'Real-life' experience in asthmatic children treated with omalizumab up to six-years follow-up

Introduction and objectives: Omalizumab is present in international guidelines for the control of severe asthma, but data on the long-term effects in children are limited. Our objective was to perform a 'eal-life' long-term trial of omalizumab in children with allergic asthma. Materials and methods: An observational single center 'real-life' study was performed. Data for treatment, lung function, side effect, asthma exacerbations and hospitalizations were recorded at six months and annually. Results: Forty-eight patients <18 years of age were enrolled. Median treatment period was 2.9 (0.5-6). Fluticasone dose for the maintenance treatment decreases significantly at six months (452mcg/day to 329.89 mcg/day, respectively). This difference was maintained throughout the follow-up. Nobody used oral corticosteroid after six months. The rate of hospital admissions and visits to the emergency department for asthma exacerbations decreased significantly in the third years and fourth years follow-up, respectively. There was an improvement in lung function. Mean values of FEV1 and FEF25-75% before treatment were 79.88 and 62.94, respectively; after six months of treatment a statistically significant change was seen with a mean FEV1 of 92.29 and FEF25-75% of 76.31 (p = 0.0001). Lung function values were above normal throughout the six years of treatment. No side effects were reported. Conclusions: Overall in 'real life' omalizumab in children reduces asthma exacerbations and hospitalizations, improves lung function, and decreases the maintenance therapy. It is shown to be safe for up to six years of treatment in children

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Asymptomatic LTP sensitisation is common in plant-food allergic children from the Northeast of Spain

BACKGROUND: The sensitisation profile at molecular level in plant-food allergy is complex. Several allergens may be involved, with different potential for severe reactions. lipid transfer proteins (LTP) are considered the most relevant plant-food allergens in adults in Mediterranean countries, but less is known in children. AIM: To describe the clinical pattern and sensitisation profile of children with plant-food allergy and LTP sensitisation from Northeast Spain. METHODS: Children with history of immediate reaction to plant-food(s), positive skin-prick-test to the culprit plant-food(s) and specific-IgE to plant-food LTPs were analysed. RESULTS: 130 children were included. 69.2% (90/130) had reacted to ≥2 taxonomically unrelated plant-foods. Peach, walnut, hazelnut and peanut were most frequently involved. Reactions severity ranged from anaphylaxis (45.4%, 59/130) to oral symptoms only. Sensitisation to a particular plant-food LTP not always caused clinical symptoms with that plant-food; 69% (40/58) and 63% (17/27) of peach- and walnut-tolerant subjects had positive rPru p 3 and nJug r 3 specific IgE, respectively. 65.4% (85/130) of children were also sensitised to storage proteins, which was associated to anaphylaxis and nut allergy. However, 60% of patients without nuts/seeds allergy were sensitised to storage proteins. Specific-IgE levels to LTPs and/or storage proteins were not useful to predict allergy (vs. tolerance) to peach, walnut, peanut or hazelnut. CONCLUSIONS: Sensitisation to LTP and/or storage proteins without clear clinical significance is relatively common. Prospective longitudinal studies are required to evaluate the relevance of these silent sensitisations over time. Caution is required when interpreting the results of molecular-based diagnostic tools in clinical practice

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A 10% liquid immunoglobulin preparation for intravenous use (Privigen (R)) in paediatric patients with primary immunodeficiencies and hypersensitivity to IVIG

BACKGROUND: The objective of this study was to evaluate safety and efficacy of Privigen®, a 10% intravenous immunoglobulin (IVIG), in a particular group of paediatric patients (highly sensitive to previous IVIG infusion) affected with Primary Immunodeficiencies (PID). MATERIAL AND METHODS: Patients (n = 8) from 3 to 17 years old diagnosed of PID who often suffered from adverse events related to the infusion to previous IVIG were switched to Privigen® in an open protocol. Data were prospectively collected regarding Privigen® administration: infusion, safety and efficacy. In parallel, data on safety and tolerance were retrospectively collected from medical charts regarding the previous 10% IVIG product used. RESULTS: 50% of the patients required premedication with previous IVIG. At the end of the study none required premedication with Privigen®. The infusion rate was lower than that recommended by the manufacturer. All patients had suffered through adverse events during previous IVIG infusion being severe in three patients and recurrent in the rest. With Privigen® only three patients suffered from an adverse event (all cases were milder than previous related). Trough levels of IgG remained stable. None suffer from any episode of bacterial infection. CONCLUSION: The present work shows that Privigen® was safe in a group of hypersensitive paediatric patients who did not tolerate the administration of a previous 10% liquid IVIG by using a particular infusion protocol slower than recommended. The number of adverse effects was smaller than published, and all cases were mild. No premedication was needed. Privigen® was also effective in this small group

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Fenotipos clínicos asociados a la deficiencia selectiva de IgA: revisión de 330 casos y propuesta de un protocolo de seguimiento / Clinical phenotypes associated with selective IgA deficiency: A review of 330 cases and a proposed follow-up protocol

La deficiencia selectiva de IgA (DSIgA) es la inmunodeficiencia primaria más frecuente. Un pequeño porcentaje presenta patología, pero a mayor edad puede asociar deficiencia de alguna subclase de IgG y mayor susceptibilidad a infecciones, enfermedades alérgicas, enfermedades autoinmunes y neoplasias. Objetivos: Describir la asociación de DSIgA con: infecciones, enfermedades alérgicas, autoinmunes y neoplasias en una población pediátrica, otras alteraciones de la inmunidad y proponer un protocolo de seguimiento. Material y métodos: Estudio retrospectivo de pacientes pediátricos (<18 años) atendidos en consultas de Inmunología y de los registros de laboratorio del Hospital Sant Joan de Déu de Barcelona con cifras de IgA<50mg/L. Se revisaron la frecuencia e intensidad de las enfermedades asociadas y los estudios inmunológicos realizados desde 1992 a 2007. Resultados: Se identifican 330 pacientes con DSIgA: 39 (11,8%) presentaron otitis de repetición, 2 con sordera como secuela; 58 (17,5%) infecciones respiratorias de vías altas repetidas y 20 (6%) tenían neumonías recurrentes, 6 de los cuales presentaron bronquiectasias y 2 requirieron lobectomía. Las enfermedades atópicas se presentaban en 62 pacientes (18,78%). Respecto a enfermedades digestivas, 21 (6,5%) presentaban diarreas crónicas, 22 (6,6%) eran celiacos, y otros 3 tenían hepatitis crónica no filiada. El 11,5% (38) de los individuos presentaron enfermedades autoinmunes (artritis crónica juvenil, diabetes mellitus, vitíligo, citopenias y enfermedad de Crohn). Se encontraron tumores en 5 pacientes (1,5%).Respecto a otras alteraciones de la inmunidad, 5 asociaron una deficiencia de subclases de IgG, y en 6 pacientes se objetivó un defecto de síntesis de anticuerpos. Conclusiones: En nuestra serie, el 56,6% de pacientes con DSIgA presenta otras comorbilidades, por orden de frecuencia, infecciones de repetición (respiratorias y óticas), enfermedades alérgicas, autoinmunes y tumores. Algunos pacientes sometidos a estudio inmunológico más amplio podrían desarrollar una forma de defecto humoral más grave como una deficiencia de subclases de IgG(AU)

Selective IgA deficiency is the most common Primary Immune Deficiency. Only a small proportion of these patients present during childhood, but this proportion increases over the years, and may be associated with an IgG subclass deficiency with increased susceptibility to respiratory and digestive tract infections. During childhood, IgA deficient patients may also refer to symptoms related to allergic and autoimmune diseases or tumours. Aims: To describe the relationship of selective IgA deficiency with infections, allergic diseases, autoimmune disorders and tumours. To investigate the presence of other immune disorders associated with selective IgA deficiency. To suggest a follow-up protocol for these patients. Methods: Retrospective study of paediatric patients (<18 years) being followed-up in the Clinical Immunology Department between 1992 and 2007, as well as laboratory records with IgA values below 50mg/L. Clinical records were reviewed (frequency and intensity of diseases associated with selective IgA deficiency) along with immunology tests performed. Results: A total of 330 paediatric patients were identified with a selective IgA deficiency: 39 (11.8%) suffered from recurrent ear infections (2 developed secondary deafness), 58 (17.5%) from recurrent upper respiratory tract infections, and 20 patients (6%) from recurrent pneumonia, 6 of whom developed secondary bronchiectasis and 2 underwent a lobectomy. A relationship with atopic disease was found in 62 (18.78%) of patients. Regarding digestive disorders, chronic diarrhoea was found in 21 (6.5%), coeliac disease in 22 (6.6%), and persistently high plasma transaminases in 3. Autoimmune manifestations were found in 38 (11.5%), juvenile chronic arthritis, type 1 diabetes, vitiligo, cytopenia, and Crohn's disease, amongst others). Tumours were identified in 5 (1.5%).An IgG sub-class deficiency was found in 5 patients (4%), and 6 patients had a confirmed deficiency in antibody production. Conclusions: In our cohort, 56.6% of patients with IgA deficiency showed other comorbidities which were, in decreasing frequency: recurrent infections (respiratory and ear infections), allergic diseases, autoimmunity and tumours. Some patients will develop a more severe humoral defect (IgG subclass deficiency with or without antibody deficiency) (AU)

Directorio de pruebas diagnósticas de las inmunodeficiencias primarias / Directory of diagnostic tests in primary immunodeficiencies

Se comentan las características clínico-inmunológicas sugestivas de las diversas formas de Inmunodeficiencias primarias (IDP). Se han recogido datos sobre los centros hospitalarios en los que se realizan estudios inmunológicos, moleculares y genéticos que permiten el diagnóstico de la mayoría de las IDP en nuestro país

The clinical and immunological characteristics that suggest diverse forms of primary immunodeficiency are discussed. Data on the hospitals that perform immunological, molecular and genetic tests for the diagnosis of most of the primary immunodeficiencies in Spain are presented

Hipereosinofilia y angioedema recurrente / Recurrent angioedema and hypereosinophilia

Introducción: Entre las diferentes causas que condicionan eosinofília, se encuentra un síndrome descrito por primera vez por Gleich en 1984 caracterizado por angioedema de cara, cuello, extremidades y tronco, aumento de peso, hipereosinofilia (60-70 %), fiebre, incremento de los niveles séricos de IgM, sin afección a órganos vitales. Observación clínica: Mujer de 17 años de edad, no alérgica, remitida por presentar angioedema tipo recurrente, inicialmente en manos y pies, actualmente facial, inicia hace 3 años, evolución progresiva con recidivas más frecuentes y graves, en el último año mensuales. Malestar general, sin fiebre. Los exámenes complementarios muestran eosinófilos 40,8 %, total de 3.300/mm 3, niveles séricos de IgM 343 mg/dl (rango normal: 53-300 mg/dl). Se descartan las posibles causas de hipereosinofília además de infiltrado de eosinófilos a órganos vitales. La paciente fue tratada con corticoides orales presentando remisión clínica y disminución de los eosinófilos (1,7 %, totales 200/ mm 3). Conclusiones: La paciente presenta las características clínicas para ser diagnosticada como un síndrome de Gleich, descartando otras entidades asociadas a hipereosinofília

Introduction: Among the various causes of eosinophilia are the syndrome first described by Gleich in 1984. This syndrome is characterized by angioedema of the face, neck, extremities and trunk, weight gain, hypereosinophilia (60-70 %), fever, and increased serum IgM levels without involvement of the vital organs. Case report: A 17-year-old non-allergic woman was referred to our hospital for further investigation of recurrent angioedema, initially of the hands and feet and subsequently of the face, with onset 3 years previously. The attacks had become more frequent and severe and had occurred monthly in the previous year. The patient also showed general malaise, without fever. Complementary investigations revealed eosinophils 40.8 %, total count 3,300/mm 3, and serum IgM levels 343 mg/dl (normal range: 53-300 mg/dl). Possible causes of hypereosinophilia and eosinophilic infiltration of vital organs were ruled out. The patient was treated with oral corticosteroids which produced clinical remission and reduction of eosinophil count (1.7 %, total 200/mm 3). Discussion: Gleich syndrome is uncommon and has well-defined clinical features and a benign course. We describe a patient who presented the clinical characteristics of this syndrome with good response to steroids and without involvement of vital organs. Conclusions: Our patient presented clinical features compatible with a diagnosis of Gleich syndrome. Other entities associated with hypereosinophilia were ruled out

Ostomastoiditis candidiásica y síndrome de hiper Ig E / Otomastoiditis candidiasica and hyper IgE syndrome

Paciente: Niña de cinco años que consulta por otorrea crónica, adenopatías cervicales y celulitis de rodilla. Además de esas lesiones, en la exploración física se observó la existencia de eccema en cuero cabelludo, cuello, región perineal y umbilical, y la persistencia de la dentición temporal y la definitiva (doble arcada dental).Las pruebas complementarias demostraron niveles de IgE de 23.969 UI/l, IgD 440U/L, IgG 23.000 mg/L, IgA 4.220 mg/L e intradermorreacción a candidina negativa, siendo normal el resto del estudio inmunológico. La tomografía axial computada (TAC) reveló la existencia de otomastoiditis bilateral. En los cultivos de secreción ótica se aisló Cándida albicans. El diagnóstico definitivo fue de síndrome de Hiper IgE. La evolución clínica fue favorable con tratamiento antibiótico y antifúngico y está en control ambulatorio periódico (AU)

Patient: A 5-year-old girl presented with chronic otorrhea, cervical adenopathies and cellulitis of the knee. In addition to these lesions, physical examination revealed eczema on the scalp, neck, perineal and umbilical regions and the persistence of deciduous teeth with adult teeth (double dental arch). Complementary investigations showed the following concentrations: IgE 23,969 UI/l, IgD 440U/L, IgG 23,000 mg/L, and IgA 4,220 mg/L. Intradermal skin testing to Candida was negative and the results of the remaining immunological studies were normal. Computerized axial tomography revealed bilateral otomastoiditis. Candida albicans was isolated from ear secretion cultures. The definitive diagnosis was hyper IgE syndrome. The patient responded favorably to antibiotic and antifungal therapy and is currently undergoing period outpatient monitoring (AU)

Alimentación del lactante con una reacción adversa a las proteínas de la leche de vaca / Feeding the infant with adverse response to cow's milk proteins

Se revisan y actualizan los conceptos actuales de la alimentación de los lactantes con reacciones adversas a las proteínas de la leche de vaca (PLV). Se propone un algoritmo terapéutico (AU)

The current concepts concerning the feeding of infants who are allergic to the proteins in cow’s milk are reviewed and a therapeutic algorithm is proposed (AU)