Narong Simakajornboon’s research while affiliated with Cincinnati Children's Hospital Medical Center and other places

Sleep-related movement disorders (SRMDs), such as restless legs syndrome (RLS) and periodic limb movement disorder (PLMD), are common in pediatric sleep practice. There is increasing literature on RLS, PLMD, and a newly described sleep disorder called “restless sleep disorder (RSD)”. We aimed to review and provide recent updates on SRMDs. A comprehensive search for relevant English-language peer-reviewed publications focused on three common SRMDs, namely, RLS, PLMD and RSD, in a variety of indices in PubMed and SCOPUS. Both relevant databases and systematic reviews are included. SRMDs, especially RLS and PLMD, are common in children and adolescents. However, they are underrecognized. Genetics, abnormal dopaminergic functions, and iron deficiency are the main pathophysiologies of RLS and PLMD. RLS and RSD may share common pathophysiologic mechanisms, as evidenced by low iron stores in both conditions. The diagnoses of RLS, PLMD, and RSD require specific clinical criteria and polysomnographic features. Several comorbid conditions have been associated with RLS, PLMD, and RSD. Iron therapy has been shown to be effective for treating RLS, PLMD, and RSD. There is increasing evidence on the effectiveness of specific medications in children with RLS and PLMD, but the data are still limited. This review summarizes the pathophysiology, clinical manifestations, diagnostic criteria, and management of RLS, PLMD, and RSD in children based on relevant and recent literature. It is important for pediatricians to recognize the clinical presentation of RLS, PLMD, and RSD to facilitate early diagnosis. Further studies are needed to examine the pathogenesis, long-term consequences, and pharmacologic therapy of RSD in children.

Current evidence suggests that iron deficiency (ID) plays a key role in the pathogenesis of conditions presenting with restlessness such as attention deficit hyperactivity disorder (ADHD) and restless legs syndrome (RLS). In clinical practice, ID and iron supplementation are not routinely considered in the diagnostic work-up and/or as a treatment option in such conditions. Therefore, we conducted a scoping literature review of ID guidelines. Of the 58 guidelines included, only 9 included RLS, and 3 included ADHD. Ferritin was the most frequently cited biomarker, though cutoff values varied between guidelines and depending on additional factors such as age, sex, and comorbidities. Recommendations surrounding measurable iron biomarkers and cutoff values varied between guidelines; moreover, despite capturing the role of inflammation as a concept, most guidelines often did not include recommendations for how to assess this. This lack of harmonization on the interpretation of iron and inflammation biomarkers raises questions about the applicability of current guidelines in clinical practice. Further, the majority of ID guidelines in this review did not include the ID-associated disorders, ADHD and RLS. As ID can be associated with altered movement patterns, a novel consensus is needed for investigating and interpreting iron status in the context of different clinical phenotypes.

Introduction Obstructive sleep apnea (OSA) in infants is treated with low flow oxygen via nasal cannula (NC), CPAP (continous positive airway pressure), or surgery. Literature supports the use of high flow NC (HFNC) in children in the outpatient setting, however there is limited data on the use of HFNC in infants. Objective The purpose of this study was to compare HFNC and low‐flow oxygen as treatments for OSA in infants. Methods A prospective pilot study was performed at two institutions. Infants with primarily OSA underwent a 3–4 h sleep study with HFNC titration at 6−14 lpm for OSA, followed by clinical polysomnography (PSG) for oxygen titration (1/8−1 lpm). Infants with primarily central apnea were excluded. Results Nine infants were enrolled, with a mean age of 1.3 ± 1.7 months. Average apnea hypopnea index (AHI), average obstructive apnea hypopnea index (OAHI) and average central apnea index during the diagnostic PSG was 17.2 ± 7/h, 13.4 ± 5.4/h and 3.7 ± 4.8/h respectively. OSA improved in 44.4% of subjects with HFNC; the mean AHI and OAHI decreased from 15.6 ± 5.65/h and 12.8 ± 4.4/h on diagnostic PSG to 5.12 ± 2.5/h and 4.25 ± 2.5/h on titration PSG. OSA improved universally with low flow oxygen; the mean AHI decreased from 17.2 ± 7/h on diagnostic PSG to 4.44 ± 3.6/h on titration PSG. Conclusion HFNC reduced OSA in some infants, though low flow oxygen reduced OSA in all subjects. Respiratory instability (high loop gain) in infants may explain why infants responded to low flow oxygen. More studies are needed to determine if HFNC is beneficial in selected groups of infants with OSA.

Objective Sleep problems constitute a common and heterogeneous complaint in pediatric palliative care (PPC), where they often contribute to disease morbidity and cause additional distress to children and adolescents and their families already facing the burden of life-threatening and life-limiting conditions. Despite the significant impact of sleep problems, clinical evidence is lacking. The application of general pediatric sleep recommendations appears insufficient to address the unique challenges of the PPC dimension in terms of disease variability, duration, comorbidities, complexity of needs, and particular features of sleep problems related to hospice care. Therefore, we initiated an international project aimed at establishing a multidisciplinary consensus. Methods A two-round Delphi approach was adopted to develop recommendations in the areas of Definition, Assessment/Monitoring, and Treatment. After selecting a panel of 72 worldwide experts, consensus (defined as ≥75% agreement) was reached through an online survey. Results At the end of the two voting sessions, we obtained 53 consensus recommendations based on expert opinion on sleep problems in PPC. Conclusions This study addresses the need to personalize sleep medicine's approach to the palliative care setting and its peculiarities. It provides the first international consensus on sleep problems in PPC and highlight the urgent need for global guidance to improve sleep-related distress in this vulnerable population and their caregivers. Our findings represent a crucial milestone that will hopefully enable the development of guidelines in the near future.

Introduction Narcolepsy affects 25-100/100,000 people in the US. Daytime sleepiness associated with pediatric narcolepsy negatively impacts quality of life (QoL), academics, vocational function, and personal safety. The Epworth sleepiness scale (ESS) is a validated tool that measures daytime sleepiness. We aimed to improve daytime sleepiness in children with narcolepsy at our center using quality improvement process to increase the percentage of narcolepsy patients with at least a 30% improvement of ESS from baseline. Methods We created a multidisciplinary quality improvement (QI) team in 2019 and used standard QI methods. Key drivers included access to care, patient and family engagement in care, follow up tracking, and complete provider documentation. We conducted plan-do-study-act (PDSA) cycles and tracked progress with a run chart. Interventions included pre-visit planning tool enhancement, tracking duration between visits and missed clinic visits, contacting patients with ≥2 missed visits, tracking prescriptions, identifying patients without referrals for behavioral/psychological support, and treating comorbid sleep disorders. Process measures included the ESS completion rate and days between visits. The primary outcome measure was improvement on the ESS. A secondary outcome measure was improvement in QoL (10% improvement in the PedsQL score). Results Between 2019 and 2023, 101 patients had 605 visits. The ESS completion rate (>90% pre-COVID), decreased during COVID to 57% and improved to a median of 70% in 2023. The ESS completion rate was lower for telehealth (36%) than in-person visits (90%). Pre-COVID, the median days between visits was below goal (< 200 days) at 156 days but increased to 210 days in December 2020. Approximately three years after project initiation, 62% of patients had at least a 30% improvement in the ESS score. Improvements were sustained over 16 months. Average decrease in ESS score was 9.9 points (score range: 0-24)since 1/2020. The median baseline PedsQL score was 64.7 (borderline unhealthy) and increased by 14%. Conclusion Using QI methods and multiple interventions, we increased the percentage of narcolepsy patients with at least a 30% improvement in the ESS score to >60%. Ongoing challenges include accommodating increased patient volume, reducing process variation in ESS completion, and standardizing documentation practices for ESS scores. Support (if any)

Introduction Increased periodic limb movements of sleep (PLMS) can occur in patients with sleep disordered breathing. Changes in PLMS following treatment for obstructive sleep apnea (OSA) are observed, but poorly understood. In adults, PLMS are typically seen in the first portion of the night whereas OSA events occur most frequently during the latter portion. To better understand the link between pediatric PLMS and OSA, we investigated whether nighttime distribution of pediatric PLMS could predict resolution of PLMS following surgical management of OSA. Methods A retrospective study was performed in children and adolescents aged 1-18-year-olds who had pre-operative PSG showing OSA (OAHI ≥1.5/hr) and significant PLMS (PLM index [PLMI] ≥5/hr) before undergoing surgical treatment for OSA and subsequent post-operative PSG. The cohort was divided into 2 groups based on post-operative PSGs; (1) sustained PLMS with PLMI index >5/hr post-operatively and (2) resolved PLMS with PLM index < 5/hr postoperatively. PLMS distribution was qualified by calculating PLMI during each third of the night of pre-operative PSG. The age, PSG parameters and PLMS distribution were compared between patients with sustained PLMS and resolved PLMS. Results Twenty-seven subjects met the criteria for entry into analysis; 14 patients with sustained PLMS and 13 patients with resolved PLMS. Sustained PLMS had higher overall PLMI compared to resolved PLMS (15.13±8.02/hr vs. 9.77±5.88/hr, p=0.03). Sustained PLMS had a higher PLMI during the first third of the night on pre-operative PSG compared to resolved PLMS (15.99±10.37/hr vs. 6.05±8.02/hr, p=0.01). There were no significant differences in PLMS in the middle and last thirds of the night, BMI, age (6.37±4.20 vs. 5.07±4.10 years) at sleep study, AHI, or OAHI (8.11 vs. 6.04). Conclusion Children with PLMS that resolved after surgical treatment for OSA have less frequent PLMS in the early part of the night and a lower overall PLMI compared to children with sustained PLMS. This suggests that PLMS occurring in the first third of the night are independent of sleep disordered breathing and are likely associated with other conditions such as sleep related movement disorders. The distribution of PLMS may help prioritize children with OSA and PLMS who require further evaluation of RLS/PLMD post-operatively. Support (if any)