Sleep Disordered Breathing in Children with Neuromuscular Disease.

Sleep disordered breathing (SDB) in children with neuromuscular disease (NMD) is more prevalent compared to the general population, and often manifests as sleep-related hypoventilation, sleep-related hypoxemia, obstructive sleep apnea, central sleep apnea, and/or disordered control of breathing. Other sleep problems include, sleep fragmentation, abnormal sleep architecture, and nocturnal seizures in certain neuromuscular diseases. The manifestation of sleep disordered breathing in children depends on the extent, type, and progression of neuromuscular weakness, and in some instances, may be the first sign of a neuromuscular weakness leading to diagnosis of an NMD. In-lab diagnostic polysomnography (PSG) remains the gold standard for the diagnosis of sleep disordered breathing in children, but poses several challenges, including access to many children with neuromuscular disease who are non-ambulatory. If SDB is untreated, it can result in significant morbidity and mortality. Hence, we aimed to perform a comprehensive review of the literature of SDB in children with NMD. This review includes pathophysiological changes during sleep, clinical evaluation, diagnosis, challenges in interpreting PSG data using American Academy of Sleep (AASM) diagnostic criteria, management of SDB, and suggests areas for future research.

Utility of Screening for Obstructive Sleep Apnea with the Pediatric Sleep Questionnaire (PSQ) in Children with Craniofacial Anomalies.

OBJECTIVE: To determine the accuracy of the Pediatric Sleep Questionnaire (PSQ) as a screening tool for obstructive sleep apnea in children with craniofacial anomalies. DESIGN: Retrospective cohort study. SETTING: Multidisciplinary cleft and craniofacial clinic at a tertiary care center. PATIENTS: Children with craniofacial anomalies 2 to ≤18 years of age who both completed a PSQ screen and underwent polysomnography (PSG) without interval surgery. MAIN OUTCOME MEASURES: Sensitivity and specificity of the PSQ in detecting an obstructive apnea-hypopnea index (AHI) ≥ 5 events/hour. RESULTS: Fifty children met study criteria, with 66% (n = 33) having an associated syndrome. Mean patient age at time of PSQ was 9.6 + 4.0 years. Overall, 33 (64%) screened positive on the PSQ, while 20 (40%) had an AHI ≥ 5. The sensitivity and specificity for identifying AHI ≥ 5 was 70% and 40%, respectively. With subgroup analysis, the sensitivity and specificity were higher (100% and 50%) in children with non-syndromic palatal clefting but lower (65% and 31%) in children with a syndrome or chromosomal anomaly. There was no correlation detected between PSQ score and AHI severity (p = 0.25). The mean obstructive AHI in the study population was 10.1 ± 22.7 despite 44% (n = 22) undergoing prior adenotonsillectomy. CONCLUSIONS: The PSQ was less sensitive and specific in detecting an AHI ≥ 5 in children with craniofacial anomalies than in a general population, and particularly poor in for children with syndrome-associated craniofacial conditions. Given the high prevalence of OSA in this patient population, a craniofacial-specific validated screening tool would be beneficial.

Clinical Characteristics and Post-Operative Outcomes in Children with Very Severe Obstructive Sleep Apnea.

Available information on clinical characteristics and post-operative outcomes in children with very severe obstructive sleep apnea (OSA) is limited. Our study evaluates the clinical features and polysomnographic (PSG) variables that predict post-operative outcomes in children with an obstructive apneal hypopnea index (AHI) of more than 25 events/hr. In this study from a single tertiary care center, we performed a retrospective chart review of patients with an AHI > 25/hr, who underwent tonsillectomy and adenoidectomy (T&A) between January 2016 and September 2021. In total, 50 children were included in the study: 26.0% (13/50) of children experienced post-operative respiratory events and four children needed intubation and ventilator support. Compared with children without respiratory events, children requiring post-operative respiratory interventions were younger (4.4 ± 5.2 vs. 8.0 ± 5.2 years; p = 0.04), had higher pre-operative AHI (73.6 ± 27.4 vs. 44.8 ± 24.9; p < 0.01), lower oxygen nadirs (70.0 ± 13.0% vs. 83.0 ± 7.0%; p < 0.01), and had lower body metabolic index Z-scores (−0.51 ± 2.1 vs. 0.66 ± 1.5; p < 0.04). Moderate to severe residual OSA was identified in 70% (24/34) of children with available post-operative PSG; younger children had better PSG outcomes. Our study shows that post-operative respiratory events are frequent in children with very severe OSA, particularly with an AHI > 40/h, younger children (<2 years of age), lower oxygen saturation (SpO2), and poor nutritional status, necessitating close monitoring.

Associations between sleep, obesity, and asthma in urban minority children.

STUDY OBJECTIVES: Although obesity, asthma, and sleep-disordered breathing are interrelated, there is limited understanding of the independent contributions of body-mass index and pulmonary function on polysomnography in children with asthma. METHODS: We conducted a retrospective chart review on 448 7- to 18-year-old children with asthma who had undergone polysomnography testing between 1/2007-12/2011 to elucidate the association between spirometry variables, body-mass index, and polysomnography parameters, adjusting for asthma and antiallergic medications. RESULTS: Obese children had poorer sleep architecture and more severe gas exchange abnormalities compared to healthy weight children. Multivariate analysis revealed an independent association of body-mass index with sleep efficiency, with more light and less deep sleep in both obese and healthy-weight children, and with baseline oxygen saturation and oxygen nadir in obese children. In obese children, forced vital capacity was independently associated with less deep sleep (time in N3 sleep) as well as with oxygen nadir, while among healthy-weight children, forced expiratory volume directly correlated but forced vital capacity inversely correlated with deep sleep. In obese children, inhaled corticosteroid was associated with baseline oxygen saturation, and montelukast was associated with lower end-tidal carbon dioxide. In healthy-weight children, inhaled corticosteroid was associated with arousal awakening index, and montelukast was associated with light sleep. Antiallergic medications were not independently associated with polysomnography parameters. CONCLUSIONS: Pulmonary function, body-mass index, and asthma medications have independent and differing influences on sleep architecture and gas exchange polysomnography parameters in obese and healthy-weight children with asthma. Asthma medications are associated with improved gas exchange in obese children and improved sleep architecture in healthy-weight children with asthma. CITATION: Conrad LA, Nandalike K, Rani S, Rastogi D. Associations between sleep, obesity, and asthma in urban minority children. J Clin Sleep Med. 2022;18(10):2377-2385.

Nasal trumpet as a long-term remedy for obstructive sleep apnea syndrome in a child.

We present a case of successful long-term use of nasal trumpet for severe obstructive sleep apnea syndrome in a child with cerebral palsy and complex medical issues. Obstructive sleep apnea syndrome is frequently seen in pediatric patients with cerebral palsy due to their abnormal airway tone and pulmonary vulnerability. Identifying children with cerebral palsy who are at risk for obstructive sleep apnea syndrome is important because its treatment can improve quality of life and seizure control. Although first-line treatment for obstructive sleep apnea syndrome is adenotonsillectomy, children with cerebral palsy are more likely to have residual obstructive sleep apnea syndrome postoperatively. Other options such as positive airway pressure therapy and other upper airway surgeries may pose significant challenges and tolerance issues, as in our patient. As demonstrated in our report, the low rate of complications and ease of use make nasal trumpets a potential long-term treatment option for children with obstructive sleep apnea syndrome who fail or cannot comply with the traditional treatment options.

Vaping in today's pandemic: E-cigarette, or vaping, product use-associated lung injury mimicking COVID-19 in teenagers presenting with respiratory distress.

The clinical presentation of children and adolescents infected with severe acute respiratory syndrome coronavirus 2 can range from asymptomatic to mild or moderate manifestations. We present a case series of three adolescents who presented during the coronavirus disease 2019 (COVID-19) pandemic with symptoms concerning for COVID-19, including fever, abdominal symptoms, cough, respiratory distress, and hypoxemia. Their laboratory results showed elevated inflammatory markers that are also commonly seen in COVID-19. The chest imaging studies mimicked COVID-19 with non-specific ground glass opacities and interstitial prominence patterns. However, severe acute respiratory syndrome coronavirus 2 testing was negative and further questioning of these adolescents and their parents revealed a history of vaping marijuana-related products leading to the eventual diagnosis of e-cigarette, or vaping, product use-associated lung injury. Our patients were successfully treated with corticosteroids. The providers caring for pediatric patients, especially adolescents, should continue to have a high index of suspicion for e-cigarette, or vaping, product use-associated lung injury in patients presenting with unexplained respiratory failure, while ruling out COVID-19.

Utility of Mid-Upper Arm Circumference in Diagnosing Malnutrition in Children With Cystic Fibrosis.

BACKGROUND: Standardized diagnostic indicators for malnutrition using growth percentile z-scores (weight for length or body mass index [BMI]) and mid-upper arm circumference (MUAC) z-scores are being used in clinical practice; however, their application to the pediatric cystic fibrosis (CF) population is not well described. In this study, we aim to compare growth percentile z-scores and MUAC z-scores in diagnosing and classifying malnutrition in children with CF and assess the relationship between their degree of malnourishment and corresponding pulmonary function tests (PFTs). METHODS: In this retrospective observational outpatient study of 49 pediatric CF patients, data were collected on baseline characteristics, anthropometrics, and PFTs over 12 months. Agreement in malnutrition diagnoses was quantified by Cohen κ statistics. Pearson test assessed the correlation between MUAC and BMI z-scores as well as PFTs and anthropometrics. Serial anthropometrics and PFTs were obtained and compared for a subset of patients (n = 28). RESULTS: Growth percentile and MUAC z-scores were positively correlated in diagnosing malnutrition (Pearson correlation r = 0.87), but MUAC z-scores identified more patients as malnourished compared with growth percentile z-scores (49% vs 12%, Cohen κ of 0.22 [95% CI, 0.04-0.40]). There was no significant relationship between anthropometrics and PFTs. MUAC z-scores increased significantly over time, but BMI z-scores did not show this trend. CONCLUSIONS: Our small-scale data suggest a promising role for MUAC z-scores in classifying malnutrition and in measuring changes in nutrition status over time in pediatric CF.

The Feasibility and Utility of Level III Portable Sleep Studies in the Pediatric Inpatient Setting.

STUDY OBJECTIVES: Sleep-disordered breathing (SDB) may significantly impact the course of medical illness in hospitalized children. Polysomnography (PSG) is the gold standard for establishing diagnosis of SDB, but its availability is limited. The aim of this study was to explore the feasibility and utility of level III portable sleep studies in hospitalized children with SDB. METHODS: A retrospective study was conducted at a tertiary hospital over the preceding 2 years in hospitalized children < 18 years who had undergone a level III sleep study using the Nox T3 system. The information obtained included demographic data, comorbidities, indication for admission and sleep study, time interval between the study ordered and done, adequacy of technical data from sleep study, study diagnosis, and subsequent management interventions for SDB. RESULTS: A total of 51 hospitalized children had these studies; 32 were female and mean age was 4.3 years. Approximately 90% of children had significant comorbidities, including neurological and craniofacial abnormalities. The majority (80%) of studies were conducted within 24 hours of the time requested and 92.1% studies had technically adequate data for analysis. Thirty-nine (76.5%) children were identified with SDB; all but one patient underwent therapy for SDB during that same hospitalization, including supplemental oxygen (48.7%), positive airway pressure therapy (23%), surgical intervention (38.2%) or caffeine (10.2%). Twelve percent of children had more than one intervention done. CONCLUSIONS: The level III portable sleep study is readily available, sufficient to diagnose SDB, and help to provide appropriate medical and/or surgical therapies in hospitalized children with complex medical conditions.

Intracapsular and Extracapsular Tonsillectomy and Adenoidectomy in Pediatric Obstructive Sleep Apnea.

IMPORTANCE: Limited information exists regarding clinical outcomes of children undergoing extracapsular tonsillectomy and adenoidectomy (ETA) or intracapsular tonsillectomy and adenoidectomy (ITA) for treatment of obstructive sleep apnea syndrome (OSAS). OBJECTIVES: To quantify polysomnography (PSG) and clinical outcomes of ETA and ITA in children with OSAS and to assess the contribution of comorbid conditions of asthma and obesity. DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort study using medical records at a tertiary pediatrics inner-city hospital. Medical records from 89 children who underwent ETA or ITA between October 1, 2008, and December 31, 2013, were analyzed. The dates of our analysis were January 6, 2014, to April 11, 2014. Inclusion criteria required no evidence of craniofacial or neurological disorders, confirmation of OSAS by PSG within the 2 years before surgery, and a second PSG within the 2 years after surgery. INTERVENTIONS: Each child underwent ETA or ITA after being evaluated by a pediatric otolaryngologist and obtaining written parental informed consent. MAIN OUTCOMES AND MEASURES: Main primary outcomes were derived from PSG. Secondary outcomes included treatment failure, defined as residual OSAS with an obstructive apnea-hypopnea index of at least 5 events per hour. Comparisons were made between and within groups. Logistic regression was used to identify factors associated with treatment failure. RESULTS: Fifty-two children underwent ETA, and 37 children underwent ITA. Children in the ETA group were older (7.5 vs 5.2 years, P = .001) and more obese (60% [31 of 52] vs 30% [11 of 37], P = .004). However, both groups had similar severity of OSAS, with median preoperative obstructive apnea-hypopnea indexes of 17.0 in the ETA group and 24.1 in the ITA group (P = .21), and similar prevalences of asthma (38% [20 of 52] vs 38% [14 of 37]). After surgery, significant improvement was noted on PSG in both groups, with no differences in any clinical outcomes. There was no association between procedure type, age, or body mass index z score and treatment failure. However, in a subset of patients with asthma and obesity, ITA was associated with residual OSAS (odds ratio, 16.5; 95% CI, 1.1-250.2; P = .04). CONCLUSIONS AND RELEVANCE: Both ETA and ITA are effective modalities to treat OSAS, with comparable surgical outcomes on short-term follow-up. However, when comorbid diagnoses of both asthma and obesity exist, OSAS is likely to be refractory to treatment with ITA compared with ETA.

Utility of flexible fiberoptic bronchoscopy for critically ill pediatric patients: A systematic review.

AIM: To investigate the diagnostic yield, therapeutic efficacy, and rate of adverse events related to flexible fiberoptic bronchoscopy (FFB) in critically ill children. METHODS: We searched PubMed, SCOPUS, OVID, and EMBASE databases through July 2014 for English language publications studying FFB performed in the intensive care unit in children < 18 years old. We identified 666 studies, of which 89 full-text studies were screened for further review. Two reviewers independently determined that 27 of these studies met inclusion criteria and extracted data. We examined the diagnostic yield of FFB among upper and lower airway evaluations, as well as the utility of bronchoalveolar lavage (BAL). RESULTS: We found that FFB led to a change in medical management in 28.9% (range 21.9%-69.2%) of critically ill children. The diagnostic yield of FFB was 82% (range 45.2%-100%). Infectious organisms were identified in 25.7% (17.6%-75%) of BALs performed, resulting in a change of antimicrobial management in 19.1% (range: 12.2%-75%). FFB successfully re-expanded atelectasis or removed mucus plugs in 60.3% (range: 23.8%-100%) of patients with atelectasis. Adverse events were reported in 12.9% (range: 0.5%-71.4%) of patients. The most common adverse effects of FFB were transient hypotension, hypoxia and/or bradycardia that resolved with minimal intervention, such as oxygen supplementation or removal of the bronchoscope. Serious adverse events were uncommon; 2.1% of adverse events required intervention such as bag-mask ventilation or intubation and atropine for hypoxia and bradycardia, normal saline boluses for hypotension, or lavage and suctioning for hemorrhage. CONCLUSION: FFB is safe and effective for diagnostic and therapeutic use in critically ill pediatric patients.

Anterior nasal resistance in obese children with obstructive sleep apnea syndrome.

OBJECTIVES/HYPOTHESIS: To evaluate nasal resistance in obese children with and without obstructive sleep apnea syndrome (OSAS), study the correlation between nasal resistance and severity of OSAS using the apnea-hypopnea index (AHI), and examine the association of gender and body mass index (BMI) with this measurement. STUDY DESIGN: Retrospective analysis. METHODS: Active anterior rhinomanometry was used to determine anterior nasal resistance (aNR) during wakefulness in the supine position during tidal breathing. Thirty obese children with OSAS (aged 13.8 ± 2.6 years, BMI z score 2.6 ± 0.4) and 32 matched obese controls (aged 13.6 ± 2.3 years, BMI z score 2.4 ± 0.4), were studied. Unpaired t tests and Spearman correlation were performed. RESULTS: The OSAS group had significantly higher aNR than the non-OSAS group during inspiration (P = .012) and expiration (P = .003). A significant correlation between inspiratory aNR and AHI was found for the OSAS group (r = 0.39, P = .04). The aNR did not correlate with BMI z score or with either gender. CONCLUSIONS: We noted a higher aNR in obese children with OSAS as compared to obese controls, and the aNR on inspiration correlated significantly with AHI. These findings suggest that a causal or augmentative effect of high inspiratory aNR may exist for obese children who exhibit OSAS. LEVEL OF EVIDENCE: 3b.

Severe obstructive sleep apnea in a child with melanocortin-4 receptor deficiency.

Obstructive sleep apnea (OSA) is a highly prevalent medical condition in obese children and is associated with significant neurocognitive, cardiovascular and metabolic derangements. Monogenic forms of obesity resulting from disruption of the leptin-melanocortin pathways have become more notable in recent years and distinguish between various obese phenotypes. However, the association of such disorders with OSA is not well established in children or adults. In this report, we describe a 23-month-old female with morbid obesity and OSA, who was found to carry a defect in the melanocortin-4 receptor (MC4R) pathway. This report emphasizes the genetic basis of obesity related to MC4R deficiency and OSA in children.

Adenotonsillectomy in obese children with obstructive sleep apnea syndrome: magnetic resonance imaging findings and considerations.

OBJECTIVE: The reasons why adenotonsillectomy (AT) is less effective treating obese children with obstructive sleep apnea syndrome (OSAS) are not understood. Thus, the aim of the study was to evaluate how anatomical factors contributing to airway obstruction are affected by AT in these children. METHODS: Twenty-seven obese children with OSAS (age 13.0 ± 2.3 y, body mass index Z-score 2.5 ± 0.3) underwent polysomnography and magnetic resonance imaging of the head during wakefulness before and after AT. Volumetric analysis of the upper airway and surrounding tissues was performed using commercial software (AMIRA®). RESULTS: Patients were followed for 6.1 ± 3.6 mo after AT. AT improved mean obstructive apnea-hypopnea index (AHI) from 23.7 ± 21.4 to 5.6 ± 8.7 (P < 0.001). Resolution of OSAS was noted in 44% (12 of 27), but only in 22% (4 of 18) of those with severe OSAS (AHI > 10). AT increased the volume of the nasopharynx and oropharynx (2.9 ± 1.3 versus 4.4 ± 0.9 cm(3), P < 0.001, and 3.2 ± 1.2 versus 4.3 ± 2.0 cm(3), P < 0.01, respectively), reduced tonsils (11.3 ± 4.3 versus 1.3 ± 1.4 cm(3), P < 0.001), but had no effect on the adenoid, lingual tonsil, or retropharyngeal nodes. A small significant increase in the volume of the soft palate and tongue was also noted (7.3 ± 2.5 versus 8.0 ± 1.9 cm(3), P = 0.02, and 88.2 ± 18.3 versus 89.3 ± 24.4 cm(3), P = 0.005, respectively). CONCLUSIONS: This is the first report to quantify volumetric changes in the upper airway in obese children with OSAS after adenotonsillectomy showing significant residual adenoid tissue and an increase in the volume of the tongue and soft palate. These findings could explain the low success rate of AT reported in obese children with OSAS and are important considerations for clinicians treating these children.

Deep cervical lymph node hypertrophy: a new paradigm in the understanding of pediatric obstructive sleep apnea.

OBJECTIVES/HYPOTHESIS: To determine if adenotonsillar hypertrophy is an isolated factor in pediatric obstructive sleep apnea (OSA), or if it is part of larger spectrum of cervical lymphoid hypertrophy. STUDY DESIGN: Prospective case control study. METHODS: A total of 70 screened patients (mean age 7.47 years) underwent polysomnography to confirm OSA, and then underwent MRI of the upper airway. Seventy-six matched controls (mean age 8.00 years) who already had an MRI underwent polysomnography. Volumetric analysis of lymphoid tissue volumes was carried out. Chi-square analysis and Student's t test were used to compare demographic data and lymph node volumes between cohorts. Fisher's Exact test and Chi-square analysis were used to compare sleep data. RESULTS: Patients and controls demonstrated no significant difference in mean age (7.47 vs. 8.00 yrs), weight (44.87 vs. 38.71 kg), height (124.68 vs. 127.65 cm), or body-mass index (23.63 vs. 20.87 kg/m(2)). OSA patients demonstrated poorer sleep measures than controls (P < 0.05) in all polysomnography categories (sleep efficiency, apnea index, apnea-hypopnea index, baseline SpO2, SpO2 nadir, baseline ETCO2, peak ETCO2 , and arousal awakening index). Children with OSA had higher lymphoid tissue volumes than controls in the retropharyngeal region (3316 vs. 2403 mm(3), P < 0.001), upper jugular region (22202 vs. 16819 mm(3), P < 0.005), and adenotonsillar region (18994 vs. 12675 mm(3), P < 0.0001). CONCLUSIONS: Children with OSA have larger volumes of deep cervical lymph nodes and adenotonsillar tissue than controls. This finding suggests a new paradigm in the understanding of pediatric OSA, and has ramifications for future research and clinical care.

Sleep and cardiometabolic function in obese adolescent girls with polycystic ovary syndrome.

OBJECTIVE: To compare the polysomnography findings and cardiometabolic function among adolescent girls with polycystic ovary syndrome (PCOS) and matched female and male controls. METHOD: Retrospective chart review of electronic medical records of 28 girls with PCOS (age: 16.8±1.9 years, body mass index (BMI) Z-score 2.4±0.4), 28 control females (age: 17.1±1.8, BMI Z-score 2.4±0.3) and 28 control males (age: 16.6±1.6, BMI Z-score 2.5±0.5) in a tertiary care centre. RESULTS: The prevalence of obstructive sleep apnoea (OSA) was higher in girls with PCOS compared to control females (16/28 (57%) vs. 4/28(14.3%), p<0.01); however, it was comparable to that of the control males (16/28(57%) vs. 21/28(75%), p=0.4). Girls with PCOS had a significantly higher prevalence of insulin resistance compared to control females and control males (20/28 (71.4%) vs. 9/22 (41.0%) (p=0.04) vs. 8/23 (34.8%) (p=0.01). Among girls with PCOS, those with OSA had significantly higher proportions of metabolic syndrome (MetS) (9/16 (56.3%) vs. 1/12 (8.3%) p=0.03), higher insulin resistance (14/16 (87.5%) vs. 6/12 (50%), p=0.04), elevated daytime systolic blood pressure (128.4±12.8 vs. 115.6±11.4, p<0.01), lower high-density lipoprotein (HDL) (38.6±8.7 vs. 49±10.9, p=0.01) and elevated triglycerides (TG) (149.7±87.7 vs. 93.3±25.8, p=0.03) compared to those without OSA. CONCLUSIONS: We report a higher prevalence of OSA and metabolic dysfunction in a selected group of obese girls with PCOS referred with sleep-related complaints compared to BMI-matched control girls without PCOS. We also report higher prevalence of cardiometabolic dysfunction in girls with PCOS and OSA compared to girls with PCOS without OSA.

Screening for sleep-disordered breathing and excessive daytime sleepiness in adolescent girls with polycystic ovarian syndrome.

OBJECTIVE: To determine the prevalence and clinical and metabolic correlates of sleep-disordered breathing (SDB) and excessive daytime sleepiness (EDS) in adolescent girls with polycystic ovarian syndrome (PCOS). STUDY DESIGN: Standardized questionnaires were administered to participants with PCOS and age-, sex-, ethnicity-, and body mass index (BMI) z score-matched controls. Medical records were reviewed for anthropometric and metabolic data. RESULTS: We studied 103 participants with PCOS (16.9 ± 1.5 years) and 90 controls (16.8 ± 1.7 years). Compared with controls, girls with PCOS had a higher prevalence of SDB (45.6% vs 27.8%; P = .01) and EDS (54.4% vs 35.6%; P < .01). Within PCOS, those with SDB had higher BMI z score (2.1 ± 0.5 vs 1.7 ± 0.6; P < .01), higher homeostatic model assessment index (5.1 ± 2.3 vs 4.1 ± 3.5; P < .01), and higher prevalence of the metabolic syndrome (MetS) (42.6% vs 16.1%; P = .003) compared with those without SDB. Similarly, participants with PCOS and EDS had a higher BMI z score (2.0 ± 0.6 vs 1.7 ± 0.6; P = .03), higher homeostatic model assessment index (5.1 ± 2.9 vs 3.8 ± 3.1; P = .01), and higher rate of MetS (39.3% vs. 14.9%; P < .01) compared with those without EDS. MetS was independently associated with SDB (OR, 3.2; 95% CI, 1.0-10.1) and EDS (OR, 4.5; 95% CI, 1.2-16). CONCLUSIONS: SDB and EDS are highly prevalent in adolescent girls with PCOS compared with matched controls. The MetS is independently associated with SDB and EDS in this group.

Upper airway structure and body fat composition in obese children with obstructive sleep apnea syndrome.

RATIONALE: Mechanisms leading to obstructive sleep apnea syndrome (OSAS) in obese children are not well understood. OBJECTIVES: The aim of the study was to determine anatomical risk factors associated with OSAS in obese children as compared with obese control subjects without OSAS. METHODS: Magnetic resonance imaging was used to determine the size of upper airway structure, and body fat composition. Paired analysis was used to compare between groups. Mixed effects regression models and conditional multiple logistic regression models were used to determine whether body mass index (BMI) Z-score was an effect modifier of each anatomic characteristic as it relates to OSAS. MEASUREMENTS AND MAIN RESULTS: We studied 22 obese subjects with OSAS (12.5 ± 2.8 yr; BMI Z-score, 2.4 ± 0.4) and 22 obese control subjects (12.3 ± 2.9 yr; BMI Z-score, 2.3 ± 0.3). As compared with control subjects, subjects with OSAS had a smaller oropharynx (P < 0.05) and larger adenoid (P < 0.01), tonsils (P < 0.05), and retropharyngeal nodes (P < 0.05). The size of lymphoid tissues correlated with severity of OSAS whereas BMI Z-score did not have a modifier effect on these tissues. Subjects with OSAS demonstrated increased size of parapharyngeal fat pads (P < 0.05) and abdominal visceral fat (P < 0.05). The size of these tissues did not correlate with severity of OSAS and BMI Z-score did not have a modifier effect on these tissues. CONCLUSIONS: Upper airway lymphoid hypertrophy is significant in obese children with OSAS. The lack of correlation of lymphoid tissue size with obesity suggests that this hypertrophy is caused by other mechanisms. Although the parapharyngeal fat pads and abdominal visceral fat are larger in obese children with OSAS we could not find a direct association with severity of OSAS or with obesity.

Foreign body aspiration in a child with unilateral lung aplasia.

OBJECTIVE: To present a case of foreign body aspiration in a child with unilateral lung aplasia and successful removal of the foreign body by bedside flexible bronchoscopy. DATA SOURCE: Case details were obtained from medical records. STUDY SELECTION: Eighteen-month-old girl with unilateral lung aplasia. DATA EXTRACTION AND SYNTHESIS: Demographic details (age) and clinical and biochemical data (blood gas) were obtained from medical records. An 18-mo-old girl with the diagnosis of right lung aplasia, who underwent aortopexy in the newborn period for severe respiratory distress, presented with acute-onset respiratory distress. The patient was treated with bronchodilators and steroids without success and rapidly progressed to respiratory failure. Flexible bronchoscopy done at the bedside showed a foreign body completely obstructing the left main bronchus. The rigid bronchoscopy was unsuccessful in extracting the foreign body because of the complex airway anatomy. The foreign body was successfully extracted by basket forceps via a flexible bronchoscope, and the patient recovered remarkably within few hours of the procedure. CONCLUSIONS: Because foreign body aspiration in a child with a unilateral lung can result in abrupt respiratory compromise and death, a high index of suspicion is necessary when these children present with acute respiratory symptoms. Although rigid bronchoscopy is the procedure of choice for the removal of foreign bodies, flexible bronchoscopy may be necessary for patients with abnormal airway anatomy.

CHIT1 mutations: genetic risk factor for severe asthma with fungal sensitization?

Fungi can exacerbate symptoms in patients with asthma. To our knowledge, genetic risk factors for fungal-associated asthma have not been described. We present here the cases of 6 children who carried the diagnosis of severe asthma with fungal sensitization, 3 of whom were treated with and responded clinically to itraconazole therapy. All 6 patients were heterozygous for a 24-base pair duplication in the CHIT1 gene, which has been associated with decreased levels of circulating chitotriosidase and susceptibility to fungal infection.

Severe asthma with fungal sensitization in a child: response to itraconazole therapy.

People with severe asthma with fungal sensitization may represent an underdiagnosed subset of patients with refractory disease. It is important to know that such patients may benefit from adjunct treatment with antifungal agents. We describe here the case of a child with refractory asthma, persistent airway obstruction, a serum immunoglobulin E level of >20000 IU/mL, and severe eosinophilic airway infiltration. Although he did not meet diagnostic criteria for allergic bronchopulmonary aspergillosis, he demonstrated evidence of sensitization to several fungi and responded dramatically to the addition of itraconazole therapy. We also discuss emerging hypotheses regarding fungal-induced asthma.