Technology-Enabled Health Care Collaboration in Pediatric Chronic Illness: Pre-Post Interventional Study for Feasibility, Acceptability, and Clinical Impact of an Electronic Health Record-Linked Platform for Patient-Clinician Partnership.

BACKGROUND: Mobile health (mHealth) technology has the potential to support the Chronic Care Model's vision of closed feedback loops and patient-clinician partnerships. OBJECTIVE: This study aims to evaluate the feasibility, acceptability, and short-term impact of an electronic health record-linked mHealth platform (Orchestra) supporting patient and clinician collaboration through real-time, bidirectional data sharing. METHODS: We conducted a 6-month prospective, pre-post, proof-of-concept study of Orchestra among patients and parents in the Cincinnati Children's Hospital inflammatory bowel disease (IBD) and cystic fibrosis (CF) clinics. Participants and clinicians used Orchestra during and between visits to complete and view patient-reported outcome (PRO) measures and previsit plans. Surveys completed at baseline and at 3- and 6-month follow-up visits plus data from the platform were used to assess outcomes including PRO completion rates, weekly platform use, disease self-efficacy, and impact on care. Analyses included descriptive statistics; pre-post comparisons; Pearson correlations; and, if applicable, effect sizes. RESULTS: We enrolled 92 participants (CF: n=52 and IBD: n=40), and 73% (67/92) completed the study. Average PRO completion was 61%, and average weekly platform use was 80%. Participants reported improvement in self-efficacy from baseline to 6 months (7.90 to 8.44; P=.006). At 6 months, most participants reported that the platform was useful (36/40, 90%) and had a positive impact on their care, including improved visit quality (33/40, 83%), visit collaboration (35/40, 88%), and visit preparation (31/40, 78%). PRO completion was positively associated with multiple indicators of care impact at 3 and 6 months. CONCLUSIONS: Use of an mHealth tool to support closed feedback loops through real-time data sharing and patient-clinician collaboration is feasible and shows indications of acceptability and promise as a strategy for improving pediatric chronic illness management.

Sleep disturbance and sleep insufficiency in primary caregivers and their children with cystic fibrosis.

BACKGROUND: Chronically ill children and their parents are at risk for sleep disorders and associated morbidity. Sleep disturbance prevalence and the relationships between parent and child sleep among children with CF are not well defined. Clarifying the presence and impact of sleep disturbances among pediatric CF patients and their parents could lead to improved health in this population. METHODS: Cross-sectional study assessing parent-reported sleep in ninety-one CF patients (mean age 8.8 years; 53.8% female) and their primary caregivers. Sleep sufficiency determined using American Academy of Sleep Medicine guidelines; correlation coefficients computed for sleep problem domains; stepwise multiple linear regression determined predictive models for sleep duration. RESULTS: Parents reported concerns about their own sleep and that of their children. Night waking and daytime sleepiness were most common in parents; prolonged sleep latency was most common for children. Most parents and children had inadequate sleep duration. School-age children had the highest frequencies of overall sleep concerns and inadequate sleep. Most parent and child sleep problem domains were significantly associated, with large effects for similar parent and child problems. Stepwise multiple linear regression demonstrated that CF caregiver/patient sleep duration was significantly predicted by insomnia symptoms. CONCLUSIONS: Many CF children and their parents experience sleep difficulties including inadequate sleep duration, with presence of sleep problems in many families whose children with CF had normal lung function. These data suggest that sleep health should be a CF Care Model component and should be a health care focus for families of children with other chronic illness.

Total pancreatectomy with islet autotransplantation in a pancreatic-sufficient cystic fibrosis patient.

For children with Cystic Fibrosis (CF) suffering from acute recurrent pancreatitis (ARP), abdominal pain can be severe, difficult to treat, impair their quality of life, affect participation at school, and can lead to chronic opioid dependence. Total pancreatectomy with islet autotransplantation (TPIAT) is an uncommon treatment that is reserved for refractory cases of ARP. We present a case of a 4 year old female with pancreatic-sufficient CF, refractory ARP, frequent hospital admissions for abdominal pain, and continued growth failure despite gastrostomy tube and parenteral nutrition. One year after successful TPIAT, the patient is insulin-independent, growing well, and has not been re-hospitalized for abdominal pain. To our knowledge, this is the youngest patient with CF to undergo TPIAT for debilitating ARP. With CFTR modulators restoring some pancreatic function, CF clinicians should have increased vigilance for the development of ARP.

Improving Clinical Outcomes and Quality of Life with Massage Therapy in Youth and Young Adults with Cystic Fibrosis: a Pilot Study.

BACKGROUND: Cystic Fibrosis (CF) is an autosomal recessive disorder of exocrine glands characterized by abnormal production of thick mucus, primarily in bronchi of the lungs. Individuals experience recurrent respiratory infections, increased work of breathing, cough and musculoskeletal changes with pain. Previous research found that massage therapy (MT) decreased pain, muscle tightness, and anxiety in individuals with CF, but did not use valid/reliable measurements of quality of life (QOL). PURPOSE: To evaluate the effects of MT on QOL and clinical outcomes in individuals 8 to 21 years old with CF. SETTING: A 622-bed nonprofit pediatric hospital in Ohio in the United States. PARTICIPANTS: Convenience sample of 24 patients with CF; 12 randomly assigned to treatment and control groups, respectively. RESEARCH DESIGN AND INTERVENTION: Prospective two-group controlled pre/post pilot study using deep tissue myofascial trigger point massage over 10 to 12 weeks. MEASUREMENTS: Pediatric Quality of Life Inventory (Peds QL 4.0); Cystic Fibrosis Questionnaire-Revised (CFQ-R); numeric rating scales (NRS) for pain, muscle tightness, ease of breathing, relaxation; pulmonary function (PFT); single breath count; thoracic excursion (TE). RESULTS: All participants were Caucasian; mean age 15.7 (SD = 3.5) years; 16 (66.6%) female. No significant differences were found in terms of age, gender, baseline pain between MT and control groups. At the final visit, compared to the control group, the children in MT group showed statistically significantly reduced muscle tightness (p = .048) with a large effect size (ω2 =0.163) and marginally statistically significantly higher levels of relaxation (p = .052), less pain (p = .076), and improved upper TE (p = .078) and lower TE (p = .056) scores with large and moderate effect sizes (ω2 = 0.156, ω2 = 0.095, ω2 = 0.083, and ω2 = 0.073). No statistically significant differences in children's and caregivers' QOL scores between the two groups were found. CONCLUSIONS: Massage therapy was found to significantly reduce muscle tightness, marginally significantly help pain, relaxation, and thoracic excursion in participants with CF.

Relationship between exercise capacity and glucose tolerance in cystic fibrosis.

BACKGROUND: Improved exercise capacity (EC) and normal glucose tolerance (NGT) are independently associated with favorable outcomes in CF, however, little information on this relationship exists in patients with CF. METHODS: Cardiopulmonary exercise tests, oral glucose tolerance tests (OGTT), and HbA1c values measured within a 12-month period were evaluated on 83 pediatric patients diagnosed with CF. Patients were categorized as having NGT, abnormal glucose tolerance (AGT), or CF-related diabetes (CFRD). RESULTS: EC decreased as severity of glucose intolerance increased across NGT, AGT, and CFRD groups (P = 0.02). Compared to patients with NGT, patients with CFRD had lower peak VO2 mL/kg/min (33.0 ± 7.3 vs 41.3 ± 9.4, P = 0.01), lower VO2 % (81 ± 20 vs 93 ± 17, P = 0.03), and higher HbA1c (6.9 ± 1.7 vs 5.4 ± 0.4, P < 0.01). There was a positive association with age and FEV1 % with EC in the 17 patients with CFRD. In the 66 patients without diabetes, peak EC was positively associated with FEV1 % and negatively associated with age, fasting insulin, and insulin 120 min. After accounting for age and FEV1 %, multivariate analyses indicated that insulin and glucose values at 120 min predicted EC. CONCLUSIONS: These data provide evidence that poor glucose tolerance is associated with lower EC in pediatric patients with CF. There was a significant relationship between glucose and insulin values obtained by OGTT with EC in a sample of non-diabetic patients with preserved lung function. Future studies are warranted to confirm these findings and investigate the potential role of exercise in the management or prevention of CFRD.

Body Sanctification and Sleep in Adolescents with Cystic Fibrosis: A Pilot Study.

Imbuing one's body with divine significance is associated with health-protective behaviors. The purpose of this study was to determine whether adolescents with a life-shortening, chronic disease (cystic fibrosis) who sanctified their bodies also received adequate sleep. Data from Daily Phone Diaries and questionnaire replies from 45 adolescents with cystic fibrosis (ages 11-19 years) were analyzed. A significant relationship between body sanctification and sleep was found, with between-gender differences. Body sanctification is an understudied construct which is associated with healthy behaviors.

Protocol of a Pilot Study of Technology-Enabled Coproduction in Pediatric Chronic Illness Care.

BACKGROUND: Pediatric chronic illness care models are traditionally organized around acute episodes of care and may not meet the needs of patients and their families. Interventions that extend the patient-clinician interaction beyond the health care visit, allow for asynchronous and bidirectional feedback loops that span visits and daily life, and facilitate seamless sharing of information are needed to support a care delivery system that is more collaborative, continuous, and data-driven. Orchestra is a mobile health technology platform and intervention designed to transform the management of chronic diseases by optimizing patient-clinician coproduction of care. OBJECTIVE: The aim of this study is to assess the feasibility, acceptability, and preliminary impact of the Orchestra technology and intervention in the context of pediatric chronic illness care. METHODS: This study will be conducted in the cystic fibrosis and inflammatory bowel disease clinics at Cincinnati Children's Hospital Medical Center. We will enroll interested patients and their caregivers to work with clinicians to use the Orchestra technology platform and care model over a 6-month period. In parallel, we will use quality improvement methods to improve processes for integrating Orchestra into clinic workflows and patient/family lifestyles. We will use surveys, interviews, technology use data, and measures of clinical outcomes to assess the feasibility, acceptability, and preliminary impact of Orchestra. Outcome measures will include assessments of: (1) enrollment and dropout rates; (2) duration of engagement/sustained use; (3) symptom and patient-reported outcome tracker completion rates; (4) perceived impact on treatment plan, communication with the clinical team, visit preparation, and overall care; (5) changes in disease self-efficacy and engagement in care; and (6) clinical outcomes and health care utilization. RESULTS: Participant recruitment began in mid-2015, with results expected in 2017. CONCLUSIONS: Chronic disease management needs a dramatic transformation to support more collaborative, effective, and patient-centered care. This study is unique in that it is testing not only the impact of technology, but also the necessary processes that facilitate patient and clinician collaboration. This pilot study is designed to examine how technology-enabled coproduction can be implemented in real-life clinical contexts. Once the Orchestra technology and intervention are optimized to ensure feasibility and acceptability, future studies can test the effectiveness of this approach to improve patient outcomes and health care value.

Gaps in evidence: Management of pediatric obstructive sleep apnea without tonsillar hypertrophy.

OBJECTIVES/HYPOTHESIS: Persistent obstructive sleep apnea (OSA) is demonstrated in 40% of children after adenotonsillectomy. We previously evaluated the basis of management decisions in children with OSA without tonsillar hypertrophy and found that 61% of decisions were non-evidence based. The aim of this study was to identify gaps in evidence for the management of children with OSA without tonsillar hypertrophy. STUDY DESIGN: Case series. METHODS: We recorded all real-time decisions made by pediatric subspecialists from eight disciplines that participated in an upper airway clinic and management conferences. Practitioners were immediately queried regarding the basis of their decisions, and non-evidence-based decisions were categorized. RESULTS: During 10 case conferences and five clinics, 507 decisions were made for 63 children (43% with Down syndrome, 20% with Pierre Robin sequence). The 309 non-evidence-based decisions most commonly pertained to follow-up timing and appropriate subspecialty clinic location (116/309, 38%) as well as timing for repeat polysomnography (35/309, 11%), especially in children at high risk for persistent OSA after treatment. Additional gaps identified included the likelihood of OSA improvement from weight loss, and effectiveness of sleep surgical procedures (i.e., lingual tonsillectomy, posterior midline glossectomy, and craniofacial surgery) alone or in combination. CONCLUSIONS: Identified gaps in evidence included timing and location of follow-up, appropriate use of polysomnography for surveillance, effectiveness of specific surgical procedures performed alone and in combination, and the use of oral appliances and continuous positive airway pressure therapy in children with Down syndrome. We also found a need for studies to compare the effectiveness of these treatment options in diverse patient populations. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:758-762, 2016.

Decision Making for Children with Obstructive Sleep Apnea without Tonsillar Hypertrophy.

OBJECTIVE: Evidence-based medicine is the gold standard practice model for patient management. Our aim was to determine whether decisions made by pediatric subspecialists regarding management of obstructive sleep apnea in children without tonsillar hypertrophy adhered to this model or were based on clinical experiences. STUDY DESIGN: Single-institution prospective study. SETTING: Multidisciplinary upper airway center in an academic pediatric hospital. SUBJECTS AND METHODS: Twelve pediatric subspecialists representing 8 specialties participating in upper airway clinics and management conferences. Real-time decisions made in treatment conferences and upper airway clinics were collected. Physicians were queried regarding the basis of their decisions, and these decisions were then classified into 10 categories. RESULTS: Over 13 days (10 case conferences, 3 half-day clinics), 324 decisions were made for 58 patients (mean age = 8.9 ± 7.4 years, mean body mass index percentile = 75 ± 29); 34% (n = 108) of decisions were evidence based; 59% (n = 193) were nonevidence based; and 7% (n = 23) were based on parental preference. Providers were able to cite specific studies for <20% of these decisions. There was no significant increase in the proportion of evidence-based decisions made over time. CONCLUSIONS: We deemed 34% of decisions regarding the management of obstructive sleep apnea in children without tonsillar hypertrophy to be evidence based and found that sharing the basis for decisions did not improve the percentage of evidence-based decisions over time. These findings reflect significant evidence gaps and highlight the need for a systematic literature evaluation to identify best practice in managing this population. We recommend that these evidence gaps be further characterized and incorporated into an agenda for future research.

Adherence Determinants in Cystic Fibrosis: Cluster Analysis of Parental Psychosocial, Religious, and/or Spiritual Factors.

RATIONALE: Cystic fibrosis is a progressive disease requiring a complex, time-consuming treatment regimen. Nonadherence may contribute to an acceleration of the disease process. Spirituality influences some parental healthcare behaviors and medical decision-making. OBJECTIVES: We hypothesized that parents of children with cystic fibrosis, when classified into groups based on adherence rates, would share certain psychosocial and religious and/or spiritual variables distinguishing them from other adherence groups. METHODS: We conducted a multisite, prospective, observational study focused on parents of children younger than 13 years old at two cystic fibrosis center sites (Site 1, n= 83; Site 2, n = 59). Religious and/or spiritual constructs, depression, and marital adjustment were measured by using previously validated questionnaires. Determinants of adherence included parental attitude toward treatment, perceived behavioral norms, motivation, and self-efficacy. Adherence patterns were measured with the Daily Phone Diary, a validated instrument used to collect adherence data. Cluster analysis identified discrete adherence patterns, including parents' completion of more treatments than prescribed. MEASUREMENTS AND MAIN RESULTS: For airway clearance therapy, four adherence groups were identified: median adherence rates of 23%, 52%, 77%, and 120%. These four groups differed significantly for parental depression, sanctification of their child's body, and self-efficacy. Three adherence groups were identified for nebulized medications: median adherence rates of 35%, 82%, and 130%. These three groups differed significantly for sanctification of their child's body and self-efficacy. CONCLUSIONS: Our results indicated that parents in each group shared psychosocial and religious and/or spiritual factors that differentiated them. Therefore, conversations about adherence likely should be tailored to baseline adherence patterns. Development of efficacious religious and/or spiritual interventions that promote adherence by caregivers of children with cystic fibrosis may be useful.

The impact of transforming healthcare delivery on cystic fibrosis outcomes: a decade of quality improvement at Cincinnati Children's Hospital.

BACKGROUND: In 2001, Cincinnati Children's Hospital embarked on a journey to improve healthcare delivery to patients with cystic fibrosis (CF). Data from the Cystic Fibrosis Foundation National Patient Registry revealed our below-average clinical outcomes, prompting us to initiate improvement interventions. OBJECTIVE: To improve clinical outcomes for patients with CF through a comprehensive quality-improvement approach directed at increasing patient centredness and improving healthcare delivery. INTERVENTIONS: In 2001, we shared our below-average outcomes with patients, families and care providers. We instituted a quality-improvement steering committee with parental and hospital leadership, and our data-management support was restructured to provide real-time clinical data to monitor our progress. In 2002, our weekly chart conference changed to a prospective planning session and individualised daily schedules were created for inpatients. In 2003, an influenza vaccination campaign was initiated and our infection-control practices were redesigned. In 2005, best-practice guidelines were developed for airway-clearance therapy. In 2007, evidence-based clinical algorithms were designed and implemented and key care-team members were added. MEASUREMENTS: Primary outcome measures were median forced expiratory volume in 1 s per cent predicted (age range 6-17 years) and median body mass index percentile (age range 2-20 years). RESULTS: From 2000 to 2010, median forced expiratory volume in 1 s increased from 81.7% to 100.1% predicted and median body mass index increased from the 35th to the 55th centile. DISCUSSION: By focusing on specific outcomes, empowering families and patients, effectively using data, and standardising care processes, we transformed the culture and delivery of care for our patients with CF and learned valuable lessons potentially translatable to other chronic-care providers.

Obstructive lung disease is common in children with syndromic and congenital scoliosis: a preliminary study.

BACKGROUND: It is well known that restrictive lung disease (RLD) is associated with scoliosis. This study identifies that obstructive lung disease (OLD) is associated with syndromic scoliosis and congenital scoliosis. METHODS: We searched a local database for patients with scoliosis who underwent a pulmonary function testing (PFT) from 2004 to 2009. All patients with congenital scoliosis or syndromic thoracolumbar scoliosis with a Cobb angle of ≥40 degrees and acceptable and repeatable PFT testing were included in the study. OLD was defined as an forced expiratory volume in the first second/forced vital capacity ratio below 95% confidence interval. Bronchoscopy videos and computed tomography scans or magnetic resonance images were reviewed to identify anatomic causes of lower airway disease. RESULTS: A total of 18 patients met the criteria for inclusion. The median age at diagnosis was 11.3 years. The median primary Cobb angle was 60 degrees. The prevalence of OLD was 33% and RLD was 57%. The 6 children with OLD underwent preoperative bronchoscopy and chest computed tomography or magnetic resonance imaging to identify anatomic causes of lower airway obstruction. The 4 children with OLD and right-sided major thoracic curves had compression of the right mainstem bronchus between the spine (posterior) and the right pulmonary artery (anterior). The 2 children with OLD and left-sided major thoracic curves had compression of the left mainstem bronchus between the spine (posterior) and the descending aorta (anterior) or the left atrium (anterior). CONCLUSIONS: In our study, the prevalence of OLD in children with congenital scoliosis or syndromic scoliosis was 33%, which was elevated when compared with the population prevalence of 2% to 5%. Mainstem airway compression from spine rotation was discovered to be the potential mechanism of disease. LEVEL OF EVIDENCE: Level IV, prognostic study investigating the effect of a patient characteristic on the outcome of disease.

Baroreflex sensitivity after adenotonsillectomy in children with obstructive sleep apnea during wakefulness and sleep.

STUDY OBJECTIVES: Children with obstructive sleep apnea have blunted baroreflex sensitivity and increased blood pressure variability. The aim of the study was to test the hypothesis that treatment of sleep apnea by adenotonsillectomy results in significant improvement of baroreflex sensitivity, lowering of blood pressure and blood pressure variability and increase vagal heart rate modulation. STUDY DESIGN: One hundred ninety-four children aged 9.6 ± 2.3 years were enrolled; 133 had obstructive sleep apnea and 61 were healthy controls. For children with sleep apnea, polysomnography with 3-lead electrocardiography and continuous blood pressure was performed before adenotonsillectomy, then 6 weeks and 6 months postoperatively. Controls underwent the same assessment at study entry and 6 months later. Spontaneous baroreflex sensitivity was measured in the time and frequency domains. Data analyses were performed for available and complete cases. RESULTS: Children with sleep apnea experienced postoperatively an increase in baroreflex sensitivity and decrease in blood pressure variability during wakefulness and sleep. A decrease in blood pressure during sleep and in heart rate during wakefulness was also measured. The improvement in baroreflex sensitivity was predicted by the change in the apnea-hypopnea and arousal indices. A normal pattern of rising baroreflex sensitivity during the night was restored in children with severe apnea after surgery. However, baroreceptor sensitivity did not completely normalize after treatment. CONCLUSION: Treatment of obstructive sleep apnea in children by adenotonsillectomy is associated with gradual improvement in known risk factors for cardiovascular disease. Complete normalization of baroreceptor sensitivity was not achieved 6 months postoperatively.

Neonates with tongue-based airway obstruction: a systematic review.

OBJECTIVE: In this systematic review, the authors summarize the current evidence in the literature regarding diagnosis, treatment, and long-term outcomes in neonates with tongue-based airway obstruction (TBAO) and assess the level of evidence of included studies. DATA SOURCES: The terms Pierre Robin syndrome/sequence, micrognathia, retrognathia, and cleft palate were combined with airway obstruction, treatment, tongue-lip plication, and osteogenesis distraction to perform an Ovid literature search, yielding 341 references. The authors excluded references containing patients with isolated choanal/nasal obstruction, patients older than 12 months, and expert opinion papers, yielding 126 articles. REVIEW METHODS: The authors searched 3 electronic databases and reference lists of existing reviews from 1980 to October 2010 for articles pertaining to the diagnosis, treatment, and outcomes of TBAO. Reviewers assigned a level of evidence score based on Oxford's Centre for Evidence Based Medicine scoring system and recorded relevant information. RESULTS: Most studies were case studies and single-center findings. The lack of standardization of diagnostic and treatment protocols and the heterogeneity of cohorts both within and between studies precluded a meta-analysis. There was little evidence beyond expert opinion and single-center evaluation regarding diagnosis, treatment, and long-term outcomes of neonates with TBAO. CONCLUSIONS: The variability in the phenotype of the cohorts studied and the absence of standardized indications for intervention preclude deriving any definitive conclusions regarding diagnostic tools to evaluate this patient population, treatment choices, or long-term outcomes. A coordinated multicenter study with a standardized diagnostic and treatment algorithm is recommended to develop evidence for the diagnosis and treatment of neonates with TBAO.

Comparison of lingual tonsil size as depicted on MR imaging between children with obstructive sleep apnea despite previous tonsillectomy and adenoidectomy and normal controls.

BACKGROUND: Cine MRI has become a useful tool in the evaluation of patients with persistent obstructive sleep apnea (OSA) despite previous surgical intervention and in patients with underlying conditions that render them susceptible to multilevel airway obstruction. Findings on cine MRI studies have also increased our understanding of the mechanisms and anatomic causes of OSA in children. OBJECTIVE: To compare lingual tonsil size between children with OSA and a group of normal controls. In addition, a subanalysis was made of the group of children with OSA comparing lingual tonsils between children with and without underlying Down syndrome. MATERIALS AND METHODS: Children with persistent OSA despite previous palatine tonsillectomy and adenoidectomy and controls without OSA underwent MR imaging with sagittal fast spin echo inversion-recovery images, and lingual tonsils were categorized as nonperceptible at imaging or present and measurable. When present, lingual tonsils were measured in the maximum anterior-posterior diameter. If lingual tonsils were greater than 10 mm in diameter and abutting both the posterior border of the tongue and the posterior pharyngeal wall, they were considered markedly enlarged. RESULTS: There were statistically significant differences between the OSA and control groups for the presence vs. nonvisualization of lingual tonsils (OSA 33% vs. control 0%, P=0.0001) and mean diameter of the lingual tonsils (OSA 9.50 mm vs. control 0.0 mm, P=0.00001). Within the OSA group, there were statistically significant differences between children with and without Down syndrome for the three lingual tonsil width categories (P=0.0070) and occurrence of markedly enlarged lingual tonsils (with Down syndrome 35% vs. without Down syndrome 3%, P=0.0035). CONCLUSIONS: Enlargement of the lingual tonsils is relatively common in children with persistent obstructive sleep apnea after palatine tonsillectomy and adenoidectomy. This is particularly true in patients with Down syndrome.

Obstructive sleep apnea: Should all children with Down syndrome be tested?

OBJECTIVES: To determine the incidence of obstructive sleep apnea syndrome in children aged 2 to 4 years with Down syndrome and to determine parents' ability to predict sleep abnormalities in this patient population. DESIGN: Prospective cohort study. SETTING: Tertiary care pediatric referral center. PATIENTS: Sixty-five children participating in a 5-year longitudinal study in which the otolaryngologic problems seen in Down syndrome were evaluated. Fifty-six completed overnight polysomnography (PSG) between 4 and 63 months of age (mean age, 42 months). INTERVENTIONS: Overnight PSG was performed. Parents also completed a questionnaire regarding their impressions of their child's sleep patterns before PSG. MAIN OUTCOME MEASURES: Polysomnograms were classified as abnormal if the obstructive index was greater than 1, if the carbon dioxide level was greater than 45 mm Hg for more than two thirds of the study or greater than 50 mm Hg for more than 10% of the study, and/or if there was unexpected hypoxemia less than 92% during sleep or repeated intermittent desaturations less than 90%. We also identified a group of children whose PSGs findings were normal except for an arousal index greater than 10 and were associated with increased work of breathing. RESULTS: The PSGs revealed that 57% of the children had abnormal results and evidence of obstructive sleep apnea syndrome. If we also include an elevated arousal index, 80% of the PSGs had abnormal results. Sixty-nine percent of parents reported no sleep problems in their children, but in this group, 54% of PSGs had abnormal results. Of the parents who reported sleep problems in their children, only 36% had abnormal sleep study results. CONCLUSION: Because of the high incidence of obstructive sleep apnea syndrome in young children with Down syndrome, and the poor correlation between parental impressions of sleep problems and PSG results, baseline PSG is recommended in all children with Down syndrome at age 3 to 4 years.

Causes of persistent obstructive sleep apnea despite previous tonsillectomy and adenoidectomy in children with down syndrome as depicted on static and dynamic cine MRI.

OBJECTIVE: Our purpose was to evaluate the causes of persistent obstructive sleep apnea despite previous tonsillectomy and adenoidectomy in children with Down syndrome as depicted on cine MRI. MATERIALS AND METHODS: Cine MRI studies performed to evaluate persistent obstructive sleep apnea despite previous tonsillectomy and adenoidectomy were reviewed. MRI was performed under sedation and included cine MR images (fast gradient-echo) obtained in the midline sagittal plane and in the axial plane at the base of the tongue and T1-weighted spin-echo and fast spin-echo inversion recovery images in the axial and sagittal planes. Imaging parameters reviewed included static and dynamic diagnoses made, frequency of recurrence and diameter of tonsillar tissue, and tongue morphology. RESULTS: Twenty-seven patients were identified (mean age, 9.9 years). Diagnoses included glossoptosis in 17 patients (63%), hypopharyngeal collapse in six (22%), recurrent and enlarged adenoid tonsils in 17 (63%), enlarged lingual tonsils in eight (30%), and macroglossia in 20 (74%). Of the 20 patients with macroglossia, 11 (55%) had absence of the normal median sulcus and 12 (60%) had evidence of fatty infiltration of the tongue musculature. CONCLUSION: Persistent obstructive sleep apnea in children with Down syndrome who have undergone previous adenoidectomy and tonsillectomy has multiple causes. The most common causes include macroglossia, glossoptosis, recurrent enlargement of the adenoid tonsils, and enlarged lingual tonsils.

Upper airway motion depicted at cine MR imaging performed during sleep: comparison between young Patients with and those without obstructive sleep apnea.

PURPOSE: To compare the patterns of dynamic airway motion depicted on cine magnetic resonance (MR) images obtained during sleep between young patients with and those without obstructive sleep apnea (OSA). MATERIALS AND METHODS: Fast gradient-echo sequences were performed in the sagittal midline by using a 1.5-T unit to create cine MR images. Cine MR images obtained during sleep in 16 young patients with OSA were compared with those obtained in 16 young patients with no airway symptoms of airway disease. The nasopharynx, oropharynx, and hypopharynx were characterized in terms of airway motion as static patent (SP), dynamic patent, intermittent collapsed (IC), or static collapsed (SC); and the maximal diameter and greatest change in diameter (in millimeters) of these airways were calculated. Adenoid tonsil size and mouth position (ie, opened or closed) were determined. Differences in the frequency of MR imaging parameters in the different anatomic regions were evaluated by using Fisher exact, chi 2, and sample t tests. RESULTS: There were statistically significant differences in the following parameters between the two groups: nasopharynx SP (P <.001) and IC (P <.001); hypopharynx SP (P <.001) and IC (P <.001); and mean change in airway diameter of the nasopharynx (P <.001) and hypopharynx (P <.001). The mean adenoid tonsil size in the patients with OSA was larger (P =.006). CONCLUSION: There are significant differences in the patterns of dynamic airway motion between young patients with and those without OSA.

Relapsed non-Hodgkin's lymphoma diagnosed by flexible bronchoscopy.

Pulmonary relapse of non-Hodgkin's lymphoma (NHL) occurred in a 10-year-old girl who presented with cough, blood-tinged sputum, and chest tightness. The diagnosis of Ki-1 (CD30) anaplastic large-cell lymphoma was established using bronchoalveolar lavage (BAL) and transbronchial biopsy (TBB). These procedures demonstrated malignant cells that stained positive for CD30 and had the t(2;5) translocation, thereby avoiding the need for an open lung biopsy.