Polysomnographic findings in children with 22q deletion & duplication syndrome: relationship to genetic diagnosis, parent-reported symptoms, and calcium levels.

PURPOSE: 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome. In the current study, we assessed the relationship between parent-reported symptoms of obstructive sleep apnea (OSA) and polysomnographic (PSG) results in patients with 22q11.2DS. Additionally, we explored the relationships between genetic diagnosis, serum calcium and ferritin levels, and PSG results. METHODS: Retrospective chart review was completed for patients enrolled in our 22q Center's registry from 2015-2021. Data extracted included: patient characteristics, parent-reported sleep symptoms from the Childhood Sleep Habits Questionnaire (CSHQ), serum calcium and ferritin levels, and results from formal PSG. RESULTS: Overall, n = 89 encounters (60 unique patients) with PSG data demonstrated that there were no differences in OSA between those with deletion vs duplication, but PLMD was more common in those with deletion (35% vs 7%, p = 0.032). In a subset of n = 24 encounters with PSG and survey data in proximity, there were no significant associations between the CSHQ sleep-disordered breathing subscale and OSA presence or severity (p = 0.842). Likewise, we found no significant associations between the individual symptoms of OSA and PSG results (all p > 0.5). In those patients with available calcium (n = 44) and ferritin (n = 17) levels, we found a significant negative correlation between serum calcium and PLMS (r = -0.446, p = 0.002), but not ferritin (r = -0.067, p = 0.797) levels. CONCLUSIONS: Parent-reported symptoms do not predict the presence or severity of OSA in children with 22q11.2DS. There was a negative correlation between serum calcium, but not ferritin, and PLMS on PSG.

Nf1-dependent tumors require a microenvironment containing Nf1+/-- and c-kit-dependent bone marrow.

Interactions between tumorigenic cells and their surrounding microenvironment are critical for tumor progression yet remain incompletely understood. Germline mutations in the NF1 tumor suppressor gene cause neurofibromatosis type 1 (NF1), a common genetic disorder characterized by complex tumors called neurofibromas. Genetic studies indicate that biallelic loss of Nf1 is required in the tumorigenic cell of origin in the embryonic Schwann cell lineage. However, in the physiologic state, Schwann cell loss of heterozygosity is not sufficient for neurofibroma formation and Nf1 haploinsufficiency in at least one additional nonneoplastic lineage is required for tumor progression. Here, we establish that Nf1 heterozygosity of bone marrow-derived cells in the tumor microenvironment is sufficient to allow neurofibroma progression in the context of Schwann cell Nf1 deficiency. Further, genetic or pharmacologic attenuation of c-kit signaling in Nf1+/- hematopoietic cells diminishes neurofibroma initiation and progression. Finally, these studies implicate mast cells as critical mediators of tumor initiation.

Survival kinetics of Listeria monocytogenes and Salmonella enterica on dehydrated enoki and wood ear mushrooms during long-term storage.

Two specialty mushrooms have recently become novel vectors for foodborne outbreaks in the U.S.: fresh enoki and dried wood ear mushrooms were linked to a listeriosis and salmonellosis outbreak, respectively. The aim of this study was to evaluate the survival kinetics of Listeria monocytogenes and Salmonella enterica on dehydrated enoki and wood ear mushrooms during long-term storage. Following heat dehydration, mushrooms were inoculated with either L. monocytogenes or S. enterica, allowed to dry for 1 h, and then stored for up to 180 d at 25 °C and 33% relative humidity. Both pathogens were enumerated from the mushrooms at intervals during the storage period. Survival kinetics of both pathogens were modeled using both the Weibull and log-linear with tail models. After inoculation and 1 h drying, both pathogen populations decreased 2.26-2.49 log CFU/g on wood ear mushrooms; no decrease was observed on enoki. Both pathogens survived during storage on both mushroom types. On wood ear mushrooms, a 2-log decrease of both pathogens occurred during storage. On enoki mushrooms, 4-log decreases of both pathogens were modeled to occur after 127.50-156.60 d. The results of this study suggest that L. monocytogenes and S. enterica can persist on dehydrated specialty mushrooms during long-term storage.

Enuresis and sleep fellowship education.

PURPOSE: Enuresis is a common sleep-related concern in school aged children that gradually decreases into adulthood. We performed a survey of sleep providers in order to assess their comfort level in managing patients with enuresis. METHODS: Survey participants were recruited via the Pedsleep listserv and sleep medicine program directors in the USA on basecamp application. The Pedsleep list includes a mixture of physicians, psychologists, and other sleep providers/researchers. RESULTS: Forty-two sleep providers completed the survey. Forty (95%) were board certified in sleep medicine, and 32 (76%) were board certified in pediatrics. Practice patterns for management of enuresis varied among respondents, with 69% who evaluate for possible contributions from other sleep disorders such as obstructive sleep apnea then refer for additional management. Nineteen (45%) respondents felt that they received inadequate or very inadequate training during their sleep fellowship for management of enuresis. While 83% of respondents worked in an academic medical center setting, none of their respective sleep clinics were the primary managing clinic at their own situation. Participants who endorsed their training as adequate/very adequate were significantly more likely to feel comfortable/very comfortable managing enuresis (90% vs 37.5%, p = 0.009). CONCLUSIONS: A large percentage of sleep providers are lacking essential training to manage enuresis patients. These results suggest the need for additional educational initiatives in this area.

Rise in Babesiosis Cases, Pennsylvania, USA, 2005-2018.

Babesiosis is an emerging infection in the state of Pennsylvania, and clinicians need to be made aware of its clinical manifestations as well as the risk factors associated with severe disease. Before 2010, our tertiary academic center in central Pennsylvania previously saw zero cases of babesiosis. We saw our first confirmed case of Babesia infection acquired in Pennsylvania in 2011; we recorded 2 confirmed cases in 2017 and 4 confirmed cases in 2018. All 4 cases from 2018 were thought to be acquired in southcentral Pennsylvania counties, whereas prior reports of cases were predominately in the southeast and northeast counties of the state.

Early administration of imatinib mesylate reduces plexiform neurofibroma tumor burden with durable results after drug discontinuation in a mouse model of neurofibromatosis type 1.

BACKGROUND: Neurofibromatosis type 1 (NF1) is a common genetic disorder characterized by plexiform neurofibromas (pNF), which are thought to be congenital tumors that arise in utero and enlarge throughout life. Genetic studies in murine models delineated an indispensable role for the stem cell factor (SCF)/c-kit pathway in pNF initiation and progression. A subsequent phase 2 clinical trial using imatinib mesylate to inhibit SCF/c-kit demonstrated tumor shrinkage in a subset of preexisting pNF; however, imatinib's role on preventing pNF development has yet to be explored. PROCEDURE: We evaluated the effect of imatinib dosed at 10-100 mg/kg/day for 12 weeks to one-month-old Nf1flox/flox ;PostnCre(+) mice, prior to onset of pNF formation. To determine durability of response, we then monitored for pNF growth at later time points, comparing imatinib- with vehicle-treated mice. We assessed gross and histopathological analysis of tumor burden. RESULTS: Imatinib administered preventatively led to a significant decrease in pNF number, even at doses as low as 10 mg/kg/day. Tumor development continued to be significantly inhibited after cessation of imatinib dosed at 50 and 100 mg/kg/day. In the cohort of treated mice that underwent prolonged follow-up, the size of residual tumors was significantly reduced as compared with age-matched littermates that received vehicle control. CONCLUSIONS: Early administration of imatinib inhibits pNF genesis in vivo, and effects are sustained after discontinuation of therapy. These findings may guide clinical use of imatinib in young NF1 patients prior to the substantial development of pNF.

Hierarchical and Mediation Analysis of Disparities in Very Short Sleep among Sexual Minority Youth in the U.S., 2015.

BACKGROUND: Insufficient sleep is associated with increased risk of chronic diseases, substance use, and unintentional injuries. Little is known about the disparities in short sleep among sexual minority youth. METHODS: A nationally representative sample of U.S. students in grades 9-12 (n = 14,703) from the 2015 Youth Risk Behavior Survey was analyzed to examine the prevalence and risk factors of short sleep. Self-reported sleep duration (very short: ≤5 h, short: 6-7 h, normal: ≥8 h per day) were compared by sex group (male vs. female) and sexual orientation (heterosexual, gay, lesbian, bisexual, and unsure). RESULTS: Of all respondents, 88.8% were heterosexual/straight, 2.0% were lesbian or gay, 6.0% were bisexual, and 3.2% were unsure about their sexual identity. Bisexual and unsure girls (36.2%, 95% CI [31.3-41.0] and 33.7%, CI [25.6-41.8], respectively) had a higher prevalence of very short sleep duration than straight girls (19.8%, CI [18.3-21.4]). Gay and unsure boys (38.5%, CI [25.6-51.5] and 33.3%, CI [23.5-32.1], respectively) had a higher prevalence of very short sleep duration than straight boys (16.5%, CI [15.1-17.9]). The effects of sexual minority status on very short sleep duration attenuated when incrementally adjusting for influencing factors, and further analysis identified that feeling sad/hopeless had the largest standardized mediation effect. CONCLUSIONS AND RELEVANCE: Sexual minority adolescents had a higher prevalence of reporting very short sleep duration as compared to their straight peers, and the effects were mediated by influencing variables including demographic factors, substance use, excessive media use, and experiences of victimization/mental health problems.

A Predictive Model for Survival of Escherichia coli O157:H7 and Generic E. coli in Soil Amended with Untreated Animal Manure.

This study aimed at developing a predictive model that captures the influences of a variety of agricultural and environmental variables and is able to predict the concentrations of enteric bacteria in soil amended with untreated Biological Soil Amendments of Animal Origin (BSAAO) under dynamic conditions. We developed and validated a Random Forest model using data from a longitudinal field study conducted in mid-Atlantic United States investigating the survival of Escherichia coli O157:H7 and generic E. coli in soils amended with untreated dairy manure, horse manure, or poultry litter. Amendment type, days of rain since the previous sampling day, and soil moisture content were identified as the most influential agricultural and environmental variables impacting concentrations of viable E. coli O157:H7 and generic E. coli recovered from amended soils. Our model results also indicated that E. coli O157:H7 and generic E. coli declined at similar rates in amended soils under dynamic field conditions.The Random Forest model accurately predicted changes in viable E. coli concentrations over time under different agricultural and environmental conditions. Our model also accurately characterized the variability of E. coli concentration in amended soil over time by providing upper and lower prediction bound estimates. Cross-validation results indicated that our model can be potentially generalized to other geographic regions and incorporated into a risk assessment for evaluating the risks associated with application of untreated BSAAO. Our model can be validated for other regions and predictive performance also can be enhanced when data sets from additional geographic regions become available.

On the Use of a Single Beam Acoustic Current Profiler for Multi-Point Velocity Measurement in a Wave and Current Basin.

Harnessing the energy of tidal currents has huge potential as a source of clean renewable energy. To do so in a reliable and cost effective way, it is critical to understand the interaction between tidal turbines, waves, and turbulent currents in the ocean. Scaled testing in a tank test provides a controlled, realistic, and highly reproducible down-scaled open ocean environment, and it is a key step in gaining this understanding. Knowledge of the hydrodynamic conditions during tests is critical and measurements at multiple locations are required to accurately characterise spatially varying flow in test tank facilities. The paper presents a laboratory technique using an acoustic velocimetry instrument, the range over-which measurements are acquired being more akin to open water applications. This enables almost simultaneous multi-point measurements of uni-directional velocity along a horizontal profile. Velocity measurements have been obtained from a horizontally mounted Single Beam Acoustic Doppler (SB-ADP) profiler deployed in the FloWave Ocean Energy Research Facility at the University of Edinburgh. These measurements have been statistically compared with point measurements obtained while using a co-located Acoustic Doppler Velocimeter (ADV). Measurements were made with both instruments under flow velocities varying from 0.6 ms-1 to 1.2 ms-1, showing that flow higher than 1 ms-1 was more suitable. Using a SB-ADP has shown the advantage of gaining 54 simultaneous measurement points of uni-directional velocity, covering a significant area with a total distance of 10 m of the test-tank, at a measurement frequency of 16 Hz. Of those measurement points, 41 were compared with co-located ADV measurements covering 8 m of the profile for a tank nominal flow velocity of 0.8 ms-1, and four distributed locations were chosen to to carry out the study at 0.6 ms-1, 1.0 ms-1, and 1.2 ms-1. The comparison with the ADV measurement showed a 2% relative bias on average.

Does Tonsillectomy Increase Obesity Risk in Children with Down Syndrome?

OBJECTIVES: To examine weight changes relative to surgical success in children with Down syndrome and obstructive sleep apnea (OSA). STUDY DESIGN: Retrospective chart review of children with Down syndrome undergoing tonsillectomy from 2005 to 2016 for OSA at a tertiary care children's hospital. Only patients with pre-and postoperative polysomnogram within 6 months of tonsillectomy were included. Demographics, weight, height, and polysomnogram data were collected. Body mass index (BMI), expressed as a percentage of the 95th percentile (%BMIp95), was calculated for 24 months prior to and following surgery. Pre-and postoperative OSA severity were also recorded. The postoperative obstructive/hypopnea index identified subjects with resolution of obstruction (obstructive/hypopnea index <2 events/hour) or persistent mild/moderate/severe obstructive apnea. Regression analyses were used to compare %BMIp95 pre- and post-tonsillectomy with %BMIp95 by OSA status following tonsillectomy. RESULTS: A total of 78 patients with Down syndrome whose mean age was 5.29 years at time of tonsillectomy were identified. There was no difference between best-fit curves of %BMI p95 pre-and post-tonsillectomy. There was no difference between best-fit curves of %BMI p95 in patients who saw resolution of OSA after tonsillectomy vs patients with residual OSA. CONCLUSIONS: Tonsillectomy neither alters the BMI trajectory of children with Down syndrome, nor changes differentially the risk for obesity in children whose OSA did or did not resolve after surgery.

Assessing readiness to drive in adolescents with narcolepsy: what are providers doing?

PURPOSE: There are no universally accepted guidelines for assessing driving readiness in adolescents with narcolepsy. The purpose of the present study was to survey pediatric sleep medicine providers regarding their current practice patterns for assessing driving readiness in adolescents with narcolepsy, knowledge of their state laws regarding physician reporting of unsafe drivers, and opinions regarding what physician duty ought to be. METHODS: This was an anonymous web-based survey distributed via the PedSleep listserv, which serves as a hub of communication for pediatric sleep medicine providers. RESULTS: A total of 52 pediatric sleep providers from 25 different states completed the survey. Eighty-eight percent of providers routinely assess driving readiness in adolescents with narcolepsy. Factors rated as "absolutely essential" by at least 50% of respondents included the following: history of previous fall-asleep crash or near miss, sleepiness (reported by patient), sleepiness (reported by caregiver), and cataplexy (reported by patient). Providers included maintenance of wakefulness testing: never (34%), if patient reports no/mild sleepiness (10%), if patient reports moderate/severe sleepiness (25%), or always regardless of patient symptoms (30%), and the median minimally acceptable result was 30 min (25-75th: 20-40 min). There was substantial lack of knowledge regarding legal obligations for reporting. CONCLUSIONS: These results demonstrate great variability in practice patterns among pediatric sleep medicine providers for assessing driving readiness in adolescents with narcolepsy. In addition, it shows limited knowledge of the providers about their respective states' laws. Further studies are required to identify the best approach to assess residual sleepiness in this population.

When morphological ability exceeds syntactic ability: A case study.

This article addresses the question of whether children classified as having a specific language impairment are such due to a particular problem in inflectional morphology. This has been claimed to be the case for some time. An effort is made here to propose that there may be an age effect behind that result. To support this possibility, a case study of a much older child with specific language impairment is presented. The participant is Tom, a child with a language impairment who underwent language intervention between the ages 9;4 and 10;3. During that period, language samples were taken at five different times (MLUs 5.3-6.0). They were analyzed to examine both Tom's morphological and syntactic abilities. The results indicated that he did quite well with English noun and verb inflections, but was unable to generate complex sentence structures. If there were a period when he had such difficulties, it was overcome by the time of the sampling. A lexical explanation is given which argues that bound morphology will appear better than syntax in cases where children are older and thus have had time to acquire the various inflected words as individual lexical items.

Nf1+/- monocytes/macrophages induce neointima formation via CCR2 activation.

Persons with neurofibromatosis type 1 (NF1) have a predisposition for premature and severe arterial stenosis. Mutations in the NF1 gene result in decreased expression of neurofibromin, a negative regulator of p21(Ras), and increases Ras signaling. Heterozygous Nf1 (Nf1(+/-)) mice develop a marked arterial stenosis characterized by proliferating smooth muscle cells (SMCs) and a predominance of infiltrating macrophages, which closely resembles arterial lesions from NF1 patients. Interestingly, lineage-restricted inactivation of a single Nf1 allele in monocytes/macrophages is sufficient to recapitulate the phenotype observed in Nf1(+/-) mice and to mobilize proinflammatory CCR2+ monocytes into the peripheral blood. Therefore, we hypothesized that CCR2 receptor activation by its primary ligand monocyte chemotactic protein-1 (MCP-1) is critical for monocyte infiltration into the arterial wall and neointima formation in Nf1(+/-) mice. MCP-1 induces a dose-responsive increase in Nf1(+/-) macrophage migration and proliferation that corresponds with activation of multiple Ras kinases. In addition, Nf1(+/-) SMCs, which express CCR2, demonstrate an enhanced proliferative response to MCP-1 when compared with WT SMCs. To interrogate the role of CCR2 activation on Nf1(+/-) neointima formation, we induced neointima formation by carotid artery ligation in Nf1(+/-) and WT mice with genetic deletion of either MCP1 or CCR2. Loss of MCP-1 or CCR2 expression effectively inhibited Nf1(+/-) neointima formation and reduced macrophage content in the arterial wall. Finally, administration of a CCR2 antagonist significantly reduced Nf1(+/-) neointima formation. These studies identify MCP-1 as a potent chemokine for Nf1(+/-) monocytes/macrophages and CCR2 as a viable therapeutic target for NF1 arterial stenosis.

Successive Surface Reactions on Hydrophilic Silica for Modified Magnetic Nanoparticle Attachment Probed by Sum-Frequency Generation Spectroscopy.

Successive surface reactions on hydrophilic silica substrates were designed and performed to immobilize ethanolamine-modified magnetic ferrite-based nanoparticle (NP) for surface characterization. The various surfaces were monitored using sum-frequency generation (SFG) spectroscopy. The surface of the hydrophilic quartz substrate was first converted to a vinyl-terminated surface by utilizing a silanization reaction, and then, the surface functional groups were converted to carboxylic-terminated groups via a thiol-ene reaction. The appearance and disappearance of the vinyl (═CH2) peak at ∼2990 cm-1 in the SFG spectra were examined to confirm the success of the silanization and thiol-ene reactions, respectively. Acyl chloride (-COCl) formation from carboxy (-COOH) functional group was then performed for further attachment of magnetic amine-functionalized magnesium ferrite nanoparticles (NPs) via amide bond formation. The scattered NPs attached on the modified silica substrate was then used to study the changes in the spectral profile of the ethanolamine modifier of the NPs for in situ lead(II) (Pb2+) adsorption at the solid-liquid interface using SFG spectroscopy. However, due to the limited number of NPs attached and sensitivity of SFG spectroscopy toward expected change in the modifier spectroscopically, no significant change was observed in the SFG spectrum of the modified silica with magnetic NPs during exposure to Pb2+ solution. Nevertheless, SFG spectroscopy as a surface technique successfully monitored the modifications from a clean fused substrate to -COCl formation that was used to immobilize the decorated magnetic nanoparticles. The method developed in this study can provide a reference for many surface or interfacial studies important for selective attachment of adsorbed organic or inorganic materials or particles.

Sleep in Children with Congenital Malformations of the Central Nervous System.

PURPOSE OF REVIEW: Congenital malformations of the central nervous system may be seen in isolation or in association with syndromes that have multiorgan involvement. Among the potential health challenges these children may face, sleep concerns are frequent and may include chronic insomnia, sleep-related breathing disorders, and circadian rhythm disorders. RECENT FINDINGS: In this review, we describe recent research into sleep disorders affecting children with congenital malformations of the CNS including visual impairment, septo-optic dysplasia, agenesis of the corpus callosum, Aicardi syndrome, Chiari malformation, spina bifida, achondroplasia, Joubert syndrome, fetal alcohol spectrum disorders, and congenital Zika syndrome. In many cases, the sleep disturbance can be directly related to observed anatomical differences in the brain (such as in apnea due to Chiari malformation), but in most syndromes, a complete understanding of the underlying pathophysiology connecting the malformation with sleep problem is still being elucidated. Our review provides a synthesis of available evidence for clinicians who treat this patient population, in whom appropriate diagnosis and management of sleep problems may improve the quality of life for both patient and caregiver.

Dihydrogen vs. hydrogen bonding in the solvation of ammonia borane by tetrahydrofuran and liquid ammonia.

The solvation structures of two systems rich in hydrogen and dihydrogen bonding interactions have been studied in detail experimentally through neutron diffraction with hydrogen/deuterium isotopic substitution. The results were analysed by an atomistic Monte Carlo simulation employing refinement to the experimental scattering data. The systems studied were the hydrogen storage material ammonia borane (NH3BH3, AB) dissolved in tetrahydrofuran (THF), and liquid ammonia (NH3), the latter in which AB shows unusually high solubility (260 g AB per 100 g NH3) and potential regeneration properties. The full orientational and positional manner in which AB-AB, AB-THF and AB-NH3 pairs interact with each other were successfully deciphered from the wide Q-range total neutron scattering data. This provided an unprecedented level of detail into such highly (di)hydrogen bonding solute-solvent interactions. In particular this allowed insight into the way in which H-B acts as a hydrogen bond acceptor. The (di)hydrogen bonding was naturally determined to dictate the intermolecular interactions, at times negating the otherwise expected tendency for polar molecules to align themselves with anti-parallel dipole moments. Several causes for the extreme solubility of AB in ammonia were determined, including the ability of ammonia to (di)hydrogen bond to both ends of the AB molecule and the small size of the ammonia molecule relative to AB and THF. The AB B-H to ammonia H dihydrogen bond was found to dominate the intermolecular interactions, occurring almost three times more often than any other hydrogen or dihydrogen bond in the system. The favourability of this interaction was seen on the bulk scale by a large decrease in AB clustering in ammonia compared to in the dihydrogen bond-less THF.

Antenatal Determinants of Bronchopulmonary Dysplasia and Late Respiratory Disease in Preterm Infants.

RATIONALE: Mechanisms contributing to chronic lung disease after preterm birth are incompletely understood. OBJECTIVES: To identify antenatal risk factors associated with increased risk for bronchopulmonary dysplasia (BPD) and respiratory disease during early childhood after preterm birth, we performed a prospective, longitudinal study of 587 preterm infants with gestational age less than 34 weeks and birth weights between 500 and 1,250 g. METHODS: Data collected included perinatal information and assessments during the neonatal intensive care unit admission and longitudinal follow-up by questionnaire until 2 years of age. MEASUREMENTS AND MAIN RESULTS: After adjusting for covariates, we found that maternal smoking prior to preterm birth increased the odds of having an infant with BPD by twofold (P = 0.02). Maternal smoking was associated with prolonged mechanical ventilation and respiratory support during the neonatal intensive care unit admission. Preexisting hypertension was associated with a twofold (P = 0.04) increase in odds for BPD. Lower gestational age and birth weight z-scores were associated with BPD. Preterm infants who were exposed to maternal smoking had higher rates of late respiratory disease during childhood. Twenty-two percent of infants diagnosed with BPD and 34% of preterm infants without BPD had no clinical signs of late respiratory disease during early childhood. CONCLUSIONS: We conclude that maternal smoking and hypertension increase the odds for developing BPD after preterm birth, and that maternal smoking is strongly associated with increased odds for late respiratory morbidities during early childhood. These findings suggest that in addition to the BPD diagnosis at 36 weeks, other factors modulate late respiratory outcomes during childhood. We speculate that measures to reduce maternal smoking not only will lower the risk for preterm birth but also will improve late respiratory morbidities after preterm birth.

A Survey of Practicing Sleep Coaches.

BACKGROUND: Sleep coaches are individuals of various backgrounds who offer services to families struggling with behavioral childhood sleep problems. We conducted a survey of coaches to further elucidate scope of practice, practice patterns, geographic distribution, education, training, and beliefs regarding qualification requirements. METHODS: A Web-based survey was completed by 142 individuals who identified as a sleep coach. RESULTS: Coaches were distributed across 17 countries and 5 continents. Overall, 65% of coaches served clients in countries beyond their home country. Within the United States, coaches were generally located in more affluent and well-educated zip codes near large metropolitan centers, 91% served clients beyond their home state, and 56% served clients internationally. Educational background varied across coaches (12% high school degree, 51% bachelor's degree, 32% master's degree, 2% doctoral degree, 1.5% JD degree). Few coaches (20%) were or had been licensed health care providers or carried malpractice insurance (38%). Coaches usually provided services for children < 4 months of age to about 6 years of age, and were much less likely to provide services for children with comorbid neurodevelopmental (32%) or significant medical disorders (19%). Coaches reported an average of 3 new and 6 total clients per week and working 20 hr per week on average. Most coaches (76%) felt that a formal sleep coach training program was the most important qualification for practice. CONCLUSIONS: These results highlight a diversity of background, training, and geographical distribution of sleep coaches, and may help inform discussions regarding guidelines for training and credentialing of sleep coaches.

Adult venous endothelium is a niche for highly proliferative and vasculogenic endothelial colony-forming cells.

OBJECTIVE: Postnatal resident endothelium of blood vessels has been proposed to represent terminally differentiated tissue that does not replicate. We previously isolated endothelial colony-forming cells (ECFCs) from human umbilical cord blood (CB) and term placenta by using colony-forming assays and immunocytochemistry. We showed that ECFCs are highly proliferative and form functioning vessels in vivo, the defining characteristics of a true endothelial progenitor cell. This exploratory investigation was conducted to determine whether the endothelium of healthy adult blood vessels contained resident ECFCs. METHODS: The endothelium of great saphenous vein (GSV) obtained from vein stripping procedures was collected with mechanical scraping, and ECFCs were isolated according to established protocols. RESULTS: GSV ECFCs incorporated acetylated low-density lipoprotein, formed tubules in Matrigel (BD Biosciences, San Jose, Calif) at 24 hours, and expressed endothelial antigens cluster of differentiation (CD) 144, CD31, CD105, and kinase insert domain receptor but not hematopoietic antigen CD45. Using cumulative population doublings and single-cell assays, we demonstrated that GSV ECFCs exhibited comparable proliferative capacities compared with CB ECFCs, including similar numbers of highly proliferative cells. When injected in collagen/fibronectin gels implanted in nonobese diabetic/severe combined immune deficiency mice, GSV ECFCs formed blood vessels with circulating murine red blood cells, demonstrating their vasculogenic potential. CONCLUSIONS: The ECFCs of the GSV contain a hierarchy of progenitor cells with a comparable number of highly proliferative clones as ECFCs of CB. The results of this investigation demonstrate that the adult endothelium contains resident progenitor cells that may have a critical role in vascular homeostasis and repair and could potentially be used as a source of autologous cells for cell therapies focusing on vasculogenesis.

Obstructive Sleep Apnea and Pulmonary Hypertension in Children.

Obstructive sleep apnea (OSA) is a common pediatric breathing disorder, affecting 1-5% of all children. Pulmonary hypertension (PH), a severe complication of OSA, is associated with significant morbidity and mortality. Despite this important relationship between OSA and PH, there is sparse literature addressing this subject in children. This review will examine the putative relationship between OSA and PH, synthesize the available literature in children, and suggest a reasonable approach, despite limited data, for clinicians. We conclude that available evidence suggests many children with OSA have evidence of PH (estimates ranging from 0% to 85%) and vice versa (estimates ranging from 6% to 24%). Furthermore, previous studies demonstrate that treatment of the OSA, either with surgery or non-invasive ventilation, ameliorates pulmonary artery pressures to the extent of cure in a substantial number of cases. Future studies are required to better delineate the true co-occurrence of these diseases and help predict which patients are at greater risk for this serious complication. Clinicians who maintain a healthy vigilance for this important interaction of disease states will likely recognize opportunities to intervene and improve prognoses in these patients.