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  • Clinical Research Article
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Death of children with Down syndrome by gestational age and cause

Pediatric Research volume 95, pages 1325–1330 (2024)Cite this article

Abstract

Background

We often encounter preterm infants with Down syndrome (DS) who die in the neonatal intensive care unit (NICU). In this study, we examined survival until NICU discharge and assessed the developmental prognosis of preterm infants with DS.

Methods

We retrospectively reviewed 416 infants with DS hospitalized during the past 27 years at our NICU.

Results

Death occurred in 8/20 (40%) infants at <32 weeks’ gestation, 11/23 (48%) at 32–33 weeks, 9/99 (9%) at 34–36 weeks, and 9/274 (3%) at >36 weeks. In total, 84% of infants who died and 25% of those who survived had a non-reassuring fetal status (p < 0.001). Sex, small-for-gestational-age status, and postnatal transport were not associated with death. The main causes of death were bronchopulmonary dysplasia in 4/8 (50%) infants at <32 weeks’ gestation, transient abnormal myelopoiesis in 11/20 (55%) and lymphatic dysplasia in 6/20 (30%) at 32–36 weeks, and varied causes at >36 weeks. Among survivors born at <34 weeks’ gestation, 6/19 (32%) aged >2 years had moderate or severe cerebral palsy.

Conclusions

These data on the high mortality and morbidity of preterm infants with DS may be useful for patient treatment and parent counseling in NICUs treating critically ill infants.

Impact

  • Most infants with Down syndrome born at <34 weeks’ gestation are born by cesarean section because of the non-reassuring fetal status.

  • The mortality rate before discharge for infants with Down syndrome born at <34 weeks’ gestation was 40%, and 30% of survivors developed moderate or severe cerebral palsy.

  • The risk of death due to bronchopulmonary dysplasia and pulmonary hypertension was high in very preterm infants with Down syndrome despite the absence of chorioamnionitis.

  • Infants with Down syndrome were born 1–2 weeks earlier than unaffected controls.

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Fig. 1: Flow diagram of patient cohort.
Fig. 2: Distribution of gestational age at birth in infants with Down syndrome.

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Acknowledgements

We thank Angela Morben, DVM, ELS, from Edanz (https://jp.edanz.com/ac), for editing a draft of this manuscript.

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Authors and Affiliations

  1. Department of Neonatology, Kanagawa Children’s Medical Center, Yokohama, Kanagawa, Japan

    Tomoyuki Shimokaze, Katsuaki Toyoshima, Tomoko Saito, Kaoru Katsumata & Takahiro Kemmotsu

  2. Department of Obstetrics and Gynecology, Kanagawa Children’s Medical Center, Yokohama, Kanagawa, Japan

    Hiroshi Ishikawa

  3. Department of Medical Genetics, Kanagawa Children’s Medical Center, Yokohama, Kanagawa, Japan

    Kenji Kurosawa

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  1. Tomoyuki Shimokaze
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Contributions

T. Shimokaze: conceptualization and design, original draft preparation. K. Toyoshima: conceptualization and supervision. T. Saito and K. Katsumata: data curation, investigation. T. Kemmotsu and H. Ishikawa: reviewing. K. Kurosawa: review of patients being followed up on an outpatient basis. All authors gave final approval for the version to be published.

Corresponding author

Correspondence to Tomoyuki Shimokaze.

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Competing interests

The authors declare no competing interests.

Ethics approval and consent to participate

The parents or guardians of infants admitted to the NICU were not required to give informed consent for the infants’ participation in the study because the analysis used anonymized clinical data that were obtained after the guardians of each patient agreed to treatment by providing their written consent. We also adopted an opt-out system and posted a summary of the study on the hospital’s website (https://kcmc.kanagawa-pho.jp/about/ethics/conduct_research).

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Shimokaze, T., Toyoshima, K., Saito, T. et al. Death of children with Down syndrome by gestational age and cause. Pediatr Res 95, 1325–1330 (2024). https://doi.org/10.1038/s41390-023-02870-1

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