Computer-Aided Diagnosis of Duchenne Muscular Dystrophy Based on Texture Pattern Recognition on Ultrasound Images Using Unsupervised Clustering Algorithms and Deep Learning.

OBJECTIVE: The feasibility of using deep learning in ultrasound imaging to predict the ambulatory status of patients with Duchenne muscular dystrophy (DMD) was previously explored for the first time. The present study further used clustering algorithms for the texture reconstruction of ultrasound images of DMD data sets and analyzed the difference in echo intensity between disease stages. METHODS: k-means (Kms) and fuzzy c-means (FCM) clustering algorithms were used to reconstruct the DMD data-set textures. Each image was reconstructed using seven texture-feature categories, six of which were used as the primary analysis items. The task of automatically identifying the ambulatory function and DMD severity was performed by establishing a machine-learning model. RESULTS: The experimental results indicated that the Gaussian Naïve Bayes and k-nearest neighbors classification models achieved an accuracy of 86.78% in ambulatory function classification. The decision-tree model achieved an identification accuracy of 83.80% in severity classification. A deep convolutional neural network model was established as the main structure of the deep-learning model while automatic auxiliary interpretation tasks of ambulatory function and severity were performed, and data augmentation was used to improve the recognition performance of the trained model. Both the visual geometry group (VGG)-16 and VGG-19 models achieved 98.53% accuracy in ambulatory-function classification. The VGG-19 model achieved 92.64% accuracy in severity classification. CONCLUSION: Regarding the overall results, the Kms and FCM clustering algorithms were used in this study to reconstruct the characteristic texture of the gastrocnemius muscle group in DMD, which was indeed helpful in quantitatively analyzing the deterioration of the gastrocnemius muscle group in patients with DMD at different stages. Subsequent combination of machine-learning and deep-learning technologies can automatically and accurately assist in identifying DMD symptoms and tracking DMD deterioration for long-term observation.

High incidence of cerebrovascular lesions on magnetic resonance imaging in pediatric COVID-19 during omicron outbreak - A retrospective case series.

BACKGROUND: The incidence of pediatric hospitalizations has significantly increased since the spread of the omicron variant of COVID-19. Changes of characteristics in respiratory and neurological symptoms have been reported. We performed a retrospective, cross-sectional study to characterize the MRI change in children with an emphasis on the change of cerebral vasculatures. METHODS: We retrospectively collected clinical and MRI data of 31 pediatric patients with neurological symptoms during the acute infection and abnormalities on MRI during the outbreak of omicron variant from April 2022 to June 2022 in Taiwan. The clinical manifestations and MRI abnormalities were collected and proportion of patients with vascular abnormalities was calculated. RESULTS: Among 31 pediatric patients with post-COVID-19 neurological symptoms, MRI abnormalities were observed in 15 (48.4%), predominantly encephalitis/encephalopathy (73.3%). Notable MRI findings included focal diffusion-weighted imaging (DWI) hyperintensity in cerebral cortex and thalamus, diffuse cortical T2/DWI hyperintensity, and lesions in the medulla, pons, cerebellum, and splenium of corpus callosum. Vascular abnormalities were seen in 12 (80%) patients with MRI abnormalities, mainly affecting the middle cerebral arteries. The spectrum of neurological manifestations ranged from seizures to Alice in Wonderland syndrome, underscoring the diverse impact of COVID-19 on pediatric patients. CONCLUSION: A high proportion of vascular abnormalities was observed in pediatric patients with neurological involvements, suggesting that vascular involvement is an important mechanism of neurological manifestations in omicron variant infection.

Computational fluid dynamics study in children with obstructive sleep apnea.

OBJECTIVES: This study aims to identify characteristics in image-based computational fluid dynamics (CFD) in children with obstructive sleep apnea (OSA). DESIGN: Diagnostic study. SETTING: Hospital-based cohort. PARTICIPANTS: Children with symptoms suggestive of OSA were recruited and underwent polysomnography. MAIN OUTCOME MEASURES: Three-dimensional models of computational fluid dynamics were derived from cone-beam computed tomography. RESULTS: A total of 68 children participated in the study (44 boys; mean age: 7.8 years), including 34 participants having moderate-to-severe OSA (apnea-hypopnea index [AHI] greater than 5 events/h), and 34 age, gender, and body mass index percentile matched participants having primary snoring (AHI less than 1). Children with moderate-to-severe OSA had a significantly higher total airway pressure (166.3 vs. 39.1 Pa, p = .009), total airway resistance (9851 vs. 2060 Newton-metre, p = .004) and velocity at a minimal cross-sectional area (65.7 vs. 8.8 metre per second, p = .017) than those with primary snoring. The optimal cut-off points for moderate-to-severe OSA were 46.2 Pa in the total airway pressure (area under the curve [AUC] = 73.2%), 2373 Newton-metre in the total airway resistance (AUC = 72.5%) and 12.6 metres per second in the velocity at a minimal cross-sectional area (AUC = 70.5%). The conditional logistic regression model revealed that total airway pressure, total airway resistance and velocity at minimal cross-sectional area were significantly associated with an increased risk of moderate-to-severe OSA. CONCLUSIONS: This study demonstrates that CFD could be a useful tool for evaluating upper airway patency in children with OSA.

Hybrid QUS Radiomics: A Multimodal-Integrated Quantitative Ultrasound Radiomics for Assessing Ambulatory Function in Duchenne Muscular Dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is a neuromuscular disorder that affects ambulatory function. Quantitative ultrasound (QUS) imaging, utilizing envelope statistics, has proven effective in diagnosing DMD. Radiomics enables the extraction of detailed features from QUS images. This study further proposes a hybrid QUS radiomics and explores its value in characterizing DMD. METHODS: Patients (n = 85) underwent ultrasound examinations of gastrocnemius through Nakagami, homodyned K (HK), and information entropy imaging. The hybrid QUS radiomics extracted, selected, and integrated the retained features derived from each QUS image for classification of ambulatory function using support vector machine. Nested five fold cross-validation of the data was conducted, with the rotational process repeated 50 times. The performance was assessed by averaging the areas under the receiver operating characteristic curve (AUROC). RESULTS: Radiomics enhanced the average AUROC of B-scan, Nakagami, HK, and entropy imaging to 0.790, 0.911, 0.869, and 0.890, respectively. By contrast, the hybrid QUS radiomics using HK and entropy images for diagnosing ambulatory function in DMD patients achieved a superior average AUROC of 0.971 (p < 0.001 compared with conventional radiomics analysis). CONCLUSIONS: The proposed hybrid QUS radiomics incorporates microstructure-related backscattering information from various envelope statistics models to effectively enhance the performance of DMD assessment.

Quantifying lower limb muscle stiffness in typically developing children and adolescents using acoustic radiation force impulse shear wave elastography (ARFI/SWE)-a pilot study.

OBJECTIVE: To investigate and quantify age-related changes in lower limb muscle stiffness in typically developing children and adolescents using acoustic radiation force impulse shear wave elastography. MATERIALS AND METHODS: Shear wave velocities of bilateral rectus femoris, tibialis anterior, and medial gastrocnemius muscles at rest were obtained in typically developing children and adolescents aged 3 to 18 years. The participants were classified into three age groups: Group 1 (children), 3 to 7 years old; Group 2, 8 to 12 (pre-adolescent); and Group 3 (adolescent), 13 to 18. The shear wave velocities of muscle were compared across the three age groups, as well as compared between right- and left-side limbs. The correlation between shear wave velocities and body weight or body mass index was assessed. RESULTS: Of the 47 participants, 21 were in Group 1, 17 in Group 2, and 9 in Group 3. There were no significant differences among the three age groups' shear wave velocities of bilateral lower limb muscles, and no significant differences between right and left sides. There was no correlation between muscle stiffness and body weight or body mass index. CONCLUSION: The present pilot study applied acoustic radiation force impulse shear wave elastography to quantify lower limb muscle stiffness in typically developing children and adolescents aged 3 to 18 years, suggesting no marked change in muscle stiffness occurs as they develop.

Acoustic radiation force impulse shear wave elastography quantifies upper limb muscle in patients with Duchenne muscular dystrophy.

We investigated whether the upper limb muscle stiffness quantified by the acoustic radiation force impulse shear wave elastography (ARFI/SWE) is a potential biomarker for age-related muscle alteration and functional decline in patients with Duchenne muscular dystrophy (DMD). 37 patients with DMD and 30 typically developing controls (TDC) were grouped by age (3-8, 9-11, and 12-18 years). ARFI/SWE measured the biceps and deltoid muscle's shear wave velocities (SWVs). Performance of Upper Limb Module (PUL 1.2 module) assessed muscle function in DMD patients. Mann Whitney test compared muscle SWVs between DMD and TDC, stratified by three age groups. We used analysis of variance with Bonferroni correction to compare muscle SWVs between DMD and TDC and correlated muscle SWVs with PUL results in the DMD group. Results showed that the SWVs of biceps differentiated DMD patients from TDC across age groups. Younger DMD patients (3-8 years) exhibited higher SWVs (p = 0.013), but older DMD patients (12-18 years) showed lower SWVS (p = 0.028) than same-aged TDC. DMD patients had decreasing biceps SWVs with age (p < 0.001), with no such age effect in TDC. The SWVs of deltoid and biceps positively correlated with PUL scores (r = 0.527 âˆ¼ 0.897, P < 0.05) and negatively correlated with PUL timed measures (r = -0.425 âˆ¼ -0.542, P < 0.05) in DMD patients. Our findings suggest that ARFI/SWE quantifying the SWVs in upper limb muscle could be a potential biomarker to differentiate DMD from TDC across ages and that DMD patients showed age-related muscle alteration and limb functional decline.

Phenotypic Heterogeneity in Patients with Mutations in the Mitochondrial Complex I Assembly Gene NDUFAF5.

BACKGROUND: Rare mutations in NADH:ubiquinone oxidoreductase complex assembly factor 5 (NDUFAF5) are linked to Leigh syndrome. OBJECTIVE: We aimed to describe clinical characteristics and functional findings in a patient cohort with NDUFAF5 mutations. METHODS: Patients with biallelic NDUFAF5 mutations were recruited from multi-centers in Taiwan. Clinical, laboratory, radiological, and follow-up features were recorded and mitochondrial assays were performed in patients' skin fibroblasts. RESULTS: Nine patients from seven unrelated pedigrees were enrolled, eight homozygous for c.836 T > G (p.Met279Arg) in NDUFAF5 and one compound heterozygous for p.Met279Arg. Onset age had a bimodal distribution. The early-onset group (age <3 years) presented with psychomotor delay, seizure, respiratory failure, and hyponatremia. The late-onset group (age ≥5 years) presented with normal development, but slowly progressive dystonia. Combing 25 previously described patients, the p.Met279Arg variant was exclusively identified in Chinese ancestry. Compared with other groups, patients with late-onset homozygous p.Met279Arg were older at onset (P = 0.008), had less developmental delay (P = 0.01), less hyponatremia (P = 0.01), and better prognosis with preserved ambulatory function into early adulthood (P = 0.01). Bilateral basal ganglia necrosis was a common radiological feature, but brainstem and spinal cord involvement was more common with early-onset patients (P = 0.02). A modifier gene analysis showed higher concomitant mutation burden in early-versus late-onset p.Met279Arg homozygous cases (P = 0.04), consistent with more impaired mitochondrial function in fibroblasts from an early-onset case than a late-onset patient. CONCLUSIONS: The p.Met279Arg variant is a common mutation in our population with phenotypic heterogeneity and divergent prognosis based on age at onset. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Five-year follow-up of a clinic-based sleep intervention for paediatric epilepsy: A randomized clinical trial.

Sleep disturbances in children with epilepsy are prevalent, and are associated with substantial adverse medical and psychosocial consequences. This study is a 5-year follow-up of a clinic-based sleep intervention study that randomized 100 toddlers and preschool-age children with epilepsy to a usual care group or a sleep intervention group. The intervention group received standard paediatric neurology care plus three education sessions during the child's routine clinic visit. The outcomes measured were: (1) child sleep by actigraphy and parental report; and (2) maternal sleep and depression. We aimed to evaluate the long-term benefits of a clinic-based sleep intervention for paediatric epilepsy. In total, 42 families (42.0%) participated. The average child's age at follow-up was 9.55 years. Thirty-eight (90.5%) children were not obtaining sufficient sleep at baseline, and 40 (95.2%) at the 5-year follow-up. The numbers of children with clinically significant sleep disturbances were 40 (95.2%) at baseline and 36 (85.7%) at the 5-year follow-up. Fourteen mothers (33.3%) had poor sleep quality and high depressive symptoms at both assessment time points. There were no differences (P > 0.05) in the child and maternal outcomes between the two trial arms. Overall, there was no evidence that a clinic-based sleep intervention that effectively improved multiple aspects of sleep in toddlers and preschool-age children with epilepsy had long-lasting beneficial effects. Our findings suggest that sleep interventions for families of children with epilepsy require ongoing reinforcement and monitoring during routine paediatric neurology care to prevent sleep problems from persisting or recurring.

Evaluating brain white matter hyperintensity, IQ scores, and plasma neurofilament light chain concentration in early-treated patients with infantile-onset Pompe disease.

PURPOSE: The study aimed to describe central nervous system (CNS) progression in patients with infantile-onset Pompe disease (IOPD) and explore the potential clinical impact and predictors. METHODS: Patients with IOPD treated with enzyme replacement therapy were longitudinally followed with brain magnetic resonance imaging (MRI) and evaluation for IQ scores from 2004 to 2021. Investigation of CNS involvement focused on white matter (WM) abnormalities and was quantified using a scoring system for metachromatic leukodystrophy. MRI scores were correlated with plasma neurofilament light chain (NfL) concentration and IQ scores. RESULTS: A total of 19 patients who started enzyme replacement therapy at a mean age of 26 days were analyzed; the median age at last examination was 12.1 (range = 1.7-19) years. MRI abnormalities were found in all patients, from supratentorial central WM to U-fibers, then to infratentorial WM, and eventually to gray matter. MRI scores progressed (n = 16) at variable rates (range = 0.8-2.7/y) and were positively correlated with age (n = 16) and negatively correlated with IQ scores (n = 8). Plasma NfL concentration was positively correlated with MRI scores (r2 = 0.8569; P < .001; n = 13). CONCLUSION: Our results suggest that the progression of CNS involvement in IOPD may be associated with neuroaxonal injury and decreased IQ scores. NfL could serve as a biomarker for CNS involvement in IOPD.

Measurements of craniofacial morphology using photogrammetry in children with sleep-disordered breathing.

OBJECTIVE: To assess the craniofacial morphology in children with sleep-disordered breathing (SDB) using nonradiation and readily accessible photogrammetry technique. METHODS: Included children aged 3-18 years with SDB-related symptoms from April 2019 to February 2020 in a tertiary center. All participants underwent craniofacial photogrammetry and overnight polysomnography (PSG). Participants were stratified into 2 groups (obstructive sleep apnea [OSA] group: apnea-hypopnea index [AHI] ≥ 1 and non-OSA group: AHI <1). Craniofacial photogrammetry was performed to derive variables of craniofacial features in standardized frontal and profile views. The 2 groups were propensity score matched based on age, sex, and body mass index (BMI) percentiles. Associations between craniofacial feature variables and OSA (AHI ≥1) likelihood were examined using logistic regression test. intraclass correlation coefficient (ICC) was used to evaluate the intrarater and interrater reliability. RESULTS: In total, 58 children were enrolled for the analysis after matching. All 3 variables representing the mandibular plane angle in the profile view were increased in the OSA group (mego-tn: 34.85 ± 5.99 vs 31.65 ± 5.96°, odds ratio [OR]: 1.10, 95% CI:1.02 to 1.18, P = .01; tn-gogn: 28.65 ± 6.38 vs 25.91 ± 5.38°, OR: 1.08, 95% CI:1.02 to 1.15, P = .012; and gome-tsup: 26.71 ± 6.13 vs 22.20 ± 5.89°, OR: 1.13, 95% CI:1.04 to 1.23, P = .003). CONCLUSIONS: Craniofacial photogrammetry revealed increased mandibular inclination in children with OSA. A steep mandibular plane with craniofacial photogrammetry is considered a potential predictor of pediatric OSA. Further investigation with a large sample size is required to clarify the validity of photogrammetry in evaluating pediatric OSA.

The diversity of hereditary neuromuscular diseases: Experiences from molecular diagnosis.

BACKGROUND: Hereditary neuromuscular diseases (NMDs) are a group of rare disorders, and the diagnosis of these diseases is a substantial burden for referral centers. Although next-generation sequencing (NGS) has identified a large number of genes associated with hereditary NMDs, the diagnostic rates still vary across centers. METHODS: Patients with a suspected hereditary NMD were referred to neuromuscular specialists at the National Taiwan University Hospital. Molecular diagnoses were performed by employing a capture panel containing 194 genes associated with NMDs. RESULTS: Among the 50 patients referred, 43 had a suspicion of myopathy, and seven had polyneuropathy. The overall diagnostic rate was 58%. Pathogenic variants in 19 genes were observed; the most frequent pathogenic variant found in this cohort (DYSF) was observed in only four patients, and 10 pathogenic variants were observed in one patient each. One case of motor neuron disease was clinically mistaken for myopathy. A positive family history increased the diagnostic rate (positive: 72.7% vs. negative: 56.3%). Fourteen patients with elevated plasma creatine kinase levels remained without a diagnosis. CONCLUSION: The application of NGS in this single-center study proves the great diversity of hereditary NMDs. A capture panel is essential, but high-quality clinical and laboratory evaluations of patients are also indispensable.

Changes in Cone-Beam Computed Tomography Pediatric Airway Measurements After Adenotonsillectomy in Patients With OSA.

Importance: Early intervention using cone-beam computed tomography (CBCT) and adenotonsillectomy for children with obstructive sleep apnea (OSA) may prevent impaired growth, adverse cardiovascular consequences, learning deficits, and poor quality of life. Objective: To assess changes in CBCT airway measurements and polysomnography (PSG) parameters that occur after adenotonsillectomy in children with OSA and to determine whether CBCT changes are correlated with apnea-hypopnea index (AHI) reduction. Design, Setting, and Participants: This prospective cohort study was conducted at a tertiary medical center from 2013 to 2016. Children aged 7 to 13 years with PSG-confirmed OSA (ie, AHI ≥1) were recruited. Data analysis was performed from March to July 2021. Exposures: All participants underwent CBCT and PSG before and after adenotonsillectomy. Main Outcomes and Measures: Changes in PSG and CBCT parameters after adenotonsillectomy were analyzed. Results: A total of 49 children (mean [SD] age, 9.5 [1.8] years; 34 boys [69.4%]) were recruited. Eighteen participants (36.7%) had obesity. After adenotonsillectomy, AHI significantly decreased from 11.4 to 1.2 events per hour (mean difference, -10.24 events per hour; 95% CI, -13.84 to -6.64 events per hour). The following CBCT parameters significantly increased: total airway volume (from 11 265 to 15 161 mm3; mean difference, 3896.6 mm3; 95% CI, 2788.0 to 5005.2 mm3), nasopharyngeal volume (from 2366 to 3826 mm3; mean difference, 1459.7 mm3; 95% CI, 1122.9 to 1796.5 mm3), minimal nasopharyngeal airway area (from 128 to 191 mm2; mean difference, 63.1 mm2; 95% CI, 47.4 to 78.8 mm2), mean nasopharyngeal airway area (from 144 to 231 mm2; mean difference, 86.8 mm2; 95% CI, 67.0 to 106.5 mm2), oropharyngeal volume (from 8898 to 11 335 mm3; mean difference, 2436.9 mm3; 95% CI, 1477.0 to 3396.8 mm3), minimal oropharyngeal airway area (from 82 to 158 mm2; mean difference, 76.2 mm2; 95% CI, 57.0 to 95.4 mm2), and mean oropharyngeal airway area (from 182 to 234 mm2; mean difference, 52.5 mm2; 95% CI, 33.6 to 71.4 mm2). Among all parameters, only body mass index percentile showed large effect size between the group with residual OSA (postoperative AHI ≥1) and the group with resolved disease, with the residual OSA group having a higher body mass index percentile (87.8 vs 61.4; mean difference, 26.33; 95% CI, 10.00 to 42.66). A quantile regression model revealed that total airway volume and minimal oropharyngeal airway area were significantly correlated with reductions in AHI. Conclusions and Relevance: These findings suggest that in children undergoing adenotonsillectomy, improvements in total airway volume and oropharyngeal minimal airway area were correlated with reduction of AHI. Future studies are needed to assess whether CBCT has a role in the evaluation of children with OSA who are being considered for adenotonsillectomy.

Duchenne muscular dystrophy newborn screening: the first 50,000 newborns screened in Taiwan.

BACKGROUND: Duchenne muscular dystrophy (DMD/Duchenne) is a progressive X-linked muscular disease with an overall incidence of 1:5,000 live male births. Recent availability in treatment for DMD raised the need of early diagnosis, and DMD became as a selective item of newborn screening (NBS) since Feb. 2021 in our center. MATERIALS AND METHODS: Dried blood spots (DBS) muscle-type creatine kinase (CK) isoform was measured with a commercialized kit with age-adjusted cutoffs. Subjects with an elevation of CK in the first screen were requested for a re-screen 2 weeks later. A DBS whole-exome sequencing (WES) panel for dystrophin and other neuromuscular-related genes was applied to confirm the diagnosis for subjects with persistent hyperCKemia. RESULTS: During a 1-year period, 50,572 newborns (male 26,130) received DMD screening at a mean age of 2 days (SD 1 day). Among them, 632 (1.2%) had an elevated CK value. A re-screen at a mean age of 14 days (SD 8 days) revealed 14 subjects with persistent hyperCKemia, and DMD was confirmed in 3 of them. The incidence of DMD in Taiwan was 1:8,710 (95% CI 1 in 2,963 to 1 in 25,610) live birth males. Results of DMD DBS also assisted in Pompe newborn screening. CONCLUSIONS: NBS for DMD enables earlier management of the disease. The high re-screening rate could potentially be waived by moving the DBS WES assay to a second-tier test. The long-term benefit and the impact of newborn screening on the prognosis of DMD, however, remain further elucidated.

Age- and gender-related characteristics in pediatric obstructive sleep apnea.

BACKGROUND: Age and gender disparities in polysomnographic findings in children are not well understood. OBJECTIVE: This study determined age and gender-related characteristics in pediatric obstructive sleep apnea (OSA). METHOD: Retrospectively, data were collected. We analyzed polysomnographic data in the following age groups: 3-6 years (n = 681), 6-9 years (n = 553), 9-12 years (n = 297), 12-15 years (n = 200), and 15-18 years (n = 111). RESULTS: A total of 1842 children were included (mean age: 8.0 years; boys: 67%; obesity: 21%). The apnea-hypopnea index (AHI) gradually increased with age (3-6, 6-9, 9-12, 12-15, and 15-18 years groups: 6.2, 5.9, 6.5, 8.1, and 9.9 event/h, respectively; p trend = 0.002). In all age groups, boys had a higher AHI than girls (7.8 vs. 4.1 events/h, p < 0.001). Children with obesity had a higher AHI than those without (12.9 vs. 4.9 events/h, p < 0.001). The mean AHI in the boys increased with age (3-6 to 15-18 years groups: 7.0-13.6 events/h, respectively; p trend < 0.001), whereas the mean AHI in the girls was not significantly different between ages (p trend = 0.492). In moderation analyses, gender was a moderator in the association between obesity and AHI, and the association between age and AHI during 12-15 and 15-18 years of ages. CONCLUSION: Male gender and obesity increase risk of OSA. Regarding age disparities, this study discovered a higher AHI in male adolescents than in young boys.

Office Blood Pressure Monitoring in Children with Obesity and Obstructive Sleep Apnea.

OBJECTIVE: To investigate the relative contributions of obesity and obstructive sleep apnea (OSA) to unfavorable blood pressure in children. STUDY DESIGN: Children aged 3-18 years with OSA-related symptoms were recruited. All children underwent office blood pressure (BP) monitoring and full-night polysomnography. Obesity was defined as a body mass index ≥95th percentile. OSA severity was divided into primary snoring (apnea-hypopnea index [AHI] <1), mild OSA (5> AHI ≥1), and moderate to severe OSA (AHI ≥5). Age- and sex-adjusted logistic regression analysis was performed to determine the associations among OSA, obesity, and elevated BP. RESULTS: This cross-sectional study enrolled 1689 children (66% boys), with a mean age of 7.9 years. Compared with children with primary snoring, children with moderate to severe OSA had significantly higher systolic BP (108.1 mmHg vs 105.6 mmHg), diastolic BP (75.0 mmHg vs 70.4 mmHg), systolic BP percentile (75.0 vs 70.4), and diastolic BP percentile (74.0 vs 69.2). The rate of unfavorable BP (ie, elevated BP or hypertension level BP) also was significantly higher in children with more severe OSA. Children with obesity had higher BP and BP percentile. Logistic regression analysis revealed that children with obesity and moderate to severe OSA have a 3-fold greater risk of unfavorable BP compared with children without obesity and primary snoring. CONCLUSIONS: We identified a 3-fold greater risk of unfavorable BP in children with obesity and moderate to severe OSA.

Ambulatory Blood Pressure Variability after Adenotonsillectomy in Childhood Sleep Apnea.

OBJECTIVE: To investigate the influence of adenotonsillectomy (T&A) on ambulatory blood pressure (BP) variability in children with obstructive sleep apnea (OSA). STUDY DESIGN: Prospective, interventional study. METHODS: Children with OSA symptoms were recruited from a tertiary center. After OSA diagnosis was confirmed (ie, apnea-hypopnea index [AHI] > 1), these children underwent T&A for treatment. We performed polysomnography and 24-hour recordings of ambulatory BP before and 3 to 6 months postoperatively. Ambulatory BP variability was presented as the standard deviation of mean blood pressure in the 24-hour monitoring of ambulatory BP. Differences in BP variability among different subgroups were tested using a multivariable linear mixed model. RESULTS: A total of 190 children were enrolled (mean age: 7.8 ± 3.3 years; 73% were boys; 34% were obese). The AHI significantly decreased from 12.3 ± 17.0 to 2.7 ± 5.5 events/hr after T&A. Overall, daytime, and nighttime ambulatory BP did not significantly change postoperatively, and overall, daytime, and nighttime ambulatory BP variability did not differ significantly preoperatively and postoperatively. In the subgroup analysis, children aged <6 years demonstrated a significantly greater decrease in ambulatory BP variability postoperatively than those aged >6 years (nighttime diastolic BP variability: 9.9 to 7.7 vs. 8.9 to 9.4). Children with hypertension also showed a significantly greater decrease in ambulatory BP variability than those without hypertension. CONCLUSIONS: We concluded that overall ambulatory BP variability does not significantly change after T&A in children with OSA. Moreover, young-aged and hypertensive children demonstrate a significant decrease in BP variability after T&A. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:2491-2497, 2022.

Hepatic Steatosis Assessment as a New Strategy for the Metabolic and Nutritional Management of Duchenne Muscular Dystrophy.

Growing evidence suggests that patients with Duchenne muscular dystrophy (DMD) have an increased risk of obesity and metabolic syndrome (MetS). The aim of this study was to investigate the potential risk factors for MetS and hepatic steatosis in patients with different stages of DMD. A total of 48 patients with DMD were enrolled and classified into three stages according to ambulatory status. Body mass index (BMI), serum fasting glucose, insulin, and lipid profiles including triglycerides (TG) and high-density lipoprotein were measured, and the homeostatic model assessment for insulin resistance (HOMA-IR) index was evaluated. Ultrasound examinations of the liver were performed to assess hepatic steatosis using the Nakagami parameter index (NPI). The results showed that BMI, TG, HOMA-IR, and ultrasound NPI differed significantly among DMD stages (p < 0.05). In contrast to the low rates of conventional MetS indices, including disturbed glucose metabolism (0%), dyslipidemia (14.28%), and insulin resistance (4.76%), a high proportion (40.48%) of the patients had significant hepatic steatosis. The ultrasound NPI increased with DMD progression, and two thirds of the non-ambulatory patients had moderate to severe hepatic steatosis. Steroid treatment was a risk factor for hepatic steatosis in ambulatory patients (p < 0.05). We recommend that DMD patients should undergo ultrasound evaluations for hepatic steatosis for better metabolic and nutritional management.

Patterns and Correlates of Changes in Emotional and Behavioral Problems Among Children with Congenital Heart Disease.

OBJECTIVE: The objective of this study was to examine patterns of changes in emotional and behavioral problems among children with congenital heart disease (CHD) and investigate associated factors in different domains. METHOD: The study sample comprised 327 pairs of children with CHD (aged 1.5-12 yrs) and their parents who participated in 2 waves of a survey in Taiwan from 2017 to 2019. The Child Behavior Checklist was used to evaluate the children's emotional and behavioral problems during both waves. The scores were later used to determine the patterns of changes in emotional and behavioral problems. Multinomial logistic regression was then applied to examine factors associated with varied patterns of changes in emotional and behavioral problems. RESULTS: Five patterns of changes in emotional and behavioral problems were identified: "persistent normal," "initial problematic," "worsening," "persistent problematic," and "subclinical." Among the factors examined, parenting stress was consistently associated with different patterns of change in emotional and behavioral problems. Specifically, children with parents who had higher levels of parenting stress were more likely to belong to the "initial problematic," "persistent problematic," or "subclinical" groups than to the "persistent normal" group. Other significant factors included sex, number of surgeries, and perceived health of parents. CONCLUSION: This study highlights the potential role of parenting stress in changes in emotional and behavioral problems. Accordingly, intervention programs aimed at relieving parenting stress may help reduce the development and worsening of emotional and behavioral problems among children with CHD.

C-reactive protein in children with obstructive sleep apnea and effects of adenotonsillectomy.

OBJECTIVE: C-reactive protein (CRP) is an important serum marker of inflammation associated with cardiovascular outcomes. This study aims to evaluate the association between CRP and childhood obstructive sleep apnea (OSA) and clarify the effects of adenotonsillectomy on serum CRP levels in children with OSA. METHODS: Children with symptoms suggestive of OSA who underwent an overnight polysomnography were recruited from a tertiary medical center. Their serum CRP levels were measured. For children who underwent adenotonsillectomy for OSA treatment, polysomnography and serum high-sensitivity CRP (hs-CRP) level measurement were conducted after surgery. RESULTS: This study included 326 children (mean age: 7.2 ±â€¯3.0 years; boys: 67%). Children with apnea-hypopnea index (AHI) > 5 events/h had significantly higher hs-CRP levels than children with AHI of 1-5 events/h and AHI < 1 event/h [median (interquartile range): 0.08 (0.03-0.25) vs 0.03 (0.02-0.14) vs 0.04 (0.01-0.10), P < 0.001]. Log-transformed hs-CRP levels were positively associated with log AHI values (r = 0.2, P < 0.001). In multiple linear regression analysis, hs-CRP levels were independently associated with AHI; 101 children with OSA (ie, AHI > 1) underwent adenotonsillectomy. In children with OSA in the study cohort, a significant reduction of hs-CRP levels did not occur after surgery [from 0.07 (0.02-0.22) to 0.08 (0.03-0.17), P = 0.716]. In children with OSA having abnormal hs-CRP levels (ie, CRP > 1 mg/dL), hs-CRP levels significantly decreased after surgery [from 1.87 (1.11-2.78) to 0.20 (0.07-1.04), P = 0.043]. CONCLUSION: Children with OSA had increased hs-CRP levels. Children with OSA and abnormal hs-CRP levels exhibited significantly reduced hs-CRP levels following adenotonsillectomy.

[Associations Between Daytime Physical Activity and Sleep Patterns in Children With Epilepsy].

BACKGROUND: Epilepsy is one of the most common chronic neurological diseases in children. Sleep disorders tend to increase the risk of seizures, and research has found that moderate physical activity may improve the quantity and quality of sleep in adults. However, the link between physical activity level and sleep patterns in toddlers and preschool-age children with epilepsy remains unclear. PURPOSE: To explore the association between level of physical activity and sleep patterns in toddlers and preschool-age children with epilepsy. METHODS: A cross-sectional, descriptive, correlational study was conducted. Ninety-eight children with epilepsy (1.5-6 years old) wore an actigraph for seven days. Additional data were collected using a health information datasheet, Children's Sleep Habits Questionnaire (CSHQ), and sleep diary, all of which were completed by the parents of each child. RESULTS: The results showed that the mean amount of time spent in moderate-to-vigorous physical activity per day was 36.00 ± 49.20 minutes and that only 23 children (23.5%) had a nighttime sleep efficiency greater than 85%. The overall CSHQ score (56.00 ± 5.69) indicated the presence of moderate to severe sleep disturbances. Multiple regression analysis showed the hours and percentage of moderate-to-vigorous physical activity to be positively associated with night sleep efficiency (ß = .54, p < .01; ß = .51, p < .01) and negatively associated with nighttime sleep hours (ß = -.55, p < .01; ß = -.52, p < .01), even after controlling for potential confounders. CONCLUSIONS / IMPLICATIONS FOR PRACTICE: Based on the findings, the sleep patterns and physical activity of children with epilepsy should be regularly assessed. Furthermore, appropriately increasing the duration of moderate-to-vigorous physical activity may improve sleep efficiency and prevent reductions in the duration of night sleep.