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Parental multiple endocrine Neoplasia Type 1 (MEN 1) is associated with increased offspring childhood mortality

Thompson, M, Hogg, P, De Paoli, A and Burgess, J ORCID: 0000-0003-4748-9571 2020 , 'Parental multiple endocrine Neoplasia Type 1 (MEN 1) is associated with increased offspring childhood mortality' , Journal of Clinical Endocrinology and Metabolism, vol. 105, no. 4 , e1106-e1114 , doi: 10.1210/clinem/dgz231.

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Abstract

Context: Information regarding the impact of parental multiple endocrine neoplasia type 1(MEN 1) on neonatal outcomes is limited to case reports.Objective: To determine the impact of parental MEN 1 on neonatal outcomes.Methods: Retrospective cohort analysis of the Tasman 1 MEN 1 kindred stratified by whetherbirth occurred before (“historical cohort”) or after (“contemporary cohort”) prospectivescreening commenced. The historical cohort included kindred members born between 1825 and1984 (n = 341 children with a MEN 1 positive (MEN 1+) parent and n = 314 children with MEN1 negative (MEN 1–) parents). The contemporary cohort included neonates (n = 52) of MEN 1+women (n = 21) managed at a tertiary referral hospital between 1985 and 2018.Results: Historical cohort: compared with MEN 1– parents, children of MEN 1+ parents weremore likely to die postpartum (HR 4.6, P = .046 at 6 months of age). Excess mortality at 15 yearsof age was observed for children of MEN 1+ mothers (HR 8.50, P = .002) and fathers (HR 3.82,P = .03). Contemporary cohort: neonates of MEN 1+ mothers were more likely to have low birthweight (28.9% vs 6.7%, P = .01), be admitted to a higher care nursery (40.4% vs 17%, P = .02),and require a longer median postnatal stay (5 vs 4 days, P = .009) than the Australian average.Isolated antenatal hypercalcemia did not significantly alter neonatal outcomes.Conclusion: Children with a MEN 1+ parent are disproportionately vulnerable postpartum. Neonates ofMEN 1+ mothers remain vulnerable despite contemporary care. The excess risk was not fully explainedby maternal MEN 1 or antenatal hypercalcemia.

Item Type: Article
Authors/Creators:Thompson, M and Hogg, P and De Paoli, A and Burgess, J
Keywords: parental, MEN 1, childhood, mortality
Journal or Publication Title: Journal of Clinical Endocrinology and Metabolism
Publisher: Endocrine Soc
ISSN: 0021-972X
DOI / ID Number: 10.1210/clinem/dgz231
Copyright Information:

Copyright 2019 Endocrine Society

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