Enfermedad de Pompe y compromiso respiratorio

Introducción: El compromiso respiratorio en la enfermedad de Pompe es una de sus manifestaciones más frecuentes, tiene un impacto negativo en la calidad de vida y facilita la aparición de múltiples complicaciones. Se puede presentar como parte evolutiva de la enfermedad o incluso ser el síntoma inicial de la patología. Contenidos: La presentación clínica del compromiso respiratorio en la enfermedad de Pompe se caracteriza por disnea, ortopnea, cefalea y tos, y sus cambios paraclínicos incluyen hipercapnia, policitemia, elevación del índice de apnea/hipopnea, así como disminución de la capacidad vital y de las presiones inspiratoria y espiratorias máximas. El compromiso respiratorio es causado principalmente por disfunción muscular (especialmente el diafragma) y alteración de la regulación del sistema nervioso central. Conclusiones: Es fundamental la valoración acuciosa inicial de los patrones respiratorios y por supuesto su seguimiento, el cual dependerá del tipo de progresión de la disfunción respiratoria (rápida o lenta) o del requerimiento específico para cada paciente (ventilación no invasiva o invasiva).

Introduction: Respiratory compromise in Pompe disease is one of the most frequent manifestations, which has a negative impact on quality of life and leads to multiple complications. This can occur as an evolutionary part of the disease, or even be the initial symptom of the pathology. Contents: Its clinical presentation is characterized by dyspnea, orthopnea, headache, and cough, and its paraclinical changes include hypercapnia, polycythemia, elevated apnea/hypopnea index, decreased vital capacity, and decreased maximum inspiratory and expiratory pressures. Respiratory compromise is caused mainly by muscular dysfunction (especially the diaphragm) and alteration of regulation of the central nervous system. Conclusions: An initial careful assessment of respiratory patterns is essential, and of course their follow-up, that will depend of the type of progression of respiratory dysfunction (rapid or slow) or the specific requirement for each patient (non-invasive or invasive ventilation).

COVID-19 knowledge, attitudes, and practices among health care workers in Latin America / Conhecimento, atitudes e práticas em relação à COVID-19 entre profissionais de saúde na América Latina

ABSTRACT Objective: To evaluate COVID-19 knowledge, attitudes, and practices among health care workers (HCWs) practicing in Latin American countries during the first surge of the COVID-19 pandemic. Methods: This was a multinational cross-sectional survey study, using an online self-administered questionnaire. The final version of the questionnaire comprised 40 questions, organized in five sections: demographic and professional characteristics; COVID-19 knowledge; attitudes toward COVID-19; COVID-19 practices; and institutional resources. Results: The study involved 251 HCWs from 19 Latin American countries who agreed to participate. In our sample, 77% of HCWs participated in some sort of institutional training on COVID-19, and 43% had a low COVID-19 knowledge score. COVID-19 knowledge was associated with the type of health center (public/private), availability of institutional training, and sources of information about COVID-19. Concerns about not providing adequate care were reported by 60% of the participants. The most commonly used ventilatory strategies were protective mechanical ventilation, alveolar recruitment maneuvers, and prone positioning, and the use of drugs to treat COVID-19 was mainly based on institutional protocols. Conclusions: In this multinational study in Latin America, almost half of HCWs had a low COVID-19 knowledge score, and the level of knowledge was associated with the type of institution, participation in institutional training, and information sources. HCWs considered that COVID-19 was very relevant, and more than half were concerned about not providing adequate care to patients.

RESUMO Objetivo: Avaliar o conhecimento, atitudes e práticas em relação à COVID-19 entre profissionais de saúde atuantes em países da América Latina durante o primeiro surto da pandemia. Métodos: Estudo multinacional transversal com uso de questionário on-line autoaplicável. A versão final do questionário foi composta por 40 perguntas, organizadas em cinco seções: características demográficas e profissionais; conhecimento sobre COVID-19; atitudes em relação à COVID-19; práticas relacionadas à COVID-19; e recursos institucionais. Resultados: O estudo envolveu 251 profissionais de saúde de 19 países da América Latina que aceitaram participar. Em nossa amostra, 77% dos profissionais de saúde participaram de algum tipo de treinamento institucional sobre COVID-19 e 43% tiveram baixa pontuação de conhecimento sobre COVID-19. O conhecimento sobre COVID-19 apresentou associação com o tipo de instituição de saúde (pública/privada), disponibilidade de treinamento institucional e fontes de informação. Receio de não prestar atendimento adequado foi relatado por 60% dos participantes. As estratégias ventilatórias mais utilizadas foram ventilação mecânica protetora, manobras de recrutamento alveolar e posição prona, e o uso de medicamentos para tratar a COVID-19 foi baseado principalmente em protocolos institucionais. Conclusões: Neste estudo multinacional na América Latina, quase metade da amostra teve baixa pontuação de conhecimento sobre COVID-19 e o nível de conhecimento apresentou associação com o tipo de instituição, participação em treinamento institucional e fontes de informação. Os profissionais de saúde consideravam a COVID-19 muito relevante, e mais da metade tinha receio de não prestar atendimento adequado aos pacientes.

A propósito de complicaciones infrecuentes de enfermedades comunes. Síndrome de Ekiri / About Infrequent complications of common diseases: Ekiri Syndrome

Resumen La infección por enterobacterias desencadena usualmente síntomas gastrointestinales caracterizados por vómito, diarrea y dolor abdominal, de severidad y curso variable. El compromiso extraintestinal esta descrito, inclusive sin las manifestaciones gastrointestinales clásicas lo que favorece diagnósticos y tratamientos tardíos pudiendo llegar a ser incapacitantes y letales. El síndrome de Ekiri, también denominado síndrome de encefalopatía letal tóxico, se caracteriza por una disfunción aguda del sistema nervioso central secundaria a infección por enterobacterias las cuales favorecen el desarrollo de microtrombos a nivel cerebral con inflamación mediada por el factor de necrosis tumoral α e IL- 1β y disfunción de la barrera hematoencefálica con una alta tasa de mortalidad. Dentro de nuestro conocimiento, presentamos el primer caso reportado en Colombia sobre el síndrome de Ekiri.

Abstract Gastrointestinal infection usually manifests with nausea, vomit and abdominal pain, all of them wit course and variable severity. Extrainstestinal compromise is described, even without gastrointestinal symptoms, what causes a delay on diagnosis and treatment, worsening the prognosis. Ekiri syndrome, also known as lethal toxic encephalopathy is characterized by an acute neurological dysfunction secondary to enterobacterial infection which favor thrombi development and local inflammation mediated by tumor necrosis factor alpha and IL-1β with blood brain barrier dysfunction and high mortality. As we know, we present the first Ekiri syndrome case reported in Colombia.

Traqueobronquitis y neumonía asociadas a ventilación mecánica en unidades de cuidado intensivo de Latinoamérica: epidemiología, curso clínico y desenlaces (Estudio LATINAVE) / Epidemiology, clinical evolution and outcomes of tracheobronchitis and pneumonia associated with mechanical ventilation in intensive care units of Latin America (LATINAVE study)

Introducción: Las infecciones asociadas a ventilación mecánica son causa importante de morbimortalidad en el paciente crítico. La diferenciación entre traqueobronquitis y neumonía no es siempre fácil, y es controvertida. Algunos trabajos describen aumento de mortalidad, mayor estancia en Unidades de Cuidado Intensivo (UCI), mayor requerimiento de ventilación mecánica e incremento de costos en pacientes con traqueobronquitis asociada a ventilador (TAV), sin diferencias significativas en pacientes con neumonía asociada a ventilador (NAV). Estos estudios no describen el comportamiento clínico y epidemiológico de la TAV y la NAV como 2 entidades independientes, por lo que es necesario describirlo. Métodos: Estudio multicéntrico de cohorte prospectiva, de pacientes adultos que desarrollaron TAV o NAV durante su estancia en UCI, entre noviembre de 2013 y octubre de 2014. A cada una de las variables demográficas, clínicas, de laboratorio y de desenlace, como tiempo de ventilación mecánica, estancia hospitalaria y muerte, se le realizó análisis descriptivo; para evaluar las diferencias entre los grupos se utilizó test de chi cuadrado, t de Student o U de Mann Whitney. Resultados: Se incluyó a 143 pacientes, con edad promedio de 55 años, 57% eran hombres, de 6 países en Latinoamérica; 63% con NAV y 37% con TAV. Las comorbilidades más frecuentes fueron cardiovascular (44%) y neurológica (30%); esta última fue más frecuente en TAV (41,5 vs. 23%; p = 0,02). No se encontró diferencia en APACHE II de ingreso. El índice SOFA fue mayor en NAV (8 vs. 5; p = 0,02). No hubo diferencias en el aislamiento microbiológico, ni en los patrones de resistencia bacteriana entre las 2 entidades. Se observó mayor número de complicaciones cardiovasculares y SDRA en pacientes con NAV. No se encontró diferencia entre los 2 grupos en estancia en UCI, los días de ventilación mecánica ni en mortalidad. Conclusiones: La prevalencia de TAV fue mayor a lo descrito hasta ahora en la literatura. No se encontraron diferencias significativas en el aislamiento microbiológico, la resistencia bacteriana ni el esquema antibiótico utilizado en los 2 grupos. Aunque la NAV cursó con mayor proporción de complicaciones médicas asociadas, el hallazgo de una estancia hospitalaria, tiempo de ventilación mecánica y mortalidad similares sustenta la importancia clínica de la TAV.

Introduction: The infections associated with mechanical ventilation are a major cause of morbidity and mortality in critically ill patients. Limited studies report increased mortality and intensive care units (ICU) stays, requirements for mechanical ventilation and higher costs in ventilator-associated tracheobronchitis (TAV) in comparison to patients with ventilator-associated pneumonia (NAV). These studies do not describe the clinical and epidemiological behavior in the same population as independent entities, so it is necessary to describe the epidemiology of patients with TAV and NAV. Methods: Multicenter cross-sectional study of adult patients who developed TAV and/or NAV during their stay in the ICU in 2013 to 2014. A descriptive analysis was performed on each of the variables. For qualitative variables we assessed differences between groups using the Chi-squared test; for continuous variables, we used Student's t test or the Mann Whitney U test. Results: A total of 147 patients from 6 countries in Latin America were included; 63% with NAV and 37% with TAV. The average age was 55 years; 57% male. The most frequent comorbidity was cardiovascular (44%) and neurological (30%), the latter was more frequent in those with TAV (41.5 vs. 23%, P = .02). No differences were found in APACHE II on entry, but the difference appears in the SOFA index (8 vs. 5, P = .02). There were no differences in microbiological isolation, or bacterial resistance patterns between the 2 entities. A greater number of cardiovascular complications and ARDS were observed in patients with NAV. The ICU stay, days on mechanical ventilation and mortality were not different between the 2 groups. Conclusions: The TAV prevalence was higher than heretofore described in the literature. No significant differences were found in the microbiological isolation, bacterial resistance and antibiotic therapy used in the 2 groups, which might suggest that therapeutic approach be similar to that recommended for NAV. No differences were observed in clinical outcomes such as hospital stay, duration of mechanical ventilation and mortality, although NAV was associated with a greater proportion of medical complications.