Transition of Adolescents With Severe Asthma From Pediatric to Adult Care in Spain: The STAR Consensus.

OBJECTIVES: To assess the degree of consensus among a multidisciplinary expert panel on the transition of adolescents with severe asthma from pediatric to adult care. METHODS: A 61-item survey was developed based on guidelines for other chronic diseases, covering transition planning, preparation, effective transfer, and follow-up. A 2-round Delphi process assessed the degree of consensus among 98 experts (49 pediatricians, 24 allergists, and 25 pulmonologists). Consensus was established with ≥70% agreement. RESULTS: Consensus was reached for 42 items (70%). Panelists were unable to agree on an age range for initiation of transition. The main goal during the transition identified by the experts is for adolescents to gain autonomy in managing severe asthma and prescribed treatments. The panelists agreed on the importance of developing an individualized plan, promoting patient autonomy, and identifying factors associated with the home environment. They agreed that the adult health care team should have expertise in severe asthma, biologics, and management of adolescent patients. Pediatric and adult health care teams should share clinical information, agree on the criteria for maintaining biological therapy, and have an on-site joint visit with the patient before the effective transfer. Adult health care professionals should closely follow the patient after the effective transfer to ensure correct inhaler technique, adherence, and attendance at health care appointments. CONCLUSION: This consensus document provides the first roadmap for Spanish pediatric and adult teams to ensure that key aspects of the transition process in severe asthma are covered. The implementation of these recommendations will improve the quality of care offered to the patient.

Transition of Adolescents With Severe Asthma From Pediatric to Adult Care in Spain: The STAR Consensus

Objective: To assess the degree of consensus among a multidisciplinary expert panel on the transition of adolescents with severe asthma from pediatric to adult care. Methods: A 61-item survey was developed based on guidelines for other chronic diseases, covering transition planning, preparation, effective transfer, and follow-up. A 2-round Delphi process assessed the degree of consensus among 98 experts (49 pediatricians, 24 allergists, and 25 pulmonologists). Consensus was established with ≥70% agreement. Results: Consensus was reached for 42 items (70%). Panelists were unable to agree on an age range for initiation of transition. The main goal during the transition identified by the experts is for adolescents to gain autonomy in managing severe asthma and prescribed treatments. The panelists agreed on the importance of developing an individualized plan, promoting patient autonomy, and identifying factors associated with the home environment. They agreed that the adult health care team should have expertise in severe asthma, biologics, and management of adolescent patients. Pediatric and adult health care teams should share clinical information, agree on the criteria for maintaining biological therapy, and have an on-site joint visit with the patient before the effective transfer. Adult health care professionals should closely follow the patient after the effective transfer to ensure correct inhaler technique, adherence, and attendance at health care appointments. Conclusions: This consensus document provides the first roadmap for Spanish pediatric and adult teams to ensure that key aspects of the transition process in severe asthma are covered. The implementation of these recommendations will improve the quality of care offered to the patient (AU)

Objetivo: Evaluar el grado de consenso con un panel multidisciplinar de expertos sobre la transición del adolescente con asma grave de los servicios de pediatría a atención de adultos. Métodos: Se elaboró un cuestionario de 61 ítems basado en recomendaciones de transición para otras patologías crónicas, abarcando la planificación de la transición, preparación, transferencia efectiva y seguimiento. Se evaluó el nivel de consenso entre 98 expertos (49 pediatras, 24 alergólogos y 25 neumólogos) mediante un proceso Delphi de dos rondas. El consenso se estableció con un acuerdo ≥70%. Resultados: Cuarenta y dos ítems (70%) alcanzaron consenso. Los panelistas no alcanzaron consenso en el rango de edad para iniciar la transición. El principal objetivo a conseguir durante la transición según los expertos fue que el adolescente gane autonomía en el manejodel asma grave y tratamientos prescritos. Asimismo, alcanzaron acuerdo en la importancia de desarrollar un plan individualizado, promover la autonomía del paciente e identificar los factores clave en el entorno familiar. Los especialistas de adultos deben tener experiencia en asma grave y tratamientos biológicos, así como en el manejo de pacientes adolescentes. Los equipos sanitarios de pediatría y de adultos deben compartir la información clínica, consensuar los criterios para mantener la terapia biológica y realizar una visita conjunta con el paciente antes de la transferencia. Los especialistas de adultos deben realizar un seguimiento estrecho del paciente tras la transferencia para asegurar una correcta técnica inhalatoria, el cumplimiento del tratamiento y la asistencia a las citas sanitarias. Conclusiones: Este documento de consenso proporciona la primera hoja de ruta en España para que los equipos especialistas de pediatría y adultos garanticen aspectos clave del proceso de transición en pacientes adolescentes con asma grave. La aplicación de estas (AU)

Experience in neuromuscular diseases in children and adolescents and their comorbidities in a tertiary hospital.

INTRODUCTION: Neuromuscular diseases include a large group of heterogeneous and rare pathologies that affect different components of the motor unit. It is essential to optimize resources to know the prevalence of comorbidities in the most frequent groups to establish an early multidisciplinary approach in a specialized setting. PATIENTS AND METHODS: Retrospective descriptive study of pediatric and adolescent patients with neuromuscular diseases (NMDs). The Inclusion criteria were NMDs patients with motor neuron involvement divided into three groups, depending on the affected component of the motor unit. Group I: involvement of the motor neuron; Group II: peripheral neuropathies; Group III: myopathies. Demographic variables, association with comorbidities, need for respiratory support, and rehabilitative treatment were collected in each group. RESULTS: Ninety-six patients who met the inclusion criteria were studied. In group I, when compared to the other two groups, a higher incidence of scoliosis (68.3%, p = 0.011), deformity of the rib cage (31.3%, p = 0.0001), chronic respiratory insufficiency (62.5%, p = 0.001) and bronchial aspiration (12.5%, p = 0.03) was detected. In this group, 50%of the patients required non-invasive mechanical ventilation (p = 0.0001). The in-hospital requirement for respiratory physiotherapy was higher in group I (75%, p = 0.001). We observed a higher incidence of scoliosis in Group III compared to Group II. CONCLUSIONS: Neuromuscular diseases with motor neuron involvement present more comorbidities and require an early approach after diagnosis to improve prognosis.

Asthma diagnosis in infants and preschool children: a systematic review of clinical guidelines

Background and aim: The definition and diagnosis of asthma are the subject of controversy that is particularly intense in the case of individuals in the first years of life, due to reasons such as the difficulty of performing objective pulmonary function tests or the high frequency with which the symptoms subside in the course of childhood. Since there is no consensus regarding the diagnosis of asthma in preschool children, a systematic review has been carried out. Materials and methods: A systematic search was made of the clinical guidelines published in the last 10 years and containing information referred to the concept or diagnosis of asthma in childhood - including the first years of life (infants and preschool children). A series of key questions were established, and each selected guide was analyzed in search of answers to those questions. The review protocol was registered in the international prospective register of systematic reviews (PROSPERO), with registration number CRD42017074872. Results: Twenty-one clinical guidelines were selected: 10 general guides (children and adults), eight pediatric guides and three guides focusing on preschool children. The immense majority accepted that asthma can be diagnosed from the first years of life, without requiring pulmonary function tests or other complementary techniques. The response to treatment and the exclusion of other alternative diagnoses are key elements for establishing the diagnosis. Only one of the guides denied the possibility of diagnosing asthma in preschool children. Conclusions: There is generalized although not unanimous agreement that asthma can be diagnosed in preschool children

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Asthma diagnosis in infants and preschool children: a systematic review of clinical guidelines.

BACKGROUND AND AIM: The definition and diagnosis of asthma are the subject of controversy that is particularly intense in the case of individuals in the first years of life, due to reasons such as the difficulty of performing objective pulmonary function tests or the high frequency with which the symptoms subside in the course of childhood. Since there is no consensus regarding the diagnosis of asthma in preschool children, a systematic review has been carried out. MATERIALS AND METHODS: A systematic search was made of the clinical guidelines published in the last 10 years and containing information referred to the concept or diagnosis of asthma in childhood - including the first years of life (infants and preschool children). A series of key questions were established, and each selected guide was analyzed in search of answers to those questions. The review protocol was registered in the international prospective register of systematic reviews (PROSPERO), with registration number CRD42017074872. RESULTS: Twenty-one clinical guidelines were selected: 10 general guides (children and adults), eight pediatric guides and three guides focusing on preschool children. The immense majority accepted that asthma can be diagnosed from the first years of life, without requiring pulmonary function tests or other complementary techniques. The response to treatment and the exclusion of other alternative diagnoses are key elements for establishing the diagnosis. Only one of the guides denied the possibility of diagnosing asthma in preschool children. CONCLUSIONS: There is generalized although not unanimous agreement that asthma can be diagnosed in preschool children.

Factors that increase wheezing risk in nursing infants in the first year of life

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PURPOSE: Juvenile systemic lupus erythematosus (JSLE) is a severe and chronic autoimmune disease of unknown origin. Inflammatory cytokines can play a pivotal role in the pathogenesis of JSLE, while their secretion is under genetic control. The current investigation was performed to analyse the associations of particular single nucleotide polymorphisms (SNPs) of interleukin-2 (IL-2) and interferon-gamma (IFN-γ) genes in a case control study. MATERIALS AND METHODS: The allele, genotype and haplotype frequencies of the polymorphic IL-2 (G/T at −330, rs2069762, and G/T at +166, rs2069763) and IFN-γ (A/T at +874, rs2430561) genes were estimated in 59 patients with JSLE by contrast with 140 healthy controls using polymerase chain reaction with sequence-specific primers method. RESULTS: Results of the analysed data revealed a negative allelic association for JSLE in IL-2 −330/T (P=0.02), as well as a positive allelic association for IL-2 −330/G (P=0.02). IL-2 GG genotype (−330) in the patient group was also significantly overrepresented (P<0.001), while IL-2 GT genotype (−330) was notably decreased in the patients with JSLE (P<0.001). Additionally, the frequency of IL-2 (−330, +166) GT haplotype was significantly higher in the patient group (P<0.001). CONCLUSION: IL-2 cytokine gene polymorphisms could affect individual susceptibility to JSLE and can take on the role of possible genetic markers for vulnerability to JSLE

Consenso para el abordaje del asma grave pediátrica en la práctica clínica habitual / Consensus-based approach for severe paediatric asthma in routine clinical practice

INTRODUCCIÓN: La identificación adecuada del paciente pediátrico con asma grave es esencial para su correcto manejo. Sin embargo, los criterios para definir el asma grave y las recomendaciones para su control varían mucho entre las distintas guías. MATERIAL Y MÉTODOS: Se elaboró una encuesta telemática para analizar las opiniones relativas a la definición y control del asma grave pediátrica. Para lograr un consenso se siguió una metodología Delphi modificada. Con los resultados se elaboraron recomendaciones prácticas. RESULTADOS: El cuestionario fue respondido por 11 neumólogos y alergólogos pediátricos expertos en asma grave. Hubo consenso en 50 de los 65 ítems planteados (76,92%). Se consideró que un paciente tiene asma grave si en el último año ha requerido 2 o más ciclos de corticoides orales, si requiere tratamiento diario con corticoides inhalados a dosis medias (con otra medicación controladora) o dosis altas (con o sin otra medicación controladora), si no responde a un tratamiento convencional optimizado, o si la enfermedad pone en riesgo su vida o deteriora gravemente su calidad de vida. La definición de asma grave también podría incluir a los pacientes que consumen recursos sanitarios de manera regular y justificada, o tienen factores psicosociales o ambientales que impiden su control. Para la monitorización, se recomienda usar cuestionarios específicos de población pediátrica (CAN o ACT). Respecto al tratamiento, se debería considerar el uso de omalizumab en un escalón anterior al de los corticoides orales. CONCLUSIONES: El presente trabajo ofrece recomendaciones consensuadas que pueden ser de utilidad en el manejo del asma grave pediátrica

INTRODUCTION: Accurate identification of paediatric patients with severe asthma is essential for an adequate management of the disease. However, criteria for defining severe asthma and recommendations for control vary among different guidelines. MATERIAL AND METHODS: An online survey was conducted to explore expert opinions about the definition and management of severe paediatric asthma. To reach a consensus agreement, a modified Delphi technique was used, and practice guidelines were prepared after the analysis of the results. RESULTS: Eleven paediatric chest disease physicians and allergy specialists with wide expertise in severe asthma responded to the survey. Consensus was reached in 50 out of 65 questions (76.92%). It was considered that a patient has severe asthma if during the previous year they have required 2 or more cycles of oral steroids, required daily treatment with medium doses of inhaled corticosteroids (with other controller medication) or high doses (with or without other controller medication), did not respond to optimised conventional treatment, or if the disease threatened the life of the patient or seriously impairs their quality of life. The definition of severe asthma may also include patients who justifiably use health resources on a regular basis, or have psychosocial or environmental factors impeding control. For monitoring, the use of questionnaires designed specifically for paediatric population, such as CAN or ACT, is recommended. As regards treatment, the use of omalizumab should be considered prior to the use of oral corticosteroids. CONCLUSIONS: This paper provides consensus recommendations that may be useful in the management of severe paediatric asthma

[Consensus-based approach for severe paediatric asthma in routine clinical practice]. / Consenso para el abordaje del asma grave pediátrica en la práctica clínica habitual.

INTRODUCTION: Accurate identification of paediatric patients with severe asthma is essential for an adequate management of the disease. However, criteria for defining severe asthma and recommendations for control vary among different guidelines. MATERIAL AND METHODS: An online survey was conducted to explore expert opinions about the definition and management of severe paediatric asthma. To reach a consensus agreement, a modified Delphi technique was used, and practice guidelines were prepared after the analysis of the results. RESULTS: Eleven paediatric chest disease physicians and allergy specialists with wide expertise in severe asthma responded to the survey. Consensus was reached in 50 out of 65 questions (76.92%). It was considered that a patient has severe asthma if during the previous year they have required 2 or more cycles of oral steroids, required daily treatment with medium doses of inhaled corticosteroids (with other controller medication) or high doses (with or without other controller medication), did not respond to optimised conventional treatment, or if the disease threatened the life of the patient or seriously impairs their quality of life. The definition of severe asthma may also include patients who justifiably use health resources on a regular basis, or have psychosocial or environmental factors impeding control. For monitoring, the use of questionnaires designed specifically for paediatric population, such as CAN or ACT, is recommended. As regards treatment, the use of omalizumab should be considered prior to the use of oral corticosteroids. CONCLUSIONS: This paper provides consensus recommendations that may be useful in the management of severe paediatric asthma.

Analysis of the local and systemic inflammatory response in hospitalized infants with respiratory syncitial virus bronchiolitis

BACKGROUND: Respiratory syncytial virus acute bronchiolitis (RSV-AB) is a major cause of hospital admission among our infants. The immune and inflammatory mechanisms involved in the RSV-AB and factors influencing severity have not been clearly established, although an imbalanced Th1 and Th2 response seems to be crucial. OBJECTIVES: To assess the local and systemic inflammatory response in RSV-AB. To find a possible marker of clinical severity and/or oxygen requirements. PATIENTS AND METHODS: Levels of nine cytokines were measured in nasopharyngeal aspirate (NPA) and peripheral blood (PB) of 45 infants with RSV-AB and 27 peer controls, including IFNγ, TNFα, VEGF, interleukins 4, 6 and 10, and chemokines (IL-8 and macrophage inflammatory proteins 1-α and 1-β). RESULTS: The levels of the analyzed cytokines and chemokines were significantly higher in the NPA of RSV-AB group, with a decrease in IL-4/IFNγ ratio. IL-6 and MIP-1β levels in NPA were directly correlated to oxygen therapy. PB showed an increase in IL-8 and a decrease in MIP-1α and MIP-1β in the RSV-AB group (only MIP-1β associated to the need for oxygen therapy). No correlation was found between cytokines and chemokines levels in NPA and PB. CONCLUSIONS: This study shows that RSV triggers an inflammatory response fundamentally at the respiratory level, with scant systemic repercussion. This local response is characterized by an increase in Th1 and Th2 cytokines, although with a relative predominance of Th1. The determination upon patient admission of IL-6 and MIP-1β levels in NPA, and of MIP-1β in PB could help predict severe forms and the need for oxygenotherapy

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Analysis of the local and systemic inflammatory response in hospitalized infants with respiratory syncitial virus bronchiolitis.

BACKGROUND: Respiratory syncytial virus acute bronchiolitis (RSV-AB) is a major cause of hospital admission among our infants. The immune and inflammatory mechanisms involved in the RSV-AB and factors influencing severity have not been clearly established, although an imbalanced Th1 and Th2 response seems to be crucial. OBJECTIVES: To assess the local and systemic inflammatory response in RSV-AB. To find a possible marker of clinical severity and/or oxygen requirements. PATIENTS AND METHODS: Levels of nine cytokines were measured in nasopharyngeal aspirate (NPA) and peripheral blood (PB) of 45 infants with RSV-AB and 27 peer controls, including IFNγ, TNFα, VEGF, interleukins 4, 6 and 10, and chemokines (IL-8 and macrophage inflammatory proteins 1-α and 1-ß). RESULTS: The levels of the analyzed cytokines and chemokines were significantly higher in the NPA of RSV-AB group, with a decrease in IL-4/IFNγ ratio. IL-6 and MIP-1ß levels in NPA were directly correlated to oxygen therapy. PB showed an increase in IL-8 and a decrease in MIP-1α and MIP-1ß in the RSV-AB group (only MIP-1ß associated to the need for oxygen therapy). No correlation was found between cytokines and chemokines levels in NPA and PB. CONCLUSIONS: This study shows that RSV triggers an inflammatory response fundamentally at the respiratory level, with scant systemic repercussion. This local response is characterized by an increase in Th1 and Th2 cytokines, although with a relative predominance of Th1. The determination upon patient admission of IL-6 and MIP-1ß levels in NPA, and of MIP-1ß in PB could help predict severe forms and the need for oxygenotherapy.

Omalizumab: Just what we needed

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Síndrome de apnea-hipopnea del sueño en la malformación de Chiari tipo I / Sleep apnea-hypopnea syndrome in type I Chiari malformation

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Asociación entre la exposición a paracetamol y el asma: estado de la cuestión y recomendaciones prácticas / Association between paracetamol exposure and asthma: update and practice guidelines

La prevalencia del asma ha aumentado en las últimas décadas, especialmente en los países desarrollados, por motivos no bien esclarecidos. En los últimos años se ha observado una asociación entre el uso de paracetamol en distintas etapas de la vida, incluyendo la gestación y la infancia, y la prevalencia de asma. El carácter observacional de los estudios publicados no permite establecer una relación causal. Sería necesario realizar ensayos clínicos para comprobar la naturaleza de la asociación, que podría deberse a la presencia de diversos factores de confusión. Las sociedades pediátricas españolas firmantes de este artículo consideran que los datos disponibles hasta la fecha no son suficientes para desaconsejar el uso de paracetamol durante la gestación ni en niños asmáticos o con riesgo de asma (AU)

Asthma prevalence has increased over the last few decades, especially in developed countries, and possibly due to different reasons. An association between paracetamol use or exposure at different periods of life, including gestation and childhood, and asthma prevalence has been observed in the last few years. Causality can not be established from observational reports, due to the arguable presence of many confounding factors and biases. Randomised trials are needed to elucidate the nature of this association. The Spanish Paediatric societies subscribing to this paper consider that current evidence is insufficient to discourage the use of paracetamol during gestation or in children with or at risk of asthma (AU)

[Association between paracetamol exposure and asthma: update and practice guidelines]. / Asociación entre la exposición a paracetamol y el asma: estado de la cuestión y recomendaciones prácticas.

Asthma prevalence has increased over the last few decades, especially in developed countries, and possibly due to different reasons. An association between paracetamol use or exposure at different periods of life, including gestation and childhood, and asthma prevalence has been observed in the last few years. Causality can not be established from observational reports, due to the arguable presence of many confounding factors and biases. Randomised trials are needed to elucidate the nature of this association. The Spanish Paediatric societies subscribing to this paper consider that current evidence is insufficient to discourage the use of paracetamol during gestation or in children with or at risk of asthma.

Síndrome de microdeleción 22q11: manifestaciones cardiorrespiratorias y utilidad de la fibrobroncoscopia / 22Q11 microdeletion syndrome: cardiorespiratory symptoms and fibrobronchoscopy

La microdeleción 22q11 es una deleción genética frecuente con variabilidad fenotípica amplia. Engloba una serie de síndromes, entre los que destaca el síndrome de DiGeorge. Las manifestaciones clínicas más frecuentemente descritas son malformaciones cardiacas, una facies característica, malformaciones palatinas, hipoparatiroidismo, inmunodeficiencia secundaria a hipoplasia tímica, retraso psicomotor y alteraciones psiquiátricas. Dentro de los signos producidos por la enfermedad, es frecuente la insuficiencia respiratoria de causa multifactorial. Las malformaciones de la vía aérea son frecuentes, aunque en la mayoría de los pacientes son leves, por lo que no suelen producir sintomatología. Sin embargo, en algunos casos pueden condicionar clínica respiratoria grave. Los casos clínicos presentados ilustran la importancia de la valoración precoz de la vía aérea mediante fibrobroncoscopia en pacientes con microdeleción 22q11 que presentan dificultad respiratoria recurrente(AU)

The 22q11 deletion syndrome is a frequent contiguous-gene deletion syndrome. This disorder has a broad spectrum of phenotypic manifestations. It includes various syndromes such as DiGeorge syndrome. The most frequent clinical manifestations are congenital cardiac defects, characteristic facies, palate malformations, hypoparathyroidism, immunodeficiency due to thymic hypoplasia, growth retardation, and behavioural and psychiatric problems. Among the symptoms observed, many patients suffer from respiratory insufficiency or failure. The origin is often multifactorial. Structural airway abnormalities are frequently found in this syndrome. In many of these patients the malformation is mild or non-existent, and remains asymptomatic. However, in some cases it can cause a severe respiratory insufficiency, being diagnosed when other disorders are ruled out. These cases illustrate the importance of early visualisation of the airway by fibrobronchoscopy in the management of the patient with 22q11 deletion syndrome who has recurrent respiratory difficulties(AU)

[22Q11 microdeletion syndrome: cardiorespiratory symptoms and fibrobronchoscopy]. / Síndrome de microdeleción 22q11: manifestaciones cardiorrespiratorias y utilidad de la fibrobroncoscopia.

The 22q11 deletion syndrome is a frequent contiguous-gene deletion syndrome. This disorder has a broad spectrum of phenotypic manifestations. It includes various syndromes such as DiGeorge syndrome. The most frequent clinical manifestations are congenital cardiac defects, characteristic facies, palate malformations, hypoparathyroidism, immunodeficiency due to thymic hypoplasia, growth retardation, and behavioural and psychiatric problems. Among the symptoms observed, many patients suffer from respiratory insufficiency or failure. The origin is often multifactorial. Structural airway abnormalities are frequently found in this syndrome. In many of these patients the malformation is mild or non-existent, and remains asymptomatic. However, in some cases it can cause a severe respiratory insufficiency, being diagnosed when other disorders are ruled out. These cases illustrate the importance of early visualisation of the airway by fibrobronchoscopy in the management of the patient with 22q11 deletion syndrome who has recurrent respiratory difficulties.