Clinical pattern and acute and long-term management of hereditary angioedema due to C1-esterase inhibitor deficiency

Background: Hereditary angioedema due to C1-esterase inhibitor deficiency (HAE-C1-INH) is a life-threatening disease. Objectives: To describe the clinical characteristics and management of patients with HAE-C1-INH during routine clinical practice. Methods: An observational, retrospective study was performed in patients with HAE-C1-INH. Demographic, clinical, and analytical data were collected from 2 periods: period A (October 2009-September 2010) and period B (October 2007-September 2009). Results: We studied 112 patients with HAE-C1-INH (57.1% females). Age at onset of symptoms was 14.4 years (lower in patients who had experienced attacks in the previous year). In period B (n=87), 62.1% of patients presented at least 1 edema attack (median, 3.5 attacks/patient/2 years), and 19.1% of attacks were treated. In period A (n=77), 58.4% of patients were on maintenance therapy. Stanozolol was the most widely used drug (48.9%), with a mean weekly dose of 6.7 mg. At least 1 attack was recorded in 72.7% of patients (median, 3.0 attacks/patient/year), and 31.5% of the attacks were treated. Treatment of acute attacks increased by 12.4%. Conclusion: Age at onset of symptoms is associated with clinical expression of disease. The higher age at onset of symptoms, the fewer number of attacks per patient and year, and the lower dose of attenuated androgens necessary to control the disease than in other series lead us to hypothesize that HAE-C1-INH could have a less severe expression in Spain. Acute attacks seem to be treated increasingly often (AU)

Antecedentes: El angioedema hereditario por déficit del inhibidor de la C1 esterasa (AEH-C1-INH) es potencialmente mortal. Objetivos: Describir las características clínicas y el manejo de pacientes con AEH-C1-INH durante la práctica clínica habitual. Métodos: Estudio retrospectivo observacional de pacientes con AEH-C1-INH. Se recogieron datos demográficos, clínicos y analíticos en los periodos A (Octubre 2009-Septiembre 2010) y B (Octubre 2007-Septiembre 2009). Resultados: Se estudiaron 112 pacientes con AEH-C1-INH (57,1% mujeres) con edad de inicio de los síntomas de 14,4 años (inferior en aquellos pacientes con ataques en el último año). En el periodo B (n=87) 62,1% tuvo al menos un ataque (mediana: 3,5 ataques/paciente /2 años) y el 19,9% de los ataques se trataron. En el periodo A (n=77) 58,4% recibieron tratamiento de mantenimiento, siendo el estanozolol el fármaco más utilizado (48,9%) (dosis media semanal 6,7mg). El 72,7% de los pacientes tuvo al menos un ataque (mediana: 3,0 ataques / paciente / año), el 31,5% se trataron. Hubo un incremento del 12,4% de tratamientos de ataques agudos. Conclusiones: La edad de inicio de los síntomas está relacionada con la expresión clínica de la enfermedad. La edad superior del inicio de los síntomas, el menor número de ataques por paciente/año, y una dosis inferior de andrógenos atenuados para controlar la enfermedad, comparado con otros países, permite hipotetizar que el AEH-C1-INH en España tendría una expresión clínica menos grave. Existe una tendencia al alza en la frecuencia de tratamiento de ataques agudos (AU)

Eliciting doses of positive challenge test in cow’s milk allergy are related to cow’s milk specific IgE levels

Background: Ingestion of small amounts of cow’smilk (CM) can elicit adverse reactions in patients with IgE-mediated CM allergy. Knowing the dose eliciting allergic reactions and the factors affecting it can be of great help in avoiding these reactions. Objective: To analyse the eliciting doses of positive challenge test in patients with CM allergy and to determine its association with the level of CM specific IgE. Methods: Ninety-eight positive challenge tests in 56 children, median age of 11 months (3-80) with IgE-mediated CM allergy were retrospectively analysed. Open oral challenge tests were carried out by gradually increasing doses of milk (2-100 ml). The relationship between challenge test doses and CM specific IgE levels were studied. Results: 18 % of the challenge tests were positive with 2 ml, 24 % with doses between 5 and 10 ml, and the other 58 % with doses between 25 and 100 ml. An inverse association between the doses of the positive challenge test and the level of CM specific- IgE was found, 13.9 kU/L (0.54-> 100 kU/L) when the challenge test was positive with the smaller dose (2 ml); and 1.73 kU/L (< 0.35-76.4 kU/L) with doses above 2 ml (p = 0.0001). The median age of the patients was 13 months (6-49) when the challenge test was positive with 2 ml vs 9 months (3-80) with doses above 2 ml (p = 0.048). Conclusion: The CM specific IgE level and patient’s age should be considered in the assessment of the eliciting doses of positive challenge test in CM allergy

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The predictive value of specific immunoglobulin E levels in serum for the outcome of the development of tolerance in cow’s milk allergy

Background: Immunoglobulin E-mediated allergy to cow’s milk protein (CMP) tends to subside over years of follow-up. The gold standard for detecting such allergy has been the oral challenge test. The development of some other test for determining the correct timing of the oral challenge test would avoid unnecessary patient discomfort. The aim of this study was to determine whether monitoring cow’s milk (CM) specific IgE levels overtime can be used as a predictor for determining when patients develop clinical tolerance. Methods: A prospective 4-year follow-up study was made of 170 patients with IgE-mediated allergy to CMP, involving periodic evaluations (12, 18, 24, 36 and 48 months) with the determination of casein and CM specific IgE on each visit, along with CM challenge testing. ROC curves were used to analyse the sensitivity, specificity and predictive values of the casein and CM specific IgE levels versus the challenge test out comes at the different moments of follow-up. Results: In the course of follow-up, 140 infants (82 %) became tolerant. Specific IgE levels to CM:2.58, 2.5, 2.7, 2.26, 5 kUA/l and to casein: 0.97, 1.22,3, 2.39, 2.73 kUA/l, respectively, predicted clinical reactivity (greatest diagnostic efficiency values) at the different analysed moments of follow-up (12, 18, 24,36 and 48 months). Conclusions: Quantification of CMP specific IgE is a useful test for diagnosing symptomatic allergy toCM in the paediatric population, and could eliminate the need to perform oral challenges tests in a significant number of children

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La leche de cabra no es una alternativa válida en pacientes con alergia a las proteínas de la leche de vaca / Goat´s milk is not valid alternative in patients allergic to cow´s milk proteins

Further reflections on streptococcal pharyngitis (AU)

Alergía a las proteínas de huevo / Allergy to egg proteins

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Syndrome auriculotemporal / Síndrome auriculotemporal

Two cases of syndrome auriculotemporal are presented in two male children that begin in the first years of life to present reactions of eritema of lineal itinerary in cheeks after eating several foods. The allergologic study with these foods was negative, reproducing the clinic after their ingesta (AU)

Se presentan 2 casos de síndrome auriculotemporal en niños varones que se inició en los primeros años de vida como reacciones eritematosas de itinerario lineal en las mejillas después de la ingestión de diversos alimentos.El estudio alergológico con estos alimentos fue negativo, reproduciéndose la clínica después de su ingesta (AU)