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1.
J Infect Dis ; 226(5): 757-765, 2022 09 13.
Article in English | MEDLINE | ID: covidwho-2029040

ABSTRACT

BACKGROUND: Down syndrome (DS) is associated with an increased risk of infections attributed to immune defects. Whether individuals with DS are at an increased risk of severe coronavirus disease 2019 (COVID-19) remains unclear. METHODS: In a matched cohort study, we evaluated the risk of COVID-19 infection and severe COVID-19 disease in individuals with DS and their matched counterparts in a pre-COVID-19 vaccination period at Kaiser Permanente Southern California. Multivariable Cox proportion hazard regression was used to investigate associations between DS and risk of COVID-19 infection and severe COVID-19 disease. RESULTS: Our cohort included 2541 individuals with DS and 10 164 without DS matched on age, sex, and race/ethnicity (51.6% female, 53.3% Hispanic, median age 25 years [interquartile range, 14-38]). Although the rate of COVID-19 infection in individuals with DS was 32% lower than their matched counterparts (adjusted hazard ratio [aHR], 0.68; 95% confidence interval [CI], .56-.83), the rate of severe COVID-19 disease was 6-fold higher (aHR, 6.14; 95% CI, 1.87-20.16). CONCLUSIONS: Although the risk of COVID-19 infection is lower, the risk of severe disease is higher in individuals with DS compared with their matched counterparts. Better infection monitoring, early treatment, and promotion of vaccine for COVID-19 are warranted for DS populations.


Subject(s)
COVID-19 , Delivery of Health Care, Integrated , Down Syndrome , Adult , COVID-19/epidemiology , COVID-19 Vaccines , Cohort Studies , Down Syndrome/complications , Down Syndrome/epidemiology , Female , Humans , Male
4.
Clin Microbiol Infect ; 28(8): 1155.e1-1155.e4, 2022 Aug.
Article in English | MEDLINE | ID: covidwho-1800136

ABSTRACT

OBJECTIVE: People with Down syndrome (DS) are particularly vulnerable to coronavirus disease 2019 (COVID-19) and show altered immune response to vaccination. We aimed to evaluate the immune response of a group of adults with DS treated with standard regimens of SARS-CoV-2 vaccine as compared with an age- and sex-matched group of persons without DS. METHODS: We compared antibody responses between 42 subjects with DS (41.6 ± 10.8 years, 57% male), and an age- and sex-matched comparison group of healthy health care workers (HCW) (41.4 ± 8.8 years, 54.8% male) after SARS-CoV-2 vaccination with the standard regimen of BNT162b2 mRNA COVID-19. Receptor binding domain (RBD) IgG antibodies were assessed at 4 time points (baseline, 21 days after the first dose, 21 days after the second dose, and 6 months after the first dose) with Siemens SARS-CoV-2 IgG (COV2G) antibody test. RESULTS: We observed significantly different antibody responses at all time points after vaccination (HCW vs. DS: 7.9 ± 3.9 vs. 1.4 ± 3.6 IU/mL at 21 days after first dose; 358.5 ± 3.8 vs. 38.1 ± 3.0 IU/mL at 21 days after second dose; 34.6 ± 2.4 vs. 7.9 ± 3.1 IU/mL at 6 months after vaccination) and a significantly different time course of decline in antibody titers between the two groups. DISCUSSION: Subjects with DS have a valid antibody response to SARS-CoV-2 vaccination. However, this response is lower than that of subjects in the HCW group. This finding could indicate a more rapid decline in the protective effects of the vaccination in subjects with DS and could suggest that people with DS may benefit from a booster dose of vaccine.


Subject(s)
COVID-19 , Down Syndrome , Viral Vaccines , Adult , Antibodies, Viral , BNT162 Vaccine , COVID-19/prevention & control , COVID-19 Vaccines , Down Syndrome/complications , Female , Humans , Immunoglobulin G , Male , SARS-CoV-2 , Vaccination
5.
Disabil Health J ; 15(3): 101278, 2022 07.
Article in English | MEDLINE | ID: covidwho-1693703

ABSTRACT

BACKGROUND: The Down syndrome population has been disproportionately affected by Coronavirus 2019 (COVID-19) in terms of experiencing severe illness and death. Societal efforts to curb the spread of COVID-19 may also have taken a heavy toll on the daily lives of individuals with Down syndrome. OBJECTIVE/HYPOTHESIS: The goal of the study was to understand how the COVID-19 pandemic has altered daily life (including residence, employment, and participation in adult disability day programs) and influenced the mood and behavior of adults with Down syndrome. METHODS: Between September 2020 and February 2021, caregivers of 171 adults with Down syndrome (aged 22-66 years) located across the United States and in the United Kingdom enrolled in the Alzheimer's Biomarker Research Consortium on Down Syndrome (ABC-DS) completed a survey. RESULTS: The residence of 17% of individuals was altered, and 89% of those who had been employed stopped working during the pandemic. One-third (33%) of individuals were reported to be more irritable or easily angered, 52% were reported to be more anxious, and 41% were reported to be more sad/depressed/unhappy relative to prepandemic. The majority of changes in mood and behavior were of modest severity. CONCLUSIONS: The COVID-19 pandemic has had widespread effects on the daily life and mood and behavior of adults with Down syndrome. In the short term, caregivers and providers should be prepared to help adults with Down syndrome with changes in daily routines, residence, employment, or adult disability day programs as society shifts away from COVID-19 safety protocols.


Subject(s)
COVID-19 , Disabled Persons , Down Syndrome , Adult , Affect , Down Syndrome/complications , Humans , Pandemics , United States/epidemiology
7.
BMJ Case Rep ; 14(11)2021 Nov 30.
Article in English | MEDLINE | ID: covidwho-1546475

ABSTRACT

Mucormycosis represents several unusual opportunistic infection caused by saprophytic aseptate fungi. There is a recent rise in cases of mucormycosis due to an increase in diabetic and immunodeficient patients like patients on long-term steroids, immunomodulators due to organ transplantation, malignancies, mainly haematological malignancies, and autoimmunity. Anatomically, mucormycosis can be localised most commonly as rhino-orbito-cerebral followed by pulmonary, disseminated, cutaneous and gastrointestinal, rarest being small intestinal. Patients with Down syndrome are immunodeficient due to their impaired immune response. Disseminated tuberculosis is also common in immunodeficient patients. We report a rare case of small intestinal mucormycosis in a patient with Down syndrome with coexisting intestinal tuberculosis. Due to the invasiveness of mucormycosis, the patient succumbed to death despite providing aggressive surgical debridement and medical management.


Subject(s)
Coinfection , Diabetes Mellitus , Down Syndrome , Mucormycosis , Tuberculosis, Gastrointestinal , Down Syndrome/complications , Humans , Mucormycosis/complications , Mucormycosis/diagnosis , Tuberculosis, Gastrointestinal/complications , Tuberculosis, Gastrointestinal/diagnosis
9.
Neuromolecular Med ; 23(4): 561-571, 2021 12.
Article in English | MEDLINE | ID: covidwho-1525619

ABSTRACT

The current SARS-CoV-2 outbreak, which causes COVID-19, is particularly devastating for individuals with chronic medical conditions, in particular those with Down Syndrome (DS) who often exhibit a higher prevalence of respiratory tract infections, immune dysregulation and potential complications. The incidence of Alzheimer's disease (AD) is much higher in DS than in the general population, possibly increasing further the risk of COVID-19 infection and its complications. Here we provide a biological overview with regard to specific susceptibility of individuals with DS to SARS-CoV-2 infection as well as data from a recent survey on the prevalence of COVID-19 among them. We see an urgent need to protect people with DS, especially those with AD, from COVID-19 and future pandemics and focus on developing protective measures, which also include interventions by health systems worldwide for reducing the negative social effects of long-term isolation and increased periods of hospitalization.


Subject(s)
COVID-19/epidemiology , COVID-19/virology , Disease Susceptibility , Down Syndrome/epidemiology , Adolescent , Adult , Alzheimer Disease/complications , Alzheimer Disease/epidemiology , Alzheimer Disease/immunology , COVID-19/complications , COVID-19/prevention & control , COVID-19 Vaccines/administration & dosage , COVID-19 Vaccines/immunology , Comorbidity , Disease Susceptibility/immunology , Disease Susceptibility/virology , Down Syndrome/complications , Down Syndrome/immunology , Female , Hospitalization , Humans , Immune System/abnormalities , Incidence , Male , Pandemics/prevention & control , Prevalence , Risk Factors , Vaccination/methods
10.
BMJ Case Rep ; 14(4)2021 Apr 15.
Article in English | MEDLINE | ID: covidwho-1388472

ABSTRACT

Neurological complications of SARS-CoV-2 continue to be recognised. In children, neurological phenomenon has been reported generally in the acute infectious period. It is possible that SARS-CoV-2 could trigger an immune-mediated post-infectious phenomenon. Here, we present a unique case of post-infectious marantic cardiac lesion causing cerebrovascular accident in a patient with Down syndrome.


Subject(s)
COVID-19/complications , Down Syndrome , Nervous System Diseases/virology , Stroke/virology , Child , Down Syndrome/complications , Down Syndrome/virology , Humans , Inflammation/complications , Inflammation/virology
11.
Rev. Bras. Saúde Mater. Infant. (Online) ; 21(supl.2): 553-557, 2021. graf
Article in English | WHO COVID, LILACS (Americas) | ID: covidwho-1328012

ABSTRACT

Abstract Introduction: although pediatric patients have comparatively fewer cases of COVID-19, children with Down Syndrome exhibit comorbidities such as immunodeficiency, diabetes and, in this perspective, are considered a population at risk for severe COVID-19. In addition, the literature also points to an unfavorable perspective on co-infection with Mycobacterium tuberculosis, considered an important comorbidity and a predictor of a worse clinical outcome. Description: female child, nine years old, with Down Syndrome, congenital heart disease and prematurity, with significant weight loss and intermittent fever for six months. A week ago, she had an intensification of fever, productive cough and mild respiratory distress. RT-PCR for SARS-CoV-2 detectable in nasopharynx swab, chest X-ray with diffuse alveolar infiltrate, chest CT with consolidations, excavation, solid micronodules in a sprouting tree pattern mainly in the right upper and lower lobes. Molecular rapid test for Mycobacterium tuberculosis detectable in gastric lavage. After specific treatment, the patient progressed well and was discharged from the hospital after 72 hours without fever and improvement in her breathing pattern. Discussion: despite the extensive pulmonary involvement, the patient did not require invasive ventilatory support and presented a satisfactory short-term outcome. Therefore, the relevance of the association of Tuberculosis and COVID-19 and other comorbidities in the pediatric age group still remains uncertain.


Resumo Introdução: apesar dos pacientes pediátricos apresentarem, comparativamente, menos casos da COVID-19, crianças com Síndrome de Down manifestam comorbidades como imunodeficiência, diabetes e, nessa perspectiva, são apontadas como população de risco para COVID-19 grave. Ademais, a literatura também sinaliza para um cenário desfavorável na coinfecção com Mycobacterium tuberculosis, considerada comorbidade importante e preditora para pior desfecho clínico. Descrição: criança de nove anos, sexo feminino, com Síndrome de Down, cardiopatia congênita e prematuridade, história de perda ponderal significativa e febre intermitente vespertina há seis meses. Há uma semana, apresentou intensificação da febre, tosse produtiva e desconforto respiratório leve. RT-PCR para SARS-CoV-2 em swab de nasofaringe detectável, radiografia de tórax com infiltrado alveolar difuso, tomografia de tórax com consolidações, focos de escavação, micronódulos sólidos em padrão de árvore em brotamento principalmente em lobos superior e inferior direitos. Teste rápido molecular para Mycobacterium tuberculosis detectável em lavado gástrico. Após início de tratamento específico, a paciente exibiu melhora clínica e de padrão respiratório e recebeu alta hospitalar após 72 horas afebril. Discussão: apesar do extenso comprometimento pulmonar, a paciente não necessitou de suporte ventilatório invasivo e apresentou um desfecho satisfatório em curto prazo. Portanto, a relevância da associação de tuberculose e COVID-19 e outras comorbidades na faixa etária pediátrica ainda permanecem incertas.


Subject(s)
Humans , Female , Child , Tuberculosis, Pulmonary/diagnosis , Comorbidity , Down Syndrome/complications , Coinfection , COVID-19/diagnosis , Mycobacterium tuberculosis , Risk Groups , Brazil/epidemiology
12.
Rev. Bras. Saúde Mater. Infant. (Online) ; 21(supl.2): 553-557, 2021. graf
Article in English | WHO COVID, LILACS (Americas) | ID: covidwho-1328011

ABSTRACT

Abstract Introduction: although pediatric patients have comparatively fewer cases of COVID-19, children with Down Syndrome exhibit comorbidities such as immunodeficiency, diabetes and, in this perspective, are considered a population at risk for severe COVID-19. In addition, the literature also points to an unfavorable perspective on co-infection with Mycobacterium tuberculosis, considered an important comorbidity and a predictor of a worse clinical outcome. Description: female child, nine years old, with Down Syndrome, congenital heart disease and prematurity, with significant weight loss and intermittent fever for six months. A week ago, she had an intensification of fever, productive cough and mild respiratory distress. RT-PCR for SARS-CoV-2 detectable in nasopharynx swab, chest X-ray with diffuse alveolar infiltrate, chest CT with consolidations, excavation, solid micronodules in a sprouting tree pattern mainly in the right upper and lower lobes. Molecular rapid test for Mycobacterium tuberculosis detectable in gastric lavage. After specific treatment, the patient progressed well and was discharged from the hospital after 72 hours without fever and improvement in her breathing pattern. Discussion: despite the extensive pulmonary involvement, the patient did not require invasive ventilatory support and presented a satisfactory short-term outcome. Therefore, the relevance of the association of Tuberculosis and COVID-19 and other comorbidities in the pediatric age group still remains uncertain.


Resumo Introdução: apesar dos pacientes pediátricos apresentarem, comparativamente, menos casos da COVID-19, crianças com Síndrome de Down manifestam comorbidades como imunodeficiência, diabetes e, nessa perspectiva, são apontadas como população de risco para COVID-19 grave. Ademais, a literatura também sinaliza para um cenário desfavorável na coinfecção com Mycobacterium tuberculosis, considerada comorbidade importante e preditora para pior desfecho clínico. Descrição: criança de nove anos, sexo feminino, com Síndrome de Down, cardiopatia congênita e prematuridade, história de perda ponderal significativa e febre intermitente vespertina há seis meses. Há uma semana, apresentou intensificação da febre, tosse produtiva e desconforto respiratório leve. RT-PCR para SARS-CoV-2 em swab de nasofaringe detectável, radiografia de tórax com infiltrado alveolar difuso, tomografia de tórax com consolidações, focos de escavação, micronódulos sólidos em padrão de árvore em brotamento principalmente em lobos superior e inferior direitos. Teste rápido molecular para Mycobacterium tuberculosis detectável em lavado gástrico. Após início de tratamento específico, a paciente exibiu melhora clínica e de padrão respiratório e recebeu alta hospitalar após 72 horas afebril. Discussão: apesar do extenso comprometimento pulmonar, a paciente não necessitou de suporte ventilatório invasivo e apresentou um desfecho satisfatório em curto prazo. Portanto, a relevância da associação de tuberculose e COVID-19 e outras comorbidades na faixa etária pediátrica ainda permanecem incertas.


Subject(s)
Humans , Female , Child , Tuberculosis, Pulmonary/diagnosis , Comorbidity , Down Syndrome/complications , Coinfection , COVID-19/diagnosis , Mycobacterium tuberculosis , Risk Groups , Brazil/epidemiology
13.
J Med Case Rep ; 15(1): 364, 2021 Jul 12.
Article in English | MEDLINE | ID: covidwho-1308103

ABSTRACT

BACKGROUND: The new coronavirus disease 2019 pandemic has spread throughout most of the world. Cerebral venous thrombosis is a rare thromboembolic disease that can present as an extrapulmonary complication in coronavirus disease 2019 infection. CASE PRESENTATION: We report the case of a Hispanic woman with Down syndrome who has coronavirus disease 2019 and presents as a complication extensive cerebral venous thrombosis. CONCLUSIONS: Cerebral venous thrombosis is a rare thromboembolic disease that can present as an extrapulmonary complication in coronavirus disease 2019 infection. In the absence of clinical and epidemiological data, it is important to carry out further investigation of the risk factors and pathophysiological causes related to the development of cerebrovascular thrombotic events in patients with Down syndrome with coronavirus disease 2019 infection.


Subject(s)
COVID-19 , Down Syndrome , Venous Thrombosis , Down Syndrome/complications , Female , Humans , Pandemics , SARS-CoV-2
14.
J Clin Immunol ; 41(7): 1457-1462, 2021 10.
Article in English | MEDLINE | ID: covidwho-1252168

ABSTRACT

While adults with Down syndrome (DS) are at increased risk of severe COVID-19 pneumonia, little is known about COVID-19 in children with DS. In children without DS, SARS-CoV-2 can rarely cause severe COVID-19 pneumonia, or an even rarer and more typically pediatric condition, multisystem inflammatory syndrome in children (MIS-C). Although the underlying mechanisms are still unknown, MIS-C is thought to be primarily immune-mediated. Here, we describe an atypical, severe form of MIS-C in two infant girls with DS who were hospitalized for over 4 months. Immunological evaluation revealed pronounced neutrophilia, B cell depletion, increased circulating IL-6 and IL-8, and elevated markers of immune activation ICAM1 and FcÉ£RI. Importantly, uninfected children with DS presented with similar but less stark immune features at steady state, possibly explaining risk of further uncontrolled inflammation following SARS-CoV-2 infection. Overall, a severe, atypical form of MIS-C may occur in children with DS.


Subject(s)
COVID-19/diagnosis , Down Syndrome/diagnosis , SARS-CoV-2/physiology , Systemic Inflammatory Response Syndrome/diagnosis , COVID-19/complications , Down Syndrome/complications , Fatal Outcome , Female , Hospitalization , Humans , Infant , Syndrome
15.
Transfusion ; 61(6): 1740-1748, 2021 06.
Article in English | MEDLINE | ID: covidwho-1243668

ABSTRACT

BACKGROUND: While convalescent plasma (CP) may benefit patients with COVID-19, fundamental questions remain regarding its efficacy, including the components of CP that may contribute to its therapeutic effect. Most current serological evaluation of CP relies on examination of total immunoglobulin or IgG-specific anti-SARS-CoV-2 antibody levels. However, IgA antibodies, which also circulate and are secreted along the respiratory mucosa, represent a relatively uncharacterized component of CP. STUDY DESIGN AND METHODS: Residual samples from patients and CP donors were assessed for IgM, IgG, and IgA anti-SARS-CoV-2 antibody titers against the receptor-binding domain responsible for viral entry. Symptom onset was obtained by chart review. RESULTS: Increased IgA anti-SARS-CoV-2 antibody levels correlated with clinical improvement and viral clearance in an infant with COVID-19, prompting a broader examination of IgA levels among CP donors and hospitalized patients. Significant heterogeneity in IgA levels was observed among CP donors, which correlated weakly with IgG levels or the results of a commonly employed serological test. Unlike IgG and IgM, IgA levels were also more likely to be variable in hospitalized patients and this variability persisted in some patients >14 days following symptom onset. IgA levels were also less likely to be sustained than IgG levels following subsequent CP donation. CONCLUSIONS: IgA levels can be very heterogenous among CP donors and hospitalized patients and do not necessarily correlate with commonly employed testing platforms. Examining isotype levels in CP and COVID-19 patients may allow for a tailored approach when seeking to fill specific gaps in humoral immunity.


Subject(s)
COVID-19/immunology , COVID-19/therapy , Convalescence , Immunoglobulin A/blood , SARS-CoV-2/immunology , Antibodies, Viral/blood , Blood Donors , Down Syndrome/complications , Down Syndrome/immunology , Down Syndrome/therapy , Female , Heart Septal Defects/complications , Heart Septal Defects/immunology , Heart Septal Defects/therapy , Humans , Immunity, Humoral/immunology , Immunization, Passive/methods , Immunoglobulin A/analysis , Immunoglobulin G/blood , Immunoglobulin M/blood , Infant , Retrospective Studies , Serologic Tests , United States
16.
J Pediatric Infect Dis Soc ; 10(4): 543-546, 2021 Apr 30.
Article in English | MEDLINE | ID: covidwho-1211412

ABSTRACT

Down syndrome (DS) predisposes to severe immunologic reaction secondary to infectious triggers. Here, we report a pediatric DS patient with coronavirus disease 2019 (COVID-19) who developed a hyperinflammatory syndrome, severe acute respiratory distress syndrome, and secondary hemophagocytic lymphohistiocytosis requiring pediatric intensive care unit admission and treatment with steroids, intravenous immunoglobulin, and remdesivir. Investigations into genetic susceptibilities for COVID-19 and severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2)-associated complications warrant systematic clinical and scientific studies. We report a pediatric Down syndrome patient with coronavirus disease 2019 (COVID-19) who developed secondary hemophagocytic lymphohistiocytosis requiring treatment with steroids, intravenous immunoglobulin, and remdesivir. Investigations into genetic susceptibilities for COVID-19-associated complications warrant systematic clinical and scientific studies.


Subject(s)
COVID-19/complications , Down Syndrome/complications , Lymphohistiocytosis, Hemophagocytic/virology , Systemic Inflammatory Response Syndrome/virology , Adenosine Monophosphate/analogs & derivatives , Adenosine Monophosphate/therapeutic use , Alanine/analogs & derivatives , Alanine/therapeutic use , Antiviral Agents/therapeutic use , COVID-19/diagnosis , COVID-19/drug therapy , COVID-19/virology , COVID-19 Testing , Child, Preschool , Critical Care , Genetic Predisposition to Disease , Glucocorticoids/therapeutic use , Humans , Immunoglobulins, Intravenous/therapeutic use , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/drug therapy , Male , Prednisolone/therapeutic use , SARS-CoV-2 , Systemic Inflammatory Response Syndrome/diagnosis , Systemic Inflammatory Response Syndrome/drug therapy
19.
Int J Infect Dis ; 103: 607-610, 2021 Feb.
Article in English | MEDLINE | ID: covidwho-1065174

ABSTRACT

We report two cases of Corona Virus Disease-19 (COVID-19) in patients with Down Syndrome (DS) and describe the identification, diagnosis, clinical course and management of the infection. Down Syndrome, which is caused by trisomy 21, is characterized by immune dysregulation, anatomical differences in the upper respiratory tract and higher rate of comorbidities. All these risk factors can contribute to more severe clinical presentations of COVID-19 in this population. It is essential to raise awareness of the clinical relevance of SARS-COV-2 infection in DS patients, as well as in other most vulnerable patients, in order to improve their management and treatment and to encourage vaccinating these individuals early, once a vaccination is available.


Subject(s)
COVID-19/etiology , Down Syndrome/complications , SARS-CoV-2 , Adult , Female , Humans , Middle Aged , Risk Factors
20.
Neurol Sci ; 42(5): 1649-1652, 2021 May.
Article in English | MEDLINE | ID: covidwho-1056021

ABSTRACT

PURPOSE: The aim of the current study was to determine whether COVID-19 is associated with a different presenting clinical picture or a more severe course of illness in people with Down syndrome (DS). METHODS: All consecutive patients who were admitted at healthcare facilities anywhere in Fars province (located in the south of Iran with a population of 4,851,000 people) from 19 February 2020 to 20 November 2020 were included. For every patient with DS, three age- and sex-matched patients with COVID-19 and without any underlying medical conditions were selected as controls. RESULTS: During the study period, 37,968 patients were hospitalized with a diagnosis of COVID-19. Eighteen patients had DS. Patients with DS were significantly more likely to be intubated [7 patients (39%)] compared with those without DS [3 patients (6%)]; p = 0.002. Patients with DS significantly more often died of COVID-19 compared with the controls [8 (44.4%) vs. 1 (1.9%); odds ratio: 24.37; 95% confidence interval 2.39-247.94; p = 0.007]. CONCLUSION: Patients with DS are among the high-risk populations with respect to severe COVID-19 and should receive the vaccine as soon as possible. Furthermore, they should receive more intensive care if they get hospitalized with the illness.


Subject(s)
COVID-19 , Down Syndrome , Down Syndrome/complications , Down Syndrome/epidemiology , Hospitalization , Humans , Iran/epidemiology , SARS-CoV-2
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