Efecto de una campaña de concienciación en el diagnóstico e impacto clínico de la inmunodeficiencia primaria / Effect of an awareness campaign on the diagnosis and clinical impact of primary immunodeficiency

Antecedentes y objetivo: Las deficiencias predominantemente de anticuerpos constituyen, en la actualidad, el grupo de inmunodeficiencias primarias (IDP) más prevalente en adultos. Son enfermedades complejas desde el punto de vista clínico, catalogadas como minoritarias y que tienen a menudo un retraso inaceptable en su diagnóstico. El objetivo de este estudio fue evaluar si un mejor conocimiento de estas entidades podía conllevar un incremento en el número de diagnósticos, una reducción en el intervalo al diagnóstico y, por ende, una disminución en la carga de enfermedad al diagnóstico.Pacientes y métodosSe diseñó un estudio de intervención casi experimental y Unicentro, que incluyó dos períodos, período 1 preintervención (1986-2008) y período 2 postintervención (2009-2018). Se efectuó un estudio descriptivo comparativo de diversas variables en ambos períodos.ResultadosSe incluyeron 116 pacientes [27 (23,3%) en el período 1 y 89 (76,7%) en el período 2]. La tasa de incidencia aumentó de forma significativa (0,204 y 1,236/100.000 habs./año; P < 0,05), el retraso en el diagnóstico tendió a ser menor (4 vs. 3,73 años), los motivos de sospecha diagnóstica se diversificaron y la carga de enfermedad al diagnóstico (expresada por bronquiectasias, espirometría alterada, capacidad de generar anticuerpos por mecanismo timo-independiente y necesidad de tratamiento substitutivo) tendió a disminuir en el período 2.ConclusionesDadas las complicaciones potencialmente graves de los pacientes con diagnóstico tardío de IDP, es necesaria la creación de unidades multidisciplinarias especializadas, la unificación de protocolos asistenciales y el diseño de intervenciones para la divulgación de esta entidad. (AU)

Background and objectives: Predominantly antibody deficiencies are the most prevalent primary immunodeficiency (PID) in adults. These are rare diseases difficult to diagnose. Therefore, they are diagnosed late. This study aims to evaluate whether an awareness campaign of PIDs among physicians is associated with an increase in number of diagnoses, a reduction in diagnostic delay and diagnosis at earlier stages.Patients and methodsA single centre, interventional, quasi-experimental study was designed that included 2 periods, period 1 pre-intervention (1986-2008) and period 2 post-intervention (2009-2018). A descriptive comparative study of variables was carried out in both periods.Results116 patients were included [27 (23.3%) in period 1 and 89 (76.7%) in period 2]. The incidence rate increased significantly (0.204 and 1.236/100,000habs./year; P < 0.05), the diagnosis delay tended to be lower (4 vs. 3.73 years). The reasons for diagnostic suspicion were diverse and the burden disease at diagnosis (expressed by bronchiectasis, altered spirometry, ability to generate antibodies by thymus-independent mechanism and need for substitute treatment) tended to decrease in period 2.ConclusionsGiven the potentially serious complications of patients with late diagnosis of PIDs, it is necessary to create specialized multidisciplinary units, to unify assistance protocols and to design interventions to increase the knowledge of these entities. (AU)

Effect of an awareness campaign on the diagnosis and clinical impact of primary immunodeficiency. / Efecto de una campaña de concienciación en el diagnóstico e impacto clínico de la inmunodeficiencia primaria.

BACKGROUND AND OBJECTIVES: Predominantly antibody deficiencies are the most prevalent primary immunodeficiency (PID) in adults. These are rare diseases difficult to diagnose. Therefore, they are diagnosed late. This study aims to evaluate whether an awareness campaign of PIDs among physicians is associated with an increase in number of diagnoses, a reduction in diagnostic delay and diagnosis at earlier stages. PATIENTS AND METHODS: A single centre, interventional, quasi-experimental study was designed that included 2 periods, period 1 pre-intervention (1986-2008) and period 2 post-intervention (2009-2018). A descriptive comparative study of variables was carried out in both periods. RESULTS: 116 patients were included [27 (23.3%) in period 1 and 89 (76.7%) in period 2]. The incidence rate increased significantly (0.204 and 1.236/100,000habs./year; P < 0.05), the diagnosis delay tended to be lower (4 vs. 3.73 years). The reasons for diagnostic suspicion were diverse and the burden disease at diagnosis (expressed by bronchiectasis, altered spirometry, ability to generate antibodies by thymus-independent mechanism and need for substitute treatment) tended to decrease in period 2. CONCLUSIONS: Given the potentially serious complications of patients with late diagnosis of PIDs, it is necessary to create specialized multidisciplinary units, to unify assistance protocols and to design interventions to increase the knowledge of these entities.

Comparative molecular and antibody typing during the investigation of an outbreak of Legionnaires' disease.

An outbreak of Legionnaires' disease with 113 confirmed cases was reported in the town of Mataró, Spain, in August 2002. In this study, we compared three different typing methods and characterized the clinical isolates by comparing them with other clinical isolates with the same ST from our own database to further characterize the outbreak. In the outbreak, a total of 16 clinical (nine patients) and 32 environmental (from four environmental sources) Legionella pneumophila isolates were analyzed by pulsed-field electrophoresis (PFGE), sequence-based typing (SBT), and monoclonal antibody typing (MAb). We compared the MAb and SBT profiles of the outbreak clinical isolates and other unrelated clinical isolates showing the same ST profile. We obtained seven different PFGE and SBT profiles and six MAb patterns from the outbreak isolates. PFGE and SBT showed 100% concordance during the outbreak. SBT proved to be highly discriminatory, particularly with the addition of the new neuA gene. One PFGE, SBT (ST-37), and Philadelphia profile was observed among the clinical isolates. Using PFGE, this ST37 Philadelphia profile was closely related to other unrelated clinical isolates. These findings suggest that the ST37 Philadelphia profile could be a virulence marker in our area. The combination of the three methodologies was useful to further characterize and obtain additional information on a very explosive outbreak. Despite the minor discrimination of PFGE versus SBT, the two genetic methods are recommended in outbreak investigations. Further studies are currently underway in this area to obtain more definitive conclusions.

Persistence of Legionella in hospital water supplies and nosocomial Legionnaires' disease.

The molecular epidemiology of clinical and environmental Legionella species isolates was studied in seven hospitals from 1989 to 2006. The number of environmental pulsed field gel electrophoresis (PFGE) patterns ranged from one to nine according to the hospital. Genomic PFGE pattern persistence was observed in 71% of the hospitals, even after 17 years in some hospitals, and the relationship between environmental and clinical isolates was established. The isolates associated with hospital-acquired Legionnaires' disease corresponded to the persistent environmental PFGE patterns of Legionella pneumophila in potable water supplies.

Bacteriemia por Staphylococcus aureus. Mortalidad y resistencia asociada a la meticilina / Staphylococcus aureus bacteremia. Mortality and resistance to methycillin

No disponible

Legionelosis. Del primer caso al brote / Legionnaire’s disease. From the first case to the outbreak

No disponible

Legionella pneumophila serogrupo 1 / Legionella pneumophilia serogroup 1

No disponible

Legionella spp.: community acquired and nosocomial infections.

PURPOSE OF REVIEW: The key points of this review are the increasingly recognized risk of home-acquired Legionnaires' disease; the significance and potential pathogenic role of other species of Legionella spp., different from L. pneumophila, and of other microorganisms that are phylogenetically close to Legionella and that have been named as Legionella-like amoebal pathogens; the breakthrough in the diagnosis of the disease caused by new commercially available urine antigen detection tests; the controversy over sensitivity and specificity of serological diagnostic methods; the recognition of a variety of possible mixed infections, particularly in the immunocompromised population; and new and controversial aspects of the therapeutic approach to legionellosis. RECENT FINDINGS: During the last year a number of articles have provided clinically relevant insights into our knowledge of Legionnaires' disease. In view of the fact that Legionella spp. have progressively become recognized as relatively common causative agents of both community-acquired and nosocomial legionellosis, this is an opportune moment for this review. SUMMARY: If domestic aquatic reservoirs were eventually confirmed as significant agents of transmission of legionellosis, the adoption of preventive measures would then be crucial. The progressive identification of other species, different from L. pneumophila, as causative agents of pneumonia should both encourage microbiologists and clinicians to improve their diagnostic methodology and increase the awareness of these infections. Finally, the awareness of mixed infections, probably far more severe and perhaps not so uncommon as previously thought, has important clinical connotations for both the diagnostic and the therapeutic approach to legionellosis in the immunosuppressed host, particularly in those cases of delayed clinical resolution.

Legionnaires' disease: update on epidemiology and management options.

Infection with Legionella spp. is an important cause of serious community- and hospital-acquired pneumonia, occurring sporadically and in outbreaks. Outbreaks of Legionnaires' disease have recently received considerable media attention, and some factors indicate that the problem will increase in future. Infection with Legionella spp. ranks among the three most common causes of severe pneumonia in the community setting, and is isolated in 1-40% of cases of hospital-acquired pneumonia. Underdiagnosis and underreporting are high. Only 2-10% of estimated cases are reported. Detection of a single case should not be considered an isolated sporadic event, but rather indicative of unrecognized cases. There are no clinical features unique to Legionnaires' disease; however, suspicion should be raised by epidemiologic information commensurate with the diagnosis and the presence of headache, confusion, hyponatremia, elevated creatine kinase and/or severe pneumonia. An arterial oxygen partial pressure <60mm Hg on presentation and progression of pulmonary infiltrates despite appropriate antibacterial therapy should always alert clinicians to this cause.Macrolides, fluoroquinolones and rifampin (rifampicin) are the most widely used drugs in treatment. Fluoroquinolones or azithromycin are the treatment of choice in immunosuppressed patients and those with severe pneumonia. Incorporation of the legionella urinary antigen test in emergency departments in hospitals and progressive improvement in this test will, in the near future, permit appropriate diagnosis and treatment of this frequent, sometimes severe, illness.

Hospital-acquired legionellosis: solutions for a preventable infection.

Hospital-acquired Legionnaires' disease has been reported from many hospitals since the first outbreak in 1976. Although cooling towers were linked to the cases of Legionnaires' disease in the years after its discovery, potable water has been the environmental source for almost all reported hospital outbreaks. Microaspiration is the major mode of transmission in hospital-acquired Legionnaires' disease; showering is not a mode of transmission. Since the clinical manifestations are non-specific, and specialised laboratory testing is required, hospital-acquired legionellosis is easily underdiagnosed. Discovery of a single case of hospital-acquired Legionnaires' disease is an important sentinel of additional undiscovered cases. Routine environmental culture of the hospital water supply for legionella has proven to be an important strategy in prevention. Documentation of legionella colonisation in the water supply would increase physician index of suspicion for Legionnaires' disease and the necessity for in-house legionella test methods would be obvious. Legionella is a common commensal of large-building water supplies. Preventive maintenance is commonly recommended; unfortunately, this measure is ineffective in minimising legionella colonisation of building water supplies. Copper-silver ionisation systems have emerged as the most successful long-term disinfection method for hospital water disinfection systems. There is a need for public-health agencies to educate the public and media that discovery of cases identifies those hospitals as providers of superior care, and that such hospitals are not negligent.

Bacillus anthracis y bioterrorismo / Bacillus anthracis and bioterrorism

No disponible