follow-up of the outcome of a randomized controlled trial of a home-based intervention programme for children with autism and developmental delay and their families.

INTRODUCTION: There is debate about the type and intensity of early childhood intervention that is most helpful for children with developmental problems. The aim of the study was to determine whether a home-based programme provided over 12 months resulted in sustained improvement in development and behaviour 12 months after the intervention ceased. The characteristics of the children and families who benefited most from the intervention were also studied. METHOD: Randomized controlled trial. Participants A total of 59 children, aged 3-5 years, attending two early childhood intervention centres in Melbourne, Australia. Intervention Half of the subjects received an additional home-based programme consisting of 40 weekly visits. MAIN OUTCOME MEASURES: Bayley Scales of Infant Development and Wechsler Preschool and Primary Scale of Intelligence Revised, Preschool Behaviour Checklist, Bayley Behaviour Rating Scale and Behaviour Screening Questionnaire. All tests administered pre-intervention, following the intervention and 12 months later. Secondary outcome measures Family stress, support and empowerment. RESULTS: Fifty-four children completed the assessments 12 months after conclusion of the intervention. Compared with the control group, improvement in aspects of cognitive development in the children who received the extra intervention was sustained 1 year later (P= 0.007) while significant behavioural differences post intervention were not. Analyses of the data by the Reliable Change Index indicated improvement of clinical significance occurred in non-verbal areas. In contrast to the control group who deteriorated, language skills in the intervention group remained stable. Improvements were significantly associated with higher stress in the families. CONCLUSION: Improvements following the provision of a home-based programme to preschool children with developmental disabilities were sustained 1 year later. Children from highly stressed families appeared to benefit most, reinforcing the importance of involving families in early childhood intervention programmes.

Auditory function at 14 years of age of very-low-birthweight.

The aim of the study was to determine audiological function at 14 years of age of very-low-birthweight (VLBW < or = 1500 g) children compared with a cohort of normal birthweight (NBW > 2499 g) children. Participants were consecutive surviving preterm children of birthweight < 1000 g born between 1977 and 1982 (n=86) and of birthweight 1000 to 1500 g born between 1980 and 1982 (n=124) and randomly selected NBW children born between 1981 and 1982 (n=60). Audiometric tests included pure tone audiometry, tympanometry, stapedius muscle reflexes, and measures of central auditory processing. Psychometric tests included measures of IQ, academic achievement, and behaviour. There were no significant differences in rates of hearing impairment, abnormal tympanograms, figure-ground problems, or digit recall between VLBW children and NBW control children. VLBW children had higher rates of some central auditory processing problems, which in turn were associated with poorer intellectual, academic, and behavioural progress.

Cognition, academic progress, behavior and self-concept at 14 years of very low birth weight children.

The aim of this study was to compare cognition, academic progress, behavior, and self-concept children of very low birth weight (VLBW, birth weight < 1501 g) born in the period 1980 to 1982 with randomly selected children of normal birth weight (NBW, birth weight > 2,499 g). At 14 years of age, 130 (84.4%) of 154 VLBW and 42 (70.0%) of 60 NBW children were assessed. Ten VLBW children and one NBW child who had cerebral palsy were excluded. VLBW children scored at a significantly lower level on all three composite scales of the Wechsler Intelligence Scale for Children, 3rd Edition. VLBW children were also significantly disadvantaged on more specific cognitive processes, including tests of visual processing and visual memory and on subtests reflecting learning and problem solving. Only in arithmetic was a difference between the groups discerned on tests of achievement. Significantly more VLBW children were rated by teachers as socially rejected and by their parents as having learning problems at school. VLBW children had significantly reduced self-esteem. VLBW children had more cognitive, academic, and behavioral problems and lower self-esteem at 14 years of age than NBW control subjects.

Newborn screening for congenital hypothyroidism, Victoria, Australia, 1977-1997. Part 1: The screening programme, demography, baseline perinatal data and diagnostic classification.

Clinical, demographic and laboratory data from infants with congenital hypothyroidism (CH) born in the Australian state of Victoria from the commencement of neonatal screening in mid-1977 until December 1988 are reported. These provide a baseline for a 12-year prospective longitudinal study on physical and neuro-psychological outcome until mid-1997, the subject of a second paper. Infants with CH were detected using a primary TT4 screening test. Demographic data were collected prospectively using a clinical assessment protocol. Nearly all affected infants underwent 99mTc pertechnetate scanning at the initial assessment to determine the underlying aetiology of their hypothyroidism. 704,723 infants were screened and 199 with permanent primary hypothyroidism (one in 3,541) were identified. The most common aetiologies were thyroid ectopia (46%), thyroid aplasia (33%), and 'dyshormonogenesis' (11%). The clinical abnormalities classically described in CH were more evident in infants with aplasia, and the striking female preponderance in infants with thyroid dysplasia (syn. dysgenesis) was confirmed. Other features included increased frequencies of 'dyshormonogenesis' in infants of parents of Middle-Eastern origin and of labour induction in infants with dysplasia. A closed posterior fontanelle was not found in any infant with thyroid aplasia.

Newborn screening for congenital hypothyroidism, Victoria, Australia, 1977-1997. Part 2: Treatment, progress and outcome.

A controlled longitudinal prospective study is reported of physical and neuropsychological progress up to 12 years in 152 children with congenital hypothyroidism (CH), detected by newborn screening in the Australian state of Victoria and born between the onset of screening in mid-1977 and December 1988. Linear growth of the CH children was normal. Throughout they were slightly heavier and the median head circumference was slightly larger compared with reference data. Those with thyroid aplasia required a marginally larger dose of thyroxine to achieve euthyroidism. Assessment of cognitive outcome in the children with permanent primary CH revealed the mean scores at 2, 5 and 8 years to be from 8.5 (p<0.001) to 10.2 (p<0.001) points lower than in a group of 60 euthyroid controls. However, there was large overlap and, of the affected children, only 10.1% at 2 years, 3.9% at 5 years and 6.8% at 8 years fell more than 2 SD below the means of the euthyroid controls. On univariate analysis, variables shown to have significant correlation with cognitive outcome at 8 years in the CH children were newborn activity, baseline TT4 and FTI, initial T4 dosage, socio-economic classification, maternal age, maternal education and presence of a serious accompanying disorder. On multiple regression analysis, significant variables were baseline bone age, maternal age and education, and presence of a serious accompanying disorder. No single thyroidal or extra-thyroidal variable could be identified to account for the discrepancy between the children with CH and the controls.

Antenatal corticosteroids and outcome at 14 years of age in children with birth weight less than 1501 grams.

OBJECTIVE: To determine whether exposure to antenatal corticosteroid therapy was associated with adverse effects on growth, sensorineural outcome, or lung function of children of birth weight <1501 g at 14 years of age. DESIGN: Cohort study. SETTING: The Royal Women's Hospital, Melbourne, Australia. SUBJECTS: One hundred fifty-four consecutive survivors born from October 1, 1980 to March 31, 1982. INTERVENTIONS: The mothers of 78 survivors (51%) had been given corticosteroids antenatally to accelerate fetal lung maturation. Treatment with antenatal corticosteroids was nonrandom. No mother received >1 course of corticosteroids. OUTCOME MEASURES: The children were assessed at 14 years of age, corrected for prematurity. All assessors were unaware of the exposure of the child to antenatal corticosteroids. The assessments included measurements of growth and neurological, cognitive, and lung function. Growth measurements were converted into z scores (standard deviation) for the appropriate age and gender. RESULTS: Of the 154 survivors, 130 (84%) were assessed at 14 years of age. Overall, the children exposed to antenatal corticosteroids were significantly taller (height z score; mean difference:.39; 95% confidence interval:.001-. 79) and had better cognitive functioning (Wechsler Intelligence Scale for Children-Third Edition Full Scale; IQ mean difference: 6. 2; 95% confidence interval:.8-11.6) than those not exposed to corticosteroids. There were no other differences in sensorineural outcomes between the groups. Lung function was not significantly different between the groups. No conclusions were altered by adjustment for confounding variables. CONCLUSIONS: Exposure to 1 course of antenatal corticosteroid therapy was associated with some clinically and statistically improved outcomes at 14 years of age in children of birth weight <1501 g, with no obvious adverse effects on growth or on sensorineural, cognitive, or lung function. corticosteroids, growth, cognitive, IQ, lung function, adolescence.

Methylxanthines and sensorineural outcome at 14 years in children < 1501 g birthweight.

OBJECTIVES: Methylxanthines, including theophylline, have been used extensively and successfully to treat apnoea in preterm infants. However, long-term consequences of such therapy are largely unknown. The aim of this study was to determine the relationship between theophylline therapy and outcome at 14 years of age in surviving preterm children of birthweight < 1501 g. METHODOLOGY: The subjects of this study were 154 consecutive survivors with birthweights < 1501 g born from 1 October 1980 to 31 March 1982; 130 (84.4%) were assessed at 14 years of age. Outcomes included motor function, psychological test scores, and growth. RESULTS: Of the 130 children assessed, 69 (53.1%) had been exposed to theophylline; 13.0% had cerebral palsy, significantly higher than 1.6% in the 61 children not exposed to theophylline (P < 0.02). This difference remained statistically significant after adjusting for potential confounding variables including the presence of cerebroventricular haemorrhage. In contrast, after adjusting for known confounding variables, children who had received theophylline achieved higher psychological test scores. There was no association between theophylline therapy and growth. CONCLUSIONS: Theophylline therapy in the newborn period is associated with some evidence of harmful, but also helpful sensorineural effects at 14 years of age.

The obstetrician and the extremely immature fetus (24-26 weeks): outcome to 5 years of age.

The aims of this study were to determine the outcome to 5 years of age for fetuses 24-26 weeks of gestational age from the obstetric viewpoint, and to determine if their outcome has improved over time. Consecutive fetuses with gestational ages from 24-26 weeks born at the Royal Women's Hospital, Melbourne, during 2 separate eras, Era 1 (1977-1982; n = 198) and Era 2 (1985-1987; n = 128) were studied and their outcome to 5 years of age determined. Fetuses referred with lethal malformations or clearly dead before the onset of labour were excluded. The stillbirth rates were similar in both eras (Era 1 23.7%, Era 2 21.9%), but the proportion of survivors to 5 years of age was much higher in Era 2 (Era 1 19.7%, Era 2 30.5%, X2 = 5.0, p < 0.03; odds ratio 1.80; 95% confidence interval [CI] 1.07 to 3.04). Overall, both the proportion and the absolute number of severely disabled children fell over time; 4 children survived with severe sensorineural disability in the 5 1/4 years of Era 1, but only one child in the 3 years of Era 2. From the obstetric viewpoint, only 1.5% of total births survived with a severe sensorineural disability, no higher than the rate expected for children born at term. Fetuses born at 24-26 weeks of gestational age need not contribute disproportionately to the number of severely disabled children in the community; furthermore, their outcome is improving over time. From the obstetrician's viewpoint, survival chances rather than sensorineural outcome should dominate decision-making at these extremely preterm gestations.

Levels of workplace exposure.

A brief description was presented of the evolution of workplace exposure monitoring. Measurements began in the 1930s and developed through particle counting to the introduction of the membrane filter, light microscope method in the mid-1960s. It is important to be aware that most data from the past were collected in relation to workplace control. The membrane filter phase contrast microscope method provides an index of exposure. Fibres with diameters less than 0.2 microns are unlikely to be counted. The method is not capable of analysing individual fibre types and consequently all fibres are counted. There have been several changes to the detail of the membrane filter method. The possible effect of these changes were presented. The Asbestos International Association (AIA) has implemented a fibre counting performance trial and also an annual return of workplace monitoring data. A WHO project is in progress with the objective of harmonizing the membrane filter method for fibre counting of all fibre types using phase contrast microscopy.

Cognition, school performance, and behavior in very low birth weight and normal birth weight children at 8 years of age: a longitudinal study.

Cognition, school performance, and behavior were assessed at 8 years of age in 132 very low birth weight (VLBW) children free of major sensorineural impairments, and the results were contrasted with a randomly selected control group of normal birth weight (NBW) children. Considering their fragile beginnings, the majority of VLBW children were developing normally and were reading and performing in most academic and social areas as well as the NBW children. However, VLBW children were significantly inferior to NBW children on tests of cognition, including tests of intelligence and visual memory, and on teacher's reports of motor skills and initiative. In addition, proportionally more VLBW children (20.5%) than NBW children (5.9%) were reported by their parents to be not coping at school.

Sensorineural outcome at 2 years for survivors of erythroblastosis treated with fetal intravascular transfusions.

OBJECTIVE: To determine the sensorineural outcome at 2 years of age in a complete cohort of survivors of fetal intravascular transfusions. METHODS: From March 1984 to May 1990, 38 of 52 consecutive fetuses (73%) suffering from severe erythroblastosis survived attempted intravascular transfusions at the Royal Women's Hospital, Melbourne. At 2 years of age, corrected for prematurity where appropriate, the survivors had a psychological assessment, including the mental developmental index of the Bayley scales, and a standardized neurodevelopmental examination. RESULTS: Only one transfused child had a severe sensorineural disability, with severe developmental delay and multiple minor motor seizures. Another child was moderately disabled with spastic hemiplegia. In neither case were complications of an intravascular transfusion the likely explanation for the disability. Only one other child had a mental developmental index in the suspect range. The remaining 35 children (92.1%) had no sensorineural disability. The overall rate of sensorineural impairments and disabilities was lower in the group transfused than in previous reports of survivors of intraperitoneal transfusions. The mean mental developmental index was significantly higher in the transfused group than in a control group of normal birth weight children. CONCLUSION: Children who survive fetal intravascular transfusions compare favorably not only with other high-risk survivors, but also with low-risk children.

Audiologic assessment of extremely low birth weight infants: a preliminary report.

The aim of this study was to assess all relevant aspects of auditory function, including acuity and perception, of a cohort of extremely low birth weight (< 1000 g) children who survived to 8 years of age; 42 of the 59 consecutive survivors born over a 4-year period from January 1, 1977, had a full auditory assessment. Of the 42 children, 4 (9.5%) had a sensorineural hearing impairment, 5 (11.9%) had a conductive hearing impairment, 24 (57.1%) had figure/ground differentiation problems, and 20 (47.6%) had a short-term auditory memory problem. The 4 children with sensorineural hearing impairments had had significantly higher maximum concentrations of bilirubin in the newborn period (median 167 mumol/L vs 138 mumol/L and had required more intensive care; at 8 years of age they were significantly disadvantaged in verbal ability. The 5 children with conductive hearing impairments were not significantly different on any perinatal or other 8-year outcome variables. The proportion with figure/ground differentiation problems (57.1%) was significantly higher than in a normative population (11.7%, chi 2 = 24.2). Extremely low birth weight children with figure/ground differentiation problems were more likely to be restless in the classroom (45.0% [9/20]) than those without these problems (16.7% [2/12]), but the difference was not statistically significant (chi 2 = 2.7). Children with short-term auditory memory problems had significantly higher maximum bilirubin concentrations in the newborn period (median 152 mumol/L vs 137.5 mumol/L). At 8 years of age they had significantly reduced intelligence and reading ability.(ABSTRACT TRUNCATED AT 250 WORDS)

Improvement in outcome for very low birthweight children: apparent or real?

OBJECTIVE: To determine whether improvement in the survival rate of infants with a birthweight of less than 1501 g was accompanied by an increase in the rate of neurological impairment or disability among the survivors. DESIGN, SETTING AND PATIENTS: Two cohorts of consecutive very low birthweight infants (birthweight less than 1501 g) in one tertiary perinatal centre were followed prospectively to eight years of age; for both cohorts, comparison groups of children of birthweight more than 1501 g were randomly selected from hospital births. INTERVENTIONS: The first cohort was born before the introduction of assisted ventilation (1966-1970), the second after assisted ventilation was well established (1980-1982). MAIN OUTCOME MEASURES: Comparisons between cohorts, at eight years of age, of the survival rates and the rates of severe sensorineural impairments and disabilities. RESULTS: The survival rate for very low birthweight infants to eight years of age almost doubled between these cohorts, from 37.1% to 67.8% (odds ratio [OR], 3.4; 95% confidence interval [CI], 2.5-4.7; chi 2 = 57.6; P much less than 0.0001). The biggest gain was the increase in non-disabled survivors at eight years of age, from 52.6% in the first cohort to 80.8% in the second cohort (OR, 3.5; 95% CI, 2.2-5.7; chi 2 = 26.7; P less than 0.0001). Furthermore, the rate of severe disabilities in survivors fell substantially, from 13.6% to 4.1% (OR, 0.31; 95% CI, 0.14-0.69; chi 2 = 8.3; P less than 0.01). Of specific impairments, the rate of severe sensorineural deafness fell substantially (3.2% to 0%: OR, 0.14, 95% CI, 0.02-0.81; chi 2 = 4.8; P less than 0.05), as did the rate of severe intellectual impairment (13.0% to 2.7%: OR, 0.25; 95% CI, 0.11-0.57; chi 2 = 10.7; P less than 0.002). Only the rate of cerebral palsy increased, but not significantly (2.6% to 6.8%; OR, 2.6; 95% CI, 0.89-7.6; chi 2 = 3.0). CONCLUSIONS: It has been possible to improve the survival rate of very low birthweight infants over time without increasing the number of severely disabled survivors. Whether the long-term outcome for these infants is continuing to improve with more recent advances in perinatal care remains to be determined.

Changing obstetric practice and 2-year outcome of the fetus of birth weight under 1000 g.

The aim of this study was to assess the outcome up to 2 years of age for the fetus of birth weight 500-999 g, over time and in association with changes in obstetric care. Two consecutive cohorts of infants of birth weight 500-999 g were compared from two eras, 1977-1982 and 1985-1987, and their outcome up to 2 years of age was determined with particular emphasis on the effect of various obstetric interventions at the time of birth, such as cesarean delivery, electronic fetal monitoring, antenatal steroid therapy, and tocolytic therapy. The outcome to 2 years was analyzed by logistic function regression to adjust for imbalances in confounding perinatal variables. In the latter era, the survival rate to 2 years increased significantly by almost 50%, and only 7% of the survivors were severely disabled. The rates of delivery by cesarean and of electronic fetal monitoring both increased significantly in the latter era, but neither was associated with the improved outcome. The only variable associated with an improved outcome that was amenable to obstetric intervention at the time of birth was antenatal steroid therapy, which was used equally in both eras. The obstetrician may aid the fetus of birth weight 500-999 g by giving the mother steroids to accelerate fetal lung maturity, but cesarean cannot be recommended as the routine mode of delivery unless there are recognized maternal or fetal indications.

Very low birth weight and growth to age 8 years. II: Head dimensions and intelligence.

The occipitofrontal circumference was measured in all available children in the following cohorts at ages 2, 5, and 8 years: group 1, consisting of 79 children with birth weight between 500 and 999 g; group 2, with 111 children with birth weight between 1000 and 1499 g; and group 3 with 56 children with birth weight greater than 2500 g; all were white with no signs of moderate or severe cerebral palsy. National Center for Health Statistics reference values indicated substantially more children with an occipitofrontal circumference lower than the 10th percentile, particularly at age 2 years, compared with Nellhaus reference data. Occipitofrontal circumference was the head measurement best correlated with the Full Scale IQ on the Wechsler Intelligence Scale for Children-Revised. Dolichocephaly, often seen in very-low-birth-weight children was unrelated to IQ, and correction of occipitofrontal circumference for dolichocephaly was rarely of clinical importance.

Survivors of extreme prematurity--outcome at 8 years of age.

Of 149 infants liveborn in a large maternity hospital in 1980 to 1982 and delivered between 24 and 29 completed weeks of gestation inclusive, 91 (61%) survived; 88 (97%) survivors were assessed at 8 years' corrected age; 77% of children were not disabled; disability was mild in 13%, moderate in 2% and severe in 4% of children. Although survival decreased with decreasing gestation, disability in survivors did not increase. An earlier assessment of the same children at approximately 2 years of age had been unduly pessimistic particularly for those born less than or equal to 26 weeks' gestation. The only other reports in the literature on outcome by gestation have all assessed the children in early childhood, and estimates of severe disability rates from these studies will probably also be too pessimistic. Since the rate of severe disabilities in infants of borderline viability is not much higher than in more mature infants the obstetrician should mainly consider survival chances for the fetus, and not be overly concerned with long-term neurological outcome, when making clinical decisions.

Outcome to 8 years of infants less than 1000 g birthweight: relationship with neonatal ventilator and oxygen therapy.

The study involved a cohort of 59 consecutive survivors with birthweights less than 1000 g, born between 1977 and 1980, to 8 years of age. The aim of the report was to determine if those survivors who had received more oxygen and ventilator therapy differed in their outcome compared with those who had received less oxygen and ventilation. Children were graded into four groups, characterized by decreasing durations of oxygen and ventilation. Children who had received less oxygen and ventilation were more likely to be below the third percentile for weight at 2, 5 and 8 years but the trends were significant only at 2 and 5 years (P = 0.006, P = 0.013 and P = 0.19 respectively). The rate of cerebral palsy was 8% at 8 years; the only children with severe or moderate disabilities from their cerebral palsy were in the lowest oxygen and ventilation group (n = 4, P less than 0.02). The frequency of hospital re-admission and the duration of re-hospitalization did not vary significantly between the four groups at any age. The rates of recurrent wheezing episodes or asthma did not vary significantly between the groups. Although the cohort as a whole had some impairment of lung function compared with healthy full-term controls, there was no significant difference between the four groups. Contrary to expectations, our findings suggest lower rates of poor growth and adverse neurological outcomes with increasing durations of oxygen and ventilation in the newborn period.

Changing two-year outcome of infants weighing 500 to 999 grams at birth: a hospital study.

Survival and neurodevelopmental outcome to 2 years were determined for two cohorts of infants weighing 500 to 999 gm at birth, born in a tertiary maternity hospital. Live births increased over time from an annual average of 48.7 in the first era (January 1977 to March 1982) to 64.6 in the second era (January 1985 to December 1987), largely from referrals of additional mothers with pregnancy complications. In the first era, 33.6% (86/256) of infants survived to 2 years; the survival rate improved significantly to 45.9% (89/194) in era 2. After adjustment for birth weight, the odds ratio for survival in era 2 versus era 1 was 1.39 (95% confidence interval = 1.12, 1.73; p less than 0.01). One known survivor in each era was not seen at 2 years of age. In the first era, 59.3% (51/86) of 2-year-old children were free of disability compared with 68.5% (61/89) in era 2 (NS), but the Mental Development Index of the Bayley Scales improved significantly, from 90.0 in era 1 to 98.0 in era 2. For infants weighing less than 800 gm at birth, not only did the 2-year survival rate improve, adjusted for birth weight (odds ratio = 1.53; 95% confidence interval = 1.06, 2.20; p less than 0.05), but there was also a significant reduction in neurologic disabilities in survivors (p = 0.03). For infants weighing 800 to 999 gm at birth, there was a significant improvement in the survival rate, adjusted for birth weight (odds ratio = 1.37; 95% confidence interval = 1.04, 1.79; p less than 0.05), but the rate of neurologic disabilities was unchanged. Increased survival in our tertiary maternity center was achieved without increasing the annual number of severely disabled 2-year-old survivors.

Changing diagnosis of cerebral palsy in very low birthweight children.

The stability of the diagnosis of cerebral palsy from 2 to 5 years of age was examined in 83 children of birthweight under 1000 gm, and 112 of birthweight 1000 to 1500 gm. In 20 2-year-old children with cerebral palsy, the diagnosis persisted in 11 (55%, 95% confidence intervals 35.1 to 76.9%); 2 of 175 children (1.1%) free of cerebral palsy at 2 years of age subsequently developed the condition. Severe or moderate cerebral palsy at 2 years persisted in all eight children (100%). In 9 of 12 children in whom cerebral palsy at 2 years had disappeared by 5 years, minor neurologic abnormalities and left-hand preference occurred frequently but mean psychologic tests scores were similar to children always free of cerebral palsy. In this cohort, cerebral palsy at 2 years was not a static condition, but overestimated later prevalence.