Implementation of Pediatric Emergency Care Applied Research Network Guidelines for Traumatic Brain Injury in a Rural Tertiary Care Center.

OBJECTIVES: To evaluate changes in imaging practices for pediatric head trauma after publication of the Pediatric Emergency Care Applied Research Network (PECARN) guidelines, explore areas for quality improvement regarding neuroradiology referrals. We also sought to determine the prevalence of incidental findings discovered on computed tomographies (CTs) attained for minor head trauma and ascertain disposition in these cases. METHODS: This retrospective study was conducted at a rural academic center and included 156 children who received CTs for head trauma between 2005 and 2015. Subjects were divided into 2 groups: pre-PECARN publication and post-PECARN publication. Electronic medical records were reviewed to determine whether or not head CTs were obtained according to PECARN guidelines. The proportion of scanned cases and incidental findings in each group was then compared. RESULTS: Significantly more subjects met PECARN criteria for head CT during the pre-PECARN period (67.1% vs 50.6%, P = 0.04). Among those who met PECARN criteria, severe mechanism of injury was the most common criterion met in both groups (43.8% pre-PECARN and 26.5% post-PECARN). Nine (5.7%) subjects had incidental findings (similar for both study periods), of which 3 prompted additional diagnostic testing or invasive intervention. Among those who did not meet PECARN criteria, the most common mechanism of injury was fall (<3 ft). CONCLUSIONS: Implementation of PECARN guidelines at our center remained limited in the 5 years after publication of this practice guide. Clinically insignificant incidental findings were often detected and may heighten patient anxiety.

Illness Experience, Self-Determination, and Recreational Activities in Pediatric Asthma.

Although asthma self-management depends on ongoing and accurate self-assessment by the patient, pediatric asthma patients have weak skills in the area of symptom perception. Before developing an asthma game targeted to improving asthma self-management and improved symptom awareness, we sought to identify gaps in existing games. To clarify the role of relatedness and autonomy in asthma health game design, we investigated symptom awareness, vocabulary, and self-determination through a series of semi-structured interviews with children suffering from asthma. Using self-determination theory as a framework, interviews were oriented to patients' illness experience and vocabulary related to symptomatology, as well as to recreational activities. Formative analysis of the interviews reveals attitudes, perceptions, and motivational factors arising in the context of childhood asthma, and it elucidates the images and vocabulary associated with both illness experience and recreational activities. Qualitative assessment of patient perspectives leads to specific recommendations for game design ideas that will support market entry of a spirometer-controlled game for children with asthma.

Digital Posturography Games Correlate with Gross Motor Function in Children with Cerebral Palsy.

OBJECTIVE: This pilot study aimed to assess whether performance on posturography games correlates with the Gross Motor Function Measure (GMFM) in children with cerebral palsy. MATERIALS AND METHODS: Simple games using static posturography technology allowed subjects to control screen events via postural sway. Game performance was compared with GMFMs using correlation analysis in a convenience sample of nine girls and six boys with cerebral palsy. Likert scales were used to obtain subjective responses to the balance games. RESULTS: GMFM scores correlated with game performance, especially measures emphasizing rhythmic sway. Twelve of the 15 subjects enjoyed the game and asserted an interest in playing again. CONCLUSIONS: Digital posturography games engage children with cerebral palsy in balance tasks, provide visual feedback in a balance control task, and have the potential to increase autonomy in balance control training among pediatric patients with cerebral palsy. This approach can support the relationship between child and therapist. The potential for interactive posturography to complement the assessment and treatment of balance in cerebral palsy bears continuing study.

Neuroimaging in the evaluation of neonatal encephalopathy.

BACKGROUND AND OBJECTIVE: Computed tomography (CT) is still used for neuroimaging of infants with known or suspected neurologic disorders. Alternative neuroimaging options that do not expose the immature brain to radiation include MRI and cranial ultrasound. We aim to characterize and compare the use and findings of neuroimaging modalities, especially CT, in infants with neonatal encephalopathy. METHODS: The Vermont Oxford Network Neonatal Encephalopathy Registry enrolled 4171 infants (≥36 weeks' gestation or treated with therapeutic hypothermia) between 2006 and 2010 who were diagnosed with encephalopathy in the first 3 days of life. Demographic, perinatal, and medical conditions were recorded, along with treatments, comorbidities, and outcomes. The modality, timing, and results of neuroimaging were also collected. RESULTS: CT scans were performed on 933 of 4107 (22.7%) infants, and 100 of 921 (10.9%) of those received multiple CT scans. Compared with MRI, CT provided less detailed evaluation of cerebral injury in areas of prognostic significance, but was more sensitive than cranial ultrasound for hemorrhage and deep brain structural abnormalities. CONCLUSIONS: CT is commonly used for neuroimaging in newborn infants with neonatal encephalopathy despite concerns over potential harm from radiation exposure. The diagnostic performance of CT is inferior to MRI in identifying neonatal brain injury. Our data suggest that using cranial ultrasound for screening, followed by MRI would be more appropriate than CT at any stage to evaluate infants with neonatal encephalopathy.

Relationship of Neonatal Oral Motor Assessment Scale to Feeding Performance of Premature Infants.

OBJECTIVE: Some premature infants require prolonged tube-feeding, beyond term equivalent gestational age. Tools that could prospectively identify such infants from among otherwise healthy patients are needed. We examined how well the Neonatal Oral Motor Assessment Scale (NOMAS) predicts premature infants' transition from tube to oral feeding. DESIGN: Data from a prospective study of sucking behavior as a predictor of feeding skills were used to examine the relationship of NOMAS and other nominal predictors to pre-defined feeding milestones. SETTING: Neonatal intensive Care Unit PATIENTS: 51 tube-fed, premature infants METHODS: NOMAS was administered soon (<72 hours) after oral feeding was initiated, and weekly thereafter, until infants reached full oral feeding. A timed measure of feeding efficiency was also done as oral feeding was initiated. A standardized, permissive protocol for feeding advance was used. Nonparametric rank sum tests and ANOVA were used to relate NOMAS, feeding efficiency, and other baseline variables to feeding milestones. RESULTS: Gestational age at birth, birth weight, and initial feeding efficiency predicted shorter transition and earlier acquisition to full oral feeding; NOMAS scores did not predict feeding outcomes. CONCLUSIONS: NOMAS was a poor predictor, while feeding efficiency and other baseline traits were better predictors of feeding skills in premature infants.

Pilot trial of spirometer games for airway clearance practice in cystic fibrosis.

BACKGROUND: Many children with cystic fibrosis (CF) adhere poorly to airway clearance techniques (ACTs), and would rather play video games that challenge their dexterity and visual tracking skills. We developed gaming technology that encourages forced expiratory maneuvers. OBJECTIVE: Following interviews regarding recreational activities and subjects' practice of ACTs, we conducted a pilot trial of spirometer games in 13 adolescents with CF, to test the hypothesis that games could increase subjects' engagement with forced expiratory breathing maneuvers and improve pulmonary function tests (PFTs). METHODS: After baseline PFTs, subjects were provided with digital spirometers and computers set up as "game only" or "control" devices. After the first of 2 periods (each > 2 weeks), the computer was set-up for the alternate condition for period 2. The t test and non-parametric correlation analyses examined use, number of expiratory high flow events (HFEs), and change in PFTs, identifying trends at P ≤ .1, significance at P < .05. RESULTS: Interviews disclosed minimal awareness of ACTs among our pediatric CF patients. Subjects used games and control software a similar percentage of days during the game (26%) and control periods (32%). There was a trend toward more minutes with the game versus control setup (P = .07), though HFE count did not differ between the 2 conditions (P = .71). Game play showed no overall effect on FEV(1), though correlation analysis showed a modest relation between minutes of play and change in FEV(1) from baseline (r = 0.50, P = .09). The game period showed a trend to increased vital capacity (P = .05). CONCLUSIONS: Spirometer games elicit forced expiratory breath maneuvers in pediatric CF patients. Improvement in PFTs may be due to improved test performance technique, though improved obstructive/restrictive lung function due to game play cannot be excluded. A formal clinical trial of this approach is planned.

A phase 1 study of nifurtimox in patients with relapsed/refractory neuroblastoma.

The primary aim of this phase 1 study was to determine the maximum tolerated dose (MTD) and evaluate the safety of nifurtimox alone and in combination with cyclophosphamide and topotecan in multiple relapsed/refractory neuroblastoma pediatric patients. The secondary aim was to evaluate the pharmacokinetics of nifurtimox and the treatment response. To these ends, we performed a phase 1 dose escalation trial of daily oral nifurtimox with toxicity monitoring to determine the MTD, followed by 3 cycles of nifurtimox in combination with cyclophosphamide and topotecan. Samples were collected to determine the pharmacokinetic parameters maximum concentration, time at which maximum concentration is reached, and area under the curve between 0 and 8 hours. Treatment response was evaluated by radiographic and radionuclide (I-metaiodobenzylguanidine) imaging, measurement of urinary catecholamines, and clearance of bone marrow disease. We determined the MTD of nifurtimox to be 30 mg/kg/d. The non-dose-limiting toxicities were mainly nausea and neuropathy. The dose-limiting toxicities of 2 patients at 40 mg/kg/d were a grade 3 pulmonary hemorrhage and a grade 3 neuropathy (reversible). Overall, nifurtimox was well tolerated by pediatric patients at a dose of 30 mg/kg/d, and tumor responses were seen both as a single agent and in combination with chemotherapy. A Phase 2 study to determine the antitumor efficacy of nifurtimox is currently underway.

A breath biofeedback computer game for children with cystic fibrosis.

The authors sought to develop and test a breath-controlled video game using a digital spirometer that, by providing visual breath biofeedback, could promote awareness of breathing techniques in children with cystic fibrosis (CF). To assess improvement in game performance during hospitalizations for CF exacerbations, the authors conducted a trial on 10 inpatients. Subjects had at least five 15-minute exposures to a breath biofeedback game that challenged them to track a moving target using their breath. Subjects reacted positively to the breath tracking challenge. Repeated-measures analysis of variance of a tracking fidelity statistic showed improvement in eye-breath coordination over 5 sessions ( P = .026). It was concluded that an electronic breath game is safe and can improve breath awareness among children with CF. This technology could also contribute to awareness of respiratory symptoms and foster social ties among CF patients.

Prospective study of non-nutritive sucking and feeding skills in premature infants.

OBJECTIVE: The aim of the present work was to assess the value of non-nutritive sucking (NNS) measures as predictors of oral feeding performance in comparison to other putative predictors of feeding skills: respiratory support, post-menstrual age (PMA) at birth and the neonatal oral motor assessment score (NOMAS). METHODS: This was a prospective, observational study. Cox proportional hazards and non-parametric rank sum tests were used to assess the relationship between NNS and feeding outcome measures. The setting was neonatal intensive care units (NICU) in rural/academic, urban/tertiary centres in the USA. In all, 51 premature infants born between 25 and 34 weeks' PMA, birth weight 1512.3+/-499.4 g, were included in this study. Interventions were measurement of NNS, standardised feeding advance schedule, performance of NOMAS, and standardised, permissive, oral feeding advance schedule. MAIN OUTCOME MEASURES: were transition time from first to full oral feeding (FOF) and gestational age at FOF. RESULTS: Higher NNS organisation scores predicted shorter transition to FOF (p<0.05): infants with a more organised suck pattern reached independent oral feeding 3 days earlier (16 vs 13 day transition) than infants with more chaotic patterns of suck bursts. Consistency of the suck waves also corresponded with feeding milestones: infants with more regular suck wave pressure deflections became competent oral feeders approximately 3 days earlier than those with irregular suck pressure waves. PMA at birth was inversely associated with PMA at FOF. NOMAS measures were not associated with outcome measures. CONCLUSIONS: Measures of NNS organisation and suck consistency constitute useful candidate predictors of feeding performance by premature infants. The results accord with previous findings linking PMA at birth with age at independent feeding.

Deprivation and dysphagia in premature infants.

The developmental trajectory of feeding features increasingly rhythmic ingestive behavior patterns. Sucking and swallowing by the fetus and infant, including fetal consumption of amniotic fluid, depend upon brainstem central pattern generators whose activity is increasingly influenced by chemosensory and oral-tactile input. This neurobiological fact underlies the clinical discovery that oral-tactile stimulation via pacifier stimulates ingestive behavior in tube-fed, premature infants and improves their feeding skills. However, little is known regarding the degree to which oral sensory deprivation may contribute to feeding problems in these patients. Evidence of deprivation-induced neuropathologic effects in rodents further underlines the potential impact of sensory deprivation in premature newborns, whose transition period from tube to oral feeding often lasts weeks beyond term equivalent gestational age. Studies exploring the link between early dysphagia and later developmental impairment could clarify experiential antecedents of cerebral injury. Trials of sensory interventions to promote feeding development are warranted.

Breast milk odor via olfactometer for tube-fed, premature infants.

Human newborns use odor cues to orient to their source of nutrition. However, tube-fed, premature infants have restricted chemosensory experience. New methods of introducing breast milk odor to tube-fed premature infants will permit empiric tests of the effect of controlled exposure to nutrient odor. We therefore developed an infant olfactometer and piloted its use in 7 tube-fed, premature infants in the neonatal intensive care unit. Since nonnutritive sucking shortens the amount of time required to wean from tube-feeding, we tested the effect of breast milk odor on nonnutritive sucking. Six out of 7 subjects responded to breast milk odor with an increase in number of sucks. Statistical analysis supported the hypothesis that breast milk odor reinforces nonnutritive sucking. These results indicate the feasibility and potential of this experimental approach, and warrant further study of the effect of controlled nutrient odor exposure on feeding behavior of premature infants.

Pentasomy 49,XXXXY associated with a Chiari type 1 malformation and cervical syrinx.

Pentasomy 49,XXXXY occurs in 1/85,000 newborn males. The origin of this particular form of aneuploidy is believed to be a result of consecutive nondisjunction events during maternal meiosis. Typical presentation consists of hypotonia, developmental delay, various dysmorphic features, and severe hypogenitalism. A 13-year-old with pentasomy 49,XXXXY and a Chiari type 1 malformation with an associated cervical syrinx is presented.

A pilot study of milk odor effect on nonnutritive sucking by premature newborns.

OBJECTIVE: To study the effect of milk odor on nonnutritive sucking by premature newborns. DESIGN: Blinded, crossover study of the effects of milk vs sham odor on nonnutritive sucking. SETTING: Urban neonatal intensive care unit. PATIENTS: Twenty-nine premature newborns with gestational age of 29 to 36 weeks. INTERVENTION: Fourteen subjects were tested with fortified breast milk odor (group 1) and 15 were tested with formula odor (group 2). For the test observation, milk odor was directed to the nose (orthonasal exposure) using a specially modified pacifier. For the control observation, water was used as a sham odor. Observations were made concurrently with tube feeding of the newborn with either fortified breast milk (group 1) or formula (group 2). MAIN OUTCOME MEASURES: Total number of sucks and sucking bursts, measured from a digital record of pressure changes within the pacifier. RESULTS: Nutrient odor increased suck bursts in group 1 subjects, with borderline statistical significance (46.6 bursts/10 min with odor [95% confidence interval (CI), 39-54] vs 35.4 bursts/10 min without odor [95% CI, 28-43]). Unexpectedly, when test and control observations were combined, subjects in group 1 showed an overall increase in number of sucks (260.4 [95% CI, 206-315]) and suck bursts (41.0 [95% CI, 36-46]) compared with group 2 subjects (144.8 [95% CI, 87-203] vs 27.4 [95% CI, 21-34]). CONCLUSIONS: Nutrient odor exposure via pacifier may stimulate nonnutritive sucking during gavage feeding of premature newborns. Further studies on the effects of nutrient odor on nonnutritive sucking by premature newborns must take into account the effects of nutrients given via gavage.