Citations

Loeys-Dietz syndrome (LDS) has been associated with multiple vascular abnormalities involving the entire arterial tree. However, limited reports regarding compromise in the aortoiliac and femoral bifurcation are available. Further, recommendations for optimal approach, thresholds for diameter at the time of surgery, and surveillance are also limited. We present a case of a 67-year-old male patient with LDS and aneurysmal aortoiliac and enlarging common femoral arteries aneurysms, who underwent open surgical repair. His past surgical history included multiple vascular interventions for lower extremity claudication and bilateral hip replacements. The right hip arthroplasty was previously removed due to infection. From the vascular standpoint, the patient underwent staged endovascular left hypogastric artery embolization and open aorto-bi-profunda bypass with a Rifampin-soaked Dacron graft. At 5-month follow-up, he remains asymptomatic with healed incisions and patent bypasses. This case highlights the challenges in managing peripheral aneurysms in LDS patients, emphasizing the need for tailored treatment strategies. While open repair is preferred, endovascular options may be considered in selected cases. Surveillance remains critical with annual cross-sectional imaging. Surgical planning is intricate due to comorbidities, anatomical complexities, and previous surgical infection. Surveillance of these patients must be strict as multiple vascular and non-vascular complications may arise. Therefore, collaborative decision-making is essential for optimal outcomes in this known high-risk population with connective tissue disorders.

Abstract Context Data on giant pheochromocytomas (PHEOs), defined based on size 10 cm or greater, are scarce. Objective This work aimed to compare presentation, management, and outcomes of patients with giant vs non–giant PHEOs. Methods A retrospective cohort study was conducted in a referral center from 2000 to 2023. Consecutive patients with giant PHEOs and randomly chosen patients with non–giant PHEO (referents) were studied at a 1:6 ratio. Outcomes included perioperative complications, metastases, and mortality. Results Of 828 patients with PHEOs, 31 (3.7%) had giant PHEO (median size 12 cm, interquartile range [IQR] 10.0-13.5 cm). In comparison to referents (n = 186, median size 4 cm, IQR, 2.9-5.0 cm), patients with giant PHEO had more symptoms of catecholamine excess (median of 2 vs 1; P = .04) and presented with a higher prevalence of severe catecholamine excess (76% vs 30%; P < .0001). Adrenalectomy was performed in 94% of patients with giant PHEOs and 100% of referents. In addition to preoperative α-adrenergic blockade (89%), metyrosine was used in 14 (7%) patients, mostly in patients with giant PHEOs (26% vs 3%; P < .0001). Patients with giant PHEOs had a higher perioperative complication rate (31% vs 10%; P = .004). During a median follow-up of 3 years, metastases developed at a higher rate in patients with giant PHEOs (45% vs 4% in referents; P < .0001). Disease-specific mortality was 7% in patients with giant PHEOs and 0% in referents (P = .02). Conclusion Patients with giant PHEOs compared to referents were more symptomatic, had a higher degree of catecholamine excess, and had a higher rate of perioperative complications. Almost half of patients with giant PHEOs developed metastases, warranting close follow-up.

AbstractEndothelin‐1 (ET‐1) and its receptors are linked to increases in sensitivity of the chemoreceptors to hypoxic stress and the development of hypertension in preclinical models. We hypothesized ET receptor antagonism would lower resting blood pressure (BP) as well as the acute BP response to chemoreflex stress. Twenty‐four men (31 ± 5 years, 26 ± 3 kg/m2) completed two study visits (control, bosentan). On each visit, BP was assessed under three conditions: (1) normoxia (FiO2 0.21), (2) chemoreflex excitation via hypoxia (FiO2 0.05–0.21), (3) chemoreflex inhibition via hyperoxia (FiO2 1.00). Bosentan increased plasma ET‐1 (0.94 ± 0.90 to 1.27 ± 0.62 pg/mL, p = 0.004), supporting receptor blockade. Resting diastolic (73 ± 5 to 69 ± 7 mmHg, p = 0.007) and mean (93 ± 7 to 88 ± 7 mmHg, p = 0.005) BP were reduced following bosentan compared to control with no change in systolic BP (p = 0.507). The mean BP response to both acute hypoxia (−0.48 ± 0.38 to −0.25 ± 0.31 mmHg/%, p = 0.004) and hyperoxia (area under the curve −93 ± 108 to −27 ± 66 AU, p = 0.018) were attenuated following bosentan. Acute ET receptor inhibition attenuates the rise in BP during chemoreflex excitation as well as the fall in BP during chemoreflex inhibition in healthy young men. These data support a role for ET‐1 in control of resting BP, possibly through a chemoreceptor‐mediated mechanism.

ABSTRACT Background Creatinine-based equations are the most used to estimate glomerular filtration rate (eGFR). The Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI), the re-expressed Lund-Malmö Revised (r-LMR) and the European Kidney Function Consortium (EKFC) equations are the most validated. The EKFC and r-LMR equations have been suggested to have better performances in young adults, but this is debated. Methods We collected data (GFR) measured by clearance of an exogenous marker (reference method), serum creatinine, age and sex from 2366 young adults (aged between 18 and 25 years) both from Europe and the USA. Results In the European cohorts (n = 1892), the bias (in mL/min/1.73 m²) was systematically better for the EKFC and r-LMR equations compared with the CKD-EPI equation [2.28, 95% confidence interval (1.59; 2.91), –2.50 (–3.85; –1.76), 17.41 (16.49; 18.47), respectively]. The percentage of estimated GFR within 30% of measured GFR (P30) was also better for EKFC and r-LMR equations compared with the CKD-EPI equation [84.4% (82.8; 86.0), 87.2% (85.7; 88.7) and 65.4% (63.3; 67.6), respectively]. In the US cohorts (n = 474), the bias for the EKFC and r-LMR equations was better than for the CKD-EPI equation in the non-Black population [0.97 (–1.69; 3.06), –2.62 (–5.14; –1.43) and 7.74 (5.97; 9.63), respectively], whereas the bias was similar in Black US individuals. P30 results were not different between the three equations in US cohorts. Analyses in sub-populations confirmed these results, except in individuals with high GFR levels (GFR ≥120 mL/min/1.73 m²) for whom the CKD-EPI equation might have a lower bias. Conclusions We demonstrated that both the EKFC and r-LMR creatinine-based equations have a better performance than the CKD-EPI equation in a young population. The only exception might be in patients with hyperfiltration.

AbstractA new generation of disease‐specific molecular imaging agents is poised to revolutionize fluorescence‐guided surgery. Pafolacianine has been approved for adult lung and ovarian cancers. We demonstrate a proof of concept for pediatric surgeons treating young adults with pulmonary metastatic sarcomas. Five successful fluorescence‐guided pulmonary metastasectomy operations were performed in young adult patients with metastatic osteosarcoma or Ewing sarcoma following administration of pafolacianine. All osteosarcoma lesions identified using standard techniques were also markedly fluorescent in patients. Novel fluorescent molecular agents targeted to tumor‐specific receptors have promise of increased sensitivity and specificity for detecting metastatic nodules and enhancing surgical clearance of disease.

INTRODUCTION: Prophylactic pancreatic stent placement (PSP) is effective for preventing pancreatitis after endoscopic retrograde cholangiopancreatography (ERCP) in high-risk cases, but the optimal technical approach to this intervention remains uncertain. METHODS: In this secondary analysis of 787 clinical trial patients who underwent successful stent placement, we studied the impact of (i) whether pancreatic wire access was achieved for the sole purpose of PSP or naturally during the conduct of the case, (ii) the amount of effort expended on PSP, (iii) stent length, (iv) stent diameter, and (v) guidewire caliber. We used logistic regression models to examine the adjusted association between each technical factor and post-ERCP pancreatitis (PEP). RESULTS: Ninety-one of the 787 patients experienced PEP. There was no clear association between PEP and whether pancreatic wire access was achieved for the sole purpose of PSP (vs occurring naturally; odds ratio [OR] 0.82, 95% confidence interval [CI] 0.37–1.84), whether substantial effort expended on stent placement (vs nonsubstantial effort; OR 1.58, 95% CI 0.73–3.45), stent length (>5 vs ≤5 cm; OR 1.01, 95% CI 0.63–1.61), stent diameter (≥5 vs <5 Fr; OR 1.13, 95% CI 0.65–1.96), or guidewire caliber (0.035 vs 0.025 in; 0.83, 95% CI 0.49–1.41). DISCUSSION: The 5 modifiable technical factors studied in this secondary analysis of large-scale randomized trial data did not appear to have a strong impact on the benefit of prophylactic PSP in preventing PEP after high-risk ERCP. Within the limitations of post hoc subgroup analysis, these findings may have important implications in procedural decision making and suggest that the benefit of PSP is robust to variations in technical approach.

Abstract The understanding and treatment of prearthritic hip disease has evolved remarkably over the past 20 years. The principal investigator and the multicenter Academic Network of Conservational Hip Outcomes Research (ANCHOR) group have had a key role in improving the quality of care for these patients suffering from the three most common prearthritic conditions: femoroacetabular impingement, developmental dysplasia of the hip, and residual Legg‐Calve‐Perthes Disease. We aim to demonstrate that based on the six quality of care dimensions as defined by Donabedian, our 20‐year academic journey has markedly improved the quality of care for young patients with prearthritic hip disease.