BACKGROUND: Neural tube defects (NTDs) are a major cause of death and disability. Periconceptional folic acid prevents up to 70% of these malformations but public health campaigns to increase use of supplements have had disappointing results: The proposed mandatory fortification of bread products in Ireland has raised concerns about possible side effects. We examined data collected on a cohort of children born with NTDs in an era before fortification/supplementation to illustrate the serious consequences in terms of survival and disability. METHODS: All 623 infants born with NTDs in the Dublin area between 1976 and 1987 were included. Information was collected on mortality and length of survival for those who died, and for those who survived at least 5 years, interview-based data were collected on age, place of residence, prevalence of hydrocephalus, degree of disability, schooling, and IQ. RESULTS: Seventy-four births (12%) were stillborn. Of the livebirths only 41% were alive at 5 years. Factors associated with mortality were type of lesion, level of lesion, presence of other defects, hydrocephalus, year of birth, and gestation. Of the children who survived to 5 years or more, 75% had a disability and 56% were severely disabled. Type of lesion and level of lesion influenced disability risk. Of the survivors, 51% of children had mobility limitations, 59% were incontinent, 42% had hydrocephalus, and 17% had intellectual disability. CONCLUSIONS: These findings illustrate the devastating consequences of NTDs and underline the importance of effective intervention programs with folic acid for prevention. Copyright 2008 Wiley-Liss, Inc.
BACKGROUND:Neural tube defects (NTDs) are a major cause of death and disability. Periconceptional folic acid prevents up to 70% of these malformations but public health campaigns to increase use of supplements have had disappointing results: The proposed mandatory fortification of bread products in Ireland has raised concerns about possible side effects. We examined data collected on a cohort of children born with NTDs in an era before fortification/supplementation to illustrate the serious consequences in terms of survival and disability. METHODS: All 623 infants born with NTDs in the Dublin area between 1976 and 1987 were included. Information was collected on mortality and length of survival for those who died, and for those who survived at least 5 years, interview-based data were collected on age, place of residence, prevalence of hydrocephalus, degree of disability, schooling, and IQ. RESULTS: Seventy-four births (12%) were stillborn. Of the livebirths only 41% were alive at 5 years. Factors associated with mortality were type of lesion, level of lesion, presence of other defects, hydrocephalus, year of birth, and gestation. Of the children who survived to 5 years or more, 75% had a disability and 56% were severely disabled. Type of lesion and level of lesion influenced disability risk. Of the survivors, 51% of children had mobility limitations, 59% were incontinent, 42% had hydrocephalus, and 17% had intellectual disability. CONCLUSIONS: These findings illustrate the devastating consequences of NTDs and underline the importance of effective intervention programs with folic acid for prevention. Copyright 2008 Wiley-Liss, Inc.
Authors: Marian K Bakker; Vijaya Kancherla; Mark A Canfield; Eva Bermejo-Sanchez; Janet D Cragan; Saeed Dastgiri; Hermien E K De Walle; Marcia L Feldkamp; Boris Groisman; Miriam Gatt; Paula Hurtado-Villa; Karin Kallen; Daniella Landau; Nathalie Lelong; Jorge S Lopez Camelo; Laura Martínez; Margery Morgan; Osvaldo M Mutchinick; Wendy N Nembhard; Anna Pierini; Anke Rissmann; Antonin Sipek; Elena Szabova; Giovanna Tagliabue; Wladimir Wertelecki; Ignacio Zarante; Pierpaolo Mastroiacovo Journal: Paediatr Perinat Epidemiol Date: 2019-10-21 Impact factor: 3.980
Authors: Ridgely Fisk Green; Julianne Byrne; Krista S Crider; Margaret Gallagher; Deborah Koontz; Robert J Berry Journal: Front Genet Date: 2013-11-06 Impact factor: 4.599
Authors: Renata Bortolus; Fenneke Blom; Francesca Filippini; Mireille N M van Poppel; Emanuele Leoncini; Denhard J de Smit; Pier Paolo Benetollo; Martina C Cornel; Hermien E K de Walle; Pierpaolo Mastroiacovo Journal: BMC Pregnancy Childbirth Date: 2014-05-13 Impact factor: 3.007