Early Childhood Care Coordination Through 211: A Randomized Clinical Trial.

BACKGROUND AND OBJECTIVES: Early intervention services can improve outcomes for children with developmental delays. Health care providers, however, often struggle to ensure timely referrals and services. We tested the effectiveness of telephone-based early childhood developmental care coordination through 211 LA, a health and human services call center serving Los Angeles County, in increasing referral and enrollment in services. METHODS: In partnership with 4 clinic systems, we recruited and randomly assigned children aged 12 to 42 months with upcoming well-child visits and without a known developmental delay, to intervention versus usual care. All children received developmental screening and usual clinic care. Intervention children also received telephone connection to a 211 LA early childhood care coordinator who made referrals and conducted follow-up. Primary outcomes at a 6-month follow-up included parent-reported referral and enrollment in developmental services. Secondary outcomes included referral and enrollment in early care and education (ECE). Logistic regression models were used to estimate the odds of outcomes, adjusted for key covariates. RESULTS: Of 565 families (282 intervention, 283 control), 512 (90.6%) provided follow-up data. Among all participants, more intervention than control children were referred to (25% vs 16%, adjusted odds ratio [AOR] 2.25, P = .003) and enrolled in (15% vs 9%, AOR 2.35, P = .008) ≥1 service, and more intervention than control children were referred to (58% vs 15%, AOR 9.06, P < .001) and enrolled in (26% vs 10%, AOR 3.75, P < .001) ECE. CONCLUSIONS: Telephone-based care coordination through 211 LA is effective in connecting young children to developmental services and ECE, offering a potentially scalable solution for gaps and disparities.

The impact of exclusive human milk diet on short-term growth of very preterm infants.

OBJECTIVES: The impact of exclusive human milk diet (EHMD) on postnatal growth remains controversial. This study aims to investigate the association between EHMD and short-term growth. METHODS: This multicenter retrospective study aims to compare growth between the EHMD and non-EHMD groups among infants <32 weeks of gestation. Primary outcomes include weight, length, and head circumference growth trajectories between birth and 34 weeks postmenstrual age. Sensitivity and subgroup analyses were performed. RESULTS: An EHMD was independently associated with poorer length growth, especially in infants born at ≥28 weeks' gestation or those exposed to hypertensive disorders of pregnancy. While initiating fortification at <26 kcal/oz on an EHMD showed inferior growth, initiating fortification at ≥26 kcal/oz was associated with improved weight growth, and similar length and head circumference growth when compared to the non-EHMD group. CONCLUSIONS: An EHMD with initial fortification at ≥26 kcal/oz may be implemented to avoid bovine milk exposure while sustaining comparable growth.

Human milk and breastfeeding during ketogenic diet therapy in infants with epilepsy: Clinical practice guideline.

Ketogenic diet therapy (KDT) is a safe and effective treatment for epilepsy and glucose transporter type 1 (GLUT1) deficiency syndrome in infancy. Complete weaning from breastfeeding is not required to implement KDT; however, breastfeeding remains uncommon. Barriers include feasibility concerns and lack of referrals to expert centres. Therefore, practical strategies are needed to help mothers and professionals overcome these barriers and facilitate the inclusion of breastfeeding and human milk during KDT. A multidisciplinary expert panel met online to address clinical concerns, systematically reviewed the literature, and conducted two international surveys to develop an expert consensus of practical recommendations for including human milk and breastfeeding in KDT. The need to educate about the nutritional benefits of human milk and to increase breastfeeding rates is emphasized. Prospective real-world registries could help to collect data on the implementation of breastfeeding and the use of human milk in KDT, while systematically including non-seizure-related outcomes, such as quality of life, and social and emotional well-being, which could improve outcomes for infants and mothers.

Development of a novel five dye insertion/deletion (INDEL) panel for ancestry determination.

The use of genetic markers, specifically Short Tandem Repeats (STRs), has been a valuable tool for identifying persons of interest. However, the ability to analyze additional markers including Single Nucleotide Polymorphisms (SNPs) and Insertion/Deletion (INDELs) polymorphisms allows laboratories to explore other investigative leads. INDELs were chosen in this study because large panels can be differentiated by size, allowing them to be genotyped by capillary electrophoresis. Moreover, these markers do not produce stutter and are smaller in size than STRs, facilitating the recovery of genetic information from degraded samples. The INDEL Ancestry Informative Markers (AIMs) in this study were selected from the 1000 Genomes Project based on a fixation index (FST) greater than 0.50, high allele frequency divergence, and genetic distance. A total of 25 INDEL-AIMs were optimized and validated according to SWGDAM guidelines in a five-dye multiplex. To validate the panel, genotyping was performed on 155 unrelated individuals from four ancestral groups (Caucasian, African, Hispanic, and East Asian). Bayesian clustering and principal component analysis (PCA) were performed revealing clear separation among three groups, with some observed overlap within the Hispanic group. Additionally, the PCA results were compared against a training set of 793 samples from the 1000 Genomes Project, demonstrating consistent results. Validation studies showed the assay to be reproducible, tolerant to common inhibitors, robust with challenging casework type samples, and sensitive down to 125 pg. In conclusion, our results demonstrated the robustness and effectiveness of a 25 loci INDEL system for ancestry inference of four ancestries commonly found in the United States.

How a Team Effectiveness Approach to Health Systems Science Can Illuminate Undergraduate Medical Education Outcomes.

ABSTRACT: Health care delivery requires physicians to operate in teams to successfully navigate complexity in caring for patients and communities. The importance of training physicians early in core concepts of working in teams (i.e., "teaming") has long been established. Over the past decade, however, little evidence of team effectiveness training for medical students has been available. The recent introduction of health systems science as a third pillar of medical education provides an opportunity to teach and prepare students to work in teams and achieve related core competencies across the medical education continuum and health care delivery settings. Although educators and health care system leaders have emphasized the teaching and learning of team-based care, conceptual models and evidence that inform effective teaming within all aspects of undergraduate medical education (including classroom, clinical, and community settings) are needed to advance the science regarding learning and working in teams. Anchoring teaming through the core foundational theory of team effectiveness and its operational components could catalyze the empirical study of medical student teams, uncover modifiable factors that lead to the evidence for improved student learning, and improve the link among competency-based assessments between undergraduate medical education and graduate medical education. In this article, authors articulate several implications for medical schools through 5 conceptual areas: admissions, the design and teaching of team effectiveness in health systems science curricula, the related competency-based assessments, and course and program evaluations. The authors then discuss the relevance of the measurable components and intended outcomes to team effectiveness in undergraduate medical education as critical to successfully prepare students for teaming in clerkships and eventually residency and clinical practice.

Development of a novel five-dye panel for human identification insertion/deletion (INDEL) polymorphisms.

DNA analysis of forensic case samples relies on short tandem repeats (STRs), a key component of the combined DNA index system (CODIS) used to identify individuals. However, limitations arise when dealing with challenging samples, prompting the exploration of alternative markers such as single nucleotide polymorphisms (SNPs) and insertion/deletion (INDELs) polymorphisms. Unlike SNPs, INDELs can be differentiated easily by size, making them compatible with electrophoresis methods. It is possible to design small INDEL amplicons (<200 bp) to enhance recovery from degraded samples. To this end, a set of INDEL Human Identification Markers (HID) was curated from the 1000 Genomes Project, employing criteria including a fixation index (FST) ≤ 0.06, minor allele frequency (MAF) >0.2, and high allele frequency divergence. A panel of 33 INDEL-HIDs was optimized and validated following the Scientific Working Group on DNA Analysis Methods (SWGDAM) guidelines, utilizing a five-dye multiplex electrophoresis system. A small sample set (n = 79 unrelated individuals) was genotyped to assess the assay's performance. The validation studies exhibited reproducibility, inhibition tolerance, ability to detect a two-person mixture from a 4:1 to 1:6 ratio, robustness with challenging samples, and sensitivity down to 125 pg of DNA. In summary, the 33-loci INDEL-HID panel exhibited robust recovery with low-template and degraded samples and proved effective for individualization within a small sample set.

Stressors and Silver Linings during COVID-19: Implications for Supporting Families with Young Children in a Post-Pandemic World.

OBJECTIVES: To better understand impacts of the COVID-19 pandemic and resulting economic and social disruptions on families, we analyzed qualitative data capturing perspectives from parents of young children. METHODS: This study analyzes interviews of parents of children aged 1-3.5 years at enrollment, recruited from four primary care systems serving mainly lower-income Hispanic families in Los Angeles, California. Interviews were conducted over 15 months beginning September 2020. Analyses focused on the open-ended question: Please describe in your own words how the COVID-19 pandemic has affected you and your family. We used iterative, multi-step processes to identify emergent qualitative themes. RESULTS: A total of 460 parent responses were collected and coded. Key themes and subthemes were tested for interrater reliability, with Kappa ranging from 0.74 to 0.91. Thematic analysis revealed two groups of responses, one emphasizing stress and one emphasizing "silver linings." Parents cited a range of stressors, from fear of COVID-19 to social isolation. Those emphasizing "silver linings" also referenced formal or informal supports - especially government/community assistance programs and childcare access - that enabled stronger family ties and positive lifestyle modifications. CONCLUSIONS FOR PRACTICE: Experiences of families with young children during COVID-19 were not uniform. Economic stability and reliable childcare may be critical mediators of family stress. Results affirm that the pandemic's impacts were distributed through channels largely built on, and possibly exacerbating, existing disparities. For lower-income families with young children, funding for public and private programs that target economic stability and childcare assistance may merit prioritization in future socio-economic disruptions.

Feasibility of text message follow-up for pediatric asthma care after an emergency department visit.

Background: Many children seen in the Emergency Department (ED) for asthma do not follow-up with their primary care provider. Text messaging via short message service (SMS) is a ubiquitous, but untested means of providing post-ED asthma follow-up care.Objective: To evaluate responses to an asthma assessment survey via SMS following an ED visit and estimate the likelihood of response by sociodemographic and clinical characteristics. Methods: We recruited 173 parents of children 2-17 years-old presenting for ED asthma care to receive a follow-up text (participation rate: 85%). One month later, parents received via SMS a 22-item survey that assessed asthma morbidity. We assessed response rates overall and by various sociodemographic and clinical characteristics, including age, parental education, and indicators of asthma severity.Results: Overall, 55% of parents (n = 95) responded to the SMS survey. In multivariable logistic regression (MLR), parents who graduated high school had a four-fold higher response rate compared to parents with less than a high school degree (OR: 4.05 (1.62, 10.13)). More parents of children with oral steroid use in the prior 12 months responded to survey items (OR: 2.53 (1.2, 5.31)). Reported asthma characteristics included: 48% uncontrolled, 22% unimproved/worse, 21% with sleep disruption, and 10% who were hospitalized for asthma.Conclusions: Text messaging may be a viable strategy to improve post-ED asthma assessment and to identify children with persistent symptoms in need of enhanced care or modification of care plans.

Factors Associated With Growth in Patients Treated With the Classic Ketogenic Diet for Drug-Resistant Epilepsy.

BACKGROUND: Suboptimal growth and malnutrition are often cited as complications of ketogenic diet therapy in patients with drug-resistant epilepsy; however, there is conflicting evidence on the factors that contribute to growth. METHODS: This is an observational, case-based study to evaluate growth in patients with drug-resistant epilepsy treated with the classic ketogenic diet for at least 12 months. Age, gender, height, weight, and body mass index (BMI) were collected at baseline and epilepsy clinic standard-of-care visits (one month, six months, and 12 months after diet initiation). Dietary intake and laboratory measures including glucose, bicarbonate, and beta-hydroxybutyrate were also collected. RESULTS: 119 patients were included. After ketogenic diet initiation, there was a significant fall in height z score from baseline to 12 months (-0.15, P = 0.001) but no other significant changes in weight, weight-for-length/BMI, or height z scores were noted between any time points within the 12 months after diet initiation. When separated by age, height z score changes were limited to those aged zero to three years. This was accompanied by a significant decrease in energy intake 12 months after treatment in this age group. When separated by diet route, weight z scores at each time point were significantly lower in the group eating by mouth than tube. CONCLUSIONS: Our study provides further evidence that the classic ketogenic diet impacts growth. Our population demonstrated restriction in linear growth in those aged zero to three years, which correlated with declines in energy intake, and weight declines limited to patients fed by mouth.

Bictegravir and Metformin Drug-Drug Interaction in People with Human Immunodeficiency Virus (HIV).

A drug-drug interaction (DDI) exists between bictegravir and metformin. Bictegravir inhibits renal organic cation transporter-2, leading to increased metformin plasma concentrations. The objective of this analysis was to evaluate the clinical implications of concomitant bictegravir and metformin administration. This was a retrospective, single-center, descriptive analysis evaluating people with human immunodeficiency virus (PWH) concurrently prescribed bictegravir and metformin between February 2018-June 2020. PWH lost to follow-up or non-adherent were excluded. Data collection included: hemoglobin A1C (HgbA1C), HIV RNA viral load, CD4 cell count, serum creatinine, and lactate. Adverse drug reactions (ADRs) were assessed by provider-documented, patient-reported symptoms of gastrointestinal (GI) intolerance and hypoglycemia. Metformin dose adjustments and discontinuations were recorded. Fifty-three PWH were included (116 screened; 63 excluded). GI intolerance was reported in three PWH (5.7%). There were no documented episodes of hypoglycemia or lactic acidosis. Five PWH had metformin dose reductions (N = 3 for unspecified reasons; N = 1 for GI intolerance) or discontinuation (N = 1 unrelated to ADRs). Both diabetes and HIV control improved (HgbA1C decreased by 0.7% with virologic control in 95% of PWH). Minimal ADRs were reported in PWH receiving concurrent metformin and bictegravir. Prescribers should be aware of this potential interaction; however, no empiric metformin total daily dose adjustment appears necessary.

Routine versus no assessment of gastric residual volumes in preterm infants receiving enteral feeding via intermittent feeding tubes: a randomized controlled trial.

OBJECTIVE: To evaluate whether eliminating routine gastric residual volume (GRV) assessments would lead to quicker attainment of full feeding volumes in preterm infants. STUDY DESIGN: This is a prospective randomized controlled trial of infants ≤32 weeks gestation and birthweight ≤1250 g admitted to a tertiary care NICU. Infants were randomized to assess or not assess GRV before enteral tube feedings. The primary outcome was time to attain full enteral feeding volume defined as 120 ml/kg/day. The Wilcoxon rank sum test was used to compare the days to reach full enteral feeds between the two groups. RESULTS: 80 infants were randomized, 39 to the GRV assessing and 41 to the No-GRV assessing group. A predetermined interim analysis at 50% enrollment showed no difference in primary outcome and the study was stopped as recommended by the Data Safety Monitoring Committee. There was no significant difference in median days to reach full enteral feeds between the two groups [GRV assessment: 12d (5) vs. No-GRV assessment:13d (9)]. There was no mortality in either group, one infant in each group developed necrotizing enterocolitis stage 2 or greater. CONCLUSION: Eliminating the practice of gastric residual volume assessment before feeding did not result in shorter time to attain full feeding.

Drosophila melanogaster frataxin: protein crystal and predicted solution structure with identification of the iron-binding regions.

Friedreich's ataxia (FRDA) is a hereditary cardiodegenerative and neurodegenerative disease that affects 1 in 50 000 Americans. FRDA arises from either a cellular inability to produce sufficient quantities or the production of a nonfunctional form of the protein frataxin, a key molecule associated with mitochondrial iron-sulfur cluster biosynthesis. Within the mitochondrial iron-sulfur cluster (ISC) assembly pathway, frataxin serves as an allosteric regulator for cysteine desulfurase, the enzyme that provides sulfur for [2Fe-2S] cluster assembly. Frataxin is a known iron-binding protein and is also linked to the delivery of ferrous ions to the scaffold protein, the ISC molecule responsible for the direct assembly of [2Fe-2S] clusters. The goal of this report is to provide structural details of the Drosophila melanogaster frataxin ortholog (Dfh), using both X-ray crystallography and nuclear magnetic resonance (NMR) spectroscopy, in order to provide the foundational insight needed to understand the structure-function correlation of the protein. Additionally, NMR iron(II) titrations were used to provide metal contacts on the protein to better understand how it binds iron and aids its delivery to the ISC scaffold protein. Here, the structural and functional similarities of Dfh to its orthologs are also outlined. Structural data show that bacterial, yeast, human and Drosophila frataxins are structurally similar, apart from a structured C-terminus in Dfh that is likely to aid in protein stability. The iron-binding location on helix 1 and strand 1 of Dfh is also conserved across orthologs.

Characteristics of the Isu1 C-terminus in relation to [2Fe-2S] cluster assembly and ISCU Myopathy.

Mitochondrial [2Fe-2S] cluster biosynthesis is driven by the coordinated activities of the Iron-Sulfur Cluster (ISC) pathway protein machinery. Within the ISC machinery, the protein that provides a structural scaffold on which [2Fe-2S] clusters are assembled is the ISCU protein in humans; this protein is referred to as the "Scaffold" protein. Truncation of the C-terminal portion of ISCU causes the fatal disease "ISCU Myopathy", which exhibits phenotypes of reduced Fe-S cluster assembly in cells. In this report, the yeast ISCU ortholog "Isu1" has been characterized to gain a better understanding of the role of the scaffold protein in relation to [2Fe-2S] assembly and ISCU Myopathy. Here we explored the biophysical characteristics of the C-terminal region of Isu1, the segment of the protein that is truncated on the human ortholog during the disease ISCU Myopathy. We characterized the role of this region in relation to iron binding, protein stability, assembly of the ISC multiprotein complex required to accomplish Fe-S cluster assembly, and finally on overall cell viability. We determined the Isu1 C-terminus is essential for the completion of the Fe-S cluster assembly but serves a function independent of protein iron binding.

Caregiving and Confidence to Avoid Hospitalization for Children with Medical Complexity.

OBJECTIVE: To test associations between parent-reported confidence to avoid hospitalization and caregiving strain, activation, and health-related quality of life (HRQOL). STUDY DESIGN: In this prospective cohort study, enrolled parents of children with medical complexity (n = 75) from 3 complex care programs received text messages (at random times every 2 weeks for 3 months) asking them to rate their confidence to avoid hospitalization in the next month. Low confidence, as measured on a 10-point Likert scale (1 = not confident; 10 = fully confident), was defined as a mean rating <5. Caregiving measures included the Caregiver Strain Questionnaire, Family Caregiver Activation in Transition (FCAT), and caregiver HRQOL (Medical Outcomes Study Short Form 12 [SF12]). Relationships between caregiving and confidence were assessed with a hierarchical logistic regression and classification and regression trees (CART) model. RESULTS: The parents were mostly mothers (77%) and were linguistically diverse (20% spoke Spanish as their primary language), and 18% had low confidence on average. Demographic and clinical variables had weaker associations with confidence. In regression models, low confidence was associated with higher caregiver strain (aOR, 3.52; 95% CI, 1.45-8.54). Better mental HRQOL was associated with lower likelihood of low confidence (aOR, 0.89; 95% CI, 0.80-0.97). In the CART model, higher strain similarly identified parents with lower confidence. In all models, low confidence was not associated with caregiver activation (FCAT) or physical HRQOL (SF12) scores. CONCLUSIONS: Parents of children with medical complexity with high strain and low mental HRQOL had low confidence in the range in which intervention to avoid hospitalization would be warranted. Future work could determine how adaptive interventions to improve confidence and prevent hospitalizations should account for strain and low mental HRQOL.

What Parents Want Doctors to Know: Responses to an Open-Ended Item on an Asthma Questionnaire.

OBJECTIVE: Unstructured parental comments could solicit important information about children's asthma, yet are rarely captured in clinical asthma questionnaires. This mixed-methods study describes parents' written responses to an open-ended question in a validated asthma questionnaire. METHODS: The Pediatric Asthma Control and Communication Instrument (PACCI) asthma questionnaire was administered to parents of children with asthma symptoms presenting to 48 pediatric primary care offices (PPCP), 1 pediatric pulmonology office, and 1 emergency department (ED). Responses to the question, "Please write down any concern or anything else you would like your doctor to know about your child's asthma" were analyzed using a phenomenological approach until thematic saturation was achieved for each site. Logistic regressions tested whether sociodemographic and clinical characteristics were associated with responding to the open-ended question. RESULTS: Of 7,988 parents who completed the PACCI, 954 (12%) responded to the open-ended question-2% in PPCP, 31% in the ED, and 50% in the pulmonary setting. More severe asthma was associated with higher odds of responding (odds ratio, 2.01; 95% confidence interval, 1.42-2.84). Based on responses provided, we identified 3 communication types: 1) clarifying symptoms, 2) asking questions, and 3) communicating distress. Responses also covered 5 asthma-related themes: 1) diagnostic uncertainty, 2) understanding asthma etiology and prognosis, 3) medication management, 4) impact on child function, and 5) personal asthma characteristics. CONCLUSION: Parents of children with severe asthma provided clarifying details, asked questions, and relayed health concerns and distress. None of these topics may be easily captured by closed-ended asthma questionnaires.

Children with under-diagnosed asthma presenting to a pediatric emergency department.

BACKGROUND: Undiagnosed asthma in children presenting to the emergency department (ED) for respiratory illnesses might be associated with subsequent asthma morbidity and repeat ED visits. OBJECTIVE: To examine the prevalence of undiagnosed asthma among children presenting for ED care, and explore associations with sociodemographic and clinical characteristics. METHODS: We surveyed parents of children ages 2-17 years seeking ED care for respiratory symptoms (including asthma) regarding sociodemographic characteristics, asthma symptoms, prior asthma care and morbidity, and prior asthma diagnosis. Undiagnosed asthma was defined as a positive screening for asthma and no prior diagnosis. We compared sociodemographic and clinical factors of those with diagnosed versus undiagnosed asthma using chi-square, t-tests and multivariable logistic regression model. RESULTS: Of 362 children, 36% had undiagnosed asthma. Undiagnosed children were younger, had younger parents, and had parents less likely to speak English versus diagnosed children (all p < 0.05). Among undiagnosed children, 42% had moderate or severe asthma and 66% reported ≥1 exacerbation in the prior 12 months. Parent-reported controller medication use was higher among diagnosed versus undiagnosed children (60% vs. 21%, p=.001). In a multivariable logistic regression (adjusting for insurance, education, income and preferred language), no controller usage (aOR 4.26), no asthma exacerbations in the prior year (aOR 2.41) and younger age (aOR 0.76) were significantly associated with undiagnosed asthma. CONCLUSION: Children presenting to the ED with undiagnosed asthma commonly experience significant prior asthma morbidity. Strategies to improve asthma diagnosis and messaging to their parents may reduce future morbidity.

Use of a validated asthma questionnaire to increase inhaled corticosteroid prescribing in the pediatric emergency department.

BACKGROUND: Asthma guidelines recommend assessment of asthma control and treatment with an ICS when appropriate. Children seen for asthma in the ED often have poorly controlled asthma. Validated questionnaires are rarely used in the ED and ICS are prescribed at less than 5% of ED asthma encounters, leaving many children at risk for continued poor outcomes. OBJECTIVE: To determine if use of a validated asthma questionnaire can increase the proportion of children who receive an ICS prescription during an ED asthma visit. METHODS: We administered a validated asthma questionnaire (Pediatric Asthma Control and Communication Instrument-ED version [PACCI-ED]) to parents of children 2 - 17 years old presenting for asthma care at a large, urban, academic pediatric ED. Based on national asthma guidelines, the PACCI-ED results were used to determine ICS dose recommendations. ED physicians reviewed the PACCI-ED results and ICS dose recommendations and chose whether to prescribe an ICS upon discharge. ICS prescribing rates during the intervention period were assessed via medical record review and compared to historical controls. We also surveyed parents to examine the association of sociodemographic factors with receipt of an ICS prescription, and surveyed physicians regarding their prescribing decisions. RESULTS: Thirteen physicians and seventy-nine children participated. Historically, the ICS prescribing rate for asthma exacerbations discharged from the ED was 13%. The intervention increased ICS prescribing to 56% (p < 0.001). Children with ≥2 asthma exacerbations in the prior year (p < 0.02) and those with moderate-severe persistent asthma (p < 0.02) were more likely to receive an ICS prescription. There were no statistically significant differences in ICS prescribing by sociodemographic characteristics. CONCLUSION: A validated asthma questionnaire increased ICS prescribing for children presenting for to the ED for asthma care. Additional strategies are needed to promote prescribing in this setting and ensure that all eligible children receive guideline-based asthma care.

Molecular Details of the Frataxin-Scaffold Interaction during Mitochondrial Fe-S Cluster Assembly.

Iron-sulfur clusters are essential to almost every life form and utilized for their unique structural and redox-targeted activities within cells during many cellular pathways. Although there are three different Fe-S cluster assembly pathways in prokaryotes (the NIF, SUF and ISC pathways) and two in eukaryotes (CIA and ISC pathways), the iron-sulfur cluster (ISC) pathway serves as the central mechanism for providing 2Fe-2S clusters, directly and indirectly, throughout the entire cell in eukaryotes. Proteins central to the eukaryotic ISC cluster assembly complex include the cysteine desulfurase, a cysteine desulfurase accessory protein, the acyl carrier protein, the scaffold protein and frataxin (in humans, NFS1, ISD11, ACP, ISCU and FXN, respectively). Recent molecular details of this complex (labeled NIAUF from the first letter from each ISC protein outlined earlier), which exists as a dimeric pentamer, have provided real structural insight into how these partner proteins arrange themselves around the cysteine desulfurase, the core dimer of the (NIAUF)2 complex. In this review, we focus on both frataxin and the scaffold within the human, fly and yeast model systems to provide a better understanding of the biophysical characteristics of each protein alone and within the FXN/ISCU complex as it exists within the larger NIAUF construct. These details support a complex dynamic interaction between the FXN and ISCU proteins when both are part of the NIAUF complex and this provides additional insight into the coordinated mechanism of Fe-S cluster assembly.

Parental preferences for survey mode of administration, interview versus self-administered, with an asthma management questionnaire.

Background: National asthma guidelines encourage use of patient surveys to aid clinical assessment. Little is known about how these should be administered in acute care settings such as the emergency department (ED).Objective: Evaluate if parents have a preference for interview versus self-administered surveys in an ED, understand the sociodemographic and clinical characteristics by choice of survey mode of administration, and assess if there is a difference by mode in the parent's perception of an asthma management tool.Methods: A research assistant (RA) surveyed parents of children 2-17 years of age seeking ED asthma care. Parents chose to either self-administer or have an RA-administered survey that included the Pediatric Asthma Control and Communication Instrument-ED version (PACCI-ED). We compared sociodemographic and clinical characteristics and perceptions about the PACCI-ED by mode of survey administration.Results: Of 174 parent participants, 60% chose interviewer-administered surveys. Parents who chose interviewer-administered versus self-administered surveys had lower income, lower educational attainment, and children with uncontrolled asthma (p < .05). Bivariate and multivariate analyses showed that parents who chose interviewer-administered versus self-administered surveys tended to rate the PACCI-ED more favorably.Conclusions: EDs wishing to systematically use an asthma survey may need to plan appropriate resources for staff to administer them, particularly if they serve populations of lower socioeconomic status.

Inhaled corticosteroid beliefs, complementary and alternative medicine in children presenting to the emergency department for asthma.

OBJECTIVE: Negative beliefs about inhaled corticosteroids (ICS) and endorsement of complementary and alternative medicine (CAM) have been associated with medication non-adherence and uncontrolled asthma. The association of CAM and negative health beliefs is not described in children in acute care settings. Our study objective is to determine the relationship between negative ICS beliefs, CAM use and poorly controlled asthma among a predominantly Latino population in an acute care setting. METHODS: The study was conducted in the pediatric emergency department of a children's hospital. During an ED asthma encounter, validated questionnaires surveyed parents about negative ICS beliefs, CAM use, and asthma health and control. We compared asthma health reports between parents who did or did not endorse negative ICS beliefs or CAM use, by chi-squared test (or a Fisher's exact test where appropriate). RESULTS: Parents of 174 children identified mostly as Latino with Medicaid insurance and high asthma morbidity. CAM endorsement and negative ICS beliefs were both associated with increased use of lifetime glucocorticoid (p = 0.03 and p=0.01 respectively). While CAM endorsement was associated with less hospitalizations (p = 0.04) and parental report of asthma "getting better" (p = 0.01), CAM users reported trouble with paying for rent or food (p = 0.02). Negative ICS beliefs and CAM endorsement were not associated with medication adherence. CONCLUSIONS: Negative ICS beliefs are associated with higher number of oral glucocorticoid courses. The association between CAM endorsement and asthma control is varied, but mostly in favor of improved control. Financial difficulties may make CAM use more likely.