Sleep related breathing disorders in the spina bifida population ages 1-20 years: A retrospective study in Arkansas.

PURPOSE: Sleep related breathing disorders (SRBD) are seen at disproportionately higher rates in children with spina bifida compared with their same aged peers. SRBD such as obstructive sleep apnea (OSA) or central apnea are associated with developmental and cognitive consequences, and sudden death. METHODS: Participants aged 1 to 20 years with a diagnosis of spina bifida were recruited from a pediatric academic hospital spinal cord specialty clinic to evaluate the prevalence and impact of sleep disorders in the spina bifida population; 76 polysomnogram (PSG) reports spanning a 3-year period were reviewed in this retrospective cohort study. RESULTS: Of the PSGs reviewed, 37 (49%) indicated the presence of SRBD, and 28 (76%) of those children required an escalation of management (surgical intervention or additional respiratory support). These results are consistent with previous studies and further emphasize the clinical impact of SRBD on children with spina bifida by describing the interventions that followed an abnormal PSG. CONCLUSION: The high prevalence of SRBD in the spina bifida population supports the need for additional research to develop sleep questionnaires specific to spina bifida that can predict abnormal PSG clinically and to determine the standard of care following an abnormal PSG, chiefly in OSA and central apnea.

Increased incidence of pediatric narcolepsy following the 2009 H1N1 pandemic: a report from the pediatric working group of the sleep research network.

This study was aimed to evaluate the yearly incidence of pediatric narcolepsy prior to and following the 2009 H1N1 pandemic and to evaluate seasonal patterns of narcolepsy onset and associations with H1N1 influenza infection in the United States. This was a multicenter retrospective study with prospective follow-up. Participants were recruited from members of the Pediatric Working Group of the Sleep Research Network including 22 sites across the United States. The main outcomes were monthly and yearly incident cases of childhood narcolepsy in the United States, and its relationship to historical H1N1 influenza data. A total of 950 participants were included in the analysis; 487 participants were male (51.3%). The mean age at onset of excessive daytime sleepiness (EDS) was 9.6  ±â€… 3.9 years. Significant trend changes in pediatric narcolepsy incidence based on EDS onset (p  <  .0001) occurred over the 1998-2016 period, peaking in 2010, reflecting a 1.6-fold increase in narcolepsy incidence. In addition, there was significant seasonal variation in narcolepsy incident cases, with increased cases in spring (p  <  .05). Cross-correlation analysis demonstrated a significant correlation between monthly H1N1 infection and monthly narcolepsy incident cases (p  =  .397, p  <  .0001) with a lag time of 8 months. We conclude that there is a significant increase in pediatric narcolepsy incidence after the 2009 H1N1 pandemic in the United States. However, the magnitude of increase is lower than reported in European countries and in China. The temporal correlation between monthly H1N1 infection and monthly narcolepsy incidence, suggests that H1N1 infection may be a contributing factor to the increased pediatric narcolepsy incidence after the 2009 H1N1 pandemics.

Management of Sleep Apnea in Children with Chiari I Malformation: A Retrospective Study.

INTRODUCTION: The literature indicates that decompression of Chiari I malformations (CM-1) may resolve symptoms of sleep apnea. This study aims to identify the incidence of obstructive sleep apnea (OSA), central sleep apnea (CSA), and mixed sleep apnea in a cohort of pediatric CM-1 patients treated at our institution. We also assessed apnea-hypopnea index and symptomatology before and after surgery to investigate if Chiari decompression is a viable treatment for sleep apnea in CM-1 patients. Improvement relative to ENT surgical intervention was also considered. METHODS: We identified 75 patients who underwent polysomnography (PSG) from our database of 465 CM-1 patients. Sleep apnea diagnosis was based on the sleep physician's overall interpretation of the PSG. Symptomatology pre- and post-surgery was analyzed. RESULTS: Of the 75 CM-1 patients that underwent PSG, 23 were diagnosed with sleep apnea. Sixteen had OSA, 6 had CSA, and 1 had mixed apnea. Twelve OSA patients received ENT intervention. Eight improved and 2 further improved after Chiari decompression. Of the 4 patients that did not improve, one of those later improved following Chiari decompression. Of the 6 CSA patients, 2 underwent Chiari decompression, but only one improved. The mixed apnea patient underwent several ENT interventions that did not relieve symptoms but improved following Chiari decompression. DISCUSSION/CONCLUSIONS: Based on our results, sleep apnea in CM-1 patients may be obstructive, central, or mixed and is likely multifactorial. A multidisciplinary approach to the management of these patients is important, including neurosurgery, otolaryngology, and sleep medicine. Future prospective studies will lend further insight into this condition and its management.

Outcomes of Pediatric Titration Sleep Studies Following Empirical Use of Positive Airway Pressure and the Effect on Adherence to Therapy.

BACKGROUND: Obstructive sleep apnea (OSA) is diagnosed through polysomnography (PSG) testing and commonly treated with positive airway pressure (PAP). The initial recommended treatment for pediatric OSA is adenotonsillectomy, but when this is contraindicated or ineffective, PAP is the next option. Children followed in our pediatric sleep disorders center who are diagnosed with OSA and meet criteria for therapy are empirically prescribed a PAP device, usually auto-titrating PAP (APAP), to avoid delays in therapy. Titration PSG is performed later to assess adequacy of settings. The aims of this study were to determine how often PSG titration results in changes to empirically prescribed PAP and to assess adherence to therapy before and after PSG titration. METHODS: A retrospective medical records review was completed for children diagnosed with OSA, prescribed PAP, and had a titration PSG within a 5-y consecutive period of 2008-2012. Demographic data, type of device, pressure settings, and adherence downloads were reviewed. Adherence was assessed before and after titration overall and compared for those who did and did not have therapy changes following titration. RESULTS: The study included 121 participants. Median age at the time of the diagnostic PSG was 11 (interquartile range [IQR] 8-14) y. Most (106, 88%) were initially prescribed APAP. Median length of time between initial and follow-up PSG was 6.4 (IQR 4.4-10.1) months. The majority (94, 78%) had therapy changes following titration. Overall, adherence percentage > 4 h per night was not significantly increased post titration (P = .47). There were no statistically significant differences in adherence between those who had therapy changes and those who did not (P = .26). CONCLUSIONS: Titration studies resulted in therapy modifications for most children. Adherence was not increased following the titration PSG. Changes in therapy did not result in increased adherence. Titration PSGs may optimize empirically prescribed settings.

Catathrenia and Treatment With Positive Airway Pressure in the Pediatric Population.

None: Catathrenia, also known as sleep-related groaning, is a relatively rare sleep disorder with characteristics consistent with loud groaning on expiration during sleep. Organic causes of catathrenia are unknown and the decision of whether or not to treat is unclear. Limited research is available concerning appropriate treatment and none of the literature focuses primarily on pediatrics. We report a series of three, male pediatric cases with catathrenia that were treated with continuous positive airway pressure (CPAP) while identifying and comparing clinical symptoms, polysomnogram findings, treatment, and patient response to treatment. Catathrenia may be associated with abnormal nocturnal oxygenation and ventilation and may lead to negative clinical daytime symptoms which may warrant treatment. If catathrenia leads to sleep disruption and negative daytime symptoms, treatment with CPAP should be considered.

Impact of Degree of Obesity on Sleep, Quality of Life, and Depression in Youth.

OBJECTIVE: Obese youth are more likely to report difficulties with sleep, depression, and quality of life (QOL). This study aims to characterize sleep problems, QOL, and symptoms of depression by degree of obesity. METHOD: The cross-sectional study was conducted in a specialized obesity clinic. Obese youth and their caregivers (N = 150) were evaluated with the Child Sleep Habits Questionnaire and Pediatric Quality of Life. Youth completed the Children's Depression Inventory. Regression models and correlations were calculated. RESULTS: Degree of obesity was predictive of increased sleep difficulties and decreased QOL scores. Children's Depression Inventory scores showed that children with more symptoms of depression had more sleep problems, and these were not associated with the degree of obesity. Adolescents with more difficulties sleeping also reported more symptoms of depression and lower QOL. CONCLUSIONS: Degree of obesity negatively affected QOL and sleep variables. Patients with greater sleeping difficulties reported more symptoms of depression.

Characteristics and surgical and clinical outcomes of severely obese children with obstructive sleep apnea.

STUDY OBJECTIVES: To describe characteristics and surgical and clinical outcomes of obese children with obstructive sleep apnea (OSA). METHODS: At our institution from 2000 to 2010, 143 obese children with an overnight polysomnography (OPSG) diagnosis of OSA, excluding children with comorbidities, were identified. Relationships between demographics, clinical findings, and the severity of OSA were assessed. Presurgery and postsurgery OPSG indices were compared. We defined cure as an apneahypopnea index (AHI) < 1.5/h on the postsurgery OPSG, and we compared the cure rates of different surgeries. RESULTS: A total of 143 children, median age 12.4 y (interquartile range [IQR] 9.6-14.9) and BMI z-scores 2.8 (IQR 2.6-2.9), were included. Seventy-eight (55%) (Median age 12 y [IQR 9-15]) underwent surgery: 1 had tonsillectomy; 1 tonsillectomy + uvulopharyngopalatoplasty (UPPP); 23 adenotonsillectomy (AT); 27 AT + UPPP; 11 adenoidectomy + UPPP; 8 UPPP; and 7 AT + turbinate trim ± tongue base suspension. Overall, surgery cured 19 children (26%), but AHI improved in the majority of children (p = 0.001). Similarly, the arousal index, PETCO2, and SpO2 nadir improved significantly (p < 0.002, p = 0.019, p < 0.001, respectively). AHI improved significantly in children with mild-to-moderate OSA in comparison to severe OSA (p < 0.001). Children with enlarged tonsils and no history of prior surgery benefitted more often from surgery (p < 0.004 and p = 0.002, respectively). AT was the only surgery reducing the AHI significantly (p = 0.008). Children did not lose weight despite intervention. Adherence with PAP was poor. CONCLUSIONS: Surgery improved OPSG indices in the majority of obese children with OSA.

Assessment and treatment of obstructive sleep-disordered breathing.

Obstructive sleep-disordered breathing (OSDB) is a condition that affects 1% to 3% of the pediatric population. These disorders are difficult to diagnosis and left untreated may be serious, including not only medical comorbidities but also cognitive, academic, behavioral, and emotional sequelae. This article is designed to bring awareness of the severity and prevalence to family physicians and pediatricians. It reviews detailed information concerning OSDB, including the predisposing factors, assessment of presenting features, and treatment.

Predictors and outcome of low initial forced expiratory volume in 1 second measurement in children with cystic fibrosis.

OBJECTIVE: To identify the characteristics of children with cystic fibrosis with low initial forced expiratory volume in 1 second (FEV1) % predicted and to investigate their outcome. STUDY DESIGN: Patients were categorized into low or high initial FEV1 groups using cluster analysis. Comparisons of the demographic and clinical data were performed between the 2 groups. RESULTS: From 122 children, 21 clustered into the low and 101 into the high FEV1 group. The mean FEV1 was 69% ± 12% predicted for the low and 95% ± 12% predicted for the high FEV1 group (P < .001). The low FEV1 group had lower body mass index percentiles (P = .003), were hospitalized more frequently (P = .001), and had been on dornase alfa longer (P = .006). Low FEV1 group had more patients with positive cultures for Pseudomonas aeruginosa (P = .002) and Stenotrophomonas maltophilia (P < .001) and had more total number of cultures positive for mucoid P. aeruginosa (P = .009) and methicillin resistant Staphylococcus aureus + P. aeruginosa (P = .005). The low FEV1 group continued to have low FEV1 measurements, their FEV1 declined slower, required more hospitalizations per year (P = .01), and had more cultures for mucoid (P = .003) and nonmucoid P. aeruginosa (P = .02) ± methicillin resistant S. aureus (P = .002) in comparison with the high FEV1 group. Poor adherence was associated with lower initial FEV1 values in females, and early, rapid decline of FEV1 in males. CONCLUSIONS: Some children with cystic fibrosis may present with poor lung function early in life and continue to have subnormal lung function associated with reduced body mass index, more frequent hospitalization, and higher rates of infection. Such children may benefit from careful evaluation and close follow-up.

Role of a respiratory therapist in improving adherence to positive airway pressure treatment in a pediatric sleep apnea clinic.

BACKGROUND: Many pediatric patients need positive airway pressure (PAP) for treatment of obstructive sleep-disordered breathing. Adherence to PAP (defined as percent of nights with PAP use of > 4 h) is often poor and not sustained long-term. With any chronic disease, education has been shown to help with patient outcomes. Education of patients and parents regarding PAP can be provided by different healthcare professionals. There is no published literature assessing the role of respiratory therapists (RTs) in improving adherence to PAP in children. We hypothesized that the addition of RT visits to a PAP clinic would improve PAP adherence. METHODS: RT services for PAP patients were introduced in a multidisciplinary pediatric sleep clinic in May 2006. We identified children who had been followed in clinic, and had adherence download information before and after introduction of RT services. We collected demographic, polysomnography, and CPAP adherence data at clinic visits. RESULTS: Forty-six subjects met criteria for inclusion. The mean ± SD age was 14.9 ± 6 y. The mean ± SD apnea-hypopnea index was 26.7 ± 30 events/h. Other than the addition of the RT intervention, all subjects continued to receive the same clinical services as before. Subjects were divided into 3 groups, based on baseline adherence: 0% use, use for 1-50% of nights, and use for > 50% of nights. There was a statistically significant improvement in PAP adherence in the subjects with baseline use of 0% and 1-50%, but no improvement in those with > 50% use at baseline. There was no significant change in PAP use at subsequent RT visits. CONCLUSIONS: Utilization at clinic visits of an RT trained in the use of PAP improved adherence in pediatric subjects with obstructive sleep-disordered breathing when their baseline PAP adherence was < 50%.

Behaviorally based adherence program for pediatric patients treated with positive airway pressure.

OBJECTIVES: Positive Airway Pressure (PAP) devices are being increasingly used in pediatric populations for problems including Obstructive Sleep Apnea. There is limited literature regarding pediatric adherence with PAP treatment or successful interventions that improve adherence. This paper describes a clinical program for pediatric PAP users which was designed to improve adherence of patients prescribed PAP therapy and involves intensive assessment and behavioral intervention. METHOD: The design and implementation of the program is described, including the purpose, goals, programmatic structure, clinical content, type and training of personnel, strengths and limitations and financial concerns as well as the challenges with the development and application of the program's protocol. RESULTS: Preliminary outcome data show short-term improvements in consistent usage for many of the patients. CONCLUSIONS: The program shows some promise and provides a framework for future program development and research.

An in-patient model for positive airway pressure desensitization: a report of 2 pediatric cases.

Application of positive airway pressure is frequently indicated in pediatric patients with a diagnosis of obstructive sleep apnea. Adherence to equipment use is often less than optimal and can be more challenging when working with children with special needs. An in-patient protocol was designed utilizing various techniques and strategies from the medical adherence literature and applied to 2 cases. This protocol utilizes specialists from various disciplines, including respiratory therapists, psychologists, physicians, nurses, and child life therapists, as well as parental involvement. This paper outlines this protocol using 2 case studies. Both patients successfully used their equipment for greater than 4 hours at night by the end of their hospital stay of 4 days and maintained or advanced these gains at follow-up. These 2 cases suggest that more research should be conducted to further evaluate the effectiveness of similar programs.

Periodic limb movements during sleep in children with narcolepsy.

STUDY OBJECTIVES: In adults with narcolepsy, periodic limb movements of sleep (PLMS) occur more frequently than in control population, and presence of increased PLMS is associated with greater sleep disruption and shorter mean sleep latency. This study was performed to determine whether PLMS are common in children with narcolepsy, and whether the presence of PLMS is associated with greater sleep disruption. DESIGN: Demographic and polysomnographic information were collected from consecutive patients diagnosed with narcolepsy identified retrospectively by diagnosis-based search. Descriptive data were compiled, and sleep characteristics of children with and without PLMS were compared. SETTING: Sleep disorders center in a children's hospital. PATIENTS: 44 patients, 6-19 years old (mean 13 years, SD 3.57), were identified. Twenty-eight were African American. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Four patients had a PLMS index (PLMI) ≥ 5/h (considered abnormal in literature). Sixteen (36%) had "any PLMS" (PLMI > 0/h). The mean PLMI was 1.3/h (SD 2.5). Sleep was significantly more disrupted, and the mean sleep latency was shorter in patients with "any PLMS" as compared to those with no PLMS. There was no correlation between the PLMI and other diagnostic criteria for narcolepsy. "Any PLMS" were present equally in children of African American and Caucasian heritage, 35.7% vs. 37.5%. CONCLUSIONS: As in adults, children with PLMS and narcolepsy have more sleep disruption and shorter mean sleep latencies than those with narcolepsy but without PLMS. Our findings also suggest that the use of adult criteria for diagnosis of "significant" PLMS in children may not be sufficiently sensitive.

Report of two patients with associated conditions in addition to cystic fibrosis.

PURPOSE: To report two patients with associated conditions in addition to cystic fibrosis. METHODS: We reviewed our database and report two patients with cystic fibrosis who had associated conditions. These patients also had novel disease causing CFTR mutations on full gene sequence analysis. RESULTS: We identified 2 patients with novel disease causing cystic fibrosis transmembrane conductance regulator mutations that we report here. A 12-year-old female with cystic fibrosis, diagnosed at 18months, had normal pulmonary function tests and chest X-ray. Her main cystic fibrosis-related health issue was poor growth. Results of cystic fibrosis transmembrane conductance regulator DNA analysis showed deltaF508; L467P; and 7T/9T. She was later diagnosed with Crohn's disease. An 11-year-old male with Rubinstein-Taybi syndrome, diagnosed with cystic fibrosis at 2years of age, had minimal findings on chest X-ray and pancreatic insufficiency. Results of his cystic fibrosis transmembrane conductance regulator DNA analysis showed deltaF508; 4329delCT; and 7T/9T. CONCLUSION: We report 2 patients with CF who had associated conditions and also had novel disease causing CFTR mutations. Associated conditions may worsen the clinical manifestations of CF and complicate medical management.

Diagnosis of pediatric obstructive sleep disordered breathing: beyond the gold standard.

Childhood obstructive sleep disordered breathing (OSDB), a sleep-related upper airway obstruction that degrades sleep quality, ventilation and/or oxygenation, in turn leads to a variety of daytime symptoms and morbidity. In the past, childhood OSDB was classified into primary snoring versus obstructive sleep apnea (OSA) syndrome and it was widely assumed that childhood OSA required treatment, while primary snoring did not. Pediatric polysomnography (PSG) was originally developed to distinguish primary snoring from childhood OSA. However, the late 1990s saw an explosion of new research on the daytime manifestations and long-term morbidity of childhood OSDB, which resulted in expanded, more comprehensive definitions of childhood OSDB syndromes. It is now clear that even primary snoring, in the absence of classically defined OSA, can be associated with neurobehavioral abnormalities in children. Thus, in retrospect, we realize that 'classical' childhood OSA was only a subset of a larger affected population and that children previously classified as having primary snoring may have serious neurobehavioral dysfunction. Now, in the era of the expanded definition of childhood OSDB, the role of PSG in the routine evaluation of the snoring child is controversial. Given that overnight PSG is widely regarded as the 'gold standard' for the diagnosis of OSDB in children, we review the current usage of PSG in children for the diagnosis of OSDB, its value, limitations and possibilities for future improvements.