RESUMO
OBJECTIVES: To assess clinically asymptomatic infants with single-ventricle physiology (SVP) for sleep-disordered breathing (SDB) in the supine and car seat positions using polysomnography. Polysomnography results also were compared with results of a standard Car Seat Challenge to measure the dependability of the standard Car Seat Challenge. STUDY DESIGN: This was an observational study of 15 infants with SVP. Polysomnography data included Obstructive Index, Central Index, Arousal Index, Apnea Hypopnea Index, and sleep efficiency. Polysomnography heart rate and oxygen saturation data were used to compare polysomnography with the standard Car Seat Challenge. RESULTS: Polysomnography demonstrated that all 15 infants had SDB and 14 had obstructive sleep apnea (Obstructive Index ≥1/hour) in both the supine and car seat positions. Infants with SVP had a statistically significant greater median Obstructive Index in the car seat compared with supine position (6.3 vs 4.2; P = .03), and median spontaneous Arousal Index was greater in the supine position compared with the car seat (20.4 vs 15.2; P = .01). Comparison of polysomnography to standard Car Seat Challenge results demonstrated 5 of 15 (33%) of infants with SVP with abnormal Obstructive Index by polysomnography would have passed a standard Car Seat Challenge. CONCLUSIONS: Infants with SVP without clinical symptoms of SDB may be at high risk for SDB that appears worse in the car seat position. The standard Car Seat Challenge is not dependable in the identification of infants with SVP and SDB. Further studies are warranted to further delineate its potential impact of SDB on the clinical outcomes of infants with SVP.
Assuntos
Doenças Assintomáticas , Cardiopatias Congênitas/fisiopatologia , Frequência Cardíaca/fisiologia , Ventrículos do Coração/anormalidades , Síndromes da Apneia do Sono/fisiopatologia , Feminino , Seguimentos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Humanos , Recém-Nascido , Masculino , Consumo de Oxigênio , Polissonografia , Estudos Prospectivos , Índice de Gravidade de Doença , Síndromes da Apneia do Sono/complicações , Síndromes da Apneia do Sono/diagnósticoRESUMO
OBJECTIVE: To describe and compare the lung function decline in patients with Duchenne muscular dystrophy on glucocorticoid therapy in contrast with glucocorticoid-naïve patients, and to define the deciles of pulmonary decline in glucocorticoid-treated patients. STUDY DESIGN: This retrospective study examined lung function of patients with Duchenne muscular dystrophy over 6 years of age followed between 2001 and 2015 at 2 centers-glucocorticoid-treated patients in Cincinnati, Ohio, and glucocorticoid-naïve patients in Paris, France. Forced vital capacity (FVC, FVC%), forced expiratory volume in 1 second, maximal inspiratory pressure, maximal expiratory pressure, and peak expiratory flow data were analyzed. Only FVC data were available for the French cohort. RESULTS: There were 170 glucocorticoid-treated patients (92%), 5 patients (2.7%) with past glucocorticoid use, and 50 French glucocorticoid-naïve patients. The peak absolute FVC was higher and was achieved at earlier ages in glucocorticoid-treated compared with glucocorticoid-naïve patients (peak FVC, 2.4 ± 0.6 L vs 1.9 ± 0.7 L; P < .0001; ages 13.5 ± 3.0 years vs 14.3 ± 2.8 years; P = .03). The peak FVC% was also higher and was achieved at earlier ages in glucocorticoid-treated patients (peak FVC%, 105.1 ± 25.1% vs 56 ± 20.9%; P < .0001; ages 11.9 ± 2.9 years vs 13.6 ± 3.2 years; P = .002). Rates of decline for both groups varied with age. Maximal rates of decline were 5.0 ± 0.26% per year (12-20 years) for glucocorticoid-treated and 5.1 ± 0.39% per year for glucocorticoid-naïve patients (11-20 years; P = .2). Deciles of FVC% decline in glucocorticoid-treated patients show that patients experience accelerated decline at variable ages. CONCLUSIONS: These data describe nonlinear rates of decline of pulmonary function in patients with Duchenne muscular dystrophy, with improved function in glucocorticoid-treated patients. FVC% deciles may be a useful tool for clinical and research use.
Assuntos
Distrofia Muscular de Duchenne/tratamento farmacológico , Testes de Função Respiratória , Adolescente , Criança , Progressão da Doença , Glucocorticoides/uso terapêutico , Humanos , Masculino , Distrofia Muscular de Duchenne/fisiopatologia , Prednisona/uso terapêutico , Pregnenodionas/uso terapêutico , Estudos RetrospectivosRESUMO
OBJECTIVES: To describe sleep-disordered breathing (SDB) in young boys with Duchenne muscular dystrophy (DMD) and its relationship with pulmonary function tests (PFTs). STUDY DESIGN: This retrospective study examined diagnostic polysomnogram and PFT data of boys younger than 18 years with DMD and treated with steroids. Spirometry, respiratory muscle strength, body mass index (BMI), sleep architecture variables, and indices of SDB were analyzed. We examined the effect of PFT measures on the risk of each type of respiratory event using logistic regression and have reported results as OR (95% CI). RESULTS: Subjects included 110 boys with DMD, mean age 11.5 (5.6-17.9) years. Mean (±SD) percent forced vital capacity predicted was 79.5% ± 29.1%. Mean BMI for all subjects was 21.9 ± 7.0 kg/m(2), and mean BMI z-score was 0.65 ± 1.93. Seventy (63.6%) subjects had obstructive sleep apnea; 37 (33.6%) subjects had central sleep apnea; 18 (17%) subjects had hypoventilation. Median (IQR) Apnea Hypopnea Index was 2.9 (1.6-6.9) and median Obstructive Index was 1.5 (0.5-3.8). Obstructive Index during rapid eye movement sleep positively correlated with BMI (r = 0.33, P = .002), BMI z-score (r = 0.22, P = .04), and age (r = 0.31, P = .004). Lower forced vital capacity was associated with increased risk of hypoventilation (OR 0.8, P = .001). CONCLUSION: SDB is common in young boys with DMD treated with steroids. It is manifest with rapid eye movement-obstructive sleep apnea, often severe, and strongly influenced by BMI.
Assuntos
Índice de Massa Corporal , Sobrepeso/etiologia , Síndromes da Apneia do Sono/complicações , Sono/fisiologia , Feminino , Humanos , MasculinoRESUMO
OBJECTIVE: To determine whether premature infants' sleep organization, total sleep time, and arousals may be modulated while on a conformational positioner that provides boundaries, customized positioning, and containment compared with standard positioning (standard crib mattress). STUDY DESIGN: A proof of concept trial using a within subject crossover design was conducted among 25 premature infants with feeding difficulties. Infants of 31.5 weeks gestational age served as their own control during overnight polysomnography at postconceptual age 38.4 weeks. Each baby received both interventions (order randomized), 1 for each one-half of the 10.5-hour study. RESULTS: Use of the conformational positioner resulted in higher sleep efficiency of 61% vs 54% for the standard mattress (P < .05). The interventions did not differ for percent active sleep, percent quiet sleep, percent indeterminate sleep, or spontaneous arousals. Sleep efficiency was higher on the conformational positioner than standard positioning for surgical subjects and for subjects with necrotizing enterocolitis or gastroschisis (n = 10). The surgical subjects (n = 9) had lower sleep efficiency, lower percentage of active sleep, and more spontaneous arousals compared with the nonsurgical group. CONCLUSIONS: The use of the conformational positioner improved sleep efficiency vs the standard mattress in premature infants with feeding difficulties. Infants requiring surgery or with gastrointestinal diagnoses may be more susceptible to environmental stress.
Assuntos
Recém-Nascido Prematuro , Posicionamento do Paciente/métodos , Sono/fisiologia , Estudos Cross-Over , Eletroencefalografia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , PolissonografiaRESUMO
OBJECTIVE: To assess sleep-disordered breathing (SDB), sleep architecture, and arousal pattern in infants with achondroplasia and to evaluate the relationship between foramen magnum size and the severity of SDB. STUDY DESIGN: A retrospective review of polysomnographic recordings and medical records was performed in infants with achondroplasia and in aged-matched control subjects. All studies were re-scored with the emphasis on respiratory events, sleep state, and arousals. In addition, the neuroimaging study of the brain (magnetic resonance imaging) was reviewed to evaluate foramen magnum diameters and to assess their relationship to SDB. RESULTS: Twenty-four infants met the criteria for entry into analysis, 12 infants with achondroplasia (A) and 12 control infants (C). There was no significant difference in age or sex. Infants with achondroplasia had a significant increase in total respiratory disturbance index (RDI; A, 13.9 +/- 10.8 versus C, 2.0 +/- 0.9; P < .05). However, there was no significant difference in percentages of active sleep, quiet sleep, or sleep efficiency. Analysis of arousals demonstrated that infants with achondroplasia had a significant decrease in both spontaneous arousal index (A, 10.5 +/- 3.5/hr versus C, 18.6 +/- 2.7; P < .0001) and respiratory arousals (A, 10.3% +/- 6.3% versus C, 27.5 +/- 9.5%; P < .0001). Evaluation of foramen magnum dimensions demonstrated smaller foramen magnum size, but there were no significant correlations between anteroposterior or transverse diameters and RDI. CONCLUSION: Infants with achondroplasia have significant SDB during early infancy. SDB in infants with achondroplasia is not associated with alteration in sleep architecture, possibly because of attenuation of the arousal response. We speculate that the concomitant increased apneic events and decreased arousal response will lead to vulnerability in these infants and may underlie the pathophysiologic mechanism of sudden unexpected death in this population.