RESUMEN
The recent pandemic prompted renewed interest in paediatric respiratory infections, including whether co-infections - particularly with RSV - have an adverse prognostic impact. We evaluated the charts of all children presenting with respiratory symptoms to our unit between October 2022 and April 2023, each of whom was subjected to a multiplex PCR assay to detect eight viral targets and one bacterial target and examine the relationships between mono- and co-infections and hospitalization outcomes. We observed that younger age and RSV infection were both associated with the need for hospitalisation and the duration of hospitalisation after adjusting for confounders. Co-infection was, however, not associated with these outcomes. Conclusion: This real-world data add to a growing consensus that RSV increases the risk of hospitalisation, while other co-infections, except for co-infection with SARS-CoV-2, do not. Given the timeframe over which our study was conducted, only a few children had SARS-CoV-2 co-infection, so we could not confirm any significant effect from this interaction. What is Known: ⢠RSV increases the risk of hospitalisation and the need tor ventilatory support, especially in very young children. What is New: ⢠Younger age and RSV infection were both associated with the need for hospitalisation and the duration of hospitalisation after adjusting for confounders. ⢠Co-infection was, however, not associated with these outcomes.
Asunto(s)
Coinfección , Infecciones por Virus Sincitial Respiratorio , Infecciones del Sistema Respiratorio , Humanos , Niño , Lactante , Preescolar , Infecciones por Virus Sincitial Respiratorio/complicaciones , Infecciones por Virus Sincitial Respiratorio/diagnóstico , Infecciones por Virus Sincitial Respiratorio/epidemiología , Coinfección/epidemiología , Factores de Riesgo , Hospitalización , Infecciones del Sistema Respiratorio/epidemiología , Infecciones del Sistema Respiratorio/complicacionesRESUMEN
Monogenic autoinflammatory diseases (AIDs) are a group of inflammatory disorders induced by deregulation of the innate immune system and characterised by inflammatory bouts with fever as well as a large spectrum of other possible manifestations involving most organs and tissues. In this context, musculoskeletal manifestations represent a frequent finding in the clinical picture of patients with AIDs and may range from less severe affections including myalgia and arthralgia to severe arthritis, muscle fasciitis, bone erosions, and joint deformities. Therefore, as diagnostic suspicions originates from a careful and detailed clinical evaluation, physicians dealing with mono- genic AIDs should bear in mind their possible muscle and joint manifestations, some of which are typical and very useful for diagnostic purposes. Indeed, their prompt recognition may reduce the diagnostic delay thus allowing an early and appropriate therapeutic management. For these reasons, the present review is aimed at providing a wide overview on the different patterns of joint and muscle affections in the four main monogenic AIDs.
Asunto(s)
Artralgia/fisiopatología , Artritis/fisiopatología , Enfermedades Autoinflamatorias Hereditarias/fisiopatología , Mialgia/fisiopatología , Artralgia/etiología , Artritis/etiología , Enfermedades Autoinflamatorias Hereditarias/complicaciones , Humanos , Enfermedades Musculoesqueléticas/etiología , Enfermedades Musculoesqueléticas/fisiopatología , Mialgia/etiologíaRESUMEN
Autoinflammatory diseases are caused by inflammasome dysregulation leading to overproduction of proinflammatory cytokines and a pathological delay in the inflammation switching off. The progress of cellular biology has partially clarified pathogenic mechanisms behind monogenic autoinflammatory diseases, whereas little is known about the polygenic ones. Although the genetic susceptibility of periodic fever, aphthous stomatitis, pharyngitis, and adenopathy (PFAPA) syndrome is still obscure, the presence of overlapping symptoms with monogenic periodic fevers, the recurrence in family members, the important role played by dysregulated interleukin- (IL-) 1ß secretion during flares, the overexpression of inflammasome-associated genes during attacks, and, last but not least, the therapeutic efficacy of IL-1ß blockade strongly indicate a potential genetic involvement in its pathogenesis, probably linked with environmental factors. PFAPA syndrome has a typical inception in the pediatric age, but a delayed onset during adulthood has been described as well. Treatments required as well as effectiveness of tonsillectomy remain controversial, even if the disease seems to have a self-limited course mostly in children. The purpose of this review is to provide an overview of this complex polygenic/multifactorial autoinflammatory disorder in which the innate immune system undoubtedly plays a basic role.
Asunto(s)
Fiebre/inmunología , Fiebre/patología , Neoplasia Endocrina Múltiple/inmunología , Neoplasia Endocrina Múltiple/patología , Faringitis/inmunología , Faringitis/patología , Estomatitis Aftosa/inmunología , Estomatitis Aftosa/patología , Adulto , Niño , Femenino , Fiebre/metabolismo , Humanos , Interleucina-1beta/metabolismo , Masculino , Neoplasia Endocrina Múltiple/metabolismo , Faringitis/metabolismo , Estomatitis Aftosa/metabolismoRESUMEN
During the initial phase of the national lockdown, we found that there were sharp decreases in admissions to two pediatric emergency departments (EDs) in northern Italy (Cremona and Novara). Here we present a detailed analysis of these admission patterns and types of admissions over a longer timeframe. ED admissions data were anonymously extracted from the departmental management software. Admissions data from 2019 and 2020 were analyzed and compared separately for each ED and combined. There was a 73.2% decrease in total admissions compared with the same period in 2019. With respect to admission diagnoses, there was a significant (p < 0.001) drop in infectious (-51%), respiratory (-25.5%), and nervous systems diseases (-50%) and injuries and poisoning (-17%) but not endocrine, metabolic, neoplastic, circulatory, or musculoskeletal diseases. White codes (patients with minor injuries for whom ED medical care is not required) significantly decreased by 56.3% (p < 0.001). Even if the COVID-19 pandemic represented an enormous healthcare burden in Italy, especially during the first months of the pandemic (late February to May), the workload of pediatric EDs was significantly reduced, especially for unnecessary accesses (white codes).
RESUMEN
Autoimmune diseases affect up to 10% of the world's population and, as a whole, they are far more common in females, although differences exist according to the single disease and also in different age groups. In childhood-onset autoimmune diseases, the sex bias is generally less evident than in adults, probably for the different hormonal milieau, being estrogens strongly implicated in the development of autoimmunity. Still, some rheumatic conditions, such as juvenile idiopathic arthritis (JIA), show a strong predilection for girls (F:M = 3-6.6:1), and differences may coexist between males and females regarding disease outcome. For example, chronic anterior uveitis associated with JIA affects more commonly girls but boys tend to have a more severe course. Systemic lupus erythematosus predominantly affects girls and women (F:M = 3-5:1 in children, F:M = 10-15:1 in adults). Behςet's disease has been reported to be more prevalent in adult males (F:M = 1:1-4); in children, there are no differences. The sex ratio is equal in children and adults for Henoch-Schönlein purpura (F:M = 1:1). A higher male-to-female ratio exists for Kawasaki disease (F:M = 1:1.1-1.6 in children, F:M = 1:1,5 in adults). Juvenile dermatomyositis (F:M = 2-5:1), systemic sclerosis (F:M = 4:1 in children, F:M = 6:1 in adults), and Takayasu arteritis (F:M = 2:1 in children, F:M = 7-9:1 in adults) are more common in girls and women then in boys and men. There is no gender bias for acute rheumatic fever in children, while in adults, the F:M ratio is 2:1. Given that estrogen levels are not different between genders during childhood, pediatric rheumatic diseases could represent good models to study other mechanisms related to the development of autoimmunity. Recently, the levels of miRNA expression, and their variation according to sex chromosomes, have been linked to the development of autoimmune diseases, with different impact among sexes. This review will focus not only on the sex bias reported in the more common rheumatic conditions of childhood, focusing on differences in incidence, but also on outcome and trying to depict the mechanisms underlying those differences.
Asunto(s)
Artritis Juvenil/epidemiología , Dermatomiositis/epidemiología , Vasculitis por IgA/epidemiología , Lupus Eritematoso Sistémico/epidemiología , Síndrome Mucocutáneo Linfonodular/epidemiología , Fiebre Reumática/epidemiología , Esclerodermia Sistémica/epidemiología , Arteritis de Takayasu/epidemiología , Adolescente , Adulto , Artritis Juvenil/fisiopatología , Autoinmunidad , Niño , Preescolar , Dermatomiositis/fisiopatología , Exposición a Riesgos Ambientales/efectos adversos , Femenino , Predisposición Genética a la Enfermedad , Humanos , Vasculitis por IgA/fisiopatología , Incidencia , Lupus Eritematoso Sistémico/fisiopatología , Masculino , Síndrome Mucocutáneo Linfonodular/fisiopatología , Prevalencia , Fiebre Reumática/fisiopatología , Esclerodermia Sistémica/fisiopatología , Factores Sexuales , Arteritis de Takayasu/fisiopatologíaRESUMEN
Monogenic autoinflammatory disorders (AIDs) are rare diseases driven by cytokine-mediated extraordinary sterile inflammation that results from the activation of innate immune pathways. The clinical hallmark of these diseases is the recurrence of stereotyped episodes of systemic- and organ-specific inflammation; the most common systems involved being the skin, musculoskeletal system, gastrointestinal tract, and central nervous system. The autoinflammatory disorders may have a profound impact on the quality of life of the affected patients, and a delayed diagnosis may lead to severe complications, the most dreadful of which is AA-Amyloidosis. This review gives an overview on the four main AIDs, namely familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, cryopyrinopathies, and mevalonate kinase deficiency, focusing on their clinical phenotype in adults and differential diagnosis, suggesting a diagnostic algorithm, and reviewing the available treatments.