Validation of an ICD-Code-Based Case Definition for Psychotic Illness Across Three Health Systems.

BACKGROUND AND HYPOTHESIS: Psychosis-associated diagnostic codes are increasingly being utilized as case definitions for electronic health record (EHR)-based algorithms to predict and detect psychosis. However, data on the validity of psychosis-related diagnostic codes is limited. We evaluated the positive predictive value (PPV) of International Classification of Diseases (ICD) codes for psychosis. STUDY DESIGN: Using EHRs at 3 health systems, ICD codes comprising primary psychotic disorders and mood disorders with psychosis were grouped into 5 higher-order groups. 1133 records were sampled for chart review using the full EHR. PPVs (the probability of chart-confirmed psychosis given ICD psychosis codes) were calculated across multiple treatment settings. STUDY RESULTS: PPVs across all diagnostic groups and hospital systems exceeded 70%: Mass General Brigham 0.72 [95% CI 0.68-0.77], Boston Children's Hospital 0.80 [0.75-0.84], and Boston Medical Center 0.83 [0.79-0.86]. Schizoaffective disorder PPVs were consistently the highest across sites (0.80-0.92) and major depressive disorder with psychosis were the most variable (0.57-0.79). To determine if the first documented code captured first-episode psychosis (FEP), we excluded cases with prior chart evidence of a diagnosis of or treatment for a psychotic illness, yielding substantially lower PPVs (0.08-0.62). CONCLUSIONS: We found that the first documented psychosis diagnostic code accurately captured true episodes of psychosis but was a poor index of FEP. These data have important implications for the case definitions used in the development of risk prediction models designed to predict or detect undiagnosed psychosis.

Rurality, Cardiovascular Risk Factors, and Early Cardiovascular Disease among Childhood, Adolescent, and Young Adult Cancer Survivors.

Background and Aims: Cardiovascular risk factors (CVRFs) later in life potentiate risk for late cardiovascular disease (CVD) from cardiotoxic treatment among survivors. This study evaluated the association of baseline CVRFs and CVD in the early survivorship period. Methods: This analysis included patients ages 0-29 at initial diagnosis and reported in the institutional cancer registry between 2010 and 2017 (n = 1228). Patients who died within five years (n = 168), those not seen in the oncology clinic (n = 312), and those with CVD within one year of diagnosis (n = 17) were excluded. CVRFs (hypertension, diabetes, dyslipidemia, and obesity) within one year of initial diagnosis, were constructed and extracted from the electronic health record based on discrete observations, ICD9/10 codes, and RxNorm codes for antihypertensives. Results: Among survivors (n = 731), 10 incident cases (1.4%) of CVD were observed between one year and five years after the initial diagnosis. Public health insurance (p = 0.04) and late effects risk strata (p = 0.01) were positively associated with CVD. Among survivors with public insurance(n = 495), two additional cases of CVD were identified from claims data with an incidence of 2.4%. Survivors from rural areas had a 4.1 times greater risk of CVD compared with survivors from urban areas (95% CI: 1.1-15.3), despite adjustment for late effects risk strata. Conclusions: Clinically computable phenotypes for CVRFs among survivors through informatics methods were feasible. Although CVRFs were not associated with CVD in the early survivorship period, survivors from rural areas were more likely to develop CVD. Implications for Survivors: Survivors from non-urban areas and those with public insurance may be particularly vulnerable to CVD.

Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab.

ABSTRACT: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome. Emapalumab, a fully human monoclonal antibody that neutralizes the proinflammatory cytokine interferon gamma, is approved in the United States to treat primary HLH (pHLH) in patients with refractory, recurrent, or progressive disease, or intolerance with conventional HLH treatments. REAL-HLH, a retrospective study, conducted across 33 US hospitals, evaluated real-world treatment patterns and outcomes in patients treated with ≥1 dose of emapalumab between 20 November 2018 and 31 October 2021. In total, 46 patients met the pHLH classification criteria. Median age at diagnosis was 1.0 year (range, 0.3-21.0). Emapalumab was initiated for treating refractory (19/46), recurrent (14/46), or progressive (7/46) pHLH. At initiation, 15 of 46 patients were in the intensive care unit, and 35 of 46 had received prior HLH-related therapies. Emapalumab treatment resulted in normalization of key laboratory parameters, including chemokine ligand 9 (24/33, 72.7%), ferritin (20/45, 44.4%), fibrinogen (37/38, 97.4%), platelets (39/46, 84.8%), and absolute neutrophil count (40/45, 88.9%). Forty-two (91.3%) patients were considered eligible for transplant. Pretransplant survival was 38 of 42 (90.5%). Thirty-one (73.8%) transplant-eligible patients proceeded to transplant, and 23 of 31 (74.2%) of those who received transplant were alive at the end of the follow-up period. Twelve-month survival probability from emapalumab initiation for the entire cohort (N = 46) was 73.1%. There were no discontinuations because of adverse events. In conclusion, results from the REAL-HLH study, which describes treatment patterns, effectiveness, and outcomes in patients with pHLH treated with emapalumab in real-world settings, are consistent with the emapalumab pivotal phase 2/3 pHLH trial.

Validation of an ICD-code-based case definition for psychotic illness across three health systems.

Background and Hypothesis: Early detection of psychosis is critical for improving outcomes. Algorithms to predict or detect psychosis using electronic health record (EHR) data depend on the validity of the case definitions used, typically based on diagnostic codes. Data on the validity of psychosis-related diagnostic codes is limited. We evaluated the positive predictive value (PPV) of International Classification of Diseases (ICD) codes for psychosis. Study Design: Using EHRs at three health systems, ICD codes comprising primary psychotic disorders and mood disorders with psychosis were grouped into five higher-order groups. 1,133 records were sampled for chart review using the full EHR. PPVs (the probability of chart-confirmed psychosis given ICD psychosis codes) were calculated across multiple treatment settings. Study Results: PPVs across all diagnostic groups and hospital systems exceeded 70%: Massachusetts General Brigham 0.72 [95% CI 0.68-0.77], Boston Children's Hospital 0.80 [0.75-0.84], and Boston Medical Center 0.83 [0.79-0.86]. Schizoaffective disorder PPVs were consistently the highest across sites (0.80-0.92) and major depressive disorder with psychosis were the most variable (0.57-0.79). To determine if the first documented code captured first-episode psychosis (FEP), we excluded cases with prior chart evidence of a diagnosis of or treatment for a psychotic illness, yielding substantially lower PPVs (0.08-0.62). Conclusions: We found that the first documented psychosis diagnostic code accurately captured true episodes of psychosis but was a poor index of FEP. These data have important implications for the development of risk prediction models designed to predict or detect undiagnosed psychosis.

Informatics tools to implement late cardiovascular risk prediction modeling for population management of high-risk childhood cancer survivors.

BACKGROUND: Clinical informatics tools to integrate data from multiple sources have the potential to catalyze population health management of childhood cancer survivors at high risk for late heart failure through the implementation of previously validated risk calculators. METHODS: The Oklahoma cohort (n = 365) harnessed data elements from Passport for Care (PFC), and the Duke cohort (n = 274) employed informatics methods to automatically extract chemotherapy exposures from electronic health record (EHR) data for survivors 18 years old and younger at diagnosis. The Childhood Cancer Survivor Study (CCSS) late cardiovascular risk calculator was implemented, and risk groups for heart failure were compared to the Children's Oncology Group (COG) and the International Guidelines Harmonization Group (IGHG) recommendations. Analysis within the Oklahoma cohort assessed disparities in guideline-adherent care. RESULTS: The Oklahoma and Duke cohorts both observed good overall concordance between the CCSS and COG risk groups for late heart failure, with weighted kappa statistics of .70 and .75, respectively. Low-risk groups showed excellent concordance (kappa > .9). Moderate and high-risk groups showed moderate concordance (kappa .44-.60). In the Oklahoma cohort, adolescents at diagnosis were significantly less likely to receive guideline-adherent echocardiogram surveillance compared with survivors younger than 13 years old at diagnosis (odds ratio [OD] 0.22; 95% confidence interval [CI]: 0.10-0.49). CONCLUSIONS: Clinical informatics tools represent a feasible approach to leverage discrete treatment-related data elements from PFC or the EHR to successfully implement previously validated late cardiovascular risk prediction models on a population health level. Concordance of CCSS, COG, and IGHG risk groups using real-world data informs current guidelines and identifies inequities in guideline-adherent care.

Highly variable hearing loss due to POU4F3 (c.37del) is revealed by longitudinal, frequency specific analyses.

Genotype-phenotype correlations add value to the management of families with hereditary hearing loss (HL), where age-related typical audiograms (ARTAs) are generated from cross-sectional regression equations and used to predict the audiogram phenotype across the lifespan. A seven-generation kindred with autosomal dominant sensorineural HL (ADSNHL) was recruited and a novel pathogenic variant in POU4F3 (c.37del) was identified by combining linkage analysis with whole exome sequencing (WES). POU4F3 is noted for large intrafamilial variation including the age of onset of HL, audiogram configuration and presence of vestibular impairment. Sequential audiograms and longitudinal analyses reveal highly variable audiogram features among POU4F3 (c.37del) carriers, limiting the utility of ARTAs for clinical prognosis and management of HL. Furthermore, a comparison of ARTAs against three previously published families (1 Israeli Jewish, 2 Dutch) reveals significant interfamilial differences, with earlier onset and slower deterioration. This is the first published report of a North American family with ADSNHL due to POU4F3, the first report of the pathogenic c.37del variant, and the first study to conduct longitudinal analysis, extending the phenotypic spectrum of DFNA15.

Rural, Large Town, and Urban Differences in Optimal Subspecialty Follow-up and Survivorship Care Plan Documentation among Childhood Cancer Survivors.

BACKGROUND: Children with cancer from rural and nonurban areas face unique challenges. Health equity for this population requires attention to geographic disparities in optimal survivorship-focused care. METHODS: The Oklahoma Childhood Cancer Survivor Cohort was based on all patients reported to the institutional cancer registry and ≤ 18 years old at diagnosis between January 1, 2005, and September 24, 2014. Suboptimal follow-up was defined as no completed oncology-related clinic visit five to 7 years after their initial diagnosis (survivors were 7-25 years old at end of the follow-up period). The primary predictor of interest was rurality. RESULTS: Ninety-four (21%) of the 449 eligible survivors received suboptimal follow-up. There were significant differences (P = 0.01) as 36% of survivors from large towns (n = 28/78) compared with 21% (n = 20/95) and 17% (n = 46/276) of survivors from small town/isolated rural and urban areas received suboptimal follow-up, respectively. Forty-five percent of adolescents at diagnosis were not seen in the clinic compared with 17% of non-adolescents (P < 0.01). An adjusted risk ratio of 2.2 (95% confidence interval, 1.5, 3.2) was observed for suboptimal follow-up among survivors from large towns, compared with survivors from urban areas. Seventy-three percent of survivors (n = 271/369) had a documented survivorship care plan with similar trends by rurality. CONCLUSIONS: Survivors from large towns and those who were adolescents at the time of diagnosis were more likely to receive suboptimal follow-up care compared with survivors from urban areas and those diagnosed younger than thirteen. IMPACT: Observed geographic disparities in survivorship care will inform interventions to promote equitable care for survivors from nonurban areas.

Prognostic significance of NT5E/CD73 in neuroblastoma and its function in CSC stemness maintenance.

Cluster of differentiation 73 (CD73), a cell surface enzyme that catalyzes adenosine monophosphate (AMP) breakdown to adenosine, is differentially expressed in cancers and has prognostic significance. We investigated its expression profile in neuroblastoma (NB), its association with NB clinical outcomes, and its influence in the regulation of cancer stem cells' (CSCs) stemness maintenance. RNA-Seq data mining (22 independent study cohorts, total n = 3836) indicated that high CD73 can predict good NB prognosis. CD73 expression (immunohistochemistry) gauged in an NB patient cohort (n = 87) showed a positive correlation with longer overall survival (OS, P = 0.0239) and relapse-free survival (RFS, P = 0.0242). Similarly, high CD73 correlated with longer OS and RFS in advanced disease stages, MYCN non-amplified (MYCN-na), and Stage-4-MYCN-na subsets. Despite no definite association in children < 2 years old (2Y), high CD73 correlated with longer OS (P = 0.0294) and RFS (P = 0.0315) in children > 2Y. Consistently, high CD73 was associated with better OS in MYCN-na, high-risk, and stage-4 subsets of children > 2Y. Multivariate analysis identified CD73 as an independent (P = 0.001) prognostic factor for NB. Silencing CD73 in patient-derived (stage 4, progressive disease) CHLA-171 and CHLA-172 cells revealed cell-line-independent activation of 58 CSC stemness maintenance molecules (QPCR profiling). Overexpressing CD73 in CHLA-20 and CHLA-90 cells with low CD73 and silencing in CHLA-171 and CHLA-172 cells with high CD73 showed that CD73 regulates epithelial to mesenchymal transition (E-Cadherin, N-Cadherin, Vimentin), stemness maintenance (Sox2, Nanog, Oct3/4), self-renewal capacity (Notch), and differentiation inhibition (leukemia inhibitory factor, LIF) proteins (confocal-immunofluorescence). These results demonstrate that high CD73 can predict good prognosis in NB, and further suggest that CD73 regulates stemness maintenance in cells that defy clinical therapy.

Non-Suicidal Self-Injury Among Adolescents: A Structural Model with Socioecological Connectedness, Bullying Victimization, and Depression.

The objective was to examine the associations of socioecological connectedness with bullying victimization and depressive symptoms in early adolescence and with non-suicidal self-injury (NSSI) in mid-adolescence, and how these might differ between genders. Diverse adolescents (N = 4115; 49.1% girls) in the 7th grade reported on connections with parents/family, peers, school, and neighborhood, as well as bullying victimization and depressive symptoms, and NSSI in 10th grade (Me = 16.1 years). Structural equation modeling with WSLMV indicated that the lower likelihood of NSSI in 10th grade was associated with higher perceptions of connections between adolescents and their families, both directly as well as indirectly through reduced bully victimization and depressive symptoms three years earlier. Higher school connectedness was indirectly associated with the lower likelihood of NSSI through bullying victimization and depressive symptoms. Paths to NSSI varied for girls and boys. Results advance the understanding of developmental pathways leading to NSSI in adolescent girls and boys.

Reduced asymmetry of the hand knob area and decreased sensorimotor u-fiber connectivity in middle-aged adults with autism.

Individuals with autism spectrum disorder (ASD) frequently present with impairments in motor skills (e.g., limb coordination, handwriting and balance), which are observed across the lifespan but remain largely untreated. Many adults with ASD may thus experience adverse motor outcomes in aging, when physical decline naturally occurs. The 'hand knob' of the sensorimotor cortex is an area that is critical for motor control of the fingers and hands. However, this region has received little attention in ASD research, especially in adults after midlife. The hand knob area of the precentral (PrChand) and postcentral (PoChand) gyri was semi-manually delineated in 49 right-handed adults (25 ASD, 24 typical comparison [TC] participants, aged 41-70 years). Using multimodal (T1-weighted, diffusion-weighted, and resting-state functional) MRI, we examined the morphology, ipsilateral connectivity and laterality of these regions. We also explored correlations between hand knob measures with motor skills and autism symptoms, and between structural and functional connectivity measures. Bayesian analyses indicated moderate evidence of group effects with greater right PrChand volume and reduced leftward laterality of PrChand and PoChand volume in the ASD relative to TC group. Furthermore, the right PoC-PrChand u-fibers showed increased mean diffusivity in the ASD group. In the ASD group, right u-fiber volume positively correlated with corresponding functional connectivity but did not survive multiple comparisons correction. Correlations of hand knob measures and behavior were observed in the ASD group but did not survive multiple comparisons correction. Our findings suggest that morphological laterality and u-fiber connectivity of the sensorimotor network, putatively involved in hand motor/premotor function, may be diminished in middle-aged adults with ASD, perhaps rendering them more vulnerable to motor decline in old age. The altered morphology may relate to atypical functional motor asymmetries found in ASD earlier in life, possibly reflecting altered functional asymmetries over time.

A Case of Cyclic Neutropenia and Associated Amyloidosis.

Cyclic neutropenia has been rarely associated with chronic inflammation and development of reactive AA amyloidosis. We report a family with cyclic neutropenia with associated renal and thyroid amyloid. A 12-year-old female presented with thyromegaly, recurrent aphthous ulcers, severe neutropenia, and renal failure. Renal and thyroid biopsies revealed abundant amyloid deposition. Presence of a heterozygous ELANE c.358 A>T gene mutation p.I120F variant with autosomal dominant inheritance confirmed the diagnosis of cyclic neutropenia. The patient's father also had neutropenia and amyloidosis with renal failure. We started filgrastim to attenuate neutropenia and thereby reduce chronic inflammation and minimize further amyloid deposition.

Evaluation of the academic achievements of clinician health services research scientists involved in "pre-K" career development award programs.

INTRODUCTION: Research career development awards (CDAs) facilitate development of clinician-scientists. This study compared the academic achievements of individuals in a structured institutional "pre-K" CDA program, the Mayo Clinic Kern Scholars program, with individuals who applied for but were not admitted to the Kern program ("Kern applicants"), and awardees of other unstructured internal CDAs. METHODS: This was a longitudinal cohort study of clinicians engaged in research at Mayo Clinic between 2010 and 2019. The primary outcome was time to the 15th new peer-reviewed publication after the program start, adjusted for baseline number of publications. Secondarily, we described successful awarding of federal funding by the NIH or VA. RESULTS: The median (IQR) number of baseline publications was highest among Kern Scholars compared to Kern Applicants or other CDA awardees [16 (12, 29) vs 5 (1, 11) and 8 (5, 16); P < 0.001]. After adjustment for baseline publications, the time to 15th new publication was significantly shorter for Kern Scholars than for the two comparator groups (P<0.001). Similar findings were observed with total new publications within 5 years (P < 0.001), as well as number of new first-/last-author publications within 5 years (P < 0.001). The overall frequency of K-awards, R-awards (or equivalent), or any funding were similar between groups, with the exception of R03 awards, which were significantly more common among Kern Scholars (P = 0.002). CONCLUSION: The Kern Scholars program is a successful training model for clinician-scientists that demonstrated comparatively greater acceleration of scholarly productivity than other internal CDA programs.

Neighborhood disadvantage, parent-adolescent relationship quality, and type 1 diabetes in late adolescents transitioning to early emerging adulthood.

OBJECTIVE: We examined whether neighborhood disadvantage predicted subsequent levels, and longitudinal trajectories, of type 1 diabetes (T1D) outcomes among late adolescents transitioning into early emerging adulthood. We also examined whether such associations occur indirectly through parent-adolescent relationship quality. RESEARCH DESIGN & METHODS: Seniors in high school with T1D (N = 236; mean age 17.76 ± 0.39 years; 61% female; 73.7% non-Latino White) completed selfreport measures of relationship quality with mothers and fathers, and adherence to their diabetes regimen; glycemic control was measured using HbA1c assay kits. Both T1D outcomes (i.e. adherence, HbA1c) were assessed annually across three time points (two years). Census tract indicators of neighborhood disadvantage (e.g., % unemployed) were culled from participant addresses at baseline linked to American Community Survey data. Structural Equation Modeling was used to estimate direct and indirect links between neighborhood disadvantage, relationship quality, and both subsequent levels (i.e., intercepts centered at Time 2), and trajectories of T1D outcomes (i.e., slopes across three time points). RESULTS: All models showed excellent fit to the data. Greater neighborhood disadvantage associated with lower relationship quality with both parents. Lower relationship quality with fathers (but not with mothers) measured at Time 1 predicted poorer levels of adherence and HbA1c at Time 2, and formed an indirect path linking neighborhood disadvantage to each Time 2 outcome. Neighborhood disadvantage and parent-adolescent relationship quality during high school did not predict longitudinal trajectories of T1D outcomes across all three time points. CONCLUSIONS: Parent-adolescent relationship quality (especially with fathers) remains important for T1D outcomes among late adolescents on the cusp of emerging adulthood, but may be at risk among those living within a socioeconomically-disadvantaged neighborhood.

'Only ever as a last resort': Mental health nurses' experiences of restrictive practices.

Nurses play a crucial role in the implementation of restrictive practices such as seclusion and restraint. Restrictive practices have been widely recognized as harmful practices and efforts to reduce their use have been in place for several years. While some reductions have been achieved, more information and insight into the perspectives and experiences of front-line mental health nursing staff is required if further changes are to be realized. Sixty-five respondents participated in an online survey to investigate Australian mental health nurses' personal experiences and opinions regarding restrictive practices. Analysis revealed restrictive practices as a complex, contested and challenging area of practice. Analysis of data revealed five main ways that restrictive practices were framed by respondents. These were as follows: as a response to fear; to maintain safety for all; a legacy of time and place; the last resort; and, a powerful source of occupational distress. In addition, findings revealed the need to support staff involved in restrictive practices. This need could be satisfied through the implementation of procedures to address post-restrictive distress at all levels of the organization. Ensuring an optimal work environment that includes appropriate staffing, availability of supportive education and structured routine debriefing of all episodes of restrictive practice is critical in achieving further reductions in seclusion and restraint.

Health-risk Behaviors and Type 1 Diabetes Outcomes in the Transition from Late Adolescence to Early Emerging Adulthood.

This study examined within- and between-person associations between health-risk behaviors (smoking, drinking, insulin withholding) and type 1 diabetes (T1D) outcomes (adherence and HbA1c) during the high-risk transition from late adolescence to early emerging adulthood utilizing a 2-year longitudinal study. Beginning in the senior year of high school, participants (n = 197) with T1D completed measures of health-risk behaviors, adherence, and HbA1c annually at three time points. Health-risk behaviors were associated with poorer diabetes outcomes during the transition from late adolescence to early emerging adulthood. These results highlight the importance of monitoring health-risk behaviors regularly and intervening to reduce health-risk behaviors during this important developmental transition.

Spatial analysis of landscape and sociodemographic factors associated with green stormwater infrastructure distribution in Baltimore, Maryland and Portland, Oregon.

This study explores the spatial distribution of green stormwater infrastructure (GSI) relative to sociodemographic and landscape characteristics in Portland, OR, and Baltimore, MD, USA at census block group (CBG) and census tract scales. GSI density is clustered in Portland, while it is randomly distributed over space in Baltimore. Variables that exhibit relationships with GSI density are varied over space, as well as between cities. In Baltimore, GSI density is significantly associated with presence of green space (+), impervious surface coverage (+), and population density (-) at the CBG scale; though these relationships vary over space. At the census tract scale in Baltimore, a different combination of indicators explains GSI density, including elevation (+), population characteristics, and building characteristics. Spatial regression analysis in Portland indicates that GSI density at the CBG scale is associated with residents identifying as White (-) and well-draining hydrologic soil groups A and B (-). At both census tract and CBG scales, GSI density is associated with median income (-) and sewer pipe density (-). Hierarchical modelling of GSI density presents significant spatial dependence as well as group dependence implicit to Portland at the census tract scale. Significant results of this model retain income and sewer pipe density as explanatory variables, while introducing the relationship between GSI density and impervious surface coverage. Overall, this research offers decision-relevant information for urban resilience in multiple environments and could serve as a reminder for cities to consider who is inherently exposed to GSI benefits.

Structural model of patient-centered communication and diabetes management in early emerging adults at the transfer to adult care.

Early emerging adulthood (ages 18-25) is a time of risk for type 1 diabetes (T1D) when relationships with parents and providers are changing. We examined whether individuals' high-quality relationships with mothers are associated with greater perceptions of patient-centered communication (PCC) with their doctor and whether PCC is associated with better adherence and glycemic control through diabetes-related self-efficacy. Additionally, we tested whether associations of PCC with self-efficacy and diabetes outcomes are stronger among those who had transferred to adult care. One-year post-high school, 217 individuals with T1D (60% women, 53% in adult care) reported perceptions of maternal relationship quality, PCC, self-efficacy, and adherence. Glycemic control was measured via HbA1c assay kits. Structural equation modeling indicated good model fit and revealed indirect paths linking higher maternal relationship quality to better adherence through higher PCC, and higher PCC to better HbA1c through adherence. Transfer status moderated the link between PCC and self-efficacy, suggesting PCC may be especially important when emerging adults transfer to adult care.

Preliminary exploration of secondhand smoke exposure in youth with Sickle Cell Disease: biochemical verification, pulmonary functioning, and health care utilization.

Exposure to secondhand smoke (SHSe) can compound an already challenging set of health problems for youth with Sickle Cell Disease (SCD). Exposure to electronic cigarettes may also impact SCD symptomology and respiratory functioning. This study built on these previous findings by a) utilizing two objective measures of SHSe, salivary cotinine and exhaled carbon monoxide and b) examining the association of SHSe with emergency department utilization, physician-reported sickle cell crises, and pulmonary functioning. Caregivers (N = 31 dyads; Mage = 37.6 years; SD = 8.5 years) of youth with SCD (Mage = 9.0 years; SD = 4.5 years) completed self-report measures of tobacco/nicotine use. Youth provided a saliva sample to assess cotinine levels, and performed pulmonary function tests. Dyads provided breath samples for measurement of eCO. A chart review was conducted to obtain medical history and utilization. Majority of youth (88%) were exposed to SHS via salivary cotinine. Interestingly, no significant associations were observed between youth cotinine levels and emergency department utilization, physician-reported sickle cell crises, or pulmonary functioning. Present findings indicate a need to assess for SHSe using objective assessment measures. Future research should investigate which specific factors contribute to increase SHSe/SHVe in youth with SCD.