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1.
Neurol Sci ; 44(7): 2231-2237, 2023 Jul.
Article in English | MEDLINE | ID: covidwho-2315562

ABSTRACT

INTRODUCTION: Myotonic dystrophy type 1 (DM1) is the most prevalent muscular dystrophy in adults. People with DM1 might represent a high-risk population for respiratory infections, including COVID-19. Our aim was to evaluate the characteristics of COVID-19 infection and vaccination rate in DM1 patients. METHODS: This cross-sectional cohort study included 89 patients from the Serbian registry for myotonic dystrophies. Mean age at testing was 48.4 ± 10.4 years with 41 (46.1%) male patients. Mean duration of the disease was 24.0 ± 10.3 years. RESULTS: COVID-19 infection was reported by 36 (40.4%) DM1 patients. Around 14% of patients had a more severe form of COVID-19 requiring hospitalization. The severity of COVID-19 was in accordance with the duration of DM1. A severe form of COVID-19 was reported in 20.8% of patients who were not vaccinated against SARS-CoV-2 and in none of the vaccinated ones. The majority of 89 tested patients (66.3%) were vaccinated against SARS-CoV-2. About half of them (54.2%) received three doses and 35.6% two doses of vaccine. Mild adverse events after vaccination were recorded in 20.3% of patients. CONCLUSIONS: The percentage of DM1 patients who suffered from COVID-19 was like in general population, but with more severe forms in DM1, especially in patients with longer DM1 duration. The study indicated an overall favorable safety profile of COVID-19 vaccines among individuals with DM1 and its ability to protect them from severe COVID-19.


Subject(s)
COVID-19 , Myotonic Dystrophy , Adult , Humans , Male , Middle Aged , Female , Myotonic Dystrophy/epidemiology , COVID-19 Vaccines , Cross-Sectional Studies , SARS-CoV-2
2.
J Infect Dev Ctries ; 17(2): 182-187, 2023 02 28.
Article in English | MEDLINE | ID: covidwho-2271464

ABSTRACT

INTRODUCTION: Steinert's disease is a rare genetic disorder characterized by progressive myotonia and multi-organ damage. It is associated with respiratory and cardiological complications often leading patients to exitus. These conditions are also traditional risk factors for severe COVID-19. SARS-CoV-2 has affected people with chronic diseases, but the impact on people with Steinert's disease is poorly defined, with only a few reported and described. More data are needed to understand whether this genetic disease is a risk factor for more serious evolution or death in patients with COVID-19. METHODOLOGY: The study describes two cases of patients with SD and COVID-19 and summarizes available evidence of the clinical outcome of COVID-19 in patients with Steinert's disease, by performing a systematic review of the literature (following PRISMA statements and performing PROSPERO registration). RESULTS: Overall, 5 cases were retrieved from the literature review, with a median age of 47 years, of whom 4 had advanced SD and unfortunately died. By contrast, the 2 patients from our clinical practice and 1 from literature had a good clinical outcomes. Mortality ranged from 57% (all cases) to 80% (only literature review). CONCLUSIONS: There is a high mortality rate in patients with both Steinert's disease and COVID-19. It highlights the importance of strengthening prevention strategies, especially vaccination. All SD with SARS-CoV-2 infection/COVID-19 patients should be identified early and treated to avoid complications. It is still unknown which treatment regimen is best to use in those patients. Studies on a greater number of patients are necessary to provide clinicians with further evidence.


Subject(s)
COVID-19 , Myotonic Dystrophy , Humans , Middle Aged , Myotonic Dystrophy/complications , Myotonic Dystrophy/genetics , COVID-19/complications , SARS-CoV-2
3.
Am J Clin Pathol ; 153(6): 725-733, 2020 05 05.
Article in English | MEDLINE | ID: covidwho-2227978

ABSTRACT

OBJECTIVES: To report the methods and findings of two complete autopsies of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive individuals who died in Oklahoma (United States) in March 2020. METHODS: Complete postmortem examinations were performed according to standard procedures in a negative-pressure autopsy suite/isolation room using personal protective equipment, including N95 masks, eye protection, and gowns. The diagnosis of coronavirus disease 2019 (COVID-19) was confirmed by real-time reverse transcriptase polymerase chain reaction testing on postmortem swabs. RESULTS: A 77-year-old obese man with a history of hypertension, splenectomy, and 6 days of fever and chills died while being transported for medical care. He tested positive for SARS-CoV-2 on postmortem nasopharyngeal and lung parenchymal swabs. Autopsy revealed diffuse alveolar damage and chronic inflammation and edema in the bronchial mucosa. A 42-year-old obese man with a history of myotonic dystrophy developed abdominal pain followed by fever, shortness of breath, and cough. Postmortem nasopharyngeal swab was positive for SARS-CoV-2; lung parenchymal swabs were negative. Autopsy showed acute bronchopneumonia with evidence of aspiration. Neither autopsy revealed viral inclusions, mucus plugging in airways, eosinophils, or myocarditis. CONCLUSIONS: SARS-CoV-2 testing can be performed at autopsy. Autopsy findings such as diffuse alveolar damage and airway inflammation reflect true virus-related pathology; other findings represent superimposed or unrelated processes.


Subject(s)
Autopsy , Coronavirus Infections/pathology , Lung/pathology , Pneumonia, Viral/pathology , Adult , Aged , Autopsy/instrumentation , Betacoronavirus/isolation & purification , COVID-19 , COVID-19 Testing , COVID-19 Vaccines , Clinical Laboratory Techniques/standards , Coronavirus Infections/complications , Coronavirus Infections/diagnosis , Diagnosis , Humans , Hypertension/complications , Male , Myotonic Dystrophy/complications , Obesity/complications , Oklahoma , Pandemics , Pneumonia, Viral/complications , Pneumonia, Viral/diagnosis , SARS-CoV-2
4.
Muscle Nerve ; 67(2): 117-123, 2023 Feb.
Article in English | MEDLINE | ID: covidwho-2148422

ABSTRACT

INTRODUCTION/AIMS: Due to muscular weakness and cardiopulmonary dysfunction, patients with muscular dystrophy (MD) have an increased risk of serious complications from coronavirus disease-2019 (COVID-19). Although vaccination is recommended, COVID-19 vaccination safety and immunogenicity in these patients are unknown. We investigated reaction frequency, post-vaccine antibody titers after two mRNA COVID-19 vaccine doses, and clinical predictors of antibody response among patients with MD. METHODS: We recruited 171 inpatients with MD receiving two BNT162b2 mRNA COVID-19 vaccine doses from seven hospitals. Blood samples were obtained from 53 inpatients before the first dose and 28 to 30 days after the second dose, and antibody titers were measured. RESULTS: Overall, 104 (60.8%) and 115 (67.6%) patients had side effects after the first and second doses, respectively. These were generally mild and self-limited. Multiple logistic regression analysis showed that a bedridden state was associated with reduced side effects (odds ratio [OR] = 0.29; 95% confidence interval [CI], 0.12 to 0.71). The antibody titers of all participants changed from negative to positive after two vaccine doses. The geometric mean titer (GMT) of the inpatients was 239 (95% CI, 159.3 to 358.7). Older age (relative risk [RR] = 0.97; 95% CI, 0.95 to 0.99) and bedridden state (RR = 0.27; 95% CI, 0.14 to 0.51) were associated with a lower antibody titer. Patients with myotonic dystrophy type 1 (DM1) had a lower GMT than patients with other MDs (RR = 0.42; 95% CI, 0.21 to 0.85). DISCUSSION: COVID-19 vaccination is safe and immunogenic in inpatients with MD. Patients with DM1 appear to have a poorer COVID-19 antibody response than those with other MDs.


Subject(s)
COVID-19 Vaccines , COVID-19 , Muscular Dystrophies , Myotonic Dystrophy , Humans , BNT162 Vaccine , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Inpatients , RNA, Messenger
5.
J Neuromuscul Dis ; 9(4): 517-523, 2022.
Article in English | MEDLINE | ID: covidwho-1902894

ABSTRACT

INTRODUCTION: In this study, we examined the long-term social and health impacts of the coronavirus disease 2019 (COVID-19) pandemic on people with muscular dystrophy. METHODS: We modified our prior COVID-19 Impact Survey to assess impacts from the continuing pandemic using feedback from muscular dystrophy experts, patients, and advocacy group/registry representatives. The survey assessed COVID-19 medical history, and the effects of the pandemic on social aspects, muscle disease, and medical care. We also used the validated 10-item Perceived Stress Scale. The de-identified, electronic survey was distributed to adults with muscular dystrophy via international patient registries and advocacy group websites from February 8, 2021 to March 22, 2021. RESULTS: Respondents (n = 1243 : 49% Facioscapulohumeral Muscular Dystrophy (FSHD); 43% Myotonic Dystrophy (DM), and 8% Limb-Girdle Muscular Dystrophy (LGMD)) were mostly women and middle-aged (range 18-90 years). Rates of COVID-19 infections were low at 8% with zero deaths. Reported recovery times were also short with only 9% reporting a recovery period greater than eight weeks, and 7% requiring hospitalization with one individual requiring a ventilator. Major challenges reported during the pandemic included stress management, particularly for those with LGMD (27%), and wearing a mask (24%). The majority reported a slight worsening of their disease state. Respondents reported moderate stress levels (stress score = 16.4; range = 0-39), with higher stress levels reported by women and those under age 30 years. Seventy-percent of participants who had telemedicine visits were satisfied with the encounters; however, most reported a preference for in-person visits. CONCLUSIONS: People with muscular dystrophy found ways to manage their stress and overcome obstacles during the COVID-19 pandemic. COVID-19 infection rates and medical complications were similar to a general population. Telemedicine visits may have a more permanent role in care.


Subject(s)
COVID-19 , Muscular Dystrophies, Limb-Girdle , Muscular Dystrophy, Facioscapulohumeral , Myotonic Dystrophy , Adult , COVID-19/epidemiology , Female , Humans , Male , Middle Aged , Myotonic Dystrophy/epidemiology , Pandemics
7.
Vasc Health Risk Manag ; 16: 463-466, 2020.
Article in English | MEDLINE | ID: covidwho-945492

ABSTRACT

SARS-CoV-2 infection is predominantly a respiratory disease with a diverse clinical spectrum. Pulmonary thromboembolic complications during COVID-19 pneumonia may be associated with a high mortality rate and post-mortem findings confirm the presence of platelet-fibrin thrombi in arterial vessels of patients together with lung tissue alterations. We present a patient transferred to the emergency department due to a syncope with no other associated symptoms, who was diagnosed with an acute pulmonary embolism (PE) concomitant with SARS-CoV-2 infection without lung infiltrates. Presenting with a PE as the only manifestation of this infection, reinforces our conception of COVID-19 as a heterogeneous disease of which we still know very little. We believe that while the virus is still circulating in our environment, we need to consider ruling out COVID-19 in all thrombotic events, even if the patients have no other risk factors.


Subject(s)
COVID-19/complications , Myotonic Dystrophy/complications , Pulmonary Embolism/etiology , COVID-19/diagnosis , COVID-19/therapy , Humans , Male , Middle Aged , Myotonic Dystrophy/diagnosis , Pulmonary Embolism/diagnosis , Pulmonary Embolism/therapy , Syncope/etiology , Treatment Outcome
9.
Acta Neurol Belg ; 121(6): 1761-1765, 2021 Dec.
Article in English | MEDLINE | ID: covidwho-866287

ABSTRACT

The coronavirus disease 2019 (COVID-19) pandemic is challenging health care systems worldwide. People with myotonic dystrophy type 1 (DM1) represent a high-risk population during infectious disease outbreaks, little is known about the potential impact of COVID-19 on patients with DM1. We studied the clinical course of COVID-19 in three hospitalized patients with myotonic dystrophy type 1 or Steinert's disease, between April 1, 2020-April 30-2020. All three had advanced Steinert's disease receiving non-invasive nocturnal home ventilatory support. Two of them lived in a residential care centre. Two patients had a limited respiratory capacity, whereas one patient had a rather preserved functional capacity but more comorbidities. Two out of three patients were obese, none of them had diabetes mellitus. Two patients received hydroxychloroquine. Despite maximal supportive care with oxygen therapy, antibiotics, intensive respiratory physiotherapy and non-invasive positive pressure ventilation, all three patients eventually died due to COVID-19. Our case series of three patients with DM1 admitted for COVID-19 confirms that they are at high risk for severe disease and poor outcome. Clinical trials are needed to define best practices and determinants of outcomes in this unique population.


Subject(s)
COVID-19/complications , Myotonic Dystrophy/complications , Adult , Female , Humans , Male , Middle Aged , Risk Factors , SARS-CoV-2
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