Associations of the COVID-19 pandemic with social well-being indicators in Mexico.

BACKGROUND: Public health measures for COVID-19 containment have implied economic and social life disruptions, which have been particularly deleterious in low- and middle-income countries (LMIC) due to high rates of informal employment, overcrowding, and barriers to accessing health services, amongst others social determinants. Mexico, a LMIC, is a country with a high COVID-19 mortality in which there has been a very limited governmental response to help mitigate such COVID-related disruptions. This study analyzes the association of the first wave of the COVID-19 crisis in Mexico with four well-being indicators: income, employment, anxiety, and food security. METHODS: It uses pooled cross-sectional data (n = 5453) of five monthly nationally representative surveys collected between April and August 2020. Probit models are estimated to assess the association of the pandemic with job loss and anxiety; a multinomial logistic regression is estimated for food security, and an ordinary least squares regression assesses the association between the pandemic and changes in household's income. RESULTS: Females were significantly associated with worse outcomes for the 4 well-being measures with an average reduction of 2.3% in household income compared to pre-COVID-19 levels, an increased probability (6.4 pp) of being in a household that had lost jobs, decreased probability of food security (6.9 pp), and an increased risk of anxiety symptoms (8.5 pp). In addition, those with lower SES and household with children also reported worse outcomes for employment, income and food security. The month variable was also statistically significant in these models suggesting that as more months of the pandemic elapsed the effects persisted. CONCLUSION: The currents study documents how the COVID-19 pandemic is associated with different well-being indicators in a LMIC. It suggests the urgent need to take actions to support vulnerable groups, particularly women, households with children and those in the lowest SES. If policy actions are not taken, the pandemic will increase social and gender disparities, and will jeopardize childhood development.

Meningococcemia en el contexto de una mononucleosis infecciosa / Meningoccocal disease in a patient with infectious mononucleosis

En la evaluación de los pacientes con síndrome febril, la detección de petequias asociada a un deterioro clínico importante debe orientar el diagnóstico de enfermedad meningocócica invasora. Sin embargo, algunos pacientes presentan un cuadro clínico sutil o atípico que dificulta el diagnóstico; los signos de alarma clínicos y analíticos son útiles para identificar a los niños que se beneficiarán de un tratamiento empírico precoz que mejore su pronóstico. Presentamos el caso de un niño de 3 años de edad, diagnosticado en nuestro centro de una enfermedad meningocócica invasora, insospechada por su evolución atípica, que acude al servicio de urgencias por presentar fiebre de 4 días de evolución, petequias y púrpura palpable, aunque manteniendo un buen estado general en todo momento (AU)

In the evaluation of patients with a febrile syndrome, detection of petechiae associated to an ill appearance should make us suspect an invasive meningococcal disease. Nevertheless, some patients present in a more subtle manner or with an atypical course that will make difficult the diagnosis. Clinical and analytical alarm sings could be useful to identify these children who would benefit from an early empirical treatment that improves their prognosis. We report a case of unsuspected invasive meningococcal disease because of unusual presentation in a 3-years-old boy. He came to the emergency room complaining of 4 days fever, petechiae and palpable purpura. The patient's clinical condition was all time preserved (AU)

Crisis asmática en los servicios de urgencias en Espa˜na, ¿cuál es nuestra práctica habitual? / Asthma crisis in emergency departments in Spain: what is our usual practice?

Introducción: A pesar de la existencia de múltiples guías y documentos de consenso sobre el asma, persiste todavía una gran variabilidad en su abordaje, situación poco analizada en los Servicios de Urgencias (SU). Objetivo: Describir el manejo habitual de las crisis asmáticas en los SU españoles, conocer el grado de variabilidad entre sus diferentes profesionales y analizar los factores que pueden influir en su manejo. Material y métodos: Se elaboró un cuestionario, basado en supuestos clínicos, que se distribuyó entre los socios de la Sociedad Española de Urgencias de Pediatría y asistentes a la reunión en 2011. Resultados: Se analizan 300 encuestas. En 190 casos, el lugar de trabajo habitual es un hospital de tercer nivel. Existe una alta coincidencia en la valoración de la gravedad de los casos. Más del 90% utiliza beta 2 agonistas inhalados en la crisis asmática leve, el 75,3% utiliza corticoides orales en crisis moderadas y el 79% utiliza bromuro de ipratropio en las crisis graves. El 98% considera importante hacer educación terapéutica en los SU. Solo 8 (2,7%) utilizan la medición del pico de flujo espiratorio máximo. Los encuestados que trabajan en un hospital de tercer nivel utilizan con más frecuencia escalas de gravedad (90,5% vs. 80%, p = 0,009) y administran corticoides parenterales con menos frecuencia (53,2% vs. 66,4%, p = 0,017). Conclusiones: Aunque un porcentaje elevado de pediatras que trabajan en los SU siguen las guías de práctica clínica, se han detectado aspectos en los que el abordaje del asma es discordante entre los diferentes profesionales(AU)

Background: Despite there being various guidelines and consensus statements on asthma, great variability still exists in its treatment approach; although this has been little discussed in Emergency Departments (ED). Objective: To describe the usual management of asthma exacerbations in Spanish ED, and to determine the degree of variability among different professionals, as well as analyse factors that may influence their management. Material and methods: A questionnaire was developed based on clinical cases, which was distributed among members of the Spanish Society of Paediatric Emergencies and attendees at the meeting in 2011. Results: A total of 300 surveys were analysed. In 190 cases the place of work was in a tertiary care hospital. There was high agreement in the assessment of the severity of the cases mentioned. Over 90% use inhaled beta 2 agonists in mild asthma exacerbations, 75.3% used oral corticosteroids in moderate exacerbations, and 79% use ipratropium bromide in severe exacerbations. Almost all (98%) considered Therapeutic Education important in the Emergency Department. Only 8 (2.7%) used peak expiratory flow measurements. Respondents working in a tertiary hospital used severity scales more frequently (90.5% vs 80%, P=0.009) and administered parenteral corticosteroids less frequently (53.2% vs 66.4%, P=0.017). Conclusions: Although a high percentage of paediatricians working in the ED follow Clinical Practice Guidelines, some different points of the approach among professionals have been identified(AU)

[Asthma crisis in emergency departments in Spain: what is our usual practice?]. / Crisis asmática en los servicios de urgencias en España, ¿cuál es nuestra práctica habitual?

BACKGROUND: Despite there being various guidelines and consensus statements on asthma, great variability still exists in its treatment approach; although this has been little discussed in Emergency Departments (ED). OBJECTIVE: To describe the usual management of asthma exacerbations in Spanish ED, and to determine the degree of variability among different professionals, as well as analyse factors that may influence their management. MATERIAL AND METHODS: A questionnaire was developed based on clinical cases, which was distributed among members of the Spanish Society of Paediatric Emergencies and attendees at the meeting in 2011. RESULTS: A total of 300 surveys were analysed. In 190 cases the place of work was in a tertiary care hospital. There was high agreement in the assessment of the severity of the cases mentioned. Over 90% use inhaled ß2 agonists in mild asthma exacerbations, 75.3% used oral corticosteroids in moderate exacerbations, and 79% use ipratropium bromide in severe exacerbations. Almost all (98%) considered Therapeutic Education important in the Emergency Department. Only 8 (2.7%) used peak expiratory flow measurements. Respondents working in a tertiary hospital used severity scales more frequently (90.5% vs 80%, P=.009) and administered parenteral corticosteroids less frequently (53.2% vs 66.4%, P=.017). CONCLUSIONS: Although a high percentage of paediatricians working in the ED follow Clinical Practice Guidelines, some different points of the approach among professionals have been identified.

Guía de práctica clínica sobre la bronquiolitis aguda: descripción de la metodología utilizada / Acute bronchiolitis clinical practice guidelines: Description of the metodological process

Una guía de práctica clínica es un conjunto de recomendaciones desarrolladas de forma sistemática para ayudar a los profesionales y a los pacientes a tomar decisiones sobre la atención sanitaria más apropiada, y a seleccionar las opciones diagnósticas o terapéuticas más adecuadas para abordar un problema de salud o una condición clínica específica. La bronquiolitis aguda es la infección del tracto respiratorio inferior más frecuente en el lactante, pero existen controversias en cuanto al manejo de esta. Es por este motivo que se selecciona la bronquiolitis como entidad para la elaboración de una guía de práctica clínica. Describimos el proceso metodológico utilizado para la elaboración de la guía de práctica clínica sobre la bronquiolitis aguda (AU)

A clinical practice guideline (CPG) is a set of systematically developed recommendations to assist practitioners and patient decisions about appropriate health care, and to select the most appropriate diagnostic or therapeutic options to address a health problem or a specific clinical condition. Acute bronchiolitis is the most common lower respiratory tract infection in infants. In spite of its hight prevalence, there is still controversy regarding its management. Therefore bronchiolitis is selected for the development of a CPG. We describe the methodological process used to develop the CPG on acute bronchiolitis (AU)

Guía de práctica clínica sobre la bronquiolitis aguda: recomendaciones para la práctica clínica / Acute bronchiolitis clinical practice guideline: recommendations for clinical practice

Existen controversias científicas en cuanto al manejo de la bronquiolitis aguda que generan una gran variabilidad en el abordaje de esta entidad. Una Guía de Práctica Clínica (GPC) es un conjunto de recomendaciones desarrolladas de forma sistemática para ayudar a los profesionales y a los pacientes a tomar decisiones basadas en la evidencia. Se ha seleccionado un grupo de grupo de trabajo (GT) formado por expertos que ha elaborado las preguntas clínicas a responder. Se ha realizado una búsqueda sistemática de la evidencia disponible sobre la bronquiolitis aguda y se ha evaluado. Posteriormente se ha resumido la información obtenida en tablas de evidencia y se han redactado unas recomendaciones a partir de éstas. El GT ha redactado un total de 46 preguntas clínicas agrupadas en apartados (diagnóstico, exploraciones complementarias, tratamiento, monitorización, prevención y evolución), que dan lugar a 133 recomendaciones. La GPC sobre la bronquiolitis aguda ofrece la oportunidad de unificar la práctica clínica habitual, basándose en la evidencia científica, lo que podría racionalizar el consumo de recursos sanitarios, mejorando la calidad asistencial y la equidad de acceso y utilización de los servicios (AU)

There are scientific controversies about the management of acute bronchiolitis that generate a great variability in the approach to this entity. A clinical practice guideline (CPG) is a set of systematically developed recommendations to assist practitioners and patients to make decisions based on evidence. A working group (WG) of experts has been selected and they have developed the clinical questions. A systematic search for evidence on the acute bronchiolitis has been done and the evidence has been assessed. The information obtained has been summarized in evidence tables and recommendations have been done from them. The WG has produced a total of 46 clinical questions grouped into sections (diagnosis, complementary tests, treatment, monitoring, prevention and evolution), resulting in 133 recommendations. The CPG on acute bronchiolitis provides an opportunity to unify clinical practice based on scientific evidence, which could rationalize the use of health resources, improving health care quality and equity of access to services (AU)

[Acute bronchiolitis clinical practice guideline: recommendations for clinical practice]. / Guía de práctica clínica sobre la bronquiolitis aguda: recomendaciones para la práctica clínica.

There are scientific controversies about the management of acute bronchiolitis that generate a great variability in the approach to this entity. A clinical practice guideline (CPG) is a set of systematically developed recommendations to assist practitioners and patients to make decisions based on evidence. A working group (WG) of experts has been selected and they have developed the clinical questions. A systematic search for evidence on the acute bronchiolitis has been done and the evidence has been assessed. The information obtained has been summarized in evidence tables and recommendations have been done from them. The WG has produced a total of 46 clinical questions grouped into sections (diagnosis, complementary tests, treatment, monitoring, prevention and evolution), resulting in 133 recommendations. The CPG on acute bronchiolitis provides an opportunity to unify clinical practice based on scientific evidence, which could rationalize the use of health resources, improving health care quality and equity of access to services.

[Acute bronchiolitis clinical practice guidelines: Description of the metodological process]. / Guía de práctica clínica sobre la bronquiolitis aguda: descripción de la metodología utilizada.

A clinical practice guideline (CPG) is a set of systematically developed recommendations to assist practitioners and patient decisions about appropriate health care, and to select the most appropriate diagnostic or therapeutic options to address a health problem or a specific clinical condition. Acute bronchiolitis is the most common lower respiratory tract infection in infants. In spite of its high prevalence, there is still controversy regarding its management. Therefore bronchiolitis is selected for the development of a CPG. We describe the methodological process used to develop the CPG on acute bronchiolitis.

Novel influenza A (H1N1) encephalitis in a 3-month-old infant.

Seasonal influenza virus infection has been associated with a variety of neurologic complications. We report a case of novel influenza A (H1N1) encephalitis in an infant aged 3 months with an upper respiratory infection, who presented seizures. The infection was confirmed in nasopharyngeal aspirate and cerebrospinal fluid. Treatment with oseltamivir was started. He was discharged without any neurologic sequelae.

Corea de Sydenham: un pasado aún presente / No disponible

No disponible

[Impact of pneumococcal vaccination on the management of infants with fever according to vaccination rates]. / Impacto de la vacunación neumocócica en el manejo del lactante con fiebre, en relación al porcentaje de vacunación.

OBJECTIVE: To calculate the impact of the heptavalent pneumococcal conjugate vaccine (PCV-7) in the management of fever without source (FWS) in infants according to vaccination rates. METHODS: The rate of pneumococcal vaccination in infants visiting 14 pediatric emergency departments (PEDs) was calculated. For the statistical analysis, two groups were established; group A: hospitals with vaccination rates of > or = 40%, and group B: hospitals with vaccination rates of < 40 %. A survey was carried out among pediatricians from 14 PEDs on the specific management of two hypothetical clinical cases of FWS (case 1: 7-month-old girl; case 2: 20-month-old girl) depending on their pneumococcal vaccination status (no vaccination, 1 o 2 doses, or 3 doses). RESULTS: In February 2005, data were collected in 1357 patients, aged 3 to 36 months; 568 (41.86 %) had received at least one dose of PCV-7. A total of 235 questionnaires were collected, 104 in group A and 131 in group B. Pneumococcal vaccination would lead (with statistically significant differences) to fewer diagnostic tests (complete blood cell counts and blood culture). This decrease would be more pronounced in group A than in group B (56.7 % vs 26.7 % and 55.8 vs 26.7 % in case 1 and 54.8 % vs 26 % and 57.7 vs 13.7 %, case 2, respectively), p < 0.05. Infants who had received complete pneumococcal vaccination would receive fewer antibiotics and would be more likely to be discharged directly from hospital (p < 0.001). CONCLUSIONS: In the previously vaccinated infant with FWS, there would be a significant reduction in the number of diagnostic tests, need for observation in the PED, rates of hospitalization and antibiotic therapy. The reduction in diagnostic tests would increase in areas with higher rates of pneumococcal vaccination.

Impacto de la vacunación neumocócica en el manejo del lactante con fiebre, en relación al porcentaje de vacunación / Impact of pneumococcal vaccination on the management of infants with fever according to vaccination rates

Objetivo. Estimar el impacto de la vacuna neumocócica conjugada heptavalente (VCN-7) en el manejo del lactante con fiebre sin focalidad (FSF) en relación al porcentaje de vacunación. Método. Se estimó el porcentaje VCN-7 en los lactantes con FSF que consultaron en 14 servicios de urgencias pediátricos (SUP). Para el análisis estadístico, se establecieron 2 grupos. Grupo A: hospitales con porcentaje de vacunación igual o superior a 40 %; y grupo B: hospitales con porcentaje de vacunación inferior a 40 %. Se realizó una encuesta sobre el manejo concreto de 2 casos clínicos hipotéticos de FSF (caso 1: niña de 7 meses; y caso 2: niña de 20 meses) en tres situaciones: sin vacunar; con 1 o 2 dosis de la VCN-7 o con 3 dosis. Resultados. Durante el mes de febrero de 2005 se recogieron en 14 SUP, datos de 1.357 lactantes (3 a 36 meses); 568 (41,86 %) habían recibido alguna dosis de VCN-7. Se reunieron 235 cuestionarios, 104 del grupo A y 131 del grupo B. La VCN-7 daría origen de forma significativa a la realización de menos estudios complementarios (hemograma y hemocultivo). Este descenso sería más acusado en el grupo A frente a grupo B (56,7 % frente a 26,7 % y 55,8 frente a 26,7 % en el caso 1 y 54,8 % frente a 26 % y 57,7 frente a 13,7 %, en el caso 2, respectivamente; p < 0,05). En ambos grupos de estudio, en los lactantes con VCN-7 completa, se reduciría la administración de antibióticos y se incrementaría el alta hospitalaria directa (p < 0,001). Conclusiones. En el lactante con FSF y VCN-7, se produciría una disminución significativa en el número de pruebas diagnósticas, la estancia del paciente en el SUP, la tasa de hospitalización y tratamiento antibiótico. La reducción en el número de pruebas diagnósticas sería más acusado en aquellas áreas con porcentajes superiores de VCN-7

Objective. To calculate the impact of the heptavalent pneumococcal conjugate vaccine (PCV-7) in the management of fever without source (FWS) in infants according to vaccination rates. Methods. The rate of pneumococcal vaccination in infants visiting 14 pediatric emergency departments (PEDs) was calculated. For the statistical analysis, two groups were established; group A: hospitals with vaccination rates of >= 40%, and group B: hospitals with vaccination rates of < 40 %. A survey was carried out among pediatricians from 14 PEDs on the specific management of two hypothetical clinical cases of FWS (case 1: 7-month-old girl; case 2: 20-month-old girl) depending on their pneumococcal vaccination status (no vaccination, 1 o 2 doses, or 3 doses). Results. In February 2005, data were collected in 1357 patients, aged 3 to 36 months; 568 (41.86 %) had received at least one dose of PCV-7. A total of 235 questionnaires were collected, 104 in group A and 131 in group B. Pneumococcal vaccination would lead (with statistically significant differences) to fewer diagnostic tests (complete blood cell counts and blood culture). This decrease would be more pronounced in group A than in group B (56.7 % vs 26.7 % and 55.8 vs 26.7 % in case 1 and 54.8 % vs 26 % and 57.7 vs 13.7 %, case 2, respectively), p < 0.05. Infants who had received complete pneumococcal vaccination would receive fewer antibiotics and would be more likely to be discharged directly from hospital (p < 0.001). Conclusions. In the previously vaccinated infant with FWS, there would be a significant reduction in the number of diagnostic tests, need for observation in the PED, rates of hospitalization and antibiotic therapy. The reduction in diagnostic tests would increase in areas with higher rates of pneumococcal vaccination

[Seat belt syndrome. Acute spinal cord injury due to incorrect use of two-point seat belts]. / Síndrome del cinturón de seguridad: lesión medular aguda por uso incorrecto del cinturón de seguridad de dos puntos.

Traumatic spinal cord injury (SCI) is a rare entity in the pediatric age group. These injuries are mainly caused by road traffic accidents (RTA), especially in children not wearing a seat belt. The use of child safety devices such as seat belt restraints has decreased morbidity and mortality in RTA but their incorrect use can also produce serious injuries that are grouped under the term "seat-belt syndrome". This syndrome associates vertebral and spinal cord injuries, intra-abdominal, cutaneous, and muscle-skeletal lesions. We present three patients with complete spinal cord and intra-abdominal injuries, requiring urgent surgery in two of them. On examination, all three patients had seat belt marks on the lower abdominal region. The three patients can be included in this syndrome and its main cause was the use of a two-point seat belt.

Síndrome del cinturón de seguridad: lesión medular aguda por uso incorrecto del cinturón de seguridad de dos puntos / Seat belt syndrome. Acute spinal cord injury due to incorrect use of two-point seat belts

Las lesiones medulares traumáticas son entidades poco frecuentes en la edad pediátrica. La principal causa son los accidentes de tráfico y se dan generalmente en pacientes no portadores del cinturón de seguridad. El uso de los dispositivos de seguridad, como el cinturón, ha disminuido la morbimortalidad en los accidentes, pero el uso incorrecto puede también producir lesiones graves que se describen dentro del denominado síndrome del cinturón de seguridad (seat-belt syndrome). Este síndrome asocia la presencia de lesiones vertebrales, medulares, de vísceras abdominales sólidas y/o huecas y lesiones musculoesqueléticas y cutáneas. Presentamos 3 casos clínicos de pacientes con lesiones medulares completas y lesiones intraabdominales, requiriendo cirugía urgente en dos de ellos. Destaca la presencia en todos ellos de la marca del cinturón de seguridad a nivel abdominal. Los 3 casos pueden ser incluidos en este síndrome y su principal causa es la utilización del cinturón con banda abdominal de 2 puntos

Traumatic spinal cord injury (SCI) is a rare entity in the pediatric age group. These injuries are mainly caused by road traffic accidents (RTA), especially in children not wearing a seat belt. The use of child safety devices such as seat belt restraints has decreased morbidity and mortality in RTA but their incorrect use can also produce serious injuries that are grouped under the term 'seat-belt syndrome'. This syndrome associates vertebral and spinal cord injuries, intra-abdominal, cutaneous, and muscle-skeletal lesions. We present three patients with complete spinal cord and intra-abdominal injuries, requiring urgent surgery in two of them. On examination, all three patients had seat belt marks on the lower abdominal region. The three patients can be included in this syndrome and its main cause was the use of a two-point seat belt

[Pediatric acute spinal cord injury]. / Lesión medular aguda en edad pediátrica.

INTRODUCTION: Pediatric acute spinal cord injury is rare but is often associated with significant disability and prolonged stay in the intensive care unit (ICU). OBJECTIVES: The main objective of this study was to determine the epidemiology, initial clinical abnormalities, diagnostic studies, treatment and outcome of acute spinal cord injuries in a tertiary level pediatric hospital. A second objective was to analyze whether early tracheostomy allows earlier discharge of these patients. PATIENTS AND METHODS: A retrospective analysis of patients with acute spinal cord injuries admitted to our pediatric ICU since 1992 was performed. RESULTS: We included 16 patients in the study, 12 of whom were boys (75 %). The patients were aged from birth to 19 years on admission to the ICU. The length of stay in the ICU was between 12 hours and 6 years. The cause of the lesion was birth trauma in four patients; one died and the remaining three live at home under mechanical ventilation. Traumatic injuries occurred in seven patients, two of whom died; a further two live in a long-term care facility and the remaining three live at home. Vascular spinal malformation occurred in two patients, and the three remaining injuries occurred during the acute postoperative period following spinal surgery. Of the 16 children, 56.2 % were tracheostomized and 83 % of the survivors live at home. CONCLUSIONS: Early tracheostomy, the availability of invasive ventilation for home use and parent education permit earlier discharge of these patients and allow more of them to live at home.

Lesión medular aguda en edad pediátrica / Pediatric acute spinal cord injury

Introducción La lesión medular aguda es una entidad de escasa frecuencia en edad pediátrica pero adquiere especial relevancia debido a las graves secuelas y a las prolongadas estancias que provoca. Objetivos Conocer la epidemiología, forma de presentación, pruebas diagnósticas, tratamiento y evolución de las lesiones medulares agudas ingresadas en un hospital pediátrico de tercer nivel. Determinar el efecto que puede tener la traqueostomía precoz en el tiempo de estancia en la Unidad. Pacientes y métodos Se trata de un estudio retrospectivo de cohortes que revisa las lesiones medulares agudas ingresadas en nuestro centro desde el año 1992. Resultados Se recogieron 16 pacientes, 12 varones (75 %) con edades al ingreso comprendidas entre las pocas horas de vida y los 19 años. Los días de ingreso en la unidad de cuidados intensivos oscilaron entre 12 h y 6 años. En 4 casos la causa fue una lesión obstétrica; uno de ellos falleció y el resto se encuentran en su domicilio con ventilación mecánica. En 7 casos la causa fue traumática; fallecieron 2 pacientes, otros dos residen en un centro sociosanitario y tres en su domicilio. En 2 casos la causa fue una malformación vascular medular y los tres últimos se presentaron en el postoperatorio inmediato de una cirugía de columna. De los 16 pacientes recogidos el 56,2 % fueron traqueostomizados y el 83 % de los supervivientes residen en su domicilio. Conclusiones La realización precoz de la traqueostomía, la posibilidad de la ventilación mecánica domiciliaria y la instrucción de las familias ha permitido reducir la estancia de estos pacientes en la Unidad y que puedan residir en su casa

Introduction Pediatric acute spinal cord injury is rare but is often associated with significant disability and prolonged stay in the intensive care unit (ICU). Objectives The main objective of this study was to determine the epidemiology, initial clinical abnormalities, diagnostic studies, treatment and outcome of acute spinal cord injuries in a tertiary level pediatric hospital. A second objective was to analyze whether early tracheostomy allows earlier discharge of these patients. Patients and methods A retrospective analysis of patients with acute spinal cord injuries admitted to our pediatric ICU since 1992 was performed. Results We included 16 patients in the study, 12 of whom were boys (75 %). The patients were aged from birth to 19 years on admission to the ICU. The length of stay in the ICU was between 12 hours and 6 years. The cause of the lesion was birth trauma in four patients; one died and the remaining three live at home under mechanical ventilation. Traumatic injuries occurred in seven patients, two of whom died; a further two live in a long-term care facility and the remaining three live at home. Vascular spinal malformation occurred in two patients, and the three remaining injuries occurred during the acute postoperative period following spinal surgery. Of the 16 children, 56.2 % were tracheostomized and 83 % of the survivors live at home. Conclusions Early tracheostomy, the availability of invasive ventilation for home use and parent education permit earlier discharge of these patients and allow more of them to live at home

Variability of immunodeficiency associated with ataxia telangiectasia and clinical evolution in 12 affected patients.

Ataxia telangiectasia (AT) is an infrequent condition, which is difficult to diagnose in children. The objective was to describe the evolution of all affected patients controlled in our hospital and to highlight the keys for an early diagnosis considering the variability of immunological disorders. The present study is a retrospective review of all patients diagnosed and controlled of AT in our hospital. Twelve patients were found, including two couples of siblings. The most frequent reason for consultation was unstable gait. Seven patients suffered repeated infections, being pneumonia the most frequent cause of infection, followed by sinusitis. One of the patients developed Burkitt's lymphoma, and another patient, Hodgkin's lymphoma, which caused the death of the patient at the age of 11. A couple of siblings aged 17 and 22 years developed insulin-resistant diabetes mellitus. The most frequent immunity disorders were the IgG deficiency and the decrease of T lymphocytes. Seven patients were treated with non-specific gamma-globulin. By the end of the follow-up, 8 patients (ages ranged 7 to 12 years) lost gait. Molecular genetic testing was conducted in patients who are still cared for in our hospital. Clinical suspicion of this entity will lead to an early diagnosis, the treatment of complications, and to provide genetic counselling for the families.