[Prolonged Guillain-Barré syndrome]. / Síndrome de Guillain-Barré prolongado.

Outcome in Guillain-Barré syndrome (GBS) in childhood is usually favorable although prolonged and severe forms may develop. We report the case of a 12-year-old boy with rapidly ascending, slowly-progressive GBS with severe cranial nerve and autonomic dysfunction. Diagnosis was based on clinical presentation, electromyography, cerebrospinal fluid analysis and plasmatic antiganglioside GM1 antibodies. Early treatment with an initial loading dose of 2 g/kg of intravenous gammaglobulins over 5 days was given with a significant but slow clinical improvement. A repeat infusion of gammaglobulins was given two months later. Response was satisfactory with motor, sensory and autonomic dysfunction recovery. A third course of immunoglobulins 28 days later produced little benefit. At the present time, 5 months after the onset of the disease, flaccid paralysis of the lower limbs and the distal portion of the upper limbs persists but autonomic instability has disappeared. The patient has been decannulated and is following an ambulatory rehabilitation program. Although repeated intravenous immunoglobulins may be useful in the treatment of prolonged GBS, the key issue is excellent intensive care unit management. Further research examining potentially more effective treatment such as beta-interferon and immunosuppressive agents is required.

Síndrome de Guillain-Barré prolongado / Prolonged Guillain-Barré syndrome

La evolución del síndrome de Guillain-Barré suele ser benigna en la infancia, aunque hay casos prolongados y graves. Comunicamos el caso de un varón de 12 años afectado de síndrome de Guillain-Barré, rápidamente ascendente, con afectación grave de pares craneales y del sistema nervioso autónomo y evolución tórpida. El diagnostico se basó en la sintomatología, la electromiografía, la bioquímica del líquido cefalorraquídeo (LCR) y la presencia de anticuerpos antigangliósido GM1 en plasma. Fue tratado precozmente con inmunoglobulina humana por vía intravenosa en dosis de 2 g/kg en 5 días; aunque la clínica mejoró, la recuperación fue muy lenta, decidiendo administrar un nuevo ciclo de inmunoglobulina a los 2 meses; la respuesta fue satisfactoria, con mejoría de la función sensitivomotora y de las manifestaciones vegetativas. Sin embargo, un nuevo ciclo administrado 28 días más tarde obtuvo escasos resultados. En la actualidad, 5 meses después del inicio del cuadro, persistía parálisis flácida de miembros inferiores y de la porción más distal de los superiores, si bien han desaparecido los trastornos vegetativos y el paciente ha podido ser descanulado, estando sometido a un programa de rehabilitación ambulatoria. Aunque la pauta con varios ciclos de inmunoglobulinas puede ser útil en el tratamiento del síndrome de Guillain-Barré de carácter prolongado, el tratamiento general y de soporte sigue siendo la base fundamental en estos pacientes, a la espera de que varios ensayos clínicos comprueben la eficacia de tratamientos alternativos como interferón beta e inmunosupresores (AU)

[Immunotherapy with an oral Alternaria extract in childhood asthma. Clinical safety and efficacy and effects on in vivo and in vitro parameters]. / Inmunoterapia con un extracto oral de Alternaria en el asma infantil. Eficacia clínica, seguridad, repercusiones sobre parámetros in vivo e in vitro.

Studies of immunotherapy with oral Alternaria extracts are scarce. We decided to perform a clinical trial of the clinical safety and efficacy of this extract as well as of its effects on in vivo and in vitro parameters in 39 patients with Alternaria allergy, aged between 7 and 17 years, who are also sensitized extract was used. Allergic activity was determined through RAST inhibition and skin prick test. Quantification of the principal allerten (Alt a 1) was performed through the 2-site binding assay, with a mean content of 34.2 ng Alt a 1/micro g protein. The parameters analyzed were the symptom-medication score, skin prick using the end-point technique, specific bronchial challenge test, peak flow, total and specific IgE and IgG4. Nineteen patiens received active treatment with oral immunotherapy and another 19 received symptomatic treatment. The initial phase of immunotherapy lasted 3 months until the maximum dose was reached. This was maintained for 12 months; the mean accumulated dos was 280,000 PNU. Significant differences were found in reduction in the symptom-medication score in the treated group after 12 months of immunotherapy. No differences were found in the control group. Immunotherapy was well tolerated with 0.42 adverse reactions per 100 doses administered. All adverse reactions were mild-to-moderate. In the treated group, papule size was significantly reduced. Values for the specific bronchial challenge test, expressed through PD20, were significantly higher in the immunotherapy group. Peak flow showed no changes in either group. Values of IgG4 were significantly higher in the immunotherapy group. Total and specific IgE levels showed no significant changes in either group. In conclusion, oral immunotherapy with Alternaria extract is clinically effective in pediatric patients. In general, the therapy was well tolerated. It modified specific cutaneous and bronchial reactivity in our sample and increased levels of specific IgG4, wich are implicated in humoral response.

Inmunoterapia con un extracto oral del Alternaria en el asma infantil. Eficacia clínica, seguridad, repercusines sobre parámetros in vivo e in vitro / Immunotherapy with an oral Alternaria extract in chilhood asthma. Clinical safety and efficacy and effects on in vivo and in vitro

Ante la escasez de trabajos con inmunoterapia oral de Alternaria, planteamos la realización de un ensayo clínico en 39 pacientes, con edades comprendidas entre 7 y 17 años, alérgicos a Alternaria, y sensibilizados también a pólenes y epitelios para evaluar su eficacia clínica y seguridad, así como las repercusiones sobre parámetros in vivo e in vitro. Se empleó un extracto estandarizado, determinando la actividad alérgica mediante RAST inhibición y prick test cutáneo. La cuantificación del alergeno principal (Alt a 1) se llevó a cabo mediante la técnica de fijación en dos lugares, siendo el contenido medio de 34,2 ng Alt a 1/ g de proteína. Los parámetros analizados fueron la puntuación de síntomas-medicación, prick test cutáneo a punto final (TC), test de bronco provocación específico (TBPE), pico de flujo (PF), IgE total y específica e IgG4.Diecinueve pacientes recibieron tratamiento activo con inmunoterapia oral (ITO) y otros diecinueve recibieron tratamiento sintomático. La fase de inicio de la inmunoterapia duró 3 meses, hasta llegar a dosis máxima, que se mantuvo durante 12 meses, alcanzando una dosis acumulada media de 280.000 PNU. Se hallaron diferencias significativas en la disminución de la puntuación de síntomas-medicación en el grupo tratado, tras los 12 meses de inmunoterapia (ITO). No se encontraron diferencias en el grupo control. La inmunoterapia fue bien tolerada, presentando 0,42 reacciones adversas (RA) por 100 dosis administradas, siendo de carácter leve-moderado exclusivamente. Se encontró disminución significativa del tamaño de pápula en el grupo tratado. El TBPE expresado mediante la PD20 mostró cifras significativamente más altas en el grupo con ITO. El pico de flujo no mostró cambios en ninguno de los dos grupos. Los valores de la IgG4 fueron significativamente más altos en el grupo con inmunoterapia. Los niveles de IgE total y específica no mostraron cambios significativos en ambos grupos. En conclusión, la Inmunoterapia Oral con extracto de Alternaria ha demostrado eficacia clínica en pacientes pediátricos, siendo en general bien tolerada, modificando la reactividad específica cutánea y bronquial con incremento de los niveles de IgG4 específica implicados en la respuesta humoral (AU)

Studies of immunotherapy with oral Alternaria extracts are scarce. We decided to perform a clinical trial of the clinical safety and efficacy of this extract as well as of its effects on in vivo and in vitro parameters in 39 patients with Alternaria allergy, aged between 7 and 17 years, who are also sensitized extract was used. Allergic activity was determined through RAST inhibition and skin prick test. Quantification of the principal allerten (Alt a 1) was performed through the 2-site binding assay, with a mean content of 34.2 ng Alt a 1/μg protein. The parameters analyzed were the symptom-medication score, skin prick using the end-point technique, specific bronchial challenge test, peak flow, total and specific IgE and IgG4. Nineteen patiens received active treatment with oral immunotherapy and another 19 received symptomatic treatment. The initial phase of immunotherapy lasted 3 months until the maximum dose was reached. This was maintained for 12 months; the mean accumulated dos was 280,000 PNU. Significant differences were found in reduction in the symptom-medication score in the treated group after 12 months of immunotherapy. No differences were found in the control group. Immunotherapy was well tolerated with 0.42 adverse reactions per 100 doses administered. All adverse reactions were mild-to-moderate. In the treated group, papule size was significantly reduced. Values for the specific bronchial challenge test, expressed through PD20, were significantly higher in the immunotherapy group. Peak flow showed no changes in either group. Values of IgG4 were significantly higher in the immunotherapy group. Total and specific IgE levels showed no significant changes in either group. In conclusion, oral immunotherapy with Alternaria extract is clinically effective in pediatric patients. In general, the therapy was well tolerated. It modified specific cutaneous and bronchial reactivity in our sample and increased levels of specific IgG4, wich are implicated in humoral response (AU)