Fairview Health Services

The impact of elexacaftor/tezacaftor/ivacaftor (ETI) on cystic fibrosis (CF) hepatobiliary involvement (CFHBI) is uncertain. The goal of this study was to investigate the changes in key liver parameters in PUSH study participants who started ETI compared to those who did not (noETI). PUSH was a prospective observational study of persons with CF (pwCF) (3-12 yo at PUSH entry). Linear mixed effect model (LMEM) with one knot tested if ETI changed the slope of trajectories of clinical parameters by comparing ETI to noETI. Index time (IT) for ETI was ETI start and time of ETI availability for noETI. Time zero in the LMEM was index time. One hundred forty-seven participants (104 ETI, 43 noETI). At IT, the groups were similar, for age, and liver parameters. Mean duration of ETI use was 22 months. The annual rate of change over time after IT in ETI compared to noETI was significantly improved for FEV1% pre (+3.4/yr p = 0.02) and wt z score (+0.06/yr, p = 0.006). There was improvement for ETI vs noETI for GGT (-15%/yr, p = 0.01) and ALT (-12%/yr, p = 0.02). Participants with CFHBI on ETI demonstrated similar trends and also had improvements in GGT and GGT to platelet ratio (GPR), but there were no differences in liver stiffness or US classification changes. GGT and GPR improve in pwCF and CFHBI who received ETI compared to noETI. There were no changes in other liver parameters. This suggests an early signal for positive impact on CFHBI, but no early improvement in fibrosis.

(1) Background: There has been an increase in the utilization of the minimally invasive vertical right axillary thoracotomy approach for repairing congenital heart defects in children recently. We aim, in the current study, to evaluate the outcomes of this approach in repairing anomalous pulmonary venous connections with or without an associated sinus venosus defect. (2) Methods: A total of 23 consecutive patients underwent surgical repair of anomalous pulmonary venous connections between April 2018 and February 2024. Perioperative and clinical follow-up data were obtained. (3) Results: The median age and weight were 36 months (1–277 months) and 14.4 kg (3.6–79.4 kg), respectively. More than half were females (13; 56.5%). There was no conversion to sternotomy. Partial anomalous pulmonary venous connections were the most frequent primary diagnoses (14; 60.9%), followed by scimitar syndrome (3; 13%), while two patients (8.7%) had total anomalous pulmonary venous connections. Repair techniques included single patch in 10 patients (43.5%), Warden in 6 (26.1%), and two-patch technique in 4 (17.4%). The median cardiopulmonary bypass and aortic cross-clamp times were 91 and 62 min, respectively. All patients were extubated in the operating room. The median length of hospital stay was 2 days. There were no mortalities or reoperations for pulmonary/systemic venous pathway obstruction. (4) Conclusions: Vertical right axillary thoracotomy is a valuable approach for repairing anomalous pulmonary venous connections with or without sinus venosus defects. All repair techniques, including Warden and scimitar, can be performed safely through this approach. The cosmetic superiority and short hospital stay make this approach worth considering.

BackgroundMany academic medical centers (AMCs) rely on systems like the Vizient Quality and Accountability Scorecard to track quality metrics such as the observed-to-expected (O/E) mortality ratio. The O/E mortality ratio calculation relies on clinical documentation. Missed documentation of diagnoses and risk factors for mortality leads to an underestimated expected mortality, which negatively affects the O/E metric.ObjectivesWe aimed to reduce our O/E mortality ratio from a median of 1.08 (± 0.10) to a median well below 0.90 within 12 months by improving the accuracy of clinical documentation.MethodsWe used a continuous quality improvement process that began with creating a rule-based tool within a standardized documentation template. The tool was designed to pull pertinent discrete electronic health record data into clinician documentation. The tool only pulled in data that were present on admission, and it especially prioritized inclusion of frequently missed risk factors according to prior coding query data. We then formed a multidisciplinary mortality review committee where providers reviewed mortality cases, made suggestions for documentation clarification, and found potential diagnoses and risk factors that the patient had which were missing from the documentation. We then leveraged the committee's expertise and feedback to improve the rule-based clinical tool.ResultsOver the 21-month period following implementation, the median O/E mortality ratio decreased by 30%, from 1.08 (± 0.10) to 0.72 (± 0.13) and consistently remained below the prior levels. Importantly, the intervention also led to a reduction in the total number of coding queries sent to clinicians, indicating a lower administrative burden for clinicians and coders.ConclusionOur interventions showed a clear improvement in the O/E mortality ratio at our AMC and in the expected mortality percentage compared with other similar institutions without significantly increasing burden on clinicians or coding specialists.

BackgroundWidespread immunity through vaccination or natural infection has altered the predictive ability of wastewater for hospitalization and mortality.MethodsBetween January 2022 and August 2024, we conducted a longitudinal observational study that aimed to examine the correlation between symptomatic COVID-19 in health care employees and SARS-CoV-2 wastewater community levels. Wastewater was analyzed by quantitative reverse transcription polymerase chain reaction for detection of SARS-CoV-2. The Fairview Employee Occupational Health office provided deidentified data.ResultsWe collected 215 wastewater samples from the Twin Cities Wastewater Treatment Plant over a 32-month interval. Over that period, there were 6879 positive SARS-CoV-2 test results reported to Fairview Employee Health from individuals who lived in the wastewater catchment area. We found that SARS-CoV-2 levels in wastewater accurately predicted the subsequent COVID-19 case count the following week in the community (P = .001).ConclusionsThese data demonstrate the utility of SARS-CoV-2 wastewater surveillance as it accurately predicts the frequency of symptomatic infection in the community.

Microbiome composition through the redundancy of oxalate-degrading bacteria, critically influence oxalate metabolism, explaining variable outcomes of Oxalobacter formigenes therapy and offers insights for improving probiotic selection and clinical trial strategies.

Abstract Congenital heart disease (CHD) is the most common and deadly birth defect, responsible for approximately 11% of infant mortality globally1 Approximately eight in 1,000 infants have CHD, while critical forms of CHD, or life-threatening CCHD, impact roughly two to four of every 1,000 births. Ultrasound is a proven, safe, and effective tool for diagnosing and assessing congenital heart defects (CHDs) and other pediatric heart conditions. Despite this, over 90% of young children lack access to pediatric ultrasound or echocardiography services in the places they are born or seen for routine care. Frontline clinicians in community and rural settings have limited tools to diagnose and manage life-threatening conditions in infants. By integrating ultrasound hardware components in a novel configuration and pairing with machine learning software, the RAPIDscan multi-model imaging device automates the image acquisition process with a repeatable, 30-second mechanical actuation “sweep” that simulates expert image capture from a single subcostal window placement to allow any level of user to screen and identify at-risk patients in minutes.

BackgroundMultiple sulfatase deficiency (MSD) is an exceptionally rare neurodegenerative disorder due to the absence or deficiency of 17 known cellular sulfatases. The activation of all these cellular sulfatases is dependent on the presence of the formylglycine-generating enzyme, which is encoded by the SUMF1 gene. Disease-causing homozygous or compound heterozygous variants in SUMF1 result in MSD. Other than symptomatic treatment, no curative therapy exists as of yet for MSD. Eight out of these 17 sulfatases are primarily localized in the lysosome.MethodsTwo siblings with attenuated MSD underwent hematopoietic cell transplantation (HCT), evaluating the possibility of lysosomal enzymatic cross-correction from the donor cells.ResultsThere is evidence of correction of currently available biomarkers within 3 months post-HCT. Untargeted metabolomics also shows continued correction of multiple biochemical abnormalities in the post-HCT period. Furthermore, this article also presents the neuropsychological outcomes of these children as well as the results of untargeted metabolomics analysis in this condition.ConclusionsThese data suggest biochemical benefits post-transplant along with slowing of disease progression. Long-term follow-up is necessary to fully evaluate the therapeutic benefit of HCT in MSD.