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| |  Study Examines Factors Improving Survival in Children With Congenital Anomalies NEW YORK -- January 22, 2010 -- Children born with congenital anomalies have a higher risk of death than healthy children. New research shows that the later the year of birth of a child with a congenital anomaly, the more likely that child is to survive. A number of factors are contributing to this trend, including medical advances in both treatments and surgical techniques, and the increasing rate of termination of the pregnancies with the worst outlook. The findings are reported in an article published online first and appearing in an upcoming edition of The Lancet. Advances in care have improved the prognosis for some congenital anomaly groups and subtypes, but there remains little knowledge about survival for many others, especially beyond the first year of life. To date, most studies in this area have reported survival in individuals with either Down’s syndrome or spina bifida. In this study, Judith Rankin, MD, Institute of Health and Society, Newcastle University, Newcastle-upon-Tyne, United Kingdom, and colleagues estimated survival up to 20 years of age for a range of congenital anomaly groups and subtypes. Information about children with at least 1 congenital anomaly, delivered between 1985 and 2003, was obtained from the UK Northern Congenital Abnormality Survey (NorCAS) -- a long running congenital abnormality register in the north of England. Anomalies were categorised by group (the system affected), subtype (the individual disorder), and syndrome according to European Surveillance of Congenital Anomalies (EUROCAT) guidelines. Local hospital and national mortality records were used to identify the survival status of liveborn children. Survival up to 20 years of age was estimated. A total of 13,758 cases of congenital anomaly were notified to NorCAS between 1985 and 2003. There were just under 11,000 livebirths, with survival data available in 99% of cases. 20-year survival was 86% in individuals born with at least 1 congenital anomaly (90% for cardiovascular system anomalies, 79% for chromosomal anomalies, 93% for urinary system anomalies, 83% for digestive system anomalies, 98% for orofacial clefts, and 66% for nervous system anomalies). Survival varied between subtypes within the same congenital anomaly group. The proportion of terminations for foetal anomaly increased throughout the study period, from 12.4% in 1985 to 18.3% in 2003. The authors pointed out several important medical advances that have improved survival in this population over the years, such as surfactant therapy and corticosteroid use in respiratory distress syndrome, intrapartum treatment for chorioamnionitis to prevent neonatal sepsis, and public health campaigns that have lowered the incidence of sudden unexpected death in infancy. More specific to congenital anomalies, there have also been substantial advances in surgical techniques, especially for cardiovascular system anomalies, and in prenatal diagnosis. “Aside from increasing the proportion of pregnancies eligible for termination, prenatal diagnosis might also improve outcome in liveborn individuals,” the authors wrote. “We found a significant temporal trend in the rate of termination of pregnancy for foetal anomaly, which explained part of the effect of year of birth on survival,” they added. “Although several mechanisms could explain this association, the most likely is the selective termination of pregnancies with the worst prognoses. This occurrence has already been posited as an important contributor to the reduction in infant mortality in the general population, with increasing rates of termination of pregnancy reducing the number of children being born alive with congenital anomalies.” Survival varied between subtypes within the same group, most notably in anomalies of the nervous, cardiovascular, and urinary systems. The authors said that this finding highlights the importance of presenting survival data for congenital anomalies by subtype because of the significant variation within congenital anomaly groups. They urge researches to report the various subtypes whenever possible. “We report robust estimates of survival up to 20 years of age for a range of congenital anomaly groups and subtypes, some of which have been infrequently reported previously,” the authors concluded. “This information will be valuable for families, genetic counsellors, and other health-care professionals when a congenital anomaly is detected, and will assist in planning for the future care needs of affected individuals.”
SOURCE: The Lancet
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