Assessment of comorbid symptoms in pediatric autonomic dysfunction.

PURPOSE: Pediatric patients with autonomic dysfunction and orthostatic intolerance (OI) often present with co-existing symptoms and signs that might or might not directly relate to the autonomic nervous system. Our objective was to identify validated screening instruments to characterize these comorbidities and their impact on youth functioning. METHODS: The Pediatric Assembly of the American Autonomic Society reviewed the current state of practice for identifying symptom comorbidities in youth with OI. The assembly includes physicians, physician-scientists, scientists, advanced practice providers, psychologists, and a statistician with expertise in pediatric disorders of OI. A total of 26 representatives from the various specialties engaged in iterative meetings to: (1) identify and then develop consensus on the symptoms to be assessed, (2) establish committees to review the literature for screening measures by member expertise, and (3) delineate the specific criteria for systematically evaluating the measures and for making measure recommendations by symptom domains. RESULTS: We review the measures evaluated and recommend one measure per system/concern so that assessment results from unrelated clinical centers are comparable. We have created a repository to apprise investigators of validated, vetted assessment tools to enhance comparisons across cohorts of youth with autonomic dysfunction and OI. CONCLUSION: This effort can facilitate collaboration among clinical settings to advance the science and clinical treatment of these youth. This effort is essential to improving management of these vulnerable patients as well as to comparing research findings from different centers.

Stillbirth evaluation: a stepwise assessment of placental pathology and autopsy.

BACKGROUND: The American Congress of Obstetricians and Gynecologists places special emphasis on autopsy as one of the most important tests for evaluation of stillbirth. Despite a recommendation of an autopsy, many families will decline the autopsy based on religious/cultural beliefs, fear of additional suffering for the child, or belief that no additional information will be obtained or of value. Further, many obstetric providers express a myriad of barriers limiting their recommendation for a perinatal autopsy despite their understanding of its value. Consequently, perinatal autopsy rates have been declining. Without the information provided by an autopsy, many women are left with unanswered questions regarding cause of death for their fetus and without clear management strategies to reduce the risk of stillbirth in future pregnancies. To avoid this scenario, it is imperative that clinicians are knowledgeable about the benefit of autopsy so they can provide clear information on its diagnostic utility and decrease potential barriers; in so doing the obstetrician can ensure that each family has the necessary information to make an informed decision. OBJECTIVE: We sought to quantify the contribution of placental pathologic examination and autopsy in identifying a cause of stillbirth and to identify how often clinical management is modified due to each result. STUDY DESIGN: This is a cohort study of all cases of stillbirth from 2009 through 2013 at a single tertiary care center. Records were reviewed in a stepwise manner: first the clinical history and laboratory results, then the placental pathologic evaluation, and finally the autopsy. At each step, a cause of death and the certainty of that etiology were coded. Clinical changes that would be recommended by information available at each step were also recorded. RESULTS: Among the 144 cases of stillbirth examined, 104 (72%) underwent autopsy and these cases constitute the cohort of study. The clinical and laboratory information alone identified a cause of death in 35 (24%). After placental pathologic examination, 88 (61%) cases had a probable cause of death identified. The addition of autopsy resulted in 78 (74%) cases having an identifiable probable cause of death. Placental examination alone changed clinical management in 52 (36%) cases. Autopsy led to additional clinical management changes in 6 (6%) cases. CONCLUSION: This stepwise assessment of the benefit of both placental pathological examination and autopsy in changing probable cause of death beyond traditional clinical history and laboratory results emphasizes the need to implement more comprehensive evaluation of all stillbirths. With the aim of providing a cause of stillbirth to the parents, and to prevent future stillbirths, it behooves health care professionals to understand the value of this more comprehensive approach and convey that information to the bereaved parents.

Longitudinal assessment of hemoglobin oxygen saturation in preterm and term infants in the first six months of life.

OBJECTIVE: To report longitudinal home recordings of hemoglobin O(2) saturation by pulse oximetry (Spo(2)) during unperturbed sleep in preterm and term infants. STUDY DESIGN: We recorded continuous pulse oximetry during the first 3 minutes of each hour of monitor use (nonevent epochs) for 103 preterm infants born at <1750 g and ≤ 34 weeks postmenstrual age (PMA), and 99 healthy term infants. RESULTS: Median baseline Spo(2) was approximately 98% for both the preterm and term groups. Episodes of intermittent hypoxemia occurred in 74% of preterm and 62% of term infants. Among infants with intermittent hypoxemia, the number of seconds/hour of monitoring <90% Spo(2) was initially significantly greater in the preterm than the term group and declined with age at a similar rate in both groups. The 75(th) to 95(th) percentiles for seconds/hour of Spo(2) <90% in preterm infants were highest at 36 weeks PMA and progressively decreased until 44 weeks PMA, after which time they did not differ from term infants. CONCLUSIONS: Clinically inapparent intermittent hypoxemia occurs in epochs unperturbed by and temporally unrelated to apnea or bradycardia events, especially in preterm infants at 36 to 44 weeks PMA.

Nighttime child care: inadequate sudden infant death syndrome risk factor knowledge, practice, and policies.

BACKGROUND: Millions of children in the US have parents who work alternative shifts. As a result, extended-hour and nighttime child care centers have increased in number to meet the needs of parents working nonstandard hours. Recognizing that 20% of sudden infant death syndrome (SIDS) occurs in child care settings and that child care providers may place infants prone, it is important to determine sleep position practices in nighttime child care centers. OBJECTIVE: To determine if nighttime child care centers 1) follow Back to Sleep recommendations; 2) are aware of the need for a safe sleep environment; and 3) have written policies directing proper SIDS risk reduction practices. DESIGN: A descriptive, cross-sectional survey of licensed child care centers in the US offering evening and nighttime care. All nighttime centers caring for infants <6 months old were recruited for the study. RESULTS: Out of 153 eligible centers, 110 centers in 27 states completed the survey. Infants were placed prone in 20% of centers, although only 1 center placed infants exclusively prone. Infants slept in cribs in 53.6% of centers, but slept in uncluttered sleep environments in only 18.2% of centers. Smoking was prohibited in 86.4% of centers. The most commonly cited reason for avoiding prone altogether was SIDS risk reduction; however, 10 centers that cited SIDS risk reduction continued to place infants prone at least some of the time, because of parental request or concerns about infant comfort. Over half (59%) of the centers had written policies; however, presence of written policy was not associated with avoidance of prone position. In over one third of centers with written policies, providers were unaware of the content of the policy. CONCLUSIONS: Twenty percent of nighttime child care centers place infants prone at least some of the time. Most providers who place infants prone do so because of lack of awareness or misinformation about safe sleep environment. Although the Back to Sleep campaign has been effective in communicating the risks of sleeping prone, nonprone positioning is not universal among nighttime child care providers. Additional educational efforts toward child care providers remain necessary. In addition, parents as advocates for their own infants need to be proactive in assuring that safe sleep practices are implemented in child care settings.

Effect of a sudden infant death syndrome risk reduction education program on risk factor compliance and information sources in primarily black urban communities.

BACKGROUND: In the US, a higher incidence of sudden infant death syndrome (SIDS) and a slower decline in the incidence of SIDS has been found among blacks when compared with white infants. The continued racial disparity in SIDS is thought to be attributable to lack of compliance with SIDS risk reduction recommendations. OBJECTIVES: To better understand the disparities in SIDS risk reduction behaviors, we sought to study compliance and information sources related to SIDS among primarily black communities in a city with a high SIDS incidence rate before and after a targeted educational campaign. DESIGN: Pre- and post-SIDS Risk Reduction Education Program telephone surveys were performed in targeted Chicago communities with at least 86% blacks. Data collection for Survey 1 was from September 22 to November 4, 1999. Data collection for Survey 2 was from November 17, 2001, to January 12, 2002, 24 months after the aggressive implementation of a comprehensive, ethnically sensitive risk reduction program. RESULTS: Survey 1 analyzed data from 480 mothers with an infant <12 months of age (327 black, 66 white, and 87 Hispanic) and Survey 2 had 472 mothers (305 black, 77 white, and 90 Hispanic). The incidence of nighttime prone sleeping at Survey 1 was 25% among black respondents, 17% in whites, and 12% in Hispanics and decreased (but not significantly) among all groups by Survey 2. Overall, in Survey 2 compared with Survey 1, fewer mothers reported putting their infants on an adult bed, sofa, or cot both during the day and at night, with the biggest change seen in black mothers for daytime naps. Despite the same educational initiative, blacks increased the use of pillows, stuffed toys, and soft bedding in the sleep environment as compared with whites. More mothers in Survey 2 than in Survey 1 said that they noticed their infants sleeping on their back during the newborn hospitalization. Significantly more black and white mothers in Survey 2 compared with Survey 1 reported that a doctor or nurse had told them what the best position was for putting their infants to sleep, and all 3 groups said that the health care providers indicated that placing the infant on its back was the best sleep position. In examining the relationship between information sources and SIDS risk behaviors, among all groups observation of sleep position in hospital had no effect on behavior after newborn discharge; however, specific instruction by a nurse or doctor in the hospital about how to properly place the infant for sleep influenced behavior after the mother left the hospital. CONCLUSIONS: The Surveys indicate the greatest impact of the SIDS risk factor educational initiative targeted at black communities was changing behaviors regarding safe sleep locations by reducing the incidence of infants placed for nighttime and daytime sleep in adult beds, sofas, or cots. Although these data indicate considerable progress as a result of the targeted educational initiative, our findings suggest that cultural explanations for specific infant care practices must be more clearly understood to close the gap between SIDS risk factor compliance and apparent knowledge about SIDS risk factors.

Infant polysomnography: reliability and validity of infant arousal assessment.

Infant arousal scoring based on the Atlas Task Force definition of transient EEG arousal was evaluated to determine (1). whether transient arousals can be identified and assessed reliably in infants and (2). whether arousal and no-arousal epochs scored previously by trained raters can be validated reliably by independent sleep experts. Phase I for inter- and intrarater reliability scoring was based on two datasets of sleep epochs selected randomly from nocturnal polysomnograms of healthy full-term, preterm, idiopathic apparent life-threatening event cases, and siblings of Sudden Infant Death Syndrome infants of 35 to 64 weeks postconceptional age. After training, test set 1 reliability was assessed and discrepancies identified. After retraining, test set 2 was scored by the same raters to determine interrater reliability. Later, three raters from the trained group rescored test set 2 to assess inter- and intrarater reliabilities. Interrater and intrarater reliability kappa's, with 95% confidence intervals, ranged from substantial to almost perfect levels of agreement. Interrater reliabilities for spontaneous arousals were initially moderate and then substantial. During the validation phase, 315 previously scored epochs were presented to four sleep experts to rate as containing arousal or no-arousal events. Interrater expert agreements were diverse and considered as noninterpretable. Concordance in sleep experts' agreements, based on identification of the previously sampled arousal and no-arousal epochs, was used as a secondary evaluative technique. Results showed agreement by two or more experts on 86% of the Collaborative Home Infant Monitoring Evaluation Study arousal scored events. Conversely, only 1% of the Collaborative Home Infant Monitoring Evaluation Study-scored no-arousal epochs were rated as an arousal. In summary, this study presents an empirically tested model with procedures and criteria for attaining improved reliability in transient EEG arousal assessments in infants using the modified Atlas Task Force standards. With training based on specific criteria, substantial inter- and intrarater agreement in identifying infant arousals was demonstrated. Corroborative validation results were too disparate for meaningful interpretation. Alternate evaluation based on concordance agreements supports reliance on infant EEG criteria for assessment. Results mandate additional confirmatory validation studies with specific training on infant EEG arousal assessment criteria.