RESUMEN
Asthma is one of the commonest chronic conditions in children and can occasionally be fatal. Little has changed regarding the risk factors for children dying from asthma in the last 30â¯years. The majority of deaths from asthma occur in children from socio-economically disadvantaged backgrounds. These should be preventable with better education of families, oversight of medication adherence and improved communication between health care professionals and families. More needs to be done to deliver basic messages more effectively about asthma management to the most vulnerable in communities around the world.
Asunto(s)
Asma , Servicios Preventivos de Salud , Factores Socioeconómicos , Asma/mortalidad , Asma/terapia , Niño , Mortalidad del Niño , Manejo de la Enfermedad , Educación en Salud , Humanos , Cumplimiento de la Medicación , Servicios Preventivos de Salud/métodos , Servicios Preventivos de Salud/organización & administración , Factores de RiesgoRESUMEN
OBJECTIVE: To describe the clinical course of children who have intermediate sweat chloride values on initial screening for cystic fibrosis (CF). STUDY DESIGN: We performed a retrospective review of children with intermediate sweat chloride values (raised immunoreactive trypsinogen/1 copy of p.F508del CF mutation on newborn screening (NBS)/sweat chloride value of 30-59 mmol/L) presenting to The Children's Hospital at Westmead over 15 years. Patients with an intermediate sweat chloride evolving to a formal diagnosis of CF (termed "delayed CF") were matched (2:1) with NBS positive patients with CF (termed "NBS positive CF"). Clinical outcomes were compared. RESULTS: Fourteen of 29 (48%, 95% CI 0.3-0.66) patients with intermediate sweat chloride value evolved to a diagnosis of CF and were matched with 28 NBS positive patients with CF. Delayed CF had less pancreatic insufficiency (OR 0.06, 95% CI 0.01-0.44, P = .006), less colonization with nonmucoid Pseudomonas aeruginosa (OR 0.04, 95% CI 0.01-0.38, P = .005), milder obstructive lung disease (forced expiratory volume in 1 second/forced vital capacity ratio), and overall disease severity (Shwachman scores) at 10 years (mean difference 5.93, 95% CI 0.39-11.46, P = .04; mean difference 4.72, 95% CI 0.9-8.53, P = .015, respectively). Nutritional outcomes were better at 2 years for delayed CF but did not persist to later ages. CONCLUSIONS: In this cohort, approximately one-half of infants with intermediate sweat chloride value were later diagnosed with CF. The clinical course of delayed CF was milder in some aspects compared with NBS positive CF. These results emphasize the importance of ongoing follow-up of infants with intermediate sweat chloride values.