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This investigation examined the first\u2010year mortality rates for 54 selected birth defects of various organ systems in Hawaii during 1986\u20131999 using data from a population\u2010based birth defects registry and evaluated the impact of the presence of chromosomal abnormalities and other structural birth defects and the year of delivery on the mortality rates. Mortality rates varied widely by defect, being highest for anencephaly (100%), trisomy 13 (82%), and trisomy 18 (74%), while no first\u2010year deaths were reported for glaucoma, bladder exstrophy, and persistent cloaca. The majority (36 of 54 or 67%) of the birth defects had a mortality rate of less than 25%. Among the 51 structural birth defects, 38 (75%) had higher first\u2010year mortality rate for cases with chromosomal abnormalities and 42 (82%) had higher first\u2010year mortality rates for cases with other major structural birth defects. The mortality rate among 1986\u20131992 deliveries was higher than the mortality rate among 1993\u20131999 deliveries for 37 (69%) of the 54 birth defects. This study indicates that first\u2010year mortality rates vary widely by type of birth defect, although the mortality rate for the majority of birth defects is relatively low. The presence of a chromosomal abnormality or other structural birth defect increases the mortality rate, and mortality rates for the majority of birth defects have declined in Hawaii during the study period. \u00a9 2003 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.20151","type":"journal-article","created":{"date-parts":[[2003,5,30]],"date-time":"2003-05-30T10:04:53Z","timestamp":1054289093000},"page":"311-318","source":"Crossref","is-referenced-by-count":47,"title":["First\u2010year mortality rates for selected birth defects, Hawaii, 1986\u20131999"],"prefix":"10.1002","volume":"119A","author":[{"given":"Mathias B.","family":"Forrester","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruth 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