RESUMEN
Surfactant protein B encoding gene mutations have been related to early onset fatal respiratory distress in full-term neonates. We report a school-aged male child homozygous for a surfactant protein B encoding gene missense mutation who presented after the neonatal period. His respiratory insufficiency responded to high dose intravenous methylprednisolone and hydroxychloroquine.
Asunto(s)
Diagnóstico Tardío , Proteinosis Alveolar Pulmonar/congénito , Proteína B Asociada a Surfactante Pulmonar/deficiencia , Niño , Marcadores Genéticos , Homocigoto , Humanos , Masculino , Mutación , Proteinosis Alveolar Pulmonar/diagnóstico , Proteinosis Alveolar Pulmonar/genética , Proteinosis Alveolar Pulmonar/terapia , Proteína B Asociada a Surfactante Pulmonar/genéticaRESUMEN
Paediatricians are in an excellent position to identify children with environmental risk, to advise their parents about the best way of reducing or preventing such risks, and to recommend actions to the responsible politicians involved. Paediatric environmental health speciality units (PEHSU) can help to qualify and support paediatricians in this task. PEHSU is defined as a unit within a paediatric hospital or clinic that is able to recognize, assess, and prevent environment-related health risks, to help other paediatric specialists in the management of such diseases in children, as well as to provide education, training, and research, putting emphasis on thorough and adequate establishment of paediatric environmental histories (PEHis) and to the application of the precautionary principle. Although activities and services provided by each PEHSU would differ depending on the centre or community where it is located, all should include training, research, medical care and community and school health.
Asunto(s)
Salud Ambiental/organización & administración , Medicina Ambiental/organización & administración , Unidades Hospitalarias/organización & administración , Pediatría/organización & administración , Niño , Exposición a Riesgos Ambientales , Europa (Continente) , HumanosRESUMEN
Symptomatic pulmonary manifestations of Kawasaki disease (KD) are uncommon. However, epidemiologic, radiologic, and histologic studies have indicated that respiratory symptoms and findings occur in KD and suggest that the KD agent may have a respiratory portal of entry. We report on three young infants with KD who developed pulmonary nodules, in addition to coronary artery aneurysms. Two patients had pathologic specimens available, one from biopsy and the other from autopsy. The nodules had predominantly mononuclear cell infiltrates, which were within the lung parenchyma and infiltrating vessel walls. Immunohistochemical studies of the nodules, using antibodies to common leukocyte antigen (LCA) and factor VIII-related antigen, confirmed the inflammatory nature of the lesions and showed capillary proliferation. IgA plasma-cell infiltration was observed in the nodule, consistent with previous KD findings of IgA plasma-cell infiltration in the vessel walls, kidneys, pancreas, and upper respiratory tract. The two patients with nonfatal KD were treated with intravenous immunoglobulin and aspirin, with resolution of the nodules. We propose that when pulmonary involvement occurs in KD, it ranges from subclinical interstitial micronodular infiltrates to larger inflammatory pulmonary nodules. These pulmonary infiltrates and nodules likely reflect the host response to the etiologic agent of KD, and may resolve with the disease process. Recognition of this pulmonary complication of KD may enable cautious observation of such lesions for spontaneous resolution.
Asunto(s)
Síndrome Mucocutáneo Linfonodular/inmunología , Síndrome Mucocutáneo Linfonodular/patología , Nódulo Pulmonar Solitario/patología , Antiinflamatorios no Esteroideos/uso terapéutico , Aspirina/uso terapéutico , Humanos , Inmunoglobulina A/análisis , Inmunoglobulinas Intravenosas/uso terapéutico , Inmunohistoquímica , Lactante , Pulmón/irrigación sanguínea , Masculino , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Neovascularización Patológica/patología , Células Plasmáticas/patología , Nódulo Pulmonar Solitario/tratamiento farmacológicoAsunto(s)
Bronquiolitis Obliterante/etiología , Virus de la Influenza B , Staphylococcus aureus Resistente a Meticilina/aislamiento & purificación , Neumonía Estafilocócica/complicaciones , Neumonía Viral/complicaciones , Infecciones Estafilocócicas/complicaciones , Adolescente , Bronquiolitis Obliterante/virología , Bronquitis , Femenino , Humanos , Neumonía Estafilocócica/microbiología , Neumonía Viral/virología , Atelectasia Pulmonar/etiología , Sobreinfección/microbiologíaRESUMEN
No disponible
Asunto(s)
Humanos , Femenino , Adolescente , Bronquitis/etiología , Bronquiolitis Obliterante/etiología , Neumonía/complicaciones , Betainfluenzavirus/patogenicidad , Sobreinfección/complicaciones , Staphylococcus aureus Resistente a Meticilina/aislamiento & purificación , Atelectasia Pulmonar/complicacionesRESUMEN
No disponible
Asunto(s)
Humanos , Masculino , Femenino , Lactante , Preescolar , Recién Nacido , Glotis , Hemangioma/tratamiento farmacológico , Neoplasias Laríngeas/tratamiento farmacológico , Propranolol/uso terapéutico , Factores de Tiempo , Resultado del TratamientoRESUMEN
No disponible
Asunto(s)
Humanos , Salud Ambiental/tendencias , Tabaquismo/prevención & control , Educación en Salud/métodos , Servicios de Salud Escolar , Estudiantes de Medicina , Cese del Uso de Tabaco/métodos , Promoción de la SaludRESUMEN
No disponible
Asunto(s)
Humanos , Recién Nacido , Displasia Broncopulmonar/terapia , /métodos , Pruebas de Función Respiratoria , Enfermedades del Prematuro/prevención & control , Índice de Severidad de la Enfermedad , Respiración Artificial/métodos , Terapia por Inhalación de Oxígeno/métodos , Broncodilatadores/uso terapéuticoRESUMEN
No disponible