{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T14:33:05Z","timestamp":1747146785433,"version":"3.40.5"},"reference-count":20,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,11,8]],"date-time":"2022-11-08T00:00:00Z","timestamp":1667865600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Birth Defects Research"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Severe microcephaly is a brain reduction defect where the delivery head circumference is &lt;3rd percentile for gestational age and sex with subsequent lifelong morbidities. Our objective was to evaluate survival among 2,704 Texas infants with severe microcephaly delivered 1999\u20132015.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Infants with severe microcephaly from the Texas Birth Defects Registry were linked to death certificates and the national death index. Survival estimates, hazard ratios (HR) and confidence intervals (CI) were calculated using the Kaplan\u2013Meier method and Cox proportional hazards models stratified by presence versus absence of co\u2010occurring defects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 496 deaths by age 4\u2009years; most (42.9%) occurred in the neonatal period, and another 39.9% died by 1\u00a0year of age. Overall infant survival was 84.8%. Lowest infant survival subgroups included those with chromosomal\/syndromic conditions (66.1%), very preterm deliveries (63.9%), or co\u2010occurring critical congenital heart defects (44.0%). Among infants with severe microcephaly and a chromosomal\/syndromic co\u2010occurring defect, the risk of death was nearly three\u2010fold higher among those with: proportionate microcephaly (i.e., small baby overall), relative to non\u2010proportionate (HR\u00a0=\u00a02.84, 95% CI\u00a0=\u00a02.17\u20133.71); low\u2010birthweight relative to normal (HR\u00a0=\u00a02.72, 95% CI\u00a0=\u00a01.92\u20133.85); critical congenital heart defects (CCHD) relative to no CCHD (HR\u00a0=\u00a02.90, 95% CI\u00a0=\u00a02.20\u20133.80). Trisomies were a leading underlying cause of death (27.5%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Overall, infants with severe microcephaly had high 4\u2010year survival rates which varied by the presence of co\u2010occurring defects. Infants with co\u2010occurring chromosomal\/syndromic anomalies have a higher risk of death by age one than those without any co\u2010occurring birth defects.<\/jats:p><\/jats:sec>","DOI":"10.1002\/bdr2.2109","type":"journal-article","created":{"date-parts":[[2022,11,8]],"date-time":"2022-11-08T10:03:43Z","timestamp":1667901823000},"page":"26-42","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Survival of infants and children born with severe microcephaly, Texas, 1999\u20132015"],"prefix":"10.1002","volume":"115","author":[{"given":"Lisa K.","family":"Marengo","sequence":"first","affiliation":[{"name":"Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services  Austin Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natalie","family":"Archer","sequence":"additional","affiliation":[{"name":"Environmental Epidemiology and Disease Registries Section Texas Department of State Health Services  Austin Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2884-2835","authenticated-orcid":false,"given":"Charlie","family":"Shumate","sequence":"additional","affiliation":[{"name":"Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services  Austin Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark A.","family":"Canfield","sequence":"additional","affiliation":[{"name":"Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services  Austin Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margaret","family":"Drummond\u2010Borg","sequence":"additional","affiliation":[{"name":"Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services  Austin Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,11,8]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181b783f7"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2016-311199"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/bdr2.1879"},{"key":"e_1_2_10_5_1","unstructured":"Centers for Disease Control and Prevention & National Center for Health Statistics. (2020).ICD\u201010 Cause\u2010of\u2010Death Lists for Tabulating Mortality Statistics (Updated September 2020 to include WHO updates to ICD\u201010 for data year 2019). Instruction Manual Part 9.https:\/\/www.cdc.gov\/nchs\/data\/dvs\/Part9InstructionManual2019-508.pdf"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/bdra.23587"},{"volume-title":"Merck manual consumer version","year":"2019","author":"Falcheck S.","key":"e_1_2_10_7_1"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2021-053793"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/bdr2.1164"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0378-3782(02)00065-8"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajog.2010.05.006"},{"key":"e_1_2_10_12_1","unstructured":"National Association for Public Health Statistics and Information Systems (NAPHSIS). (2021).State and Territorial Exchange of Vital Events (STEVE). Retrieved fromhttps:\/\/www.naphsis.org\/steve"},{"key":"e_1_2_10_13_1","unstructured":"National Birth Defects Prevention Network (NBDPN)(2016).NBDPN Abstractor's Instructions Final Draft of February 28 2016 Appendix 3.1 on Congenital Microcephaly. Retrieved fromhttps:\/\/www.nbdpn.org\/docs\/NBDPN_Case_Definition_-_Surveillance_Microcephaly_2016Feb28_Final_DRAFT.pdf"},{"key":"e_1_2_10_14_1","unstructured":"National Birth Defects Prevention Network (NBDPN). (2017).Appendix 3.1 Birth Defects Descriptions for NBDPN Core Recommended and Extended Conditions Updated March 2017. Botto L. Carey J. Cassell C. Colarusso T. Cragan J. Feldkamp M. Frias J. Lin A. Mai C. Olney R. Stanton C. Siffel Chttps:\/\/www.nbdpn.org\/docs\/Appendix_3_1_BirthDefectsDescriptions_2017MAR24.pdf"},{"key":"e_1_2_10_15_1","unstructured":"National Center on Birth Defects and Developmental Disabilities (NCBDDD). Centers for Disease Control and Prevention. (2020).Facts about Microcephaly. Retrieved fromhttps:\/\/www.cdc.gov\/ncbddd\/birthdefects\/microcephaly.html"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/tera.1073"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/bdra.20696"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamanetworkopen.2022.6739"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/aje\/kwm345"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.12425"},{"key":"e_1_2_10_21_1","unstructured":"World Health Organization.International Fetal and Newborn Growth Consortium for the 21st Century Child Growth Standards for birth to 5\u2009years.https:\/\/www.who.int\/tools\/child-growth-standards\/standards\/head-circumference-for-age.2021.World Health Organization;\u00a9Copyright 2009\u20132021 INTERGROWTH\u201021st. 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