Citations

Abstract Objectives The impact of mild autonomous cortisol secretion (MACS) on cognition is incompletely characterized. We aimed to assess cognition in patients with MACS, identify factors associated with lower cognition, and determine the impact of adrenalectomy on cognition. Methods We conducted a cross-sectional study (4/2019 to 10/2022) and a longitudinal cohort study (10/2021 to 9/2023) of adults with MACS and referent subjects. Cognition was assessed with National Institute of Health Toolbox Cognition Battery and reported as standardized T scores (adjusted for age, sex, race/ethnicity, and education) for fluid, crystallized, and total composite cognition. Participants were assessed with urine steroid profiling (liquid chromatography-tandem mass spectrometry), frailty index, Beck’s Depression Inventory (BDI), and Short Form-36 (SF-36). Results A total of 84 patients (median age 57 years, 67% women) and 201 referent subjects (median age 63 years, 58% women) were included. Patients with MACS demonstrated lower mean (SD) total composite (50.9 [9.3] vs 55.4 [8.9], P < .001) and fluid composite T score (49.3 [10.6] vs 55.2 [10.2], P < .001), but not crystallized composite T score (52.3 [8.4] vs 54.0 [8.4], P = .130) when compared with referent subjects. Multivariable analysis adjusted for age, sex, BDI, SF-36, frailty index, total glucocorticoids, and androgens demonstrated that MACS status was associated with lower cognition. When cognition was reassessed in 37 patients post-adrenalectomy and 31 referent subjects, adjusted group differences of cognition change were similar except for a higher comparative increase in attention and executive functioning in patients with MACS. Conclusion Patients with MACS demonstrate lower cognition than referent subjects. Adrenalectomy for MACS had a minor positive impact on cognition.

Glucagon-like peptide-1 (GLP-1) receptor agonists are medications used to treat type 2 diabetes and may aid in weight loss efforts. The surgical management of patients taking GLP-1 agonists differs from others due to the slowed gastric emptying effects of GLP-1 medications. Patients taking these medications may have a higher risk of pulmonary aspiration during hand surgery than other patients, which affects their presurgical management. This article reviews GLP-1 agonists and their role in diabetes and weight loss, as well as current recommendations for surgical management of patients taking GLP-1 agonists.

Background and Objective Rehabilitation therapy plays an important role in treating physical and functional impairments observed in individuals undergoing hematopoietic stem cell transplants (HSCT). This study assessed the rehabilitation practices implemented in the HSCT population internationally. Materials and Methods A 48-question online survey comprising questions soliciting information regarding patient characteristics, therapy details (timing, indication, and administering providers), outcome measures, and precautions were developed by an international group of cancer rehabilitation physicians. As reported by European registries, surveys were administered to personnel providing care to patients receiving HSCT at cancer centers, which comprised the top 10% of HSCT volume. In addition, emails were sent to National Medical Societies and registries in the Latin America, Asia, and Pacific regions. Results Forty-three institutions from 18 countries responded to the survey. Half of the centers provided referrals for rehabilitation therapy at the time of admission. Referrals were provided for functional decline (84.5%), risk of falls (53.3%), and discharge planning (42.2%). Rehabilitation therapies were administered by physical therapists (93.0%), occupational therapists (34.9%), therapy aides (14.0%), and speech-language pathologists (11.6%). Approximately 95% of the surveyed centers used objective functional measures such as sit-to-stand (46.5%), grip strength (46.5%), and 6-min walk/gait speed (both 34.9%). The blood counts were monitored to determine the appropriateness of the therapy modalities. Conclusion Rehabilitation practices varied internationally; however, most centers provided skilled therapy during hospitalization for HSCT, utilized objective and patient-reported outcomes, and monitored blood counts to determine the safety of administering therapy.

Abstract OBJECTIVES The Ross procedure is currently receiving renewed interest. Its function and durability depend on preservation of pulmonary valve anatomy; limited data exist on normal pulmonary valve geometry. The objective was to compare aortic and pulmonary root and cusp dimensions in adults with normal tricuspid aortic and pulmonary valves. METHODS We reviewed 507 coronary computed tomography studies, selecting those with adequate visibility of both pulmonary and aortic roots for further analysis. Diastolic aortic and pulmonary root and cusp dimensions were measured. Root dimensions at different phases of the cardiac cycle were measured in 3 patients. RESULTS We analysed studies of 50 patients with the mean age of 54 years [standard deviation (SD): 16]. In end-diastole, pulmonary root had a smaller sinutubular to basal ring ratio than the aortic root [0.82 (SD: 0.09) vs 1.14 (SD: 0.12), P < 0.001]. Aortic and pulmonary cusps had similar dimensions; however, pulmonary cusp effective height was lower [5.9 mm (SD: 1.6) vs 8.4 mm (SD: 1.2), P < 0.001]. Pulmonary basal ring perimeter was largest at end-diastole and smallest at end-systole, with the relative difference of 23.5% (SD: 2.7). CONCLUSIONS The pulmonary root has a similar cusp size compared to the aortic root, but a different shape, resulting in a lower pulmonary cusp effective height. The perimeter of the pulmonary basal ring changes during the cardiac cycle. These findings suggest that stabilizing the autograft to normal aortic, rather than pulmonary, root dimensions should result in normal autograft cusp configuration. Computed tomography angiography could become a tool for personalized planning of the Ross procedure.

Abstract Context Diagnosing adrenal insufficiency (AI) often requires complex testing, which can be time-consuming and expensive. Dehydroepiandrosterone sulfate (DHEAS) is a promising marker of hypothalamic-pituitary-adrenal axis function; however, its diagnostic performance has not yet been evaluated in a large-scale study. Objective Evaluate the performance of DHEAS and baseline cortisol in assessing AI. Design Single-center retrospective cohort study. Setting Referral center. Patients Adults who underwent cosyntropin stimulation testing (CST) between 2005 and 2023 and had DHEAS measured within 3 months prior to CST. Main Outcome Measures Area under receiver operating characteristic curve (AUROC) for DHEAS and baseline cortisol. Prevalence of AI based on various DHEAS and baseline cortisol concentrations. Results Among 1135 patients, 195 (17.2%) had AI. Both baseline cortisol and DHEAS independently had good diagnostic performance with AUROC 0.81 [95% confidence interval (CI) 0.77-0.84 and 0.81 (95% CI 0.78-0.85)], respectively. Time of CST performance had no significant effect on diagnostic accuracy of baseline cortisol while recent glucocorticoid use decreased diagnostic performance of DHEAS (AUROC 0.72 vs 0.83). Only 1.2% of patients with baseline cortisol ≥10 mcg/dL had AI based on CST. Among patients with baseline cortisol between 5 and 9.9 mcg/dL, only 1.3% had AI if DHEAS was ≥60 mcg/dL. Conversely, the majority (72.2%) of patients with both baseline cortisol <5 mcg/dL and DHEAS <25 mcg/dL were found to have AI. Conclusion DHEAS has good diagnostic performance in assessing AI. Measuring both baseline cortisol and DHEAS concentrations may eliminate the need for further dynamic testing in many patients.

Background Bicuspid aortic valve (BAV) is the most common congenital heart defect in adults, often leading to complications such as thoracic aortic aneurysms and aortic stenosis. While BAV is frequently associated with 22q11.2 deletion syndrome (22q11.2DS), the contribution of rare copy number variants (CNVs) in this region to non-syndromic BAV is less clear. This study is aimed to assess the role of rare 22q11.2 CNVs in patients with early-onset BAV (EBAV) and to determine whether these variants are linked to an increased risk of complications. Methods Whole genome microarray genotyping was conducted on 272 patients with BAV with early onset valve or aortic disease (EBAV) and 272 biological relatives. CNVs were detected using three independent algorithms, focusing on the 22q11.2 region (18–24 Mb). CNV burden in the EBAV cohort was compared with unselected European ancestry controls. Results Rare duplications and deletions within the 22q11.2 region, particularly involving genes associated with cardiac development, were identified in 7.4% of EBAV probands. These CNVs were significantly enriched compared with the general population and segregated with BAV in families. Individuals carrying rare 22q11.2 CNVs had a higher prevalence of psychiatric diagnoses and learning difficulties, although they did not exhibit the typical features of 22q11.2DS. Importantly, these CNVs were associated with early onset or complex BAV cases, underscoring their potential clinical relevance. Conclusions Rare 22q11.2 CNVs play a role in non-syndromic BAV, particularly in cases with early onset or complex presentations. CNV screening could be considered as part of risk stratification for patients with BAV, helping to predict complications and guide management. Trial registration number NCT01823432 .