Is the Pediatric Sleep Questionnaire sensitive for sleep-disordered breathing in children with complex chronic disease?

PURPOSE: Sleep-disordered breathing (SDB) is a disease defined by breathing or breathing irregularities while asleep. The current study examines the association between results of polysomnography (PSG) and the Pediatric Sleep Questionnaire (PSQ), and the specificity and sensitivity of the PSQ for obstructive sleep apnea (OSA) in patients with chronic illnesses. METHODS: Demographic and clinical attributes, in addition to PSQ and PSG outcomes were examined retrospectively among patients who underwent polysomnography (PSG) at our facility between 2012 and 2021. RESULTS: Of 745 patients included in the study, 462 (62%) were male. The median age was 81 months (34-151 months). 117 of the patients (15/8%) had chronic lung disease, and 80 (10.7%) had cerebral palsy. The most common indications for PSG were symptoms of OSA (n = 426; 57.1%). According to obstructive apnea-hypopnea index (AHI), 361 patients (48.5%) had normal PSG. The median PSQ score was 0.40 (0.22-0.57). The sensitivity and specificity of the PSQ were 71.8% and 40.4%, respectively, for individuals aged 2 to 18 years. Among the disease subgroups, the cerebral palsy group had the highest sensitivity of PSQ (88.8%) for diagnosis of OSA. CONCLUSION: Questionnaires for evaluating SDB are not sensitive or specific for identification of OSA in children with chronic conditions, and PSG remains the best method.

Changing clinical characteristics of non-cystic fibrosis bronchiectasis in children.

BACKGROUND: The prevalence of non-cystic fibrosis (CF) bronchiectasis is increasing in both developed and developing countries in recent years. Although the main features remain similar, etiologies seem to change. Our aim was to evaluate the clinical and laboratory characteristics of our recent non-CF bronchiectasis patients and to compare these with our historical cohort in 2001. METHODS: One hundred four children with non-CF bronchiectasis followed between 2002 and 2019 were enrolled. Age of diagnosis, underlying etiology and microorganisms in sputum culture were recorded. Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last 12 months. RESULTS: Mean FEV1 and FVC %predicted at presentation improved compared to historical cohort (76.6 ± 17.1 vs. 63.3 ± 22.1 and 76.6 ± 15.1 vs. 67.3 ± 23.1, respectively; p <  0.001). There was a significant decrease in pulmonary exacerbation rate from 6.05 ± 2.88 at presentation to 3.23 ± 2.08 during follow-up (p <  0.0001). In 80.8% of patients, an underlying etiology was identified. There was an increase in primary ciliary dyskinesia (PCD) (32.7% vs. 6.3%; p = 0.001), decrease in idiopathic cases (19.2% vs. 37.8%; p = 0.03) with no change in postinfectious and immunodeficiencies as underlying etiology. Sputum cultures were positive in 77.9% of patients which was 46.9% in the historical cohort (p = 0.001). CONCLUSION: Baseline pulmonary function tests were better and distribution of underlying etiology had changed with a remarkable increase in diagnosis of PCD in the recent cohort.

Improvements in body mass index of children with cystic fibrosis following implementation of a standardized nutritional algorithm: A quality improvement project.

BACKGROUND: A collaboration between the University of Michigan (UM) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated in MU through conducting Quality Improvement projects (QIP). The global aim was to improve nutritional status of children with CF (cwCF), with a specific aim to increase the mean BMI percentile (BMIp) for cwCF by 10 percentile points in 12 months. METHODS: Body mass index (BMI) percentiles of cwCF were categorized as: nutritionally adequate (BMIp ≥ 50%); at risk (BMIp 25%-49%); urgently at risk (BMIp 10%-25%); critically at risk (BMIp < 10%). Appropriate interventions were made according to BMIp category every three months. Forced expiratory volume in one-second percent predicted (FEV1pp), and health-related quality of life (HRQoL) were evaluated. RESULTS: One hundred and eight-two cwCF with a mean age of 9.1 ± 4.3 years were included in the project. Baseline BMIp increased from 25.6 to 37.2 at the 12th month (p < 0.001). In the critically at-risk group BMIp increased from 3.6 to 20.5 (p < 0.001), in the urgently at risk group from 15.9 to 30.8 (p < 0.001), in the at risk group from 37.0 to 44.2 (p < 0.079) and in the nutritionally adequate group the increase was from 66.8 to 69.5 (p < 0.301). FEV1pp also improved significantly, from 81.3 ± 20.6 to 85.9 ± 20.8 (p < 0.001). Physical functioning, eating problems, and respiratory symptoms domains of the HRQoL evaluation improved (p < 0.05). CONCLUSION: This project has led to significant improvements in BMIp, FEV1pp and HRQoL of cwCF; similar projects could easily be implemented by centers in other developing countries.

Depression, anxiety, and sleep quality of caregivers of children with spinal muscular atrophy.

BACKGROUND: The aim of this study was to evaluate the prevalence of anxiety, depression, sleep, and associated factors in caregivers of children with spinal muscular atrophy (SMA). MATERIALS AND METHODS: Beck Depression Inventory (BDI), the State-Trait Anxiety Inventory-State (STAI-S), the State-Trait Anxiety Inventory-Trait (STAI-T), and Pittsburgh Sleep Quality Index (PSQI) were used to assess the anxiety, depression, and sleep quality of the caregivers of children with SMA. Higher scores indicated worse outcome for all three questionnaires. RESULTS: Fifty-six caregivers of children with SMA were included in the study. Median age of children was 6 (3.2-10) years and mean age of the caregivers was 37.0 ± 6.5 years. Median scores of the BDI, STAI-S, STAI-T, and PSQI were 12 (7.2-17), 35.5 (31-44), 40.5 (35-48), and 7.0 (5.0-10.0), respectively. There was a positive correlation between BDI and PSQI scores (p < 0.05). There was a negative correlation between the age of the caregivers and PSQI, BDI, STAI-T scores (p = 0.01, r = -0.341; p = 0.006, r = -0.364; p = 0.003, r = -0.395, respectively). There was a negative correlation between the age of the patients and the PSQI scores of the caregivers (p = 0.01, r = -0.33). There was a negative correlation between BDI scores and household income (p = 0.01, r = -0.34). CONCLUSION: Caregivers of children with SMA had elevated depression and anxiety levels and they also had decreased sleep quality. Economic and social support resources are needed to help caregivers of those children.