Publications

This study is to determine peripheral sample collection timing impact on selectivity index (SI) and success of unstimulated adrenal venous sampling (AVS) for primary aldosteronism (PA) subtyping. In this study, a retrospective discovery and a prospective validation arm were conducted. 74 patients undergoing AVS before and after-ACTH stimulation were reviewed. Discovery dataset was divided into 1) "pre" group, peripherals collected 30-min before AVS and 2) "post" group, peripherals collected 1-min after AVS. SIs were calculated using 30-min-pre and 1-min-post values. Patients with samples having SIs < 2 and SIs ≥ 5 on before and after-ACTH were classified as false negative and those with SI ≥ 2 and SI ≥ 5 on before and after-ACTH as true positive. Data was analyzed using Chi-squared test. For validation, 27 patients were enrolled prospectively as a paired group. In each, two peripherals were collected approximately 30 min before and 1 min after AVS. Cortisol was compared using Wilcoxon matched-pair signed rank test. Retrospectively, 38% of "30 min-pre" patients had SI < 2 in right adrenals, compared to 14% in "1 min-post" patients (P = 0.007). For the left, 45.9% of "30 min pre" patients had SIs < 2 compared to 13.5% in "1 min-post" patients, (P = 0.002). Prospectively, peripheral cortisol 1 min post decreased by 28% compared to 30-min pre (median, 6.4 to 4.6 μmol/L; P < 0.001). SIs increased 40% bilaterally (P < 0.001). In conclusion, in unstimulated AVS, collecting peripheral samples after sampling the adrenal veins is more likely to give more accurate SI than before sampling the adrenal veins.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal lung disease leading to end-stage lung disease (ESLD). Single lung transplantation (SLT) is the primary treatment option for IPF; however, the native lung continues to influence post-transplant outcomes.

OBJECTIVES: To determine whether the native lung continues to deteriorate under post-transplantation immunosuppression treatment by assessing chest computed tomography (CT) and perfusion scans.

METHODS: We conducted a single-center retrospective analysis of patients who underwent SLT for IPF between 2016 and 2023. Serial chest CT scans assessed native lung changes. CT signs of fibrosis were scored for severity according to published criteria for defining pulmonary fibrosis disease progression. Lung volumes and perfusion were calculated.

RESULTS: Among 57 patients (mean age 57 years; 33% female), 42% died during follow-up (median survival 95 months). The most common immunosuppressive regimen (54% of patients) included prednisone, calcineurin inhibitor, and mycophenolate mofetil. CT analysis demonstrated that in 41/57 (72%) patients, fibrosis signs continued to deteriorate. There was also a significant correlation decline in native lung volume and perfusion scans over time (P = 0.0003, P < 0.0001, respectively) (r = 0.82, P = 0.03).

CONCLUSIONS: Fibrotic progression in the native lung persists after SLT as demonstrated by both chest CT and nuclear perfusion scan, thus highlighting the importance of ongoing monitoring for accuracy and complications assessment, integrating it into routine surveillance, and ensuring it is consistently considered in post-transplant assessments.

PURPOSE: This study aims to evaluate the safety and effectiveness of TIPS-PVR in patients with cirrhosis with acute PVT.

MATERIALS AND METHODS: A retrospective analysis of cirrhotic patients with acute PVT who underwent TIPS-PVR at a single academic institution. Patients were categorized by PVT etiology and thrombus extent according to AASLD criteria. Outcomes assessed included technical success, one-year patency of the TIPS and porto-mesenteric venous system, need for TIPS reintervention, symptom recurrence, and overall survival. Overall survival was defined as the time from TIPS-PVR to death or last follow-up.

RESULTS: Fifty cirrhotic patients underwent TIPS-PVR for acute PVT. Technical success was 100%. Superior mesenteric vein (SMV) involvement was seen in 30/50 (60%), and splenic vein in 11/50 (22%). At one year, 32 patients had follow-up. Primary patency was 75% (24/32), primary-assisted patency 94% (30/32) for TIPS and 97% (31/32) for the portal vein. Patency rates improved at one year: Main portal vein from 18 to 97% (p < 0.001), SMV from 50 to 91% (p < 0.001), splenic vein from 78 to 100% (p = 0.0108). 9 patients underwent liver transplantation after TIPS-PVR. There were no grade 4 or grade 5 CIRSE adverse events. There were five Grade 6 events after the procedure. Overall survival was 78% at 12 months, with 95% CI 64-87%.

CONCLUSIONS: TIPS-PVR appears to be a safe and effective procedure for cirrhotic patients with acute PVT, offering a viable option for restoring portal venous flow.

Preprocedural chest or cardiac imaging for cardiothoracic surgery is focused on the imaging necessary to inform the performance of a surgical procedure after an initial diagnosis and the decision to operate has been made with consideration of patient comorbidities and anesthesia risk. The diverse range of noncoronary cardiac surgeries, coronary cardiac surgeries, and thoracic surgeries each have their own unique surgical techniques, risks, and complications, which can further vary between patients undergoing first time or repeat cardiothoracic surgery. This document reviews the literature for preprocedural chest or cardiac imaging in patients with and without a history of cardiothoracic surgery. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

PURPOSE: Hepatocellular carcinoma (HCC) is a major cause of cancer mortality in sub-Saharan Africa, with most cases arising from chronic hepatitis B virus (HBV) or hepatitis C virus (HCV) infections. Despite Rwanda's national hepatitis guidelines recommending biannual surveillance with abdominal ultrasound (US) and alpha-fetoprotein (AFP) testing, the extent of adherence remains unknown. This study aimed to assess adherence to HCC surveillance guidelines among HBV- and HCV-infected patients at a national referral hospital in Rwanda.

METHODS: We conducted a retrospective descriptive study of HBV- and HCV-infected patients attending the Rwanda Military Referral and Teaching Hospital between January 2022 and December 2024. Surveillance adherence was assessed based on the proportion of time covered by abdominal US and AFP testing, with coverage categorized as optimal (100%), intermediate (50%-99%), or poor (<50%).

RESULTS: Among 388 patients (mean age, 41.9 years; 73.4% male), 82.7% were HBV-infected. Over one third (31.4%) and nearly half (44.6%) of the patients never received an US or AFP test, respectively. Only 15.5% achieved optimal US coverage, and 12.1% had optimal AFP coverage. Surveillance coverage was worse in patients age 31-50 years and those residing in Kigali. Paradoxically, patients from rural provinces demonstrated better adherence. Thirteen patients (3.4%) had liver lesions detected on US, although lesion status was undocumented in 44.6% of cases.

CONCLUSION: Adherence to HCC surveillance guidelines in Rwanda is suboptimal, with significant gaps across age groups and regions. These findings underscore the need for integrating HCC surveillance into routine hepatitis care and leveraging Rwanda's decentralized health system to improve early cancer detection.

BACKGROUND: New-onset postoperative atrial fibrillation (POAF) complicates 30% of cardiac surgeries. Although POAF is often transient, structured follow-up care of patients with POAF may identify those with paroxysmal or persistent atrial fibrillation (AF) who will benefit from evidence-based therapies.

METHODS: This retrospective study includes patients seen in a clinic dedicated to patients with POAF after cardiac surgery between 2020 and 2024. Per the clinic's operating procedure, patients wore a 14-day continuous ambulatory electrocardiogram (ECG) monitor fpr 2 months after surgery and were assessed thereafter in clinic. The primary outcome was recurrent AF lasting ≥ 30 seconds, captured by 14-day continuous ambulatory ECG or during clinical care.

RESULTS: The cohort included 881 patients, with a mean age of 68 ± 9 years, and a median Congestive Heart Failure, Hypertension, Age ≥ 75 Years, Diabetes Mellitus, Stroke, Vascular Disease, Age 65 to 74 Years, Sex Category (CHA2DS2-VASc) score of 2 (interquartile range [IQR] 1-3); 529 patients (60.0%) underwent isolated coronary artery bypass grafting. At discharge, 798 patients (90.6%) were prescribed amiodarone, and 435 (49.4%) were prescribed oral anticoagulation. The mean time between discharge and 14-day continuous ambulatory ECG monitor was 72 days (IQR 61-84). AF recurrence was detected in 94 patients (10.7%); 30 patients (36.1%) were not receiving oral anticoagulation at the time of recurrence. Among patients with recurrence detected by 14-day continuous ambulatory ECG, the median duration was 10 hours (IQR 2-253). Left atrial volume index was the only independent predictor of AF recurrence. Following the clinic visit, oral anticoagulation was continued in 122 patients (28.2%).

CONCLUSIONS: Among patients with POAF following cardiac surgery, 1 in 10 have AF recurrence, as determined by a structured 14-day continuous ambulatory ECG monitor utilized 2-3 months postoperatively.

Group 3 medulloblastoma (G3MB) is a devastating disease of the central nervous system (CNS) that primarily affects infants and children. Chimeric antigen receptor (CAR) T cell therapy holds the promise to improve outcomes for CNS malignancies, but few studies have focused specifically on G3MB. We used publicly available datasets to demonstrate EphA2 and B7-H3 expression in primary G3MB and validated expression in patient-derived cell lines. EphA2-CAR T cells had greater cytolytic activity, persistence, and TH1 cytokine production than B7-H3-CAR T cells in coculture assays with MYC-driven G3MB cell lines in vitro. In vivo, EphA2-CAR T cells demonstrated superior tumor control and improved survival compared to B7-H3-CAR T cells in 2 of 3 orthotopic G3MB models. B7-H3-CAR T cells outperformed EphA2-CAR T cells in one model in which the antigen density of EphA2 was 5-fold lower than for B7-H3. The limited antitumor activity of EphA2-CAR T cells could be overcome with second genetic modifications that increase T cell functionality including deletion of DNMT3A or the expression of a constitutively active IL-18 chimeric cytokine receptor. Thus, our study nominates EphA2-CAR T cells as a promising alternative to B7-H3-CAR T cells, which are actively being explored in clinical studies for medulloblastoma.

Hyperactive RAS signaling, induced by mutations in NRAS, HRAS, or KRAS, drives tumorigenesis in most PAX3/7::FOXO1 fusion-negative rhabdomyosarcomas (FN-RMS). Despite the frequency of these mutations, indirect RAS pathway-directed therapies have been ineffective for RAS-driven RMS. Farnesyltransferase (FTase) inhibitors (FTIs), such as tipifarnib, inhibit HRAS membrane localization and blunt RAS effector signaling, leading to an antitumor effect in HRAS-mutant FN-RMS preclinical models. However, the effect is not durable. Here, we investigated the mechanisms of adaptive resistance that limit the activity of FTIs, revealing that response to FTIs was limited by adaptive feedback reactivation of ERK signaling and upregulation of wild-type (WT) RAS. The combination of HRAS suppression with FTI and MEK inhibition (MEKi) impaired ERK reactivation and reduced ERK transcriptional output in HRAS-mutant RMS models. Co-targeting FTase and MEK restrained tumor progression and induced terminal myogenic differentiation. These findings highlight an effective combinatorial strategy and support its preclinical translation for patients with HRAS-mutant RMS.

A rudimentary horn is a type of congenital uterine anomaly associated with a unicornuate uterus. Rudimentary horn pregnancies (RHPs) are extremely rare and the majority of cases result in second trimester rupture necessitating emergent laparotomy with associated high maternal morbidity and delivery of a previable fetus. We report the case of a 33-year-old G5P0131 female who presented at 15 weeks and 4 days gestational age with pelvic and back pain and imaging favoring an abdominal pregnancy. The patient underwent an exploratory laparotomy with left salpingectomy and excision of an unruptured extrauterine pregnancy that was confirmed to be an RHP on pathologic examination. Postsurgery review of the imaging demonstrated several features consistent with the diagnosis of an RHP that were not initially identified. Furthermore, the patient's medical and surgical histories were notable for several risk factors associated with congenital uterine anomalies that should have heightened the clinical suspicion for an RHP. This case emphasizes how increased familiarity with the risk factors and imaging findings associated with rudimentary horns and RHPs may lead to an earlier and more accurate diagnosis, more timely and appropriate treatment, and ultimately a reduction in maternal and fetal morbidity and mortality.

Recent reductions in U.S. global health funding have disrupted essential programs in sub-Saharan Africa (SSA), highlighting the region's vulnerability to external financing shocks. The suspension of USAID initiatives has affected disease control, maternal care, and health system operations across 47 countries, raising urgent questions about how to sustain progress without reliable donor support. This commentary examines the potential of Public-Private Partnerships (PPPs)-structured collaborations in which governments and private actors share financing, risk, and managerial responsibility-to strengthen domestic capacity. Drawing on examples from Senegal, Nigeria, and Kenya, we explore how service, concession, financing, and technology-focused PPPs can mobilize additional resources, expand access, and improve service delivery. We also address key challenges, including governance risks, fiscal constraints, and shifting global power dynamics. While not a substitute for aid, well-designed PPPs aligned with national priorities can support more resilient, equitable, and self-reliant health systems in SSA.