RESUMEN
INTRODUCTION: The health-related quality of life in X-linked agammaglobulinemia was investigated in 25 children and adolescents patients through the Italian version of Pediatric Quality of Life Inventory 4.0 Generic Core Scale for patients aged less then 18 years, comparing child perception to that of the parents and the physician's evaluation. The data were compared with the ones of 80 healthy controls and the literature data of a group of patients with rheumatic diseases. DISCUSSION: The agammaglobulinemia subjects perceived a lower global quality of life than the healthy subjects, but significantly higher than the rheumatic diseases controls. The clinical relevance of health-related quality of life assessment in X-linked agammaglobulinemia pediatric patients is discussed.
Asunto(s)
Agammaglobulinemia/psicología , Enfermedades Genéticas Ligadas al Cromosoma X/psicología , Calidad de Vida , Actividades Cotidianas , Adolescente , Agammaglobulinemia/tratamiento farmacológico , Agammaglobulinemia/genética , Niño , Preescolar , Femenino , Enfermedades Genéticas Ligadas al Cromosoma X/tratamiento farmacológico , Enfermedades Genéticas Ligadas al Cromosoma X/genética , Estado de Salud , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Masculino , Mutación/genética , Padres , Factores Socioeconómicos , Encuestas y CuestionariosAsunto(s)
Anticuerpos Monoclonales/efectos adversos , Colitis Ulcerosa/tratamiento farmacológico , Herpes Zóster/inmunología , Vasculitis por IgA/inducido químicamente , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores , Niño , Colitis Ulcerosa/inmunología , Femenino , Humanos , Vasculitis por IgA/inmunología , Huésped Inmunocomprometido , InfliximabRESUMEN
After the introduction of Nasal Continuous Positive Airways Pressure (nCPAP) in a neonatal intensive care unit, the incidence of complications and outcomes of newborns was explored. The clinical and nursing records of 64 newborns treated with O2 and Hood's cuff (January 1996-June 1999) were compared to those of 68 patients treated with nCPAP (July 1999-July 2002). Newborns < 32 weeks, with congenital abnormalities and major myocardiopathies were excluded. The two groups were comparable for the main characteristics. Newborns treated with nCPAP needed less intubations (7 vs 14, p 0.004) and had less transferrals to more specialised structures (12 vs 26, p 0.001). The number of complications is comparable with the exception of a larger number of desaturations in the nCPAP group (20 vs 5, p 0.001) that anyhow, did not require resuscitation. The nCPAP allowed a reduction of the number of intubations and therefore newborns could be cared for in our centre, thus reducing the disadvantage of a transferral to a more specialised centre. This advantage, associated to the comparable number of complications, may be attributable to the short duration of the treatment but also to the attentive and appropriate nursing care.