Infants with Congenital Adrenal Hyperplasia Exhibit Thalamic Discrepancies in Early Brain Structure.

INTRODUCTION: Patients with classical congenital adrenal hyperplasia (CAH) have prenatal and postnatal hormonal imbalances. To characterize the ontogeny of reported brain and behavior changes in older children with CAH, we aimed to study the brain structure in infants with CAH compared to healthy controls. METHODS: We performed neuroimaging in 16 infants with classical CAH due to 21-hydroxylase deficiency (8 males, gestational age 38.2 ± 1.7 weeks, post-conceptional age [PCA] 42.2 ± 3.0 weeks) and 14 control infants (9 males, gestational age 38.5 ± 1.8 weeks, PCA 42.5 ± 2.4 weeks) utilizing 3-Tesla magnetic resonance imaging. Regional brain volumes were adjusted for PCA and sex, along with an additional adjustment for total brain volume (TBV), for group comparisons by regression analyses (mean, 95% confidence interval [CI]). The degree to which each brain region was differentiated between CAH and control infants was examined by relaimpo analyses, adjusting for all other brain regions, PCA, and sex. RESULTS: Infants with CAH had significantly smaller thalamic volumes (8,606 mm3, 95% CI [8,209, 9,002]) compared to age-matched control infants (9,215 mm3, 95% CI [8,783, 9,647]; ß = -609; p = 0.02) which remained smaller after further adjustment for TBV. Upon further adjustment for TBV, the temporal lobe was larger in infants with CAH (66,817 mm3, CI [65,957, 67,677]) compared to controls (65,616 mm3, CI [64,680, 66,551]; ß = 1,202, p = 0.03). The brain regions most differentiated between CAH versus controls were the thalamus (22%) and parietal lobe (10%). CONCLUSIONS: Infants with CAH exhibit smaller thalamic regions from early life, suggesting a prenatal influence on brain development in CAH. Thalamic emergence at 8-14 weeks makes the region particularly vulnerable to changes in the intrauterine environment, with potential implications for later maturing brain regions. These changes may take time to manifest, meriting longitudinal study through adolescence in CAH.

Factor Analysis of the Einstein Neonatal Neurobehavioral Assessment Scale in Infants with Congenital Heart Disease and Healthy Controls.

Background: Administration of the Einstein Neonatal Neurobehavioral Assessment Scale (ENNAS) can be time-consuming, and items can be highly correlated. We aimed to determine: (1) its factor analytic structure; (2) the validity of the factor structure; and (3) the associations of physiologic measures with factor scores. Methods: A factor analysis reduced 21 ENNAS items into 5 factors in 57 congenital heart disease (CHD) and 35 healthy infants. Multiple linear regressions examined the association of factor scores with group, gestational age, and physiologic variables. Results: 5-factor solution: 1 (Orienting Reflex), 2 (Extensor Axial Tone), 3 (Primitive Reflexes), 4 (Flexor Tone), 5 (Reflexive Tone Around Extremity Joints). Moderate to strong evidence supported: face, discriminant, and construct validity of these factors, with Factor 2 having the strongest. Conclusions: Components of Factor 2 may provide similar information about neonatal development, thus reducing the time for and burden of administration for researchers and clinicians.

Variations in Care for Breastfed Infants Admitted to US Children's Hospitals: A Multicenter Survey of Inpatient Providers.

BACKGROUND: Studies have revealed an association between hospitalization of breastfed infants and weaning posthospitalization. It is unknown what steps inpatient providers at children's hospitals are currently taking to support breastfeeding mothers of hospitalized infants, their comfort providing breastfeeding counseling, and what training they receive. METHODS: We conducted a multicenter survey study of pediatric providers who care for infants hospitalized at 3 urban, tertiary-care children's hospitals over a 12-month period. A convenience sample of nurses, residents, and attending physicians agreed to participate. Participants completed a 24-question questionnaire addressing provider practices, comfort with breastfeeding counseling, and previous breastfeeding education. Data were summarized as medians (interquartile ranges) and frequencies (percentages). Kruskal-Wallis and χ2 tests were used to compare between provider types. RESULTS: A total of 361 out of 1097 (33%) eligible providers completed the survey: 133 (21%) nurses, 166 (45%) residents, and 62 (63%) attending physicians. Provider practices varied by provider type. We observed a general trend that providers do not routinely review breastfeeding techniques, directly observe feeds, or use standardized breastfeeding assessment tools. Residents and attending physicians were more likely than nurses to feel comfortable with breastfeeding counseling (P = .02). Residents were more likely than nurses and attending physicians to have received breastfeeding education in the last 3 years (P < .001). CONCLUSIONS: Practices, comfort, and previous education varied by provider type. There was a general pattern that providers do not routinely perform certain practices. Further studies are needed to determine if inpatient provider practices affect weaning posthospitalization and if inpatient quality improvement initiatives will help mothers continue breastfeeding posthospitalization.

Number needed to eat: pizza and resident conference attendance.

The didactic conference is a common part of the resident education curriculum. Given the demands of clinical responsibilities and restrictions on duty hours, maximising education is a challenge faced by all residency programmes. To date, little research exists with respect to how the provision of complimentary food affects physician and resident conference attendance. The objective of this study was to determine whether complimentary food improves resident arrival times and attendance at educational conferences and, furthermore, to test whether this provision is a potentially cost-effective tool for improving education. A retrospective review of 36 resident educational Friday noon conferences, including 1043 resident arrivals, was performed. Data were analysed for total attendance, arrival times, number needed to eat (NNE) and the percentage of residents arriving on time, and compared between days on which food was and was not provided. Median attendance was 3.7% higher (p = 0.04) on days on which food was provided, at a cost of US$46 for each additional resident in attendance. Arrival times were also statistically significantly improved when food was provided, with a median improvement of 0.7 minutes (p = 0.02) and an 11.0% increase in on-time arrivals (p < 0.001). The NNE was 10.6. Complimentary food improves both attendance and arrival times by a small, but statistically significant, degree. The provision of complimentary food can be considered as an incentive for attendance and on-time arrival at didactic educational sessions, although more cost-effective modalities may exist.

ALTE and Feeding Intolerance as a Presentation of Double Aortic Arch.

Many children who are admitted to pediatric hospitals with the chief complaint of apparent life-threatening event (ALTE) are, in fact, well appearing by the time the inpatient medical team evaluates the patient. This presents a diagnostic and therapeutic challenge. We describe a case of a six-month-old full-term female presenting with an ALTE and found to have a double aortic arch, a congenital anomaly that usually presents with a more progressive onset of symptoms such as chronic cough, positional stridor, and feeding difficulties. This case highlights the importance of maintaining a broad differential in a patient presenting with findings of tracheoesophageal pathology on clinical exam.