Pregnancy outcomes in women with neurofibromatosis 1: a Danish population-based cohort study.


Journal

Journal of medical genetics
ISSN: 1468-6244
Titre abrégé: J Med Genet
Pays: England
ID NLM: 2985087R

Informations de publication

Date de publication:
03 2022
Historique:
received: 21 05 2020
revised: 21 10 2020
accepted: 21 12 2020
pubmed: 27 1 2021
medline: 10 5 2022
entrez: 26 1 2021
Statut: ppublish

Résumé

The probability of a pregnancy, live birth, stillbirth and abortion has never been assessed in women with neurofibromatosis 1 (NF1) in a large population-based study. We included 1006 women (15-49 years) registered with NF1 in the Danish National Patient Registry or followed in two national Centers for Rare Diseases and 10 020 women from the Danish population. Information on pregnancy outcomes was ascertained from health registries. Cumulative incidence, mean cumulative count, hazard ratios (HRs) and proportion ratios (PRs) with 95% CIs were calculated. The cumulative incidence of a first pregnancy at age 50 years was slightly lower in women with NF1 (74%; 95% CI 70 to 77) than in population comparisons (78%; 95% CI 77 to 79). When all pregnancies were included, two pregnancies were expected per woman at age of 50 years, irrespective of a NF1 diagnosis. The hazard of a pregnancy did not differ between women with NF1 (HR 1.03; 95% CI 0.95 to 1.11) and the comparisons after adjustment for somatic and psychiatric disease. The proportion of pregnancies that resulted in a live birth was 63% (783/1252) among women NF1 and 68% (8432/12 465) among the comparisons, yielding a PR of 0.95 (95% CI 0.90 to 1.00). The proportions of stillbirths (PR 2.83; 95% CI 1.63 to 4.93) and spontaneous abortions (PR 1.40; 95% CI 1.09 to 1.79) were increased in women with NF1. A similar hazard for pregnancy was observed for women with NF1 and population comparisons after adjustment for potential medical consequences of NF1. However, women with NF1 experienced more spontaneous abortions and stillbirths.

Sections du résumé

BACKGROUND
The probability of a pregnancy, live birth, stillbirth and abortion has never been assessed in women with neurofibromatosis 1 (NF1) in a large population-based study.
METHODS
We included 1006 women (15-49 years) registered with NF1 in the Danish National Patient Registry or followed in two national Centers for Rare Diseases and 10 020 women from the Danish population. Information on pregnancy outcomes was ascertained from health registries. Cumulative incidence, mean cumulative count, hazard ratios (HRs) and proportion ratios (PRs) with 95% CIs were calculated.
RESULTS
The cumulative incidence of a first pregnancy at age 50 years was slightly lower in women with NF1 (74%; 95% CI 70 to 77) than in population comparisons (78%; 95% CI 77 to 79). When all pregnancies were included, two pregnancies were expected per woman at age of 50 years, irrespective of a NF1 diagnosis. The hazard of a pregnancy did not differ between women with NF1 (HR 1.03; 95% CI 0.95 to 1.11) and the comparisons after adjustment for somatic and psychiatric disease. The proportion of pregnancies that resulted in a live birth was 63% (783/1252) among women NF1 and 68% (8432/12 465) among the comparisons, yielding a PR of 0.95 (95% CI 0.90 to 1.00). The proportions of stillbirths (PR 2.83; 95% CI 1.63 to 4.93) and spontaneous abortions (PR 1.40; 95% CI 1.09 to 1.79) were increased in women with NF1.
CONCLUSIONS
A similar hazard for pregnancy was observed for women with NF1 and population comparisons after adjustment for potential medical consequences of NF1. However, women with NF1 experienced more spontaneous abortions and stillbirths.

Identifiants

pubmed: 33495305
pii: jmedgenet-2020-107201
doi: 10.1136/jmedgenet-2020-107201
doi:

Types de publication

Journal Article Research Support, U.S. Gov't, Non-P.H.S.

Langues

eng

Sous-ensembles de citation

IM

Pagination

237-242

Informations de copyright

© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Auteurs

Line Kenborg (L)

Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark kenborg@cancer.dk.

Cristina Boschini (C)

Statistics and Data Analysis, Danish Cancer Society Research Center, Copenhagen, Denmark.

Pernille E Bidstrup (PE)

Psychological Aspects of Cancer, Danish Cancer Society Research Center, Copenhagen, Denmark.

Susanne O Dalton (SO)

Survivorship and Inequality in Cancer, Danish Cancer Society Research Center, Copenhagen, Denmark.

Karoline Doser (K)

Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark.

Thomas T Nielsen (TT)

Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark.

Anja Krøyer (A)

Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark.

Christoffer Johansen (C)

Psychological Aspects of Cancer, Danish Cancer Society Research Center, Copenhagen, Denmark.
Department of Oncology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.

Kirsten Frederiksen (K)

Statistics and Data Analysis, Danish Cancer Society Research Center, Copenhagen, Denmark.

Sven Asger Sørensen (SA)

Department of Neurogenetics, Institute of Cellular and Molecular Medicine, University of Copenhagen, Copenhagen, Denmark.

Hanne Hove (H)

Department of Clinical Genetics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.

John R Østergaard (JR)

Center for Rare Diseases, Department of Pediatrics and Adolescent Medicine, Aarhus University Hospital, Aarhus, Denmark.

John J Mulvihill (JJ)

Department of Pediatrics, University of Oklahoma, Oklahoma City, OK, USA.

Jeanette F Winther (JF)

Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark.
Department of Clinical Medicine, Faculty of Health, Aarhus University, Aarhus, Denmark.

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