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.

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.

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.

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.

Prevalence and factors linked to allergic rhinitis in 10 and 11-year-old children in Almería. Isaac Phase II, Spain.

BACKGROUND: Allergic rhinitis affects 10-30% of children in developed countries and has increased in frequency over the last few decades, probably due to changes in the environment and life style. AIM: To assess the prevalence, severity, and factors linked to rhinitis in 10 and 11-year-old children from Almeria (Spain). METHODS: As part of ISAAC II, a cross-sectional survey was conducted among a representative sample of 1143 schoolchildren in spring and autumn of 2001, using homologated questionnaires and skin-prick testing. RESULTS: The overall prevalence of rhinitis and rhinoconjunctivitis were 38.9% and 24.8%, respectively, 17.9% had medically diagnosed rhinitis. During the previous year symptoms disturbed daily activities and school attendance in some measure in 40% and 26% of children with rhinitis, respectively. The risk factors found in the multiple logistic regression analysis were atopy (OR 2.57; 95% CI 1.92-3.42); cat contact at home during first year of life (OR 2.4 95% CI 1.13-5.12); prior medical diagnosis of asthma (OR 2.2; 95% CI 1.22-4.02); nocturnal cough in absence of colds (OR 1.9; 95% CI 1.25-2.97); diagnosis of rhinitis in one of the parents (OR 1.8; 95% CI 1.31-2.59); wheezing at any time (OR 1.6; 95% CI 1.18-2.28); and nursery school attendance (OR 1.5; 95% CI 1.21-2.5). CONCLUSIONS: The prevalence of rhinitis found is superior to that of other centres participating in the ISAAC Phases I and II, and coexists with asthma and eczema in many children. The independent risk factors associated to rhinitis are in accordance with previous reports.

Prevalence and factors linked to atopic eczema in 10- and 11-year-old schoolchildren. Isaac 2 in Almeria, Spain.

BACKGROUND: Atopic eczema affects 5-10% of the Spanish paediatric population, and has increased in frequency over the last few decades, probably due to changes in the environment and lifestyle. Phase II of the ISAAC (International Study of Asthma and Allergies in Childhood) uses a standardised methodology to establish the prevalence of allergic disorders and factors linked to them in each centre. OBJECTIVES: To assess the prevalence and severity of atopic eczema, and to establish factors linked to atopic eczema in 10-11 year-old school children in the city of Almeria (South-East coast of Spain). MATERIAL AND METHODS: An ecological study was carried out as part of ISAAC II, using homologated questionnaires and allergic tests in 1143 schoolchildren. Statistic association was assessed by means of chi(2) test, and then logistic regression analysis was performed with the most significant variables from the univariant analysis. RESULTS: The prevalence of atopic eczema was 11.4%. The risk factors found in the multiple logistic regression analysis were: personal antecedents of severe asthma (OR 19 CI 95% 1.35-266) and severe rhinitis (OR 7.7 CI 95% 1.79-33), fungi in bedroom during the first year of life (OR 4.2 CI 95% 1.17-15.1) and atopic eczema in one parent (OR 5.2 CI 95% 2.69-10.1). CONCLUSIONS: The prevalence of atopic eczema is similar to that found in other studies within ISAAC Phase I. The most important risk factors for atopic eczema are family and personal history of other atopic diseases and the presence of fungi in the home.

[Safety of MMR immunization in egg-allergic children]. / Seguridad de la vacunación triple vírica en pacientes con alergia al huevo.

In Spain, for many years allergy to eggs was considered to contraindicate vaccines cultured in fibroblasts from chick embryos such as the measles, mumps, rubella (MMR) and influenza vaccines. Consequently, an alternative vaccine (Triviraten Berna) incubated in diploid human cells has been systematically administered to children who are allergic to eggs, without questioning tolerance to the standard MMR vaccine. After Biotech Bern Laboratory discontinued the production of Triviraten, this alternative was no longer available for children with egg allergy, who should receive a first dose of the MMR vaccine at the age of 15 months and a second one (booster dose) at the age of 3 years. In this context, from November 2004 to June 2005, a single dose of the MMR vaccine was administered to 40 children with allergy to eggs and none showed an adverse reaction. We conclude that this vaccine can be safely administered to this group of patients.

[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.

[Cardiac tumors. Experience with two cases]. / Tumores intrapericárdicos.

Cardiac tumors are rare neoplasms with an incidence of 0.027 cases per 100. Up to 90 % of these tumors are benign but, because of their location, they can be lethal, despite their histology. Most appear in infants aged less than 1 year. Teratomas, which are relatively frequent in the pediatric age group, are usually located in the ovaries, sacrococcygeal area, thorax, and retroperitoneal area. Intrapericardial location is less frequent. In this localization, the clinical manifestation depends on compression of adjacent structures and determines the therapeutic attitude in each case. Imaging studies should be performed and the definitive diagnosis is obtained after surgical resection, which is the treatment of choice. The differential diagnosis should include other tumors of the anterior mediastinum, thymus hypertrophy, pericardial effusion and primary cardiac disorders such as pericardial mesothelioma, Ebstein malformation, and intrapericardial bronchogenic cysts. We present two patients who were diagnosed in the first year of life in our hospital. Both children developed pericardial tumours. In one patient, the mass decreased and disappeared while in the other, who received a later diagnosis, surgical treatment was required.

[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.

[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.

Prevalence and factors linked to atopy in 10-and 11-year-old children in Almería, Spain.

BACKGROUND: During the last decades there has been an increase in both allergic diseases and allergic sensitisation, probably due to changes in the environment and living habits. ISAAC Phase II was designed to establish the prevalence and associated factors to asthma and allergic disorders in childhood. AIM: To assess the prevalence and factors linked to atopy in 10-11 year-old children from Almería (Spain). METHODS: As a part of ISAAC II, a survey was conducted among a sample of 1143 schoolchildren using standardised questionnaires and skin-prick testing. RESULTS: The overall prevalence of atopy was 42.5%. Most common sensitisations were to Dermatophagoides pteronyssinus (36.2%), D. farinae (32.3%), cat (10.8%), Alternaria (7%), grass (6%), and tree pollen (1.7%). 34.9% of these sensitisations could be regarded as subclinical sensitisations. The fractions of asthma, rhinitis and eczema attributable to atopy were 49.2%, 40.4% y 18.6%, respectively. After multivariate analysis, the risk of atopy was significantly lower among females (OR 0.62, CI 95% 0.45-0.86); children with older siblings (OR 0.67; CI 95% 0.49-0.92); intestinal parasites (OR 0.68; CI 95% 0.48-0.97); contact with farm animals in the past (OR 0.48 CI 95% 0.23-0.99); or other animals at present (OR 0.53 CI 95% 0.30-0.95). To have an allergic father (OR 2.96 CI 95% 1.77-4.94) was the only significant risk factor. CONCLUSIONS: We found several independent factors which significantly protect against atopic sensitisation. These protective factors were not the same for asthma, rhinitis or eczema, suggesting that other factors could interact to influence atopy and act against such protective factors.

Prevalence and associated factors of allergic rhinitis and atopic dermatitis in children.

Allergic disorders are the chronic diseases of greatest pediatric morbidity, affecting over 25 % of the pediatric population. Indeed, this situation has been referred to as an "allergic epidemic". In comparison with asthma, atopic dermatitis and allergic rhinitis have been less extensively investigated, although this does not mean that they should be regarded as minor disorders but rather as alterations that affect the quality of life of the patients and their families, which generate considerable direct and indirect costs. Despite an important research effort, the reason for this allergic epidemic is not well known. These are multifactor disorders without a single causal agent, in which the most important component is the genetic predisposition of the patient (atopy), modulated by environmental factors, exposure to allergens, infections and irritants, among others. A confounding element is the fact that the concept of allergic diseases encompasses phenotypes of rhinitis, atopic dermatitis or asthma in which no IgE-mediated atopic mechanism is demonstrated, and which can manifest in a way similar to true allergic phenotypes. Differentiation between the two is difficult to establish on the basis of self-administered questionnaires alone, in the absence of a precise etiological diagnosis. The present article reviews the numerous factors suggested to be responsible for the increase in allergic diseases recorded in the last few decades, and for the differences in prevalence observed among centres. For most of these factors the results published in the literature are contradictory, in some cases due to a lack of control of the associated interacting or confounding factors. Consensus exists for only some of these causal factors, such as the established parallelism between the increase in allergic diseases and the reduction in infectious processes on one hand, and the increase in particles generated by diesel fuel combustion on the other. In addition, the implicated factors could act differently (and in some cases even antagonically) upon atopy and on the different disease phenotypes, thereby complicating the study of these interactions even further.

[Cross reactivity between fish and shellfish]. / Reactividad cruzada entre pescados y mariscos.

In Spain, fish allergy represents 18 % of all cases of food allergy in children while reactions caused by crustacea and mollusks account for 3.8 % and 1.6 % respectively. Cross-reactivity is defined as the recognition of distinct antigens by the same IgE antibody, demonstrable by in vivo and in vitro tests, which clinically manifests as reactions caused by antigens homologous to different species. Subclinical sensitization can also occur, giving rise to patients sensitized to particular fish or shellfish but who do not present symptoms on consumption.Cod and shrimp have been the models used to study allergy to fish and crustacea respectively. The major allergens responsible for cross-reactivity among distinct species of fish and amphibians are proteins that control calcium flow in the muscular sarcoplasm of these animals, called parvalbumins, with a molecular weight of approximately 12 kD and an isoelectric point of 4.75, resistant to the action of heat and enzymatic digestion. Recently, recombinant carp parvalbumin has been reproduced, confirming that this allergen contains 70 % of the IgE epitopes present in natural extract of cod, tuna and salmon, which makes it a valid tool in the diagnosis of patients with fish allergy. Moreover, this recombinant allergen could constitute the basis for the development of immunotherapy against food allergy. In the case of shellfish, a non-taxonomic group that includes crustacea and mollusks, the major allergen is tropomyosin, an essential protein in muscle contraction both in invertebrates and vertebrates. In invertebrates, tropomyosins, which have a molecular weight of between 38 and 41 kD, show great homology in their amino acid sequence and are the panallergens responsible for cross-reactions between crustacea, insects, mites, nematodes, and different classes of mollusks. It is estimated that 50 % of individuals allergic to some type of fish are at risk for reacting to a second species, while those allergic to some type of crustacea present a risk of 75 % due to the greater similarity among tropomyosins than among parvalbumins. In addition, up to 40 % of patients sensitized to one or more fish do not present symptoms on consuming other species, the best tolerated of which belong to the Scombroidea family (which includes tuna).

[Pleurodesis with tetracyclines after fontan operation]. / Pleurodesis con tetraciclinas tras intervención de Fontan.

Chemical pleurodesis consists of the fusion of both pleural layers after contact with certain substances, and is used in the treatment of prolonged or recurrent pleural effusions refractory to other therapeutic approaches. This procedure has been extensively used in adults with malignant pleural effusion but has rarely been used in children. The Fontan technique is used in the treatment of tricuspid atresia and other heart diseases that act as a single ventricle. This technique frequently presents complications due to fluid retention (edema, ascites, pleural and pericardial effusions and protein-losing enteropathy). With persistent or recurrent pleural effusion, chemical pleurodesis through the drainage tube might constitute a safe and reliable alternative treatment. We report the case of a female patient with tricuspid atresia, who presented recurrent pleural effusion after undergoing the Fontan procedure. The disorder was resolved by chemical pleurodesis with tetracycline.