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1.
Cleft Palate Craniofac J ; 57(7): 808-818, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-31973553

RESUMEN

OBJECTIVE: To determine pre- and postoperative prevalence of obstructive sleep apnea (OSA) in patients with 22q11.2 deletion syndrome (DS) undergoing wide posterior pharyngeal flap (PPF) surgery for velopharyngeal dysfunction (VPD). DESIGN: Retrospective study using pre- and postoperative polysomnography (PSG) to determine prevalence of OSA. Medical records were reviewed for patients' medical comorbidities. Parents were surveyed about snoring. SETTING: Academic tertiary care pediatric hospital. PATIENTS: Forty patients with laboratory confirmed 22q11.2DS followed over a 6-year period. INTERVENTIONS: Pre- and postoperative PSG, speech evaluation, and parent surveys. MAIN OUTCOME MEASURE: Severity and prevalence of OSA, defined by obstructive apnea hypopnea index (OAHI), before and after PPF surgery to determine whether PPF is associated with increased risk of OSA. RESULTS: Mean OAHI did not change significantly after PPF surgery (1.1/h vs 2.1/h, P = .330). Prevalence of clinically significant OSA (OAHI ≥ 5) was identical pre- and postoperatively (2 of 40), with both cases having severe-range OSA requiring positive airway pressure therapy. All other patients had mild-range OSA. Nasal resonance was graded as severe preoperatively in 85% of patients. None were graded as severe postoperatively. No single patient factor or parent-reported concern predicted risk of OSA (OAHI ≥ 1.5). CONCLUSIONS: Patients with 22q11.2DS are medically complex and are at increased risk of OSA at baseline. Wide PPF surgery for severe VPD does not significantly increase risk of OSA. Careful perioperative planning is essential to optimize both speech and sleep outcomes.


Asunto(s)
Síndrome de DiGeorge , Apnea Obstructiva del Sueño , Niño , Humanos , Faringe , Estudios Retrospectivos
2.
Paediatr Respir Rev ; 25: 9-13, 2018 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-28216256

RESUMEN

Kleine-Levin syndrome (KLS) is a rare disorder characterized by discrete episodes of hypersomnia associated with cognitive and behavioural abnormalities, as well as normal alertness and function between episodes. The prevalence of KLS may be underestimated as it is often misdiagnosed and managed as another sleep disorder, neurological disorder or psychiatric condition. KLS is more typically seen in adolescence than at other ages, and is more common in males than in females. There are currently neither standard biomarkers nor specific imaging study findings, making the diagnosis of KLS a challenge. Furthermore, there are no consistently effective therapies. The prognosis, however, is felt to be overall favorable, as episodes become progressively milder and less frequent before resolving entirely in most patients.


Asunto(s)
Síndrome de Kleine-Levin , Adolescente , Factores de Edad , Síntomas Conductuales , Cognición , Diagnóstico Diferencial , Femenino , Humanos , Síndrome de Kleine-Levin/diagnóstico , Síndrome de Kleine-Levin/psicología , Masculino , Pronóstico
3.
Br J Haematol ; 163(1): 112-7, 2013 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23869561

RESUMEN

Sickle cell disease (SCD) is a disorder known to impact the respiratory system. We sought to identify respiratory muscle force and lung volume relationships in a paediatric SCD population. Thirty-four SCD-SS subjects underwent pulmonary function testing. Height, weight, age, and gender-adjusted percent predicted maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) values were compared to spirometry and lung volumes. Statistical analyses were performed using Pearson's correlation coefficient and paired two-tailed t-test. The mean ± standard deviation (SD) MIP and MEP was 69·6 ± 31·6 cm H2 O and 66·9 ± 22·9 cm H2 O, respectively, and mean ± SD percent predicted MIP (101·3 ± 45·9) exceeded MEP (72·1 ± 26·0) (P = 0·002). MIP correlated with forced vital capacity (FVC; r = 0·51, P = 0·001) and TLC (r = 0·54, P < 0·0001). MEP also correlated with FVC (r = 0·43, P = 0·011) and total lung capacity (TLC; r = 0·42, P = 0·013). Pearson's correlation coefficient testing yielded relationships between MIP and MEP (r = 0·64, P < 0·0001). SCD-SS patients showed correlations between respiratory muscle force and lung volume, and reduced percent predicted expiratory muscle force compared to inspiratory muscle force. Respiratory muscle strength may affect lung volumes in these patients, and expiratory muscles may be more susceptible than the diaphragm to SCD-induced vaso-occlusive damage.


Asunto(s)
Anemia de Células Falciformes/fisiopatología , Fuerza Muscular , Músculos Respiratorios/fisiopatología , Capacidad Pulmonar Total , Adolescente , Niño , Femenino , Humanos , Masculino , Estudios Prospectivos , Pruebas de Función Respiratoria , Espirometría
4.
Am J Med Genet A ; 158A(9): 2152-61, 2012 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-22847950

RESUMEN

We describe 19 unrelated individuals with submicroscopic deletions involving 10p15.3 characterized by chromosomal microarray (CMA). Interestingly, to our knowledge, only two individuals with isolated, submicroscopic 10p15.3 deletion have been reported to date; however, only limited clinical information is available for these probands and the deleted region has not been molecularly mapped. Comprehensive clinical history was obtained for 12 of the 19 individuals described in this study. Common features among these 12 individuals include: cognitive/behavioral/developmental differences (11/11), speech delay/language disorder (10/10), motor delay (10/10), craniofacial dysmorphism (9/12), hypotonia (7/11), brain anomalies (4/6) and seizures (3/7). Parental studies were performed for nine of the 19 individuals; the 10p15.3 deletion was de novo in seven of the probands, not maternally inherited in one proband and inherited from an apparently affected mother in one proband. Molecular mapping of the 19 individuals reported in this study has identified two genes, ZMYND11 (OMIM 608668) and DIP2C (OMIM 611380; UCSC Genome Browser), mapping within 10p15.3 which are most commonly deleted. Although no single gene has been identified which is deleted in all 19 individuals studied, the deleted region in all but one individual includes ZMYND11 and the deleted region in all but one other individual includes DIP2C. There is not a clearly identifiable phenotypic difference between these two individuals and the size of the deleted region does not generally predict clinical features. Little is currently known about these genes complicating a direct genotype/phenotype correlation at this time. These data however, suggest that ZMYND11 and/or DIP2C haploinsufficiency contributes to the clinical features associated with 10p15 deletions in probands described in this study.


Asunto(s)
Deleción Cromosómica , Cromosomas Humanos Par 10 , Telómero , Niño , Femenino , Humanos , Lactante , Recién Nacido , Masculino
5.
Behav Sleep Med ; 9(3): 184-93, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21722013

RESUMEN

Actigraphy provides a non-invasive objective means to assess sleep-wake cycles. In young children, parent logs can also be useful for obtaining sleep-wake information. The authors hypothesized that actigraphy and parent logs were both equally valid instruments in healthy preschool-aged children. The authors studied 59 children aged 3 to 5 years in full-time day care. Each child was screened for medical problems and developmental delays before being fitted with an actigraphy watch, which was worn for 1 week. Parents maintained logs of sleep and wakefulness during the same period, with input from day care workers. In general, parents overestimated the amount of nighttime sleep measured by actigraphy by 13% to 22% (all significant). Although there was no difference in sleep onset times, parents reported later rise times on the weekend and fewer nighttime awakenings. There was no significant difference between parent logs and actigraphy with regard to daytime napping. The authors conclude that parent logs are best utilized in assessing daytime sleep and sleep onset, whereas actigraphy should be used to assess nighttime sleep and sleep offset time.


Asunto(s)
Actigrafía/métodos , Registros Médicos/estadística & datos numéricos , Adulto , Preescolar , Femenino , Humanos , Masculino , Padres , Sueño , Vigilia
6.
J Sleep Res ; 19(4): 559-66, 2010 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-20545836

RESUMEN

Obesity continues to be a major public health issue. In adolescents, there are limited studies on the relationship between obesity and sleep duration. We found hypothesized that an average sleep duration of <6 h in adolescents was associated with obesity. Data were from the National Longitudinal Study of Adolescent Health (ADD Health); a survey of 90,000 youths, aged 12-18 years; surveyed in several waves. The sample population for our study was 13,568. Weighted multiple logistic regression was used to identify the relationship between obesity at Wave II and sleep duration, having adjusted for skipping breakfast ≥ 2/week; race, gender, parental income, TV ≥ 2 h per day, depression, and obesity at Wave I. At Wave I, the mean age was 15.96 ± 0.11 years; mean sleep hours were 7.91 ± 0.04. At Waves I and II, respectively, 10.6 and 11.2% of adolescents were obese. Adjusted analyses suggest that the effect of shortened sleep duration in Wave I was not significantly predictive of obesity in Wave II (P < 0.218). Longitudinally, depression and TV ≥ 2 h per day at Wave I was associated with a higher risk of obesity at Wave II in adjusted analyses. Depressed adolescents were almost twice as likely to be obese (OR = 1.84, 95% CI = 1.25-2.72); adolescents who watched TV ≥ 2 h per day were 37% more likely to be obese (OR = 1.37, 95% CI = 1.09-1.72). Environmental factors including TV ≥ 2 h per day and depression were significantly associated with obesity; shortened sleep duration was not. Future longitudinal studies in adolescents are needed to determine whether timing of television watching directly influences sleep patterns and, ultimately, obesity.


Asunto(s)
Obesidad/etiología , Privación de Sueño/complicaciones , Adolescente , Factores de Edad , Conducta Alimentaria/fisiología , Femenino , Humanos , Modelos Logísticos , Estudios Longitudinales , Masculino , Actividad Motora/fisiología , Estado Nutricional/fisiología , Obesidad/fisiopatología , Grupos Raciales , Factores Sexuales , Sueño/fisiología , Privación de Sueño/fisiopatología , Estados Unidos
7.
J Atten Disord ; 23(4): 333-340, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-25646024

RESUMEN

OBJECTIVE: The aim of this study is to understand the impact of a 5-day period of nap restriction on sleep patterns and cognitive function in typically developing preschoolers, aged 3 to 4 years. METHOD: Following 1 week of baseline assessment, 28 children were randomly assigned to either a "napping as usual" group ( n = 15) or a 5-day period of nap restriction ( n = 13). Sleep was assessed with sleep logs and actigraphy; cognition was assessed at baseline and at the end of the intervention week. RESULTS: No group differences in sleep or cognitive function were observed at baseline. For the no-nap group, the 5-day period of daytime nap restriction resulted in increased nighttime sleep. Children in the no-nap group also showed a significant improvement in attentional control compared with baseline, whereas no such changes were observed among children in the napping-as-usual group. CONCLUSION: In preschool children who typically take naps, short-term nap restriction is associated with increased nighttime sleep and may contribute to improved attentional function.


Asunto(s)
Trastorno por Déficit de Atención con Hiperactividad/fisiopatología , Atención/fisiología , Sueño/fisiología , Actigrafía , Análisis de Varianza , Preescolar , Ritmo Circadiano/fisiología , Cognición/fisiología , Femenino , Humanos , Masculino , Proyectos Piloto , Privación de Sueño/fisiopatología
8.
Pediatr Neurol ; 39(3): 162-9, 2008 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-18725060

RESUMEN

Rapid eye movement sleep distribution changes during development, but little is known about rapid eye movement latency variation in childhood by age, sex, or pathologic sleep states. We hypothesized that: (1) rapid eye movement latency would differ in normal children by age, with a younger cohort (1-10 years) demonstrating shorter rapid eye movement latency than an older group (>10-18 years); (2) rapid eye movement latency in children would differ from typical adult rapid eye movement latency; and (3) intrinsic sleep disorders (narcolepsy, pediatric obstructive sleep apnea syndrome) would disrupt normal developmental patterns of rapid eye movement latency. A retrospective chart review included data from clinic visits and of rapid eye movement latency and other parameters measured by overnight polysomnography. Participants included 98 control children, 90 children with obstructive sleep apnea syndrome, and 13 children with narcolepsy. There were no statistically significant main effects of age category or sex on rapid eye movement latency. Rapid eye movement latency, however, exhibited a significant inverse correlation with age within the older control children. Healthy children exhibited rapid eye movement latencies significantly longer than adults. Normal control patients demonstrated significantly longer rapid eye movement latency than obstructive sleep apnea syndrome and narcolepsy patients.


Asunto(s)
Polisomnografía/métodos , Apnea Obstructiva del Sueño/fisiopatología , Sueño REM/fisiología , Sueño/fisiología , Adolescente , Factores de Edad , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Masculino , Narcolepsia/fisiopatología , Factores Sexuales , Apnea Obstructiva del Sueño/patología , Fases del Sueño/fisiología
9.
Behav Sleep Med ; 6(3): 147-57, 2008.
Artículo en Inglés | MEDLINE | ID: mdl-18629686

RESUMEN

Sleep-related dissociative disorders are parasomnias that can emerge at any point during the sleep period and are associated with traumatic life experiences. No reports to date have described this parasomnia in a school-age child. This case report discusses presentation, diagnosis, and treatment of a 6-year-old girl with this diagnosis. Sleep-related dissociative disorder should be considered in young children who have experienced traumatic life events and who present with abnormal sleep patterns and bizarre behavior. Early identification and intensive psychotherapy can be efficacious for these children.


Asunto(s)
Trastornos Disociativos/complicaciones , Trastorno de la Conducta del Sueño REM/complicaciones , Niño , Manual Diagnóstico y Estadístico de los Trastornos Mentales , Trastornos Disociativos/diagnóstico , Electroencefalografía , Femenino , Humanos , Polisomnografía , Trastorno de la Conducta del Sueño REM/diagnóstico , Fases del Sueño , Tomografía Computarizada por Rayos X
10.
Sleep ; 30(2): 141-51, 2007 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-17326539

RESUMEN

Parasomnias in childhood are common, and often more frequent than in adults. The large number of parasomnias underscore that sleep is not simply a quiescent state, but can involve complex episodes of movement, ranging from subtle to dramatic and complex. Clinicians should be aware that many pediatric parasomnias are benign, self-limited, and may not persist into late childhood or adolescence. Importantly, parasomnias in childhood often differ in type from adults. Nevertheless, parasomnias across ages can be classified as: 1) disorders of arousal (from non-rapid eye movement, or NREM, sleep); 2) parasomnias usually associated with REM sleep; and 3) other parasomnias. We detail here issues in the clinical diagosis, evaluation, and management of multiple pediatric parasomnias. The further study of parasomnias in children may help elucidate the multi-factorial etiologies of these fascinating conditions, shedding light on the potential genetic bases as well as environmental contributions.


Asunto(s)
Parasomnias/fisiopatología , Niño , Electromiografía , Humanos , Parasomnias/diagnóstico , Parasomnias/terapia , Polisomnografía , Recurrencia , Factores de Riesgo , Fases del Sueño/fisiología
11.
Nurs Clin North Am ; 37(4): 693-706, 2002 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-12587368

RESUMEN

Sleep disorders are common in childhood, and may affect multiple aspects of a child's life and the lives of other family members. A sleep disorder assessment should begin with detailed sleep history and a review of interrelated health issues. Factors contributing to disturbed sleep may be discovered or confirmed by a thorough physical examination. Thereafter, appropriate ancillary testing can provide support for a specific clinical diagnosis. The spectrum of childhood sleep disorders includes OSA, narcolepsy, RLS/PLMD, sleep onset association disorder, and parasomnias. Diagnosing sleep disorders in children remains a challenge; however, a multidisciplinary approach may provide an opportunity for productive collaboration and, thereby, more effective patient management. Centers treating pediatric sleep disorders may include providers from a variety of disciplines in pediatric healthcare, such as child psychology, pulmonology, neurology, psychiatry, nursing, and otolaryngology. Over the last decade, research in pediatric sleep disorders has expanded greatly, paralleled by an increased awareness of the importance of adequate, restorative sleep in childhood.


Asunto(s)
Trastornos del Sueño-Vigilia , Edad de Inicio , Niño , Preescolar , Humanos , Narcolepsia/diagnóstico , Narcolepsia/terapia , Parasomnias/diagnóstico , Parasomnias/terapia , Apnea Obstructiva del Sueño/diagnóstico , Apnea Obstructiva del Sueño/terapia , Trastornos del Sueño-Vigilia/diagnóstico , Trastornos del Sueño-Vigilia/terapia
12.
Sleep ; 37(8): 1349-52, 2014 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-25083015

RESUMEN

STUDY OBJECTIVES: Although the American Academy of Sleep Medicine (AASM) mandates that periodic limb movements during sleep (PLMS) be scored on every polysomnogram, and considers a periodic limb movement index (PLMI) > 5/h abnormal in children, there is a lack of community-derived data regarding the prevalence of PLMS in children, and no data to support this cutoff value. Therefore, the aim of this study was to determine the prevalence of PLMS in a sample of normal children. DESIGN: Retrospective study. PARTICIPANTS: 195 healthy, non-snoring children aged 5-17 years, recruited from the community, who underwent polysomnography for research purposes. METHODS: PLMS were scored using the AASM 2007 criteria. MEASUREMENTS AND RESULTS: The group age (median [IQR]) was 12.9 [10-15] years, and 58% were male. Sleep architecture was normal, and the obstructive apnea hypopnea index was 0.1 [0-0.3]/h. The median PLMI was 0/h, ranging from 0 to 35.5/h. Fifteen (7.7%) subjects had a PLMI > 5/h, and only 3 (1.5%) met the adult pathologic criterion of more than 15/h. Use of the 95th percentile PLMI cutoff of 7.2/h produced little difference in categorization between groups. Children with a PLMI > 5/h had a higher arousal index than those with a lower PLMI (11.6 [8.8-14.6] vs 8.1 [6.1-9.9]/h, respectively, P = 0.003). CONCLUSIONS: This study provides normative data to the field and supports the clinical periodic limb movement index cutoff of > 5/h based on both prevalence and the correlate of increased sleep fragmentation. Periodic limb movements during sleep are infrequent in normal children recruited from the community. CITATION: Marcus CL, Traylor J, Gallagher PR, Brooks LJ, Huang J, Koren D, Katz L, Mason TB, Tapia IE. Prevalence of periodic limb movements during sleep in normal children.


Asunto(s)
Extremidades/fisiología , Movimiento , Sueño/fisiología , Adolescente , Niño , Femenino , Voluntarios Sanos , Humanos , Masculino , Síndrome de Mioclonía Nocturna/epidemiología , Síndrome de Mioclonía Nocturna/fisiopatología , Polisomnografía , Prevalencia , Valores de Referencia , Estudios Retrospectivos , Privación de Sueño/fisiopatología
13.
Sleep Med ; 13(8): 1013-20, 2012 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-22841030

RESUMEN

OBJECTIVES: To test agreement and define differences in periodic limb movements in sleep (PLMS) measured by polysomnography and an ankle activity monitor, and to describe PLMS variability across nights, feasibility of home monitoring, and correlates of PLMS in children with sickle cell disease (SCD). METHODS: Twenty children with SCD and restless legs syndrome (RLS) symptoms or polysomnography-documented PLMS underwent concurrent attended polysomnography and ankle activity monitoring over one to two nights and home activity monitoring for three nights. Serum iron and ferritin were measured pre- and post-polysomnography. RESULTS: Adequate sensitivity (1.00), specificity (0.69), and mean bias (5.0±7.4 PLMS/h) for identifying elevated PLMS by activity monitor were obtained when scoring the period from sleep onset to offset rather than time in bed per manufacturer recommendation, and using a cut-point of 10 PLMS/h. Compared to activity monitor, only polysomnographic PLMS demonstrated periodicity, at inter-movement intervals (IMI) 20-35 s; the activity monitor overscored PLMS at the beginning and end of sleep and at shorter IMI (5-15s; p≤0.003), suggesting misclassification of nonperiodic leg movements as PLMS by activity monitor. PLMS varied across four nights by 16.1±13.4 PLMS/h. Post-polysomnography ferritin was associated (positively) with PLMS (p=0.034); RLS symptoms were not. CONCLUSIONS: Ankle activity monitoring is a valid screening measure for PLMS in children with SCD and can readily be performed at home. Interpretation should incorporate a threshold for elevated PLMS of 10/h and scoring from sleep onset to offset, which could be identified with concurrent wrist actigraphy, to better account for true PLMS.


Asunto(s)
Actigrafía/normas , Anemia de Células Falciformes/complicaciones , Monitoreo Fisiológico/normas , Polisomnografía/normas , Síndrome de las Piernas Inquietas , Actigrafía/métodos , Adolescente , Articulación del Tobillo/fisiología , Niño , Preescolar , Estudios de Factibilidad , Femenino , Ferritinas/sangre , Humanos , Hierro/sangre , Masculino , Monitoreo Fisiológico/métodos , Estudios Prospectivos , Reproducibilidad de los Resultados , Síndrome de las Piernas Inquietas/complicaciones , Síndrome de las Piernas Inquietas/diagnóstico , Síndrome de las Piernas Inquietas/fisiopatología , Sensibilidad y Especificidad
14.
Pediatr Pulmonol ; 47(8): 808-15, 2012 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-22328189

RESUMEN

Assessment of oxyhemoglobin saturation in patients with sickle cell disease (SCD) is vital for prompt recognition of hypoxemia. The accuracy of pulse oximeter measurements of blood oxygenation in SCD patients is variable, partially due to carboxyhemoglobin (COHb) and methemoglobin (MetHb), which decrease the oxygen content of blood. This study evaluated the accuracy and reliability of a non-invasive pulse co-oximeter in measuring COHb and MetHb percentages (SpCO and SpMet) in children with SCD. We hypothesized that measurements of COHb and MetHb by non-invasive pulse co-oximetry agree within acceptable clinical accuracy with those made by invasive whole blood co-oximetry. Fifty children with SCD-SS underwent pulse co-oximetry and blood co-oximetry while breathing room air. Non-invasive COHb and MetHb readings were compared to the corresponding blood measurements. The pulse co-oximeter bias was 0.1% for COHb and -0.22% for MetHb. The precision of the measured SpCO was ± 2.1% within a COHb range of 0.4-6.1%, and the precision of the measured SpMet was ± 0.33% within a MetHb range of 0.1-1.1%. Non-invasive pulse co-oximetry was useful in measuring COHb and MetHb levels in children with SCD. Although the non-invasive technique slightly overestimated the invasive COHb measurements and slightly underestimated the invasive MetHb measurements, there was close agreement between the two methods.


Asunto(s)
Anemia de Células Falciformes/sangre , Carboxihemoglobina/análisis , Metahemoglobina/análisis , Oximetría/métodos , Adolescente , Análisis de los Gases de la Sangre , Niño , Preescolar , Femenino , Humanos , Masculino , Reproducibilidad de los Resultados , Espectrofotometría
15.
Chest ; 142(1): 94-100, 2012 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-22241762

RESUMEN

BACKGROUND: The prevalence of obstructive sleep apnea syndrome (OSAS) is higher in children with sickle cell disease (SCD) as compared with the general pediatric population. It has been speculated that overgrowth of the adenoid and tonsils is an important contributor. METHODS: The current study used MRI to evaluate such an association. We studied 36 subjects with SCD (aged 6.9 ± 4.3 years) and 36 control subjects (aged 6.6 ± 3.4 years). RESULTS: Compared with control subjects, children with SCD had a significantly smaller upper airway (2.8 ± 1.2 cm(3) vs 3.7 ± 1.6 cm(3), P < .01), and significantly larger adenoid (8.4 ± 4.1 cm(3) vs 6.0 ± 2.2 cm(3), P < .01), tonsils (7.0 ± 4.3 cm(3) vs 5.1 ± 1.9 cm(3), P < .01), retropharyngeal nodes (3.0 ± 1.9 cm(3) vs 2.2 ± 0.9 cm(3), P < .05), and deep cervical nodes (15.7 ± 5.7 cm(3) vs 12.7 ± 4.0 cm(3), P < .05). Polysomnography showed that 19.4% (seven of 36) of children with SCD had OSAS compared with 0% (zero of 20) of control subjects (P < .05) and that in children with SCD the apnea-hypopnea index correlated positively with upper airway lymphoid tissues size (r = 0.57, P < 001). In addition, children with SCD had lower arterial oxygen saturation nadir (84.3% ± 12.3% vs 91.2% ± 4.2%, P < .05), increased peak end-tidal CO(2) (53.4 ± 8.5 mm Hg vs 42.3 ± 5.3 mm Hg, P < .001), and increased arousals (13.7 ± 4.7 events/h vs 10.8 ± 3.8 events/h, P < .05). CONCLUSIONS: Children with SCD have reduced upper airway size due to overgrowth of the surrounding lymphoid tissues, which may explain their predisposition to OSAS.


Asunto(s)
Tonsila Faríngea/patología , Anemia de Células Falciformes/patología , Tejido Linfoide/patología , Tonsila Palatina/patología , Adolescente , Anemia de Células Falciformes/complicaciones , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Polisomnografía , Prevalencia , Apnea Obstructiva del Sueño/diagnóstico , Apnea Obstructiva del Sueño/epidemiología
16.
Sleep ; 34(7): 899-908, 2011 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-21731140

RESUMEN

STUDY OBJECTIVES: To describe the rate, distribution and correlates of periodic limb movements in sleep (PLMS) in children with sickle cell disease (SCD). DESIGN: Prospective, cross-sectional. SETTING: Hospital-based sleep laboratory. PARTICIPANTS: Sixty-four children aged 2-18 years with SCD, hemoglobin SS-type who had an overnight polysomnogram and a parent-completed Pediatric Sleep Questionnaire. Mean age was 8.4 years (SD 4.8); 50% were male. INTERVENTIONS: N/A. MEASUREMENTS AND RESULTS: The mean PLMS index was 3.7 (6.6) and ranged from 0 to 31.8, with 23.4% of the sample having PLMS ≥ 5/h. Sleep efficiency was decreased (P = 0.03), and the total arousal index (P = 0.003) and PLMS arousal index (P < 0.001) were increased in children with PLMS ≥ 5/h compared to those with PLMS < 5/h. PLMS were most frequent in NREM stage 2 sleep and during the fourth hour of sleep. Inter-movement interval duration peaked at 25-30 s. "Growing pains worst in bed" or "restlessness of the legs", suggesting restless legs syndrome (RLS), were reported in 12.5% of the total sample and were more common in children with elevated PLMS. A PLMS score for identifying elevated PLMS in children, based on items from the Pediatric Sleep Questionnaire, did not significantly predict PLMS ≥ 5/h. CONCLUSIONS: Elevated PLMS are common in children with SCD and are associated with sleep disruption and symptoms of RLS. Future research into the time structure of PLMS, their causes and consequences, and development of a disease-specific sleep disorders screening questionnaire, is needed in children with SCD.


Asunto(s)
Anemia de Células Falciformes/complicaciones , Síndrome de las Piernas Inquietas/etiología , Fases del Sueño/fisiología , Adolescente , Anemia de Células Falciformes/fisiopatología , Niño , Preescolar , Estudios Transversales , Extremidades/fisiopatología , Femenino , Humanos , Lactante , Masculino , Movimiento , Polisomnografía , Estudios Prospectivos , Síndrome de las Piernas Inquietas/epidemiología
17.
J Clin Sleep Med ; 7(1): 81-7, 2011 Feb 15.
Artículo en Inglés | MEDLINE | ID: mdl-21344042

RESUMEN

STUDY OBJECTIVES: The gold-standard test used to diagnose childhood obstructive sleep apnea is polysomnography. However, this test requires an overnight stay at a sleep laboratory and the attachment of multiple sensors to the patient. The long-term impact of this testing on the child and family are not known. We hypothesized that polysomnography does not precipitate acute or chronic psychological effects in children. METHODS: A consecutive cohort of children who had undergone sleep studies 2 to 4 months prior to the interview were administered a standardized questionnaire via telephone. RESULTS: Of the 118 families that were eligible to participate, 67% could be contacted and agreed to participate; 87% of respondents reported the experience to have been satisfactory (mean Likert score of 8.6 ± 2.0 [SD] on a scale of 1-10). Similar levels of satisfaction were reported by parents of children with developmental delay or those who were younger than 3 years. The night's sleep was considered typical in 68% of cases. Sleep was less likely to be typical in children younger than 3 years (47%, p = 0.043). Eight percent of children experienced pain during the study. By caregiver report, of those children who remembered the sleep study, memories were positive in 84%. No child had evidence of serious long-term psychological issues. CONCLUSIONS: The vast majority of children and families found the polysomnography experience to be satisfactory, with no psychological sequelae. However, many children, especially those younger than 3 years, demonstrated sleep patterns different from their usual sleep. The clinical relevance of this finding merits further study. Further research evaluating the generalizability of this study is also needed.


Asunto(s)
Monitoreo Fisiológico/métodos , Polisomnografía/métodos , Relaciones Profesional-Familia , Síndromes de la Apnea del Sueño/diagnóstico , Adolescente , Niño , Preescolar , Femenino , Hospitalización , Humanos , Lactante , Entrevistas como Asunto , Modelos Lineales , Masculino , Relaciones Padres-Hijo , Satisfacción del Paciente , Pediatría , Síndromes de la Apnea del Sueño/psicología , Estadísticas no Paramétricas , Encuestas y Cuestionarios , Adulto Joven
18.
Sleep Med ; 12(9): 892-7, 2011 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-21940205

RESUMEN

OBJECTIVE: To analyze sleep in children with Williams Syndrome (WS) compared to normal healthy controls in order to determine whether particular sleep features are characteristic of WS, and to explore associations between disturbed sleep and behavior. METHODS: Thirty-five children with genetically-confirmed WS and 35 matched controls underwent overnight polysomnography and performance testing in the Sleep Center at the Children's Hospital of Philadelphia. Parents completed questionnaires regarding the subjects' sleep and behavior. RESULTS: WS subjects had significantly different sleep than controls, with decreased sleep efficiency, increased respiratory-related arousals and increased slow wave sleep on overnight polysomnography. WS subjects were also noted to have more difficulty falling asleep, with greater restlessness and more arousals from sleep than controls. Fifty-two percent of WS subjects had features of attention deficit-hyperactivity disorder. CONCLUSION: Children with WS had significantly different sleep than controls in our sample. These differences demonstrated in our study may reflect genetic influences on sleep.


Asunto(s)
Trastornos del Sueño-Vigilia/genética , Trastornos del Sueño-Vigilia/fisiopatología , Sueño REM/fisiología , Síndrome de Williams/genética , Síndrome de Williams/fisiopatología , Adolescente , Trastorno por Déficit de Atención con Hiperactividad/genética , Trastorno por Déficit de Atención con Hiperactividad/fisiopatología , Niño , Conducta Infantil , Preescolar , Femenino , Humanos , Masculino , Padres/psicología , Polisomnografía , Trastornos del Sueño-Vigilia/diagnóstico , Encuestas y Cuestionarios
19.
Sleep ; 34(6): 773-8, 2011 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-21629365

RESUMEN

STUDY OBJECTIVES: The prevalence of obstructive sleep apnea syndrome (OSAS) in sickle cell disease (SCD) has been reported to be higher than that in the general pediatric population. However, not all subjects with SCD develop OSAS. We hypothesized that SCD patients with OSAS have a blunted neuromuscular response to subatmospheric pressure loads during sleep, making them more likely to develop upper airway collapse. DESIGN: Subjects with SCD with and without OSAS underwent pressure-flow measurements during sleep using intraoral surface electrodes to measure genioglossal EMG (EMGgg). Two techniques were applied to decrease the nasal pressure (P(N)) to subatmospheric levels, resulting in an activated and relatively hypotonic upper airway. The area under the curve of the inspiratory EMGgg moving time average was analyzed. EMGgg activity was expressed as a percentage of baseline. Changes in EMGgg in response to decrements in nasal pressure were expressed as the slope of the EMGgg vs. nasal pressure (slope of EMGgg-P(N)). SETTING: Sleep laboratory. PARTICIPANTS: 4 children with SCD and OSAS and 18 children with SCD but without OSAS. RESULTS: THE MAJOR FINDINGS OF THIS STUDY WERE: (1) using the activated but not the hypotonic technique, the slope of EMGgg-P(N) was more negative in SCD controls than SCD OSAS; (2) the slope of EMGgg-P(N) was significantly lower using the activated technique compared to the hypotonic technique in SCD controls only; (3) similarly, the critical closing pressure, Pcrit, was more negative using the activated technique than the hypotonic technique in SCD controls but not in SCD OSAS. CONCLUSION: This preliminary study has shown that children with SCD but without OSAS have more prominent upper airway reflexes than children with SCD and OSAS.


Asunto(s)
Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/fisiopatología , Apnea Obstructiva del Sueño/epidemiología , Adolescente , Estudios de Casos y Controles , Niño , Electromiografía , Femenino , Humanos , Masculino , Polisomnografía , Mecánica Respiratoria/fisiología , Músculos Respiratorios/fisiopatología , Sistema Respiratorio/fisiopatología , Apnea Obstructiva del Sueño/diagnóstico , Apnea Obstructiva del Sueño/fisiopatología , Lengua/fisiopatología
20.
Pediatrics ; 123(6): e1005-10, 2009 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-19482732

RESUMEN

OBJECTIVE: Adolescents may not receive the sleep they need. New media technology and new, popular energy drinks may be implicated in sleep deficits. In this pilot study we quantified nighttime technology use and caffeine consumption to determine effects on sleep duration and daytime behaviors in adolescents. We hypothesized that with increased technology use, adolescents increase caffeine consumption, resulting in insufficient sleep duration. PATIENTS AND METHODS: Subjects were recruited from a pediatric office in a proximal suburb of Philadelphia, Pennsylvania. Inclusion criteria for this study were middle and high school subjects aged 12 to 18 years old. The questionnaire, Adolescent Sleep, Caffeine Intake, and Technology Use, was developed by the investigators to measure adolescents' intake of caffeinated drinks, use of nighttime media-related technology, and sleep behaviors. Descriptive statistics characterized the subjects, their caffeine and technology use, and sleep variables. Regression models assessed the relationships between caffeine, technology use, and sleep variables, having adjusted for age, race, gender, and BMI. RESULTS: Sleep was significantly related to the multitasking index. Teenagers getting 8 to 10 hours of sleep on school nights tended to have 1.5- to 2-fold lower multitasking indices compared with those getting less sleep. Thirty-three percent of the teenagers reported falling asleep during school. Caffeine consumption tended to be 76% higher by those who fell asleep. The log-transformed multitasking index was significantly related to falling asleep during school and with difficulties falling asleep on weeknights. CONCLUSIONS: Many adolescents used multiple forms of technology late into the night and concurrently consumed caffeinated beverages. Subsequently, their ability to stay alert and fully functional throughout the day was impaired by excessive daytime sleepiness. Future studies should measure more than television hours when evaluating the impact of nighttime activities on sleep patterns in adolescents.


Asunto(s)
Atención , Cafeína/administración & dosificación , Ritmo Circadiano , Estilo de Vida , Medios de Comunicación de Masas/estadística & datos numéricos , Microcomputadores/estadística & datos numéricos , Psicología del Adolescente , Privación de Sueño/epidemiología , Privación de Sueño/psicología , Vigilia , Adolescente , Niño , Femenino , Encuestas Epidemiológicas , Humanos , Masculino , Philadelphia , Proyectos Piloto , Revisión de Utilización de Recursos/estadística & datos numéricos
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