D espite advancements in medicine, preterm infants continue to experience high neonatal mortality and morbidity, with many having lifelong neurodevelopmental deficits, such as cerebral palsy (CP) and impaired cognitive abilities. The risk of impairment increases with decreasing gestational age at birth and many studies of short-term and long-term outcomes have focused on infants born at <33-week gestation. There are few data, however, on neonates born at 33 or 34 weeks. Issues include optimal places for delivery of preterm infants and the optimal gestational age for delivery in women with such pregnancy complications as gestational hypertension, intrauterine growth restriction (IUGR), and preterm premature rupture of membranes. In this population-based study of infants born at 30 to 34 weeks, neonatal mortality and morbidity and 5-year neurodevelopmental outcomes were assessed.All live and stillbirths occurring between 30 and 32 weeks in 1997 (n = 2020) and all between 33 and 34 weeks in April and October of 1997 (n = 457) in all maternity units in 9 French regions were included in the EPIPAGE study. Data on the mother, pregnancy, and neonate were recorded on standardized forms and included maternal complications, gestational age at birth, adverse neonatal outcomes, and treatments used in the neonatal unit. Survivors had a medical and neuropsychological assessment at 5 years of age.Of the 2467 infants enrolled in the study, 2018 were eligible for follow-up and 1461 were evaluated at 5 years. The distribution of pregnancy complications varied with gestational age in that the proportion of pregnancies with singletons and IUGR or hypertension decreased from 26% at 30 weeks to 18% at 34 weeks. On the other hand, the proportion with preterm premature rupture of membranes increased from 19% at 30 weeks to 26% at 34 weeks. The rate of cesarean births, antenatal corticosteroid therapy, and birth in tertiary-care facilities decreased as gestational age increased. So did the rate of adverse neonatal outcomes, although such morbidities as respiratory distress syndrome and bronchopulmonary dysplasia continued through all birth ages. Hospital length of stay also decreased with increasing gestational age; however, 27% of infants born at 34 weeks required admission to the neonatal intensive care unit; 7% needed endotracheal ventilation; 16% received antibiotics for >7 d and 5%, parenteral nutrition for >10 d; 9% received caffeine for apneas; and 47% had phototherapy for jaundice. The CP rate was 6.3% in the 30-week group, 8.7% at 31 weeks, 4.1% at 32 weeks, 3.7% at 33weeks, and 0.7% at 34 weeks (P<0.01). The proportion of children with Mental Processing Composite scores below 70 ranged from 10% in the 30-week and 31-week groups to 5% in the 34-week group, with the mean Mental Processing Composite score increasing significantly from 94 at 30 weeks to 98 at 34weeks. There was little difference among age groups as to visual and hearing deficiencies. In multivariate analysis, only gestational age at birth and pregnancy complication...

Food fortification with folic acid was associated with a significant reduction in the rate of neural-tube defects in Canada. The decrease was greatest in areas in which the baseline rate was high.

Although an increase in older mothers was observed in most registers, the prevalence of DS births remained stable in most registers as a result of increasing use of prenatal diagnostic procedures and ToP with DS.

The prevalence for multiple congenital contractures in Alberta is 1/8700 for 1980-1996 and 1/4300 for 1997-2007. Rates for the three groups were calculated. Specific diagnostic categories were found in groups II and III of 43% and 65%, respectively. Mortality is high, especially in the first month of life (45% total losses). New classification and coding systems are proposed.

The birth prevalence of limb deficiencies in Alberta has been fluctuating. The objectives were to examine patterns and temporal trends of congenital limb deficiencies in Alberta and compare rates with those of other jurisdictions. The Alberta Congenital Anomalies Surveillance System data on live births, stillbirths, and terminations of pregnancy (<20 weeks gestation) occurring between 1980 through 2012 with the ICD-10 Royal College of Paediatrics and Child Health Adaptations codes Q71-Q73 (limb reduction defects), Q79.80 (congenital constriction bands), and Q87.24 (sirenomelia syndrome) were reviewed. Cases were classified as having amelia, transverse, longitudinal (preaxial, postaxial, central, or mixed), intercalary, split hand/split foot, complex, or other type of limb deficiency. Phenotypes were classified as associated, which included cases with a known etiology and cases with at least one other type of anomaly, or isolated. From 1980 through 2012, 795 cases were ascertained from 1,411,652 live births and stillbirths, giving a prevalence of 5.6/10,000 total births. Mixed longitudinal deficiencies were the most common (22.4%). The upper limbs (63.9%) were affected more often than the lower limbs (25.3%). Isolated limb deficiencies occurred in 43.6% of cases, 28.4% had Mendelian or other known conditions, 21.9% had multiple congenital anomalies, 5.4% had chromosome abnormalities and 0.6% were due to teratogens. The associated group, showed a significant increasing trend (P =  0.023). While the overall limb deficiency rates show very little differences across diverse populations and differing time periods, comparisons of subgroups should be made with caution, because variations in terminology and classification contribute to reported differences.

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Results confirmed the etiologic heterogeneity of spina bifida and the more pronounced effect of folic acid in decreasing the risk of the more severe clinical presentations.

Objectives: Fortification of grain products with folic acid has been shown to significantly reduce the occurrence of neural tube defects (NTDs) in Canada and elsewhere. However, the impact on non-NTD anomalies has not been well studied.Methods: Using the Alberta Congenital Anomalies Surveillance System (ACASS), we examined changes in occurrence of select congenital anomalies where folic acid supplementation with multivitamins had previously been suggested to have an effect. Anomalies documented in the ACASS 1992-1996 (pre-fortification) were compared to 1999-2003 (post-fortification).Results: A significant decrease in spina bifida (OR 0.51, 95% CI 0.36-0.73) and ostium secundum atrial septal defects (OR 0.80, 95% CI 0.69-0.93) was evident, but there was a significant increase in obstructive defects of the renal pelvis and ureter (OR 1.45, 95% CI 1.24-1.70), abdominal wall defects (OR 1.40, 95% CI 1.04-1.88) and pyloric stenosis (OR 1.49, 95% CI 1.18-1.89). Conclusions:Consistent with other studies, a 50% reduction in spina bifida was associated with the post-fortification time period. Supporting the possibility that folic acid fortification may play a role in preventing other birth defects, a 20% reduction in atrial septal defects was also associated. The increase in abdominal wall defects, most notably gastroschisis, is likely related to pre-existing increasing trends documented in several regions around the world. The increase in pyloric stenosis and obstructive urinary tract defects was not expected and any causal relationship with folic acid fortification remains unclear. Similar studies by other birth defects surveillance systems in Canada and elsewhere are needed to confirm these trends.