Oral Presentation ESA-SRB-AOTA 2019

Parental multiple endocrine neoplasia type 1 (MEN 1) is associated with increased offspring mortality postpartum: the impact of maternal MEN 1 and antenatal hypercalcemia (#130)

Michael Thompson 1 2 , Prue Hogg 3 , Tony De Paoli 1 4 , John Burgess 1 5
  1. School of Medicine, University of Tasmania, Hobart
  2. Austin Health, Heidelberg, VICTORIA, Australia
  3. General Medicine, Royal Hobart Hospital, Hobart
  4. Department of Paediatrics, Royal Hobart Hospital, Hobart
  5. Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart

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 1825-1984 (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 all neonates (n=52) of MEN 1+ women (n=28) born at a tertiary referral hospital between 1985-2018. Data was retrieved from births, deaths and marriages registries, medical records and the Australian Institute of Health and Welfare.

 

Results:

Historical cohort: compared to MEN 1- parents, children of MEN 1+ parents were more likely to die before 15 years of age in multivariable analysis (HR 4.6, p=0.005). Excess mortality was attributable to children of MEN 1+ mothers (HR 4.52, p=0.021) and fathers (HR 3.76, p=0.029) and primarily accrued postpartum (HR 4.48, p=0.039 at 3 months). Deaths due to neoplasia were not significantly increased (1 vs 0 death, p=1.0).

Contemporary cohort: neonates of MEN 1+ mothers were more likely to be low birth weight (28.9% vs 6.7%, p=0.01), admitted to a higher care nursery (40.4% vs 17%, p=0.02) and had a longer median postnatal length of stay (5 vs 4 days, p=0.009) compared to the Australian average. Neonatal hypoglycemia (76%) and infection (15%) occurred frequently whereas hypocalcemia requiring intravenous calcium treatment did not (3.8%).  Isolated antenatal hypercalcemia did not significantly alter neonatal outcomes.

 

Conclusion:

Children of MEN 1+ parents are disproportionately vulnerable and most at risk postpartum. The excess risk was not attributable to maternal MEN 1 or antenatal hypercalcemia alone.