Global, regional, and national burden of disorders affecting the nervous system, 1990–2021: a systematic analysis for the Global Burden of Disease Study 2021

BackgroundDisorders affecting the nervous system are diverse and include neurodevelopmental disorders, late-life neurodegeneration, and newly emergent conditions, such as cognitive impairment following COVID-19. Previous publications from the Global Burden of Disease, Injuries, and Risk Factor Study estimated the burden of 15 neurological conditions in 2015 and 2016, but these analyses did not include neurodevelopmental disorders, as defined by the International Classification of Diseases (ICD)-11, or a subset of cases of congenital, neonatal, and infectious conditions that cause neurological damage. Here, we estimate nervous system health loss caused by 37 unique conditions and their associated risk factors globally, regionally, and nationally from 1990 to 2021.MethodsWe estimated mortality, prevalence, years lived with disability (YLDs), years of life lost (YLLs), and disability-adjusted life-years (DALYs), with corresponding 95% uncertainty intervals (UIs), by age and sex in 204 countries and territories, from 1990 to 2021. We included morbidity and deaths due to neurological conditions, for which health loss is directly due to damage to the CNS or peripheral nervous system. We also isolated neurological health loss from conditions for which nervous system morbidity is a consequence, but not the primary feature, including a subset of congenital conditions (ie, chromosomal anomalies and congenital birth defects), neonatal conditions (ie, jaundice, preterm birth, and sepsis), infectious diseases (ie, COVID-19, cystic echinococcosis, malaria, syphilis, and Zika virus disease), and diabetic neuropathy. By conducting a sequela-level analysis of the health outcomes for these conditions, only cases where nervous system damage occurred were included, and YLDs were recalculated to isolate the non-fatal burden directly attributable to nervous system health loss. A comorbidity correction was used to calculate total prevalence of all conditions that affect the nervous system combined.FindingsGlobally, the 37 conditions affecting the nervous system were collectively ranked as the leading group cause of DALYs in 2021 (443 million, 95% UI 378–521), affecting 3·40 billion (3·20–3·62) individuals (43·1%, 40·5–45·9 of the global population); global DALY counts attributed to these conditions increased by 18·2% (8·7–26·7) between 1990 and 2021. Age-standardised rates of deaths per 100 000 people attributed to these conditions decreased from 1990 to 2021 by 33·6% (27·6–38·8), and age-standardised rates of DALYs attributed to these conditions decreased by 27·0% (21·5–32·4). Age-standardised prevalence was almost stable, with a change of 1·5% (0·7–2·4). The ten conditions with the highest age-standardised DALYs in 2021 were stroke, neonatal encephalopathy, migraine, Alzheimer's disease and other dementias, diabetic neuropathy, meningitis, epilepsy, neurological complications due to preterm birth, autism spectrum disorder, and nervous system cancer.InterpretationAs the leading cause of overall disease burden in the world, with increasing global DALY counts, effective prevention, treatment, and rehabilitation strategies for disorders affecting the nervous system are needed

Assessment of Left Atrial Ejection Force in Mildly Asphyxiated Newborns

Objective: Asphyxia-induced cardiac insult is one of the major causes of mortality and morbidity in the course of perinatal asphyxia. Nowadays, a remarkable trend of interest is sensed introducing a plausible modality for early detection of cardiac insults at the beginning stages of asphyxia. In this study we aimed to evaluate diagnostic utility of transmitral Doppler–derived parameters as well as left atrial ejection force index as a marker of left atrial contractile function in these patients. Methods: In a prospective study selected cases of 26 asphyxiated newborns with preserved systolic function underwent conventional transmitral Doppler flow echocardiographic assessment. Left atrial ejection force index was further calculated for all patients. Data was compared with normal ranges of healthy newborns in order to clarify the diagnostic utility of these parameters for determining minor cardiac insults in this age group. Findings: We found that mildly asphyxiated newborns showed an increase in the values of left atrial ejection force index (5.44±2.12 kilodyne vs. 6.66±2.17 kilodyne, P= 0.02) and left atrial filling fraction (39%±10% vs. 45%±8%, P= 0.01). Furthermore, the acceleration and deceleration rate of early filling flow peak velocity were decreased in this group of asphyxiated newborns. Conclusion: Assessment of left atrial ejection force in mildly asphyxiated newborns reveals that newborns with even mild asphyxia, although could not be categorized in conventional grading system, suffer to some extent from a ventricular filling abnormality. This type of latent ventricular filling abnormality could simply be unmasked by calculation of atrial ejection force index

Evaluation of mobile phone-based tele-monitoring of cystic fibrosis patients during the COVID-19 pandemic: A 3-year experience in Iran

Background: Telemedicine has been used for cystic fibrosis (CF) in a wide range of signs and symptoms even before the COVID 19 pandemic, however, little is known about the health consequences and use of specific health care for cystic CF. This study aimed to evaluate the evolution of clinical trends and data related to mobile based monitoring activities in CF patients at home for 3 years. Methods: This is a semi experimental single group study. Forty five CF patients under 7 years' old who were referred to the Masih Daneshvari Hospital between 2018 and 2021 were selected. A mobile phone_based customized Short Message Service (SMS) application used to monitor patients. Remotely monitored variables included the amount and color of sputum, cough, wheezing, and shortness of breath at rest. SPSS using Chi square and Friedman tests. Results: The condition of patients based on the number and type of cough increased sputum, decreased appetite, fatty stool, fever and dyspnea, headache, noninvasive ventilation, and drug comfortably remained almost unchanged in the study of the 1st, 2nd, and 3rd years, and the studied parameters did not show a significant difference (P > 0.05). Of course, the number of outpatient visits decreased significantly (P value: 0.02). The respiratory rate and arterial oxygen saturation variables were almost the same in three consecutive annual measurements (P values: 0.544 and 0.639, respectively). Conclusion: Telemedicine is a method that is useful in the follow up of chronic diseases such as CF and improves the quality of life and reduces the deterioration of lung function; therefore, there is less need for invasive treatments in the long run, and a fundamental change in referral motivation brings to the hospital

Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome

Abstract Background STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. Method Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. Result Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. Conclusion Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies

LPS-Responsive Beige-Like Anchor Gene Mutation Associated With Possible Bronchiolitis Obliterans Organizing Pneumonia Associated With Hypogammaglobulinemia and Normal IgM Phenotype and Low Number of B Cells

LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. In spite of therapeutic doses of antibiotics, antivirals, and antifungal agents, in addition to immunoglobulin replacement therapy, he developed disseminated involvement of both lungs with peripheral nodules; transbronchial lung biopsy revealed possible bronchiolitis obliterans organizing pneumonia (BOOP). Combined homozygosity mapping and exome sequencing identified a homozygous LRBA mutation in this patient (p.Asp248Glufs*2). Such clinical and immunological findings have not been described to date and illustrate the broad and variable clinical phenotype of human LRBA deficiency