PMID- 31781766
OWN - NLM
STAT- MEDLINE
DCOM- 20210104
LR  - 20210104
IS  - 1945-7197 (Electronic)
IS  - 0021-972X (Linking)
VI  - 105
IP  - 4
DP  - 2020 Apr 1
TI  - Parental Multiple Endocrine Neoplasia Type 1 (MEN 1) Is Associated with Increased 
      Offspring Childhood Mortality.
LID - dgz231 [pii]
LID - 10.1210/clinem/dgz231 [doi]
AB  - 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 whether birth occurred before ("historical cohort") or after ("contemporary 
      cohort") prospective screening commenced. The historical cohort included kindred 
      members born between 1825 and 1984 (n = 341 children with a MEN 1 positive (MEN 
      1+) parent and n = 314 children with MEN 1 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 were more likely 
      to die postpartum (HR 4.6, P = .046 at 6 months of age). Excess mortality at 15 
      years of 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 birth weight (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 of MEN 1+ mothers remain vulnerable despite contemporary 
      care. The excess risk was not fully explained by maternal MEN 1 or antenatal 
      hypercalcemia.
CI  - (c) Endocrine Society 2019. All rights reserved. For permissions, please e-mail: 
      journals.permissions@oup.com.
FAU - Thompson, Michael
AU  - Thompson M
AD  - Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, 
      Tasmania.
AD  - School of Medicine, University of Tasmania, Hobart, Tasmania.
FAU - Hogg, Prudence
AU  - Hogg P
AD  - Department of General Medicine, Royal Hobart Hospital, Hobart, Tasmania.
FAU - De Paoli, Antonio
AU  - De Paoli A
AD  - School of Medicine, University of Tasmania, Hobart, Tasmania.
AD  - Department of Paediatrics, Royal Hobart Hospital, Hobart, Tasmania.
FAU - Burgess, John
AU  - Burgess J
AD  - Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, 
      Tasmania.
AD  - School of Medicine, University of Tasmania, Hobart, Tasmania.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
SB  - IM
MH  - Adolescent
MH  - Child
MH  - Child Mortality/*trends
MH  - Child, Preschool
MH  - Cohort Studies
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*physiopathology
MH  - *Parents
MH  - Pregnancy
MH  - Prognosis
MH  - Prospective Studies
MH  - Retrospective Studies
MH  - Survival Rate
OTO - NOTNLM
OT  - MEN 1
OT  - childhood
OT  - multiple endocrine neoplasia type 1
OT  - neonatal
OT  - survival
EDAT- 2019/11/30 06:00
MHDA- 2021/01/05 06:00
CRDT- 2019/11/30 06:00
PHST- 2019/09/06 00:00 [received]
PHST- 2019/11/26 00:00 [accepted]
PHST- 2019/11/30 06:00 [pubmed]
PHST- 2021/01/05 06:00 [medline]
PHST- 2019/11/30 06:00 [entrez]
AID - 5645537 [pii]
AID - 10.1210/clinem/dgz231 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2020 Apr 1;105(4):dgz231. doi: 10.1210/clinem/dgz231.