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Bedard T.,Alberta Congenital Anomalies Surveillance System
Journal of registry management | Year: 2012

The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10 CM) will be implemented on October 1, 2013 in the United States by institutions such as hospitals and insurance companies, and by surveillance programs and registries. The Alberta Congenital Anomalies Surveillance System (ACASS) experienced a transition in 2000, changing from the British Paediatric Association version of ICD-9 (ICD-9 BPA) to the Royal College of Paediatrics and Child Health adaptation of ICD-10 (ICD-10 RCPCH). Although the United States will use ICD-10 CM, the experiences discussed are applicable to birth defects programs in the United States. ACASS is funded by the Alberta Ministry of Health known as Alberta Health and Wellness (AHW) and is primarily a passive system covering approximately 50,000 annual births in the province of Alberta. Hospitals in Alberta changed from ICD-9 to an enhanced version of ICD-10 developed by the Canadian Institute for Health Information (ICD-10 CA) in 2002. Both ICD-10 RCPCH and ICD-10 CA are comparable; however, ICD-10 RCPCH offers a more detailed breakdown of some congenital anomaly categories. Although the implementation date for ICD-10 CA was to be in 2002, Alberta hospitals were aware in 1999 that the change would occur. This 3-year period allowed for preparation by ACASS prior to the required implementation.

Bedard T.,Alberta Congenital Anomalies Surveillance System
American Journal of Medical Genetics, Part A | Year: 2016

After the thalidomide epidemic in the early 1960s, many jurisdictions developed congenital anomaly surveillance systems. Congenital limb deficiencies can act as indicators of potential teratogens. The classification of congenital limb deficiencies is essential to determine the precise cause or causes of this anomaly. This article describes the different terminology and classification that have been used over time and the need for a consensus. While there are a variety of studies examining the epidemiology and etiology of congenital limb deficiencies, there is an inconsistent use of terminology and classification which makes comparisons between studies challenging. © 2016 Wiley Periodicals, Inc.

Orioli I.M.,Institute Biologia | Orioli I.M.,Inagemp Instituto Nacional Of Genetica Medica Populacional | Amar E.,Rhone Alps Registry of Birth Defects REMERA | Arteaga-Vazquez J.,Instituto Nacional Of Ciencias Medicas Y Nutricion Salvador Zubiran | And 20 more authors.
American Journal of Medical Genetics, Part C: Seminars in Medical Genetics | Year: 2011

Sirenomelia is a very rare limb anomaly in which the normally paired lower limbs are replaced by a single midline limb. This study describes the prevalence, associated malformations, and maternal characteristics among cases with sirenomelia. Data originated from 19 birth defect surveillance system members of the International Clearinghouse for Birth Defects Surveillance and Research, and were reported according to a single pre-established protocol. Cases were clinically evaluated locally and reviewed centrally. A total of 249 cases with sirenomelia were identified among 25,290,172 births, for a prevalence of 0.98 per 100,000, with higher prevalence in the Mexican registry. An increase of sirenomelia prevalence with maternal age less than 20 years was statistically significant. The proportion of twinning was 9%, higher than the 1% expected. Sex was ambiguous in 47% of cases, and no different from expectation in the rest. The proportion of cases born alive, premature, and weighting less than 2,500g were 47%, 71.2%, and 88.2%, respectively. Half of the cases with sirenomelia also presented with genital, large bowel, and urinary defects. About 10-15% of the cases had lower spinal column defects, single or anomalous umbilical artery, upper limb, cardiac, and central nervous system defects. There was a greater than expected association of sirenomelia with other very rare defects such as bladder exstrophy, cyclopia/holoprosencephaly, and acardia-acephalus. The application of the new biological network analysis approach, including molecular results, to these associated very rare diseases is suggested for future studies. © 2011 Wiley Periodicals, Inc.

Mutchinick O.M.,Instituto Nacional Of Ciencias Medicas Y Nutricion Salvador Zubiran | Luna-Munoz L.,Instituto Nacional Of Ciencias Medicas Y Nutricion Salvador Zubiran | Amar E.,Rhone Alps Registry of Birth Defects REMERA | Bakker M.K.,University of Groningen | And 21 more authors.
American Journal of Medical Genetics, Part C: Seminars in Medical Genetics | Year: 2011

Conjoined twins (CT) are a very rare developmental accident of uncertain etiology. Prevalence has been previously estimated to be 1 in 50,000 to 1 in 100,000 births. The process by which monozygotic twins do not fully separate but form CT is not well understood. The purpose of the present study was to analyze diverse epidemiological aspects of CT, including the different variables listed in the Introduction Section of this issue of the Journal. The study was made possible using the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) structure. This multicenter worldwide research includes the largest sample of CT ever studied. A total of 383 carefully reviewed sets of CT obtained from 26,138,837 births reported by 21 Clearinghouse Surveillance Programs (SP) were included in the analysis. Total prevalence was 1.47 per 100,000 births (95% CI: 1.32-1.62). Salient findings including an evident variation in prevalence among SPs: a marked variation in the type of pregnancy outcome, a similarity in the proportion of CT types among programs: a significant female predominance in CT: particularly of the thoracopagus type and a significant male predominance in parapagus and parasitic types: significant differences in prevalence by ethnicity and an apparent increasing prevalence trend in South American countries. No genetic, environmental or demographic significant associated factors were identified. Further work in epidemiology and molecular research is necessary to understand the etiology and pathogenesis involved in the development of this fascinating phenomenon of nature. © 2011 Wiley Periodicals, Inc.

Lowry R.B.,Alberta Congenital Anomalies Surveillance System | Lowry R.B.,University of Calgary | Lowry R.B.,Alberta Childrens Hospital Research Institute | Sibbald B.,Alberta Congenital Anomalies Surveillance System | Bedard T.,Alberta Congenital Anomalies Surveillance System
Cleft Palate-Craniofacial Journal | Year: 2014

Objective: To determine the prevalence and trends of orofacial clefts in Alberta (Canada) over a 33-year period (1980 through 2011) and to determine whether the trends differ for subcategories of orofacial clefts for the period from 1997 through 2011.Design: A prevalence study based on the Alberta Congenital Anomalies Surveillance System, which has multiple sources of ascertainment, capability of verification, and an upper age limit of 1 year.Inclusion: All live born and stillborn babies and fetal deaths less than 20 weeks' gestation (including terminations of pregnancy) born in Alberta of mothers who reside in Alberta.Results and Conclusions: Rates for cleft lip with or without cleft palate and cleft palate only have been very stable over the 33-year period (1980 through 2011). These rates include all clefts (isolated, syndromes, recognizable conditions, chromosomal and multiple congenital anomalies). Ascertainment of fetal deaths less than 20 weeks' gestation began in 1997. There are trends for the 1997 through 2011 cohort with a marginally significant increase for cleft lip with or without cleft palate in the isolated category and a significant decrease for cleft palate, mainly in the associated groups. The impact of folic acid fortification and/or multivitamins/folic acid supplementation reports in the literature have shown no consensus with respect to a change in the prevalence of orofacial clefts. It is unclear whether folic acid fortification has had any impact in Alberta. © Copyright 2014 American Cleft Palate-Craniofacial Association.

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