Assessing air travel safety in neuromuscular disease: standard versus prolonged hypoxic challenge tests.

PURPOSE: The hypoxic challenge test (HCT) is used to evaluate safety for air travel in individuals with respiratory disease by breathing in 15% oxygen for 20 min. Our aim was to determine if a prolonged HCT, lasting 120 min, identified more individuals with neuromuscular disease at potential risk than the standard HCT lasting 20 min. METHODS: This was a cross-sectional study. All of the clinical testing took place at SickKids, Toronto, Canada. Patients were included in the study if they had a diagnosis of NMD, greater than 6 years of age, resting oxygen saturation ≥ 94%, and partial pressure of carbon dioxide (pCO2) ≤ 45 mmHg. Notable exclusion criteria were left ventricular ejection fraction < 30%, presence of a tracheostomy, and use of non-invasive ventilation for more than 12 h daily. Participants underwent a standard HCT as well as the prolonged HCT on the same day. RESULTS: Twenty-three patients consented to the study. One patient was withdrawn because he was unable to follow the study procedures. The 22 study participants had a mean age of 14.9 years (standard deviation (SD) of 5 years). Seventeen (77%) participants were male. Two participants were withdrawn on the day of testing due to hypercapnia. Twenty participants completed the standard and prolonged HCTs. None of the participants had a positive standard or prolonged HCT. CONCLUSION: Our results suggest that performing a standard or prolonged HCT may, in fact, not be of clinical utility in individuals with less severe NMD.

Volume-assured pressure support mode for noninvasive ventilation: can it improve overnight adherence in children with neuromuscular disease?

PURPOSE: Volume-assured pressure support in noninvasive ventilation (VAPS-NIV) is a newer mode providing automatic pressure support adjustment to ensure a constant alveolar ventilation. Previous studies have shown that NIV effectiveness depends on patient adherence and tolerance. The aim of this study was to determine the adherence and efficacy of VAPS-NIV compared to spontaneous-time (S/T) mode in pediatric patients with neuromuscular disease (NMD). METHODS: This was a prospective observational study. Children with NMD who utilized NIV at home for ≥ 3 months were recruited from the Long-term ventilation clinic at The Hospital for Sick Children, Toronto, Canada, from July 1, 2015, to July 1, 2019. Baseline characteristics, date of initiation of NIV, and pulmonary function tests were recorded. Polysomnogram (PSG) data and adherence were recorded and analyzed comparing VAPS and S/T modes. RESULTS: Twenty children with NMD (17 male, 85%) were enrolled. The mean (SD) age at initiation of NIV was 11.6 ± 4.6 years. The median (IQR) duration of ventilation was 1.36 (0.80-2.98) years. The mean average daily usage and the median daily usage for VAPS mode and S/T mode were 8.4 ± 1.6 versus 7.2 ± 2.5 h (p = 0.012) and 8.6 ± 1.4 versus 7.8 ± 2.1 h (p = 0.022), respectively. There was no difference in sleep architecture, gas exchange, or parent proxy report of NIV tolerance between S/T and VAPS modes. CONCLUSION: VAPS was associated with an improvement in adherence to therapy in children with NMD compared to S/T mode. Longitudinal studies are required to evaluate long-term clinical outcomes using VAPS mode in children with NMD.

The impact of polysomnograms and family-centred decision making in children with medical complexity.

Objectives: To determine whether a change in clinical management (e.g., new tracheostomy or adenotonsillectomy) occurred following a polysomnogram (PSG) in children with medical complexity (CMC) and to explore whether families' goals of care (regarding results and treatment implications) were discussed prior to the completion of a PSG. Methods: All CMC enrolled in a complex care program at the Hospital for Sick Children, Canada, who underwent a baseline PSG from 2009 to 2015 were identified. Exclusion criteria included (1) PSGs for ventilation titration and (2) PSGs outside the study time frame. Health records were retrospectively reviewed to determine demographics, medical histories, families' wishes, PSG results, and their impact on clinical care. Descriptive statistics were used to summarize results. Results: Of 145 patients identified, 96 patients met inclusion criteria. Fifty (52%) were male. Median age was 3 years. Forty-eight (50%) were diagnosed with clinically significant (i.e., moderate to severe obstructive sleep apnea, central sleep apnea, and/or hypoventilation) sleep-related breathing disorders. Of those diagnosed, 9 (19%) had surgery, 25 (52%) underwent respiratory technology initiation, and 3 (6%) underwent both. In the remaining 11 (23%) patients, treatment was either considered too risky or did not align with the families' wishes. Only 3 of 96 patients had clear documentation of their families' wishes prior to PSG completion. Conclusion: Recognizing the burden of medical tests for both the child and the health care system, a process of shared-decision making that includes clarifying a family's wishes may be prudent prior to conducting a PSG.

Intermittent nocturnal hypoxia and metabolic risk in obese adolescents with obstructive sleep apnea.

PURPOSE: There is conflicting data regarding the independent associations of obstructive sleep apnea (OSA) with metabolic risk in obese youth. Previous studies have not consistently addressed central adiposity, specifically elevated waist to height ratio (WHtR), which is associated with metabolic risk independent of body mass index. OBJECTIVE: The objective of this study was to determine the independent effects of the obstructive apnea-hypopnea index (OAHI) and associated indices of nocturnal hypoxia on metabolic function in obese youth after adjusting for WHtR. METHODS: Subjects had standardized anthropometric measurements. Fasting blood included insulin, glucose, glycated hemoglobin, alanine transferase, and aspartate transaminase. Insulin resistance was quantified with the homeostatic model assessment. Overnight polysomnography determined the OAHI and nocturnal oxygenation indices. RESULTS: Of the 75 recruited subjects, 23% were diagnosed with OSA. Adjusting for age, gender, and WHtR in multivariable linear regression models, a higher oxygen desaturation index was associated with a higher fasting insulin (coefficient [standard error] = 48.076 [11.255], p < 0.001), higher glycated hemoglobin (coefficient [standard error] = 0.097 [0.041], p = 0.02), higher insulin resistance (coefficient [standard error] = 1.516 [0.364], p < 0.001), elevated alanine transferase (coefficient [standard error] = 11.631 [2.770], p < 0.001), and aspartate transaminase (coefficient [standard error] = 4.880 [1.444], p = 0.001). However, there were no significant associations between OAHI, glucose metabolism, and liver enzymes. CONCLUSION: Intermittent nocturnal hypoxia rather than the OAHI was associated with metabolic risk in obese youth after adjusting for WHtR. Measures of abdominal adiposity such as WHtR should be considered in future studies that evaluate the impact of OSA on metabolic health.

Prevalence of obstructive sleep apnea among obese toddlers and preschool children.

BACKGROUND: Obstructive sleep apnea (OSA) is a common disorder estimated at 1-5% in the school-aged children. With the obesity prevalence reaching staggering rates globally, OSA in obese adolescents is estimated to be 4-5-folds higher than their lean peers. There is a paucity of data regarding obesity-related OSA in children 6 years and less. This is particularly relevant as OSA is associated with neurocognitive deficits. The aim of this study is to evaluate the prevalence of OSA among obese toddlers and preschool children and further to determine what other factors may be associated with the presence of OSA. METHODS: A retrospective study involving children ≤6 years, identified from two Canadian pediatric tertiary care centers who had an in-lab polysomnography (PSG). Obesity was defined by a BMI of > 95th percentile for age and gender or a z-score of > 2. OSA was diagnosed if the obstructive apnea-hypopnea index (OAHI) was greater than 2 events per hour. RESULTS: There were 60 participants included; the mean age was 4.4 years (standard deviation [SD] ± 1.7), mean BMI z-score was 3.0 (SD ± 1.2). Of these, 22/60 (36.6%) had OSA. Compared with the non-OSA group, the OSA group had a higher Epworth sleepiness score (p = 0.03) and were more likely to snore (p = 0.01). CONCLUSION: Young obese children should be assessed for OSA. A history of snoring and daytime sleepiness may be useful indicators to facilitate triage for a PSG, especially in resource-limited settings.

A retrospective analysis of clinical characteristics of childhood narcolepsy.

AIM: Narcolepsy, encompassing excessive daytime sleepiness (EDS), cataplexy, sleep paralysis and hypnogogic hallucinations, was previously considered rare in childhood. Recently, cases of childhood narcolepsy have increased significantly and the reasons for this may include the increasing awareness of narcolepsy as well as the H1N1 vaccination. The aim of this study was to describe the clinical characteristics of childhood narcolepsy, specifically focusing on cataplexy subtypes that may facilitate early recognition of narcolepsy. METHODS: We retrospectively reviewed and analyzed the medical records of 33 children diagnosed with narcolepsy at the Hospital for Sick Children, in Toronto, Ontario. All patients were seen prior to 18 years of age and symptoms were self-reported by parents and/or children themselves. RESULTS: At presentation, 32 of 33 children reported EDS and 28 of 33 reported cataplexy. Among the 28 patients with cataplexy, 18 of 28 reported cataplexy referred to as 'cataplectic facies' (e.g., facial hypotonia and/or tongue protrusion) while 10 of 28 patients reported characteristic cataplexy, defined as bilateral loss of muscle tone. Children with cataplectic facies reported higher BMI z-scores compared to those with characteristic cataplexy, 1.8 and 0.8, respectively. Children with cataplectic facies also tended to be younger than those with characteristic cataplexy, 9.2 and 11.8 years of age, respectively. Cataplectic facies appear to be related to narcolepsy close to disease onset. CONCLUSIONS: Children, especially young, obese children, presenting with a history of EDS with associated facial hypotonia or tongue protrusion raises the index of suspicion of narcolepsy and should prompt a referral to a specialized sleep facility to establish the diagnosis.

Plastic bronchitis: a rare complication of long-term haemodialysis catheter placement in a child.

BACKGROUND: Despite peritoneal dialysis being the preferred mode of renal replacement therapy in neonates and infants, long-term haemodialysis may be necessary in a minority of patients with its attendant risks. CASE DIAGNOSIS/TREATMENT: This case identifies plastic bronchitis as a rare yet serious complication of long-term large bore vascular access when a vessel-sparing approach is not possible. CONCLUSIONS: An appropriately sized catheter should be used for the dialytic therapy required and to optimize access survival.

Intelligent volume-assured pressured support (iVAPS) for the treatment of congenital central hypoventilation syndrome.

PURPOSE: Congenital central hypoventilation syndrome (CCHS) is characterized by ventilatory insensitivity to hypercapnia and hypoxemia during sleep and/or wakefulness. Management of CCHS includes a long-term ventilation. However, ventilation can be challenging given differences in the control of breathing during different sleep stages. Intelligent volume-assured pressure support (iVAPS) is a mode of Bi-level positive airway pressure (BPAP) ventilation in which the pressure support is modulated to ensure a constant alveolar ventilation. The aim of this study was to determine if BPAP with iVAPS mode is more effective at controlling hypercapnia than BPAP with spontaneous/timed (S/T) mode. METHODS: A retrospective chart review of CCHS patients who underwent both a titration polysomnogram (PSG) with standard BPAP S/T mode and a consecutive follow-up study with BPAP iVAPS mode at The Hospital for Sick Children, Toronto, Canada, between January 1, 2013 and September 30, 2015 were included. Comparisons were made between S/T mode and iVAPS mode. RESULTS: Eight (four males) children with CCHS were included. The median (IQR) age at the time of PSG using Bi-level ventilation with S/T mode for study participants was 10.0 (IQR 8.4, 11.6) years followed by PSGs with iVAPS mode, median age 10.6 (IQR 9.1, 12.5) years. The non-rapid eye movement (NREM) peak transcutaneous CO2 (tcCO2) median (IQR) for iVAPS was 43.0 (40.0-46.0-) mmHg versus 46.5 (45.0-48.0) mmHg for S/T mode, (p value <0.05). CONCLUSION: iVAPS was associated with a reduction in the maximum tcCO2 during NREM sleep as compared to traditional S/T mode. Prospective, longitudinal studies are needed to evaluate the benefits of BPAP therapy iVAPS mode for the treatment of pediatric CCHS.

Sleep-disordered breathing in children with chronic kidney disease.

BACKGROUND: The aim of our study was to ascertain the prevalence and type of sleep-disordered breathing (SDB) in paediatric patients with severe chronic kidney disease (CKD) based on the results of polysomnograms (PSGs). METHODS: Overnight PSGs were conducted on children with CKD stages 3-5 (dialysis dependent). Data were collected on patient demographics from the medical records. Study participants and/or their caregivers completed the paediatric modification of the Epworth Sleepiness Scale Score, the Pediatric Sleep Questionnaire (PSQ) and the Pediatric Quality of Life Inventory at the time of the PSG. RESULTS: Nineteen children were included in the study, of whom seven were on dialysis. The median (interquartile range) age at the time of the PSG was 13.5 (5.4-16.5) years, and eight (42%) of the children were male. There was a 37% (n = 7) prevalence of SDB in this cohort based on the PSG results. Central sleep apnea and obstructive sleep apnea were found in three children each. The PSQ scores did not correlate with the obstructive apnea-hypopnea index. CONCLUSIONS: There was a high prevalence of SDB in this cohort of children with CKD. The PSG and validated sleep questionnaires yielded discordant results, reinforcing the limitations of diagnosing SDB in the CKD population based solely on sleep questionnaires.

The diagnostic yield of rescreening for arteriovenous malformations in children with hereditary hemorrhagic telangiectasia.

The diagnostic yield of rescreening children with hereditary hemorrhagic telangiectasia at regular intervals for arteriovenous malformations is unclear. Here, we show that when children with initially negative screening were reassessed after 5 years, no new arteriovenous malformations were detected suggesting that longer intervals between screenings may be adequate.

Sickle Cell Disease Phenotypes and Obstructive Sleep Apnea; Are They Related?

OBJECTIVE: This study aims to compare the polysomnographic features between Arab-Indian and Benin phenotypes of sickle cell disease (SCD). MATERIALS AND METHODS: This prospective cross-sectional study was conducted in the Children's Hospital at King Fahad MedicalCity, in Riyadhwhere childrenwere recruited fromthe pediatric hematology clinic and pediatric sleepmedicine. All families were approached and patients who met the inclusion criteria and agreed to participate were included in the study. RESULTS: Eighty four children (37 of whom were females) with SCD were included in the study. Their median (interquartile) age was 9 (6.65, 11) years and their body mass index z score was -1.45 (-2.195, -1.45). The evidence of obstructive sleep apnea (OSA) was more prominent in the Benin phenotype (66.7%) in comparison to those of the Arab-Indian (35.2%) phenotype ( p = 0.006). Additionally, 56.7% of Benin had moderate to severe OSA whereas Arab-Indian had 18% with a ( p = 0.0003). Controlling for other factors, the odds ratio (confidence interval) of having OSA in Benin phenotype was 4.68 (1.42-15.38) times higher as compared to Arab-Indian phenotype. CONCLUSION: The risk of having OSA as well as the severity of OSA is higher in Benin phenotype as compared to Arab-Indian phenotype which indicates the presence of potential OSA risk factors other than the SCD itself.

14 and 6 Hz like spike wave activity is a common finding in young patients with Prader-Willi syndrome.

STUDY OBJECTIVES: Our aim was to characterize the 14 and 6 like spike wave activity seen on electroencephalograms (EEG) in children with Prader-Willi syndrome (PWS) undergoing polysomnograms. METHODS: We performed a retrospective review of children with PWS and healthy controls who underwent diagnostic polysomnograms between January 1, 2007, and December 31, 2020, at SickKids, Toronto, Canada. EEGs from the polysomnograms were reviewed for the presence of the 14 and 6 like spike wave activity and its characteristics. Clinical correlation of the EEG variant with sleep-disordered breathing indices from the polysomnograms was also evaluated. RESULTS: A total of 94 children with PWS and 50 healthy controls were included. The median age and interquartile range for the cohort was 1.42 (0.6, 4.2) years. There were 50 (53.2%) males in the PWS cohort. The EEG variant prevalence in this cohort was 51.0% (n = 48) in children with PWS and 0% for the healthy controls. 14 and 6 Hz like spike wave activity was bilateral in 52% (25/48) children with PWS. The waves had a negative deflection in almost all patients, 44/48 (92%), with PWS. It was predominantly located in the frontal leads for children with PWS, 23/48 (47.9%). It most frequently occurred during non-rapid eye movement stage 2 sleep for children with PWS, 25/48 (52.0%). The mean (standard deviation) frequency was 6.8 (0.97) Hz. The median (interquartile range) length of the waves was 1.1 (0.8, 1.4) seconds in children with PWS. There was no correlation between the presence of the EEG variant and sleep-disordered breathing indices in children with PWS. CONCLUSIONS: The 14 and 6 Hz like spike wave activity EEG variant was present in more than 50% of a pediatric cohort of children with PWS compared with 0% in healthy children. This EEG variant did not appear to be associated with sleep-disordered breathing indices in children with PWS and is of unknown clinical significance. CITATION: Alzaid M, Sunkonkit K, Massicotte C, Otsubo H, Amin R, Al-Saleh S. 14 and 6 Hz like spike wave activity is a common finding in young patients with Prader-Willi syndrome. J Clin Sleep Med. 2024;20(8):1227-1232.

Prevalence of pulmonary arteriovenous malformations in children versus adults with hereditary hemorrhagic telangiectasia.

It is unclear whether pulmonary arteriovenous malformations (PAVMs) in hereditary hemorrhagic telangiectasia develop later in life or are preformed in childhood. We show that the prevalence of PAVMs in children is similar to that in their parents, providing evidence against de novo PAVM formation in hereditary hemorrhagic telangiectasia.

Longitudinal evaluation of sleep-disordered breathing in children with Prader-Willi Syndrome during 2 years of growth hormone therapy.

OBJECTIVE: To review longitudinal polysomnography data to assess sleep-related disordered breathing (SRDB) before and up to 2 years after initiation of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). STUDY DESIGN: This was a retrospective review of systematic polysomnography evaluations performed in children with PWS before and at 6 weeks, 6 months, 1 year, and 2 years after initiation of GH therapy. RESULTS: A total of 15 children with PWS were reviewed. At baseline, the median age was 3.7 years (range, 0.8-15.4 years), and the median body mass index percentile was 82.4 (range, 0-100). GH was discontinued in 2 of these 15 children owing to the occurrence of severe obstructive sleep apnea after 6 weeks of GH therapy. The remaining 13 children who were followed for up to 2 years on GH therapy demonstrated no statistically significant trends over time for any adverse sleep-related outcomes, specifically obstructive or central sleep apnea. CONCLUSION: In young children with PWS with known SRDB at baseline, the first few weeks after initiation of GH therapy may represent a vulnerable time for the development of significant SRDB. However, most children with PWS did not show significant changes in SRDB after 2 years of GH therapy. We conclude that long-term GH therapy appears to be safe after an initial period of increased risk in the context of SRDB in children with PWS.

Polysomnography in hospitalized children: Characteristics and clinical practice at a single tertiary care center.

BACKGROUND: Polysomnography (PSG) is the gold standard for the diagnosis of pediatric sleep-disordered breathing (SDB). However, the literature characterizing the indications for inpatient PSGs and the impact on clinical decision-making is limited. OBJECTIVE: To determine the indications, results, and outcomes for children undergoing inpatient PSGs at our institution. METHODS: We performed a retrospective review of children aged 0-18 years who underwent inpatient diagnostic PSGs between July 2018 and July 2021 at SickKids, Toronto, Canada. Baseline characteristics, indications, and management were reviewed and characterized by descriptive statistics. RESULTS: Eighty-eight inpatient PSGs were performed in 75 children (male 62.7%). Median (interquartile range) age and body mass index z-score were 1.5 (0.2, 10.8) years and 0.27 (-1.58, 2.66), respectively. The most common indication for inpatient PSG was initiation and titration of ventilation (n = 34/75, 45.3%). Of the 75 children, 48 (64%) had multiple complex chronic conditions (CCCs). Sixty children (80%) underwent a baseline PSG for either the entire night or a portion of the night. Of these studies, 54 (90%) had clinically significant SDB of which isolated obstructive sleep apnea (OSA; 17/60, 28.3%) was the most common. The following management was undertaken for the 54 patients with SDB; respiratory technology (88.9%), surgical intervention (31.5%), positional therapy (1.9%), intranasal steroids (3.7%), and no further intervention (5.6%), respectively. CONCLUSIONS: Our study highlights that inpatient PSG was an important diagnostic tool resulting in directed medical and surgical management. Future multicenter studies are needed to compare indications for inpatient PSGs across institutions to develop evidence-based clinical practice guidelines.

Lower airway nitric oxide is increased in children with sickle cell disease.

OBJECTIVES: To determine alveolar and airways nitric oxide (NO) levels in children with sickle cell disease (SCD). STUDY DESIGN: Multiple flows fractional exhaled NO (FE(NO)), bronchial NO flux (J'aw(NO)), and alveolar NO concentration (Ca(NO)) were determined prospectively in 16 non-atopic children with SCD in a tertiary ambulatory clinic and compared with those in 10 children with primary ciliary dyskinesia and 22 healthy control subjects. Differences in FE(NO), J'aw(NO), and Ca(NO) were compared with mixed model analysis and Mann-Whitney tests. RESULTS: Children with SCD had reference range FE(NO) at 50 mL/sec, but FE(NO) was elevated across all flows compared with healthy control subjects (mean difference=2.10±0.91 parts per billion, P=.03). Subjects with SCD had increased J'aw(NO) (1177±533 picoliters per second versus 833±343 picolitres per second, P=.03), and Ca(NO) was no different from control subjects. In contrast, children with primary ciliary dyskinesia had decreased FE(NO) (mean difference=3.36±1.24 parts per billion, P<.01) and J'aw(NO) (507±259 picoliters per second versus 833±343 picoliters per second, P<.01). CONCLUSIONS: Lower airways NO is increased in children with SCD. Elevation of J'aw(NO) may represent dysregulation of NO metabolism or subclinical airways inflammation.

Utility of contrast echocardiography for pulmonary arteriovenous malformation screening in pediatric hereditary hemorrhagic telangiectasia.

OBJECTIVE: To evaluate the utility of transthoracic contrast echocardiography (TTCE) as a screening tool for pulmonary arteriovenous malformations (PAVMs) in children with hereditary hemorrhagic telangiectasia (HHT). STUDY DESIGN: This was a single-center study of children who underwent baseline screening for PAVMs using both TTCE and chest computed tomography (CT) for evaluation of HHT. The CT and TTCE results were prospectively reviewed independently by 2 radiologists and 2 cardiologists blinded to the study results. RESULTS: Both intraobserver and interobserver agreement for interpreting TTCE results were excellent (κ = 0.97 and 0.92, respectively) and higher than the interobserver agreement for CT interpretation (κ = 0.75). The sensitivity and specificity of TTCE to predict PAVMs were 1 and 0.82, respectively, and the positive predictive and negative predictive values were 0.39 and 1, respectively. CONCLUSION: TTCE is a sensitive test for PAVMs in children with suspected HHT and can be a useful initial screening tool in pediatric HHT.

Asymmetric dimethylarginine contributes to airway nitric oxide deficiency in patients with cystic fibrosis.

RATIONALE: Airway nitric oxide is reduced in cystic fibrosis airways. Asymmetric dimethylarginine is an endogenous nitric oxide synthase inhibitor that may contribute to nitric oxide deficiency in cystic fibrosis. OBJECTIVES: To test the hypothesis that asymmetric dimethylarginine is increased in cystic fibrosis and contributes to nitric oxide deficiency and airway obstruction. METHODS: The concentrations of asymmetric dimethylarginine, symmetric dimethylarginine, and l-arginine were measured in sputum of clinically stable patients with cystic fibrosis, in patients with cystic fibrosis before and after treatment for a pulmonary exacerbation, and in healthy control subjects, using liquid chromatography-tandem mass spectrometry. MEASUREMENTS AND MAIN RESULTS: Asymmetric dimethylarginine was increased in cystic fibrosis compared with control sputum, and the l-arginine/asymmetric dimethylarginine ratio was decreased. Symmetric dimethylarginine exceeded asymmetric dimethylarginine concentrations in control sputum, but this ratio was reversed in cystic fibrosis. Treatment for pulmonary exacerbation resulted in a decrease in sputum asymmetric dimethylarginine and an improved l-arginine/asymmetric dimethylarginine ratio. The treatment-related decrease in asymmetric dimethylarginine correlated significantly with an increase in sputum nitric oxide metabolites and improvement in pulmonary function. The activity of the asymmetric dimethylarginine-metabolizing enzyme, dimethylarginine dimethylaminohydrolase, was higher in cystic fibrosis sputum before rather than after treatment, suggesting that the accumulation of asymmetric dimethylarginine is caused by increased production, not decreased degradation, of asymmetric dimethylarginine. CONCLUSIONS: Asymmetric dimethylarginine is increased in cystic fibrosis airways and may contribute to airway obstruction in patients with cystic fibrosis by reducing nitric oxide formation.

Risks and Benefits of Adenotonsillectomy in Children With Cerebral Palsy With Obstructive Sleep Apnea: A Systematic Review.

OBJECTIVES/HYPOTHESIS: Assess the risks and benefits of adenotonsillectomy (AT) for obstructive sleep apnea (OSA) in children with cerebral palsy (CP). STUDY DESIGN: Systematic review. METHODS: We conducted a systematic review of Medline, Embase, and Cochrane Central Registry from 1946 to 2021. Broad search concepts included cerebral palsy, pediatric, tonsillectomy/adenoidectomy, and sleep. Additional articles were identified by searching reference lists. Studies on the safety and efficacy of AT for OSA management in children with CP were included. RESULTS: Fifteen articles met inclusion criteria. Articles were classified into one or more of four themes: intraoperative risk (n = 1), postoperative risk (n = 3), postoperative care requirements (n = 6), and surgical outcomes (n = 7). No intraoperative anesthetic complications were reported. Postoperatively, respiratory complications including pneumonia were common and necessitated additional airway management. Following AT, children with CP required close postoperative observation, experienced increased lengths of stay, and had increased odds of unplanned intensive care unit (ICU) admission. Benefits following AT were improvement in OSA as measured by a reduction in obstructive apnea-hypopnea index (OAHI) as well as improved quality of life in some; however, many patients went on to require tracheostomy due to persistent OSA. CONCLUSIONS: Children with CP who undergo AT have a significant risk of developing a postoperative respiratory complication. Realistic counseling of families around increased perioperative risks in this population is imperative and close postoperative monitoring is critical. Many children will obtain a reduction in OAHI, but additional surgical management is often required, including tracheostomy. Further research is needed to determine the best management strategy for OSA in children with CP. Laryngoscope, 132:687-694, 2022.

Sputum induction in routine clinical care of children with cystic fibrosis.

OBJECTIVE: To determine the microbiological yield of induced sputum (IS) samples compared with conventional airway samples, spontaneously expectorated sputum and throat swabs, in children with cystic fibrosis (CF) attending an outpatient clinic. STUDY DESIGN: Ninety-five children with CF (75 able to spontaneously expectorate sputum) were included in this prospective cross-sectional comparative study. After obtaining expectorated sputum or throat swabs samples, IS was obtained by performing the sputum induction procedure using an eFlow device (PARI, Starnberg, Germany). CF bacterial culture results were compared between the two procedures. RESULTS: Differences in culture results between samples were observed in 25 of 94 (27%) patients. IS had a significantly higher yield for CF pathogens, with 80% of the differences being due to detection of additional organisms in IS samples. Overall, SI was well tolerated, but 12 of 95 cases had a >20% postinduction decline in forced expiratory volume in 1 second. The whole SI procedure took 30 to 85 minutes of clinic time, and its estimated additional cost was $150 (US)/patient. CONCLUSIONS: Induced sputum has a higher microbiological yield compared with the conventional samples in children with CF, even in patients capable of expectorating sputum spontaneously. Although sputum induction is safe and tolerable, it is time-consuming and expensive in routine clinical settings.