RESUMEN
OBJECTIVE: To measure sleep patterns and quality, objectively and subjectively, in clinically stable children with cystic fibrosis (CF) and healthy control children, and to examine the relationship between sleep quality and disease severity. STUDY DESIGN: Clinically stable children with CF and healthy control children (7-18 years of age) were recruited. Sleep patterns and quality were measured at home with actigraphy (14 days). Overnight peripheral capillary oxygen saturation was measured via the use of pulse oximetry. Daytime sleepiness was evaluated by the Pediatric Daytime Sleepiness Scale (PDSS) and subjective sleep quality by the Sleep Disturbance Scale for Children and Obstructive Sleep Apnea-18. RESULTS: A total of 87 children with CF and 55 control children were recruited with no differences in age or sex. Children with CF had significantly lower total sleep time and sleep efficiency than control children due to frequent awakenings and more wake after sleep onset. In children with CF, forced expiratory volume in 1 second and overnight peripheral capillary oxygen saturation nadir correlated positively with total sleep time and sleep efficiency and negatively with frequency of awakenings and wake after sleep onset. Patients with CF had significantly greater Sleep Disturbance Scale for Children (45 vs 35; P < .001), Obstructive Sleep Apnea-18 (35 vs 24; P < .001), and PDSS scores (14 vs 11; P < .001). There was a negative correlation between PDSS and forced expiratory volume in 1 second (r = -0.23; P < .05). CONCLUSIONS: Even in periods of clinical stability, children with CF get less sleep than their peers due to more time in wakefulness during the night rather than less time spent in bed. Objective measures of sleep disturbance and subjective daytime sleepiness were related to disease severity. In contrast, parents of children with CF report high levels of sleep disturbance unrelated to disease severity.
Asunto(s)
Fibrosis Quística/complicaciones , Trastornos del Sueño-Vigilia/epidemiología , Trastornos del Sueño-Vigilia/etiología , Encuestas y Cuestionarios , Actigrafía/métodos , Adolescente , Distribución por Edad , Australia , Estudios de Casos y Controles , Niño , Fibrosis Quística/diagnóstico , Femenino , Volumen Espiratorio Forzado , Humanos , Incidencia , Masculino , Oximetría/métodos , Polisomnografía/métodos , Pronóstico , Intercambio Gaseoso Pulmonar , Valores de Referencia , Medición de Riesgo , Índice de Severidad de la Enfermedad , Distribución por Sexo , Trastornos del Sueño-Vigilia/diagnóstico , Estadísticas no Paramétricas , Centros de Atención TerciariaRESUMEN
OBJECTIVES: To determine whether bronchoalveolar lavage (BAL)-directed therapy for infants and young children with cystic fibrosis (CF), rather than standard therapy, was justified on the grounds of a decrease in average costs and whether the use of BAL reduced treatment costs associated with hospital admissions. STUDY DESIGN: Costs were assessed in a randomized controlled trial conducted in Australia and New Zealand on infants diagnosed with CF after newborn screening and assigned to receive either BAL-directed or standard therapy until they reached 5 years of age. A health care funder perspective was adopted. Resource use measurement was based on standardized data collection forms administered for patients across all sites. Unit costs were obtained primarily from government schedules. RESULTS: Mean costs per child during the study period were Australian dollars (AUD)92â860 in BAL-directed therapy group and AUD90â958 in standard therapy group (mean difference AUD1902, 95% CI AUD-27â782 to 31â586, P = .90). Mean hospital costs per child during the study period were AUD57â302 in the BAL-directed therapy group and AUD66â590 in the standard therapy group (mean difference AUD-9288; 95% CI AUD-35â252 to 16â676, P = .48). CONCLUSIONS: BAL-directed therapy did not result in either lower mean hospital admission costs or mean costs overall compared with managing patients with CF by a standard protocol based upon clinical features and oropharyngeal culture results alone. Following on our previous findings that BAL-directed treatment offers no clinical advantage over standard therapy at age 5 years, flexible bronchoscopy with BAL cannot be recommended for the routine management of preschool children with CF on the basis of overall cost savings.
Asunto(s)
Lavado Broncoalveolar/economía , Fibrosis Quística/economía , Fibrosis Quística/terapia , Preescolar , Costos y Análisis de Costo , Humanos , Lactante , Admisión del Paciente/economía , Admisión del Paciente/estadística & datos numéricosRESUMEN
OBJECTIVE: To determine whether a defect in energy metabolism exists in infants with cystic fibrosis (CF). DESIGN: Unselected, newly-diagnosed subjects with CF (n = 46) and 24 healthy infants aged <20 weeks had measurements of resting energy expenditure (REE), total energy expenditure (TEE) (n = 25), and body composition. Metabolizable energy intake (MEI) was calculated. Genotype, energy intake, and pancreatic status was determined in all subjects with CF, and 24 underwent bronchial lavage. RESULTS: At diagnosis, infants with CF detected by newborn screening had significant anthropometric deficits (mean [SD] z-weight = 0.5 [1.0], z-length = 0.7 [1.3]) associated with pancreatic insufficiency. Their REE, TEE, or MEI (absolute measurements, per unit body weight or fat-free mass) were not increased. No relationship between REE, TEE, or MEI and Delta F(508) genotype, and no proportional differences in individual components of MEI between subjects with CF and controls, or between subjects with CF who were homozygotes or compound heterozygotes for Delta F(508) were observed. REE and TEE were not correlated with bronchial infection or inflammation. CONCLUSION: Growth impairment during the first weeks of life in infants with CF is associated with pancreatic insufficiency. However, there is no evidence for a defect of energy metabolism related to Delta F(508), and in infants with CF, minimal lung disease is unaccompanied by increased energy expenditure.