RESUMEN
Introducción: El déficit congénito de surfactante es una entidad de diagnóstico inhabitual en recién nacidos. Se reporta un caso clínico de déficit de proteína B del surfactante, se revisa el estudio, tratamiento y diagnóstico diferencial de los déficit de proteínas del surfactante y enfermedad crónica intersticial de la infancia. Caso clínico: Recién nacido de término que cursa dificultad respiratoria, con velamiento pulmonar recurrente y respuesta transitoria a administración de surfactante. El estudio inmunohistoquímico y genético confirmaron diagnóstico de déficit de proteína B de surfactante. Conclusiones: La enfermedad pulmonar congénita requiere un alto índice de sospecha. El déficit de proteína B de surfactante genera un cuadro clínico progresivo y mortal en la mayoría de los casos, al igual que el déficit de transportador ATP binding cassette, sub-family A member 3 (ABCA3). El déficit de proteína C es insidioso y puede presentarse con un patrón radiológico pulmonar intersticial. Debido a la similitud en el patrón histológico, el estudio genético permite una mayor certeza en el pronóstico y la posibilidad de entregar un adecuado consejo genético.
Introduction: Congenital surfactant deficiency is a condition infrequently diagnosed in newborns. A clinical case is presented of surfactant protein B deficiency. A review is performed on the study, treatment and differential diagnosis of surfactant protein deficiencies and infant chronic interstitial lung disease. Case report: The case is presented of a term newborn that developed respiratory distress, recurrent pulmonary opacification, and a transient response to the administration of surfactant. Immunohistochemical and genetic studies confirmed the diagnosis of surfactant protein B deficiency. Conclusions: Pulmonary congenital anomalies require a high index of suspicion. Surfactant protein B deficiency is clinically progressive and fatal in the majority of the cases, similar to that of ATP binding cassette subfamily A member 3 (ABCA3) deficiency. Protein C deficiency is insidious and may present with a radiological pulmonary interstitial pattern. Due to the similarity in the histological pattern, genetic studies help to achieve greater certainty in the prognosis and the possibility of providing adequate genetic counselling.
Asunto(s)
Humanos , Masculino , Recién Nacido , Proteinosis Alveolar Pulmonar/congénito , Síndrome de Dificultad Respiratoria del Recién Nacido/etiología , Surfactantes Pulmonares/administración & dosificación , Proteína B Asociada a Surfactante Pulmonar/deficiencia , Proteinosis Alveolar Pulmonar/complicaciones , Proteinosis Alveolar Pulmonar/diagnóstico , Proteinosis Alveolar Pulmonar/tratamiento farmacológico , Síndrome de Dificultad Respiratoria del Recién Nacido/genética , Diagnóstico DiferencialRESUMEN
INTRODUCTION: Congenital surfactant deficiency is a condition infrequently diagnosed in newborns. A clinical case is presented of surfactant protein B deficiency. A review is performed on the study, treatment and differential diagnosis of surfactant protein deficiencies and infant chronic interstitial lung disease. CASE REPORT: The case is presented of a term newborn that developed respiratory distress, recurrent pulmonary opacification, and a transient response to the administration of surfactant. Immunohistochemical and genetic studies confirmed the diagnosis of surfactant protein B deficiency. CONCLUSIONS: Pulmonary congenital anomalies require a high index of suspicion. Surfactant protein B deficiency is clinically progressive and fatal in the majority of the cases, similar to that of ATP binding cassette subfamily A member 3 (ABCA3) deficiency. Protein C deficiency is insidious and may present with a radiological pulmonary interstitial pattern. Due to the similarity in the histological pattern, genetic studies help to achieve greater certainty in the prognosis and the possibility of providing adequate genetic counselling.
Asunto(s)
Proteinosis Alveolar Pulmonar/congénito , Proteína B Asociada a Surfactante Pulmonar/deficiencia , Surfactantes Pulmonares/administración & dosificación , Síndrome de Dificultad Respiratoria del Recién Nacido/etiología , Diagnóstico Diferencial , Humanos , Recién Nacido , Masculino , Proteinosis Alveolar Pulmonar/complicaciones , Proteinosis Alveolar Pulmonar/diagnóstico , Proteinosis Alveolar Pulmonar/tratamiento farmacológico , Síndrome de Dificultad Respiratoria del Recién Nacido/genéticaRESUMEN
We study the transmission of diffuse light through thick slits. For perfectly conducting slits and in-plane s-polarized illumination, the transmittance curves present a staircaselike behavior as a function of the aperture width, where the steps mark the appearance of new propagation modes. In contrast, with p-polarized illumination the transmittance increases linearly with the aperture width, with only some perturbations in the positions that correspond to the appearance of new modes. Out-of-plane incidence and more realistic assumptions about the slit, such as finite conductivity and roughness, are also discussed.