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. 2021 Mar:33:100769.
doi: 10.1016/j.eclinm.2021.100769. Epub 2021 Feb 22.

Medical vulnerability of individuals with Down syndrome to severe COVID-19-data from the Trisomy 21 Research Society and the UK ISARIC4C survey

Anke Hüls  1 , Alberto C S Costa  2 , Mara Dierssen  3   4   5 , R Asaad Baksh  6   7 , Stefania Bargagna  8 , Nicole T Baumer  9 , Ana Claudia Brandão  10 , Angelo Carfi  11 , Maria Carmona-Iragui  12   13 , Brian Allen Chicoine  14 , Sujay Ghosh  15 , Monica Lakhanpaul  16   17 , Coral Manso  18 , Miguel-Angel Mayer  19 , Maria Del Carmen Ortega  20 , Diego Real de Asua  21 , Anne-Sophie Rebillat  22 , Lauren Ashley Russell  23 , Giuseppina Sgandurra  24   25 , Diletta Valentini  26 , Stephanie L Sherman  27 , Andre Strydom  6   7   28 ; T21RS COVID-19 Initiative
Affiliations

Medical vulnerability of individuals with Down syndrome to severe COVID-19-data from the Trisomy 21 Research Society and the UK ISARIC4C survey

Anke Hüls et al. EClinicalMedicine. 2021 Mar.

Abstract

Background: Health conditions, immune dysfunction, and premature aging associated with trisomy 21 (Down syndrome, DS) may impact the clinical course of COVID-19.

Methods: The T21RS COVID-19 Initiative launched an international survey for clinicians or caregivers on patients with COVID-19 and DS. Data collected between April and October 2020 (N=1046) were analysed and compared with the UK ISARIC4C survey of hospitalized COVID-19 patients with and without DS.

Findings: The mean age of COVID-19 patients with DS in the T21RS survey was 29 years (SD = 18). Similar to the general population, the most frequent signs and symptoms of COVID-19 were fever, cough, and shortness of breath. Joint/muscle pain and vomiting or nausea were less frequent (p < 0.01), whereas altered consciousness/confusion were more frequent (p < 0.01). Risk factors for hospitalization and mortality were similar to the general population with the addition of congenital heart defects as a risk factor for hospitalization. Mortality rates showed a rapid increase from age 40 and were higher in patients with DS (T21RS DS versus non-DS patients: risk ratio (RR) = 3.5 (95%-CI=2.6;4.4), ISARIC4C DS versus non-DS patients: RR = 2.9 (95%-CI=2.1;3.8)) even after adjusting for known risk factors for COVID-19 mortality.

Interpretation: Leading signs/symptoms of COVID-19 and risk factors for severe disease course are similar to the general population. However, individuals with DS present significantly higher rates of medical complications and mortality, especially from age 40.

Funding: Down Syndrome Affiliates in Action, DSMIG-USA, GiGi's Playhouse, Jerome Lejeune Foundation, LuMind IDSC Foundation, The Matthew Foundation, NDSS, National Task Group on Intellectual Disabilities and Dementia Practices.

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Conflict of interest statement

Dr. Chicoine reports other from Woodbine House Publishing, other from Various Grateful Families of Patients, outside the submitted work; Dr. Strydom reports grants from MRC, grants from EC - Horizon 2020, during the conduct of the study; grants and personal fees from AC Immune, other from ProMIS Neurosciences, outside the submitted work; DRdA has been partially supported by the Spanish Fondo de Investigación Sanitaria-Instituto Carlos III (FIS-ISCIII), grant no. PI19/00634. All other authors have nothing to declare.

Figures

Fig 1
Fig. 1
Signs and symptoms reported among the COVID-19 cases with Down syndrome grouped by age (T21RS survey). The signs and symptoms “not eating or drinking”, “conjunctivitis” and “skin lesions” were added in the second wave of the survey (smaller sample size for these symptoms).
Fig 2
Fig. 2
Mortality rates among patients hospitalized with COVID-19. A. Age distribution of the proportion of deaths among individuals with DS (combined data from the T21RS and ISARIC4C surveys), who were hospitalized with COVID-19, in comparison to hospitalized cases of COVID-19 from the general population (combined data from the UK ISARIC4C survey (patients without DS), NYC and Spain [24]). B., C. and D. Mortality rates among hospitalized individuals. Hospitalized individuals with Down syndrome from the UK ISARIC4C and the T21RS surveys are compared to matched individuals without Down syndrome (controls) from the ISARIC4C survey. The 100 individuals with Down syndrome reported through the UK ISARIC4C survey were matched to 400 individuals without Down syndrome (controls) from the same survey (matching 1:4) as well as to 100 individuals with Down syndrome from the T21RS survey (matching 1:1). As samples were matched based on age, gender and ethnicity, mortality rates are corrected for these characteristics (see supplementary tables S2 and S3 for more information on the matched samples). We had information on the outcome of disease in 91/100 individuals from the matched T21RS dataset. B. All matched individuals. C. Matched individuals younger than 40 years of age. D. Matched individuals 40 years of age or older.
Fig 3
Fig. 3
Risk factors associated with adverse outcomes of COVID-19 in individuals with Down syndrome from the T21RS survey. Associations with A. hospitalization and B. mortality in symptomatic COVID-19 patients with Down syndrome from the T21RS survey estimated in adjusted logistic regression models (odds ratios (OR) and 95%-confidence intervals (95%-CI)). Associations with age and gender were adjusted for the data source (caregiver versus clinician survey) and associations with living situation, level of IDD and comorbidities were adjusted for age, gender, data source and country of residence. Abbreviations: IDD, intellectual and developmental disabilities; ref, reference category.
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