The spectrum of the VATER association has been debated ever since its description more than two decades ago. To assess the spectrum of congenital anomalies associated with VATER while minimizing the distortions due to small samples and referral patterns typical of clinical series, we studied infants with VATER association reported to the combined registry of infants with multiple congenital anomalies from 17 birth defects registries worldwide that are part of the International Clearinghouse for Birth Defects Monitoring Systems (ICB-DMS). Among approximately 10 million infants born from 1983 through 1991, the ICB-DMS registered 2,295 infants with 3 or more of 25 unrelated major congenital anomalies of unknown cause. Of these infants, 286 had the VATER association, defined as at least three of the five VATER anomalies (vertebral defects, anal atresia, esophageal atresia, renal defects, and radial-ray limb deficiency), when we expected 219 (P<0.001). Of these 286 infants, 51 had at least four VATER anomalies, and 8 had all five anomalies. We found that preaxial but not other limb anomalies were significantly associated with any combination of the four nonlimb VATER anomalies (P<0.001). Of the 286 infants with VATER association, 214 (74.8%) had additional defects. Genital defects, cardiovascular anomalies, and small intestinal atresias were positively associated with VATER association (P<0.001). Infants with VATER association that included both renal anomalies and anorectal atresia were significantly more likely to have genital defects. Finally, a subset of infants with VATER association also had defects described in other associations, including diaphragmatic defects, oral clefts, bladder exstrophy, omphalocele, and neural tube defects. These results offer evidence for the specificity of the VATER association, suggest the existence of distinct subsets within the association, and raise the question of a common pathway for patterns of VATER and other types of defects in at least a subset of infants with multiple congenital anomalies.
Objective To develop national birthweight percentiles by gestational age for male and female singleton infants born in Australia, and to compare the birthweight percentiles of Indigenous and non‐Indigenous infants. Design and setting Cross‐sectional study of singleton live births to Australian‐born mothers from 1991 to 1994. Main outcome measures Birthweight percentiles by gestational age. Results During 1991‐1994 Australian‐born women gave birth to 769077 live singleton infants. Of these, 28230 (3.7%) were reported as births to Aboriginal or Torres Strait Islander women. Birthweight was missing for 581 (0.1%) births and gestational age was missing for 3014 (0.4%). An additional 3283 (0.4%) births were excluded because the recorded birthweights were extreme outliers for their recorded gestational ages. Indigenous women were more likely to be recorded as giving birth preterm (<37 weeks' gestation) than non‐Indigenous women (11.6% v. 5.4%) and were more likely to give birth to small‐for‐gestational‐age infants at term. After 34 weeks' gestation, the median birthweights of Indigenous infants were consistently lower than those of non‐Indigenous infants. At 40 weeks' gestation the difference in the median birthweights between these two groups was 160 g for males and 130g for females. Conclusions We present recent birthweight percentiles by gestational age based on national data in Australia. These percentiles provide current Australian norms for clinicians and researchers, and can provide a baseline for monitoring Indigenous perinatal outcomes.
Summary:Purpose: The study goal was to assess teratogenic effects of antiepileptic drugs (AEDs) through the use of a surveillance system (MADRE) of infants with malformations.Methods: Information on all malformed infants (1990)(1991)(1992)(1993)(1994)(1995)(1996) with maternal first-trimester drug exposure was collected by the International Clearinghouse for Birth Defects and Monitoring Systems (ICBDMS). Cases were defined as infants presenting with a specific malformation, and controls were defined as infants presenting with any other birth defect. Exposure was defined by the use of AEDs during the first trimester of pregnancy. The association of AEDs with malformations was then estimated by calculating the odds ratios with 95% confidence intervals and testing their homogeneity among registries.Results: Among 8005 cases of malformations, 299 infants were exposed in utero to AEDs. Of those exposed to monotherapy, 65 were exposed to phenobarbital, 10 to methylphenobarbital, 80 to valproic acid, 46 to carbamazepine, 24 to phenytoin, and 16 to other AEDs. Associations were found for spina bifida with valproic acid. Infants exposed to phenobarbital and to methylphenobarbital showed an increased risk of oral clefts. Cardiac malformations were found to be associated with phenobarbital, methylphenobarbital, valproic acid, and carbamazepine. Hypospadias was associated with valproic acid. Porencephaly and other specified anomalies of brain, anomalies of face, coarctation of aorta, and limb reduction defects were found to be associated with valproic acid.Conclusions: Using the MADRE system, we confirmed known teratogenic effects of AEDs. We also found increased risks for malformations that had never been reported associated with AEDs or for which the association was suggested by case reports. Key Words: Antiepileptic drugs-Teratogenesis- Malformation registries-Risk factorsThe assessment of the teratogenic effects of antiepileptic drugs (AEDs) through epidemiological studies has often provided conflicting results (1-5). Cohort studies are limited by the low incidence of congenital malformations and thus reach adequate power only if a large multicenter approach is adopted. On the other hand, case-control studies on birth defects usually select Accepted June 1, 2000. Address correspondence and reprint requests to Prof. Pierpaolo Mastroiacovo at International Centre for Birth Defects, via Pilo Albertelli 9, 00195 Roma, Italy. E-mail: icbd@icbd.org healthy infants as controls, and there is a major risk of recall bias (i.e., the quality of the information given by the mothers regarding exposures during pregnancy differs between cases and controls). A registry-based system represents a relatively simple and inexpensive way of gathering information on intrauterine drug exposure in a large number of malformed infants (6). The use of controls presenting with malformations other than the malformation under study may reduce the recall bias and thus the overestimation of the effect.The main objective of our study was to test the effe...
The results of this study suggest that school hearing screenings may be provided using telehealth technology. This study did find that 5 students performed differently to pure-tone screenings administered by the telehealth protocol in contrast to on-site hearing screening services. Further research is necessary to identify factors leading to false responses to pure-tone hearing screening when telehealth technology is used. In addition, telehealth hearing screening protocols should be conducted with participants of different age groups and experiencing a wide range of hearing loss to further clarify the value of telehealth technology.
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