Pulmonary Complications of Everolimus in Liver Transplant Patients: A 10-Year Experience.

This retrospective study aims to evaluate the safety of everolimus when used as part of the immunosuppression regimen in patients who underwent liver transplant from 2009 to 2019 at a tertiary liver transplant center. Patients were divided into two groups: those who received everolimus as part of the post-transplant regimen and those who did not. The primary safety outcome measured was the development of new pulmonary complications that had been associated with everolimus use in prior studies. Lung function was determined by pulmonary function tests if available or CT scans of the chest. Secondary outcomes measured included everolimus discontinuation rates and survival rates. During the study period, 450 patients underwent liver transplant; 35% of patients received everolimus (n=156) and 65% of patients did not receive everolimus (n=292). Primary safety outcome of pulmonary complications was seen in 3.9% of patients who received everolimus (n=6) and 6.3% of the control group patients who did not receive everolimus (n=19). The association between everolimus use and new pulmonary complications was not significant with a chi-square statistic of 1.33 (p=0.249). Overall, 51.3% of patients who received everolimus during their post-transplant course discontinued the medication (n=80). Everolimus is safe from a pulmonary toxicity standpoint in liver transplant immunosuppression regimens as there was no significant difference found in pulmonary complications between patients who received the medication and those who did not.

Systemic right ventricle complications in levo-transposition of the great arteries: A case report and review of literature.

BACKGROUND: Congenitally corrected levo-transposition of the great arteries (L-TGA) is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy. In L-TGA, the double discordance, atrioventricular and ventriculoarterial, create an acyanotic milieu which allows patients to survive their early decades, however, progressive systemic right ventricle (sRV) dysfunction creates complications later in life. sRV dysfunction and remodeling predisposes patients to intracardiac thrombus (ICT) formation. CASE SUMMARY: A 40-year-old male with L-TGA presented with symptoms of acute decompensated heart failure. In childhood, he had surgical repair of a ventricular septal defect. In adulthood, he developed sRV dysfunction, systemic tricuspid valve (sTV) regurgitation, and left-bundle branch block for which he underwent cardiac resynchronization therapy. Transthoracic echocardiogram showed a sRV ejection fraction of 40%, severe sTV regurgitation, and a newly identified sRV ICT. ICT was confirmed by ultrasound-enhancing agents and transesophageal echocardiography. Our patient was optimized with guideline-directed medical therapy and diuresis. Anticoagulation was achieved with a vitamin K antagonist (VKA) and he was later referred for evaluation by advanced heart failure and heart transplant services. CONCLUSION: Anticoagulation with VKA is the mainstay of treatment in the absence of conclusive data supporting direct oral anticoagulant use in ICT in patients with congenital heart disease. This case illustrates the natural history of L-TGA and highlights the importance of surveillance and monitoring with dedicated cardiac imaging to identify complications.

Full-thickness resection device for management of lesions involving the appendiceal orifice: Systematic review and meta-analysis.

Background and study aims Endoscopic resection of lesions involving the appendiceal orifice is technically challenging and is commonly referred for surgical resection. However, post-resection appendicitis is a concern. Many studies have varying rates of post-procedure appendicitis. We aim to report the rate of post-resection appendicitis by performing a systematic review and meta-analysis. Methods Studies that involved the use of a full-thickness resection device (FTRD) for management of appendiceal polyps were included. The primary outcome was appendicitis after FTRD and a subgroup analysis was performed on studies that only included FTRD performed at the appendiceal orifice. Results Appendicitis was encountered in 15% (95%CI: [11-21]) of the patients with 61% (95% CI: [44-76]) requiring surgical management. Pooled rates of technical success, histologic FTR, and histologic R0 resection in this sub-group (n=123) were 92% (95% CI: [85-96]), 98% (95% CI: [93-100]), and 72% (95% CI: [64-84%]), respectively. Post-resection histopathological evaluation revealed a mean resected specimen size of 16.8 ± 5.4 mm, with non-neoplastic pathology in 9 (7%), adenomas in 103 (84%), adenomas + high-grade dysplasia (HGD) in nine (7%), and adenocarcinoma in two (2%). The pooled rate for non-appendicitis-related surgical management (technical failure and/or high-risk lesions) was 11 % (CI: 7-17). Conclusions FTRD appears to be an effective method for managing appendiceal lesions. However, appendicitis post-resection occurs in a non-trivial number of patients and the R0 resection rate in appendiceal lesions is only 72%. Therefore, caution should be employed in the use of this technique, considering the relative risks of surgical intervention in each patient.

Gastrointestinal Stromal Tumor Presenting as Food Impaction and Pseudo-Achalasia.

Gastrointestinal stromal tumors (GISTs) are one of the most common mesenchymal tumors of the gastrointestinal tract. Studies report the incidence of GIST to be 14.5 per million, with 18% being diagnosed incidentally. The most common location is the stomach while the esophagus is the rarest, representing only 0.7% of cases. The clinical manifestations of GISTs vary. Most patients present with bleeding and gastric discomfort. Some may present with life-threatening hematemesis or melena. We present a unique case of a GIST presenting as pseudo-achalasia and food impaction.

A Case of a Large and Rare Incidental Pleural Tumor in an Elderly Female.

Solitary fibrous tumors are very rare in the pleura, and they are generally found incidentally. Even though they can potentially become malignant and metastasize, they have minimal clinical symptoms and can still be benign. Due to the low incidence of these tumors, there is no standard of therapy beyond surgical resection. We present an asymptomatic case of a large, rapidly expanding solitary fibrous tumor of the pleura in an elderly female.

The First Collective Examination of Immunosuppressive Practices Among American Intestinal Transplant Centers.

Background: Unlike other solid organs, no standardized treatment algorithms exist for intestinal transplantation (ITx). We established a consortium of American ITx centers to evaluate current practices. Methods: All American centers performing ITx during the past 3 y were invited to participate. As a consortium, we generated questions to evaluate and collect data from each institution. The data were compiled and analyzed. Results: Ten centers participated, performing 211 ITx during the past 3 y (range, 3-46; mean 21.1). Induction regimens varied widely. Thymoglobulin was the most common, used in the plurality of patients (85/211; 40.3%), but there was no consensus regimen. Similarly, regimens for the treatment of acute cellular rejection, antibody-mediated rejection, and graft-versus-host disease varied significantly between centers. We also evaluated differences in maintenance immunosuppression protocols, desensitization regimens, mammalian target of rapamycin use, antimetabolite use, and posttransplantation surveillance practices. Maintenance tacrolimus levels, stoma presence, and scoping frequency were not associated with differences in rejection events. Definitive association between treatments and outcomes, including graft and patient survival, was not the intention of this initial collaboration and is prevented by the lack of patient-level data and the presence of confounders. However, we identified trends regarding rejection episodes after various induction strategies that require further investigation in our subsequent collaborations. Conclusions: This initial collaboration reveals the extreme heterogeneity of practices among American ITx centers. Future collaboration will explore patient-level data, stratified by age and transplant type (isolated intestine versus multivisceral), to explore the association between treatment regimens and outcomes.

Disparities in Referrals to End-of-Life Care in Eligible Hepatocellular Carcinoma Patients.

BACKGROUND: Hepatocellular Carcinoma (HCC) is a malignancy with increasing incidence and morbidity. For patients with a poor prognosis, engagement with advanced care planning and end-of life (EOL) services (I.e., palliative care, hospice) can address physical, financial, and social complications of a terminal diagnosis. Minimal data exist on the demographics of the patients being referred to and enrolling in EOL services for HCC. AIMS: We aim to report the relationship between demographics and EOL service referral. METHODS: Retrospective review of a prospectively maintained high-volume liver center registry of patients diagnosed with HCC from 2004 to 2022. EOL services eligible patients were defined as BCLC stage C or D, evidence of metastases, and/or transplant ineligible. RESULTS: Black patients were more likely to be referred than white patients (OR 1.47 (1.03, 2.11)). Once referred, patients were significantly more likely to be enrolled if they had insurance coverage, though no other factors in models were significant. There were no significant differences in survival among those referred who did or did not enroll, after controlling for other factors. CONCLUSION: Black patients were more likely to be referred compared to white patients and patients who were insured were more likely to be enrolled. Whether this is indicative of black patients being appropriately referred at a higher rate, being offered EOL care instead of aggressive treatment, or other unknown factors warrants further study.

Splenic Artery Embolism in Liver Transplant Patients: A Single-Center Experience.

BACKGROUND: Hypersplenism, portal hypertension, and ascites have been seen after liver transplants. Patients are usually treated medically with refractory patients potentially undergoing splenectomy. Splenic artery embolism (SAE) is an alternative that can be performed to limit the surgical intervention that may have the benefit of improving portal hypertension. Few studies have studied the effect on main portal vein (MPV) velocities and hepatic artery resistive indices (HARIs) which may be beneficial as markers of portal hypertension. PURPOSE: This study aims to evaluate the efficacy and safety of interventional radiology (IR)-guided SAE for the management of portal hypertension in patients who have had liver transplants. METHODS: A retrospective analysis was conducted on liver transplant patients who had undergone IR-guided SAE post-transplant at a single tertiary transplant center from 2012 to 2022. The primary outcome of intervention efficacy was quantified by peak HARIs and MPV velocities. Ultrasound with Doppler obtained before and after the intervention was reviewed for these parameters. Secondary outcomes included adverse events at the time of the procedure and within one year of the procedure, the need for splenectomy, and spleen size. RESULTS: Twenty-eight patients met the criteria for inclusion. The mean age of patients was 52.5 years (21-71 years) and the time after transplant was 149.5 days (2-1588 days). About 96.4% of SAEs were technically successful (n=27). Twenty-one patients had MPV velocities available, and 24 had peak HARIs available. In these patients, HARIs decreased by an average of 0.063 (95% CI 0.014-0.112) after SAE. MPV velocity decreased by an average of 47.2 cm/s (95% CI 27.3-67.1) after SAE. About 10.4% of patients (n=3) developed a procedure-related complication, all of which were femoral access site aneurysms. No (0) patients suffered from bleeding, infections, or abscesses after the procedure. About 10.7% of patients (n=3) required splenectomy after SAE: one splenectomy was due to technical failure and two were due to refractory symptoms. CONCLUSION: We performed one of the first analyses on MPV and RI and showed that our patients saw an improvement post-embolization with a theoretical improvement in portal hypertension. The complication rate and risk of infection seem to be acceptable risks, making SAE a feasible option for management.

False Reassurance From a Stool Test: A Delayed Cancer Diagnosis.

Colorectal cancer (CRC) is an increasingly prevalent condition in the United States and the world. Numerous screening tools have been created to help prevent and identify early cases of CRC, which have led to better outcomes for patients. These screening tools range from stool tests to more invasive procedures like a colonoscopy. With this array of screening options, patients are often presented with a bevy of choices in their primary care clinic and may have difficulty understanding the difference between screening and treatment. Popular culture has also influenced these decisions as both traditional media and social media have weighed in on the experience with these screening tools. We present a unique case where our patient tested negative on a stool screening exam and proceeded to be diagnosed with CRC within the negative screening period. The case was complicated by the patient's reluctance to undergo a colonoscopy and a unique combination of symptoms that led to a difficult diagnosis.

Application of Impella Mechanical Circulatory Support Devices in Transcatheter Aortic Valve Replacement and Balloon Aortic Valvuloplasty: A Single-Center Experience.

BACKGROUND/PURPOSE: Percutaneous valve interventions for aortic stenosis (AS) include transcatheter aortic valve replacement (TAVR) and balloon aortic valvuloplasty (BAV). Intraprocedural mechanical circulatory support (MCS) with Impella devices (Abiomed, Danvers, MA) is used in select high-risk patients, although data regarding its efficacy is limited. This study sought to evaluate the clinical outcomes of Impella use in patients with AS who underwent TAVR and BAV at a quaternary-care center. METHODS/MATERIALS: All patients with severe AS who underwent TAVR and BAV with Impella between 2013 and 2020 were included. Patient demographics, outcomes, complications, and 30-day mortality data was analyzed. RESULTS: Over the study period 2680 procedures were performed, 1965 TAVR and 715 BAV. 120 utilized Impella support, 26 TAVR and 94 BAV. Among TAVR Impella cases, justifications for MCS included cardiogenic shock (53.9 %), cardiac arrest (19.2 %), and coronary occlusion (15.4 %). Among BAV Impella cases, justifications for MCS included cardiogenic shock (55.3 %) and protected percutaneous coronary intervention (43.6 %). The 30-day mortality rate in TAVR Impella was 34.6 % and in BAV Impella was 28 %. BAV Impella cases involving cardiogenic shock had a higher rate of 45 %. Impella remained in-use past 24 h from the procedure in 32.2 % cases. Vascular access-related complications occurred in 4.8 % cases and bleeding complications occurred in 1.5 % cases. Conversion to open-heart surgery occurred in 0.7 % cases. CONCLUSIONS: MCS is an option for high-risk patients with severe AS who require TAVR and BAV. Despite hemodynamic support, the 30-day mortality rate remained high especially in cases where support was employed for cardiogenic shock.

Malignant Cardiac Tamponade: A Complication of Untreated Breast Cancer.

Carcinomatous pericarditis is a rare complication of locally aggressive breast cancer in which malignant cells directly extend into the pericardium causing inflammation and creating a pericardial effusion. A 40-year-old woman with untreated metastatic breast cancer presented to an outpatient clinic in significant distress with symptoms of progressive shortness of breath and bilateral leg swelling. An urgent echocardiogram demonstrated a large pericardial effusion with echocardiographic evidence of cardiac tamponade. She underwent emergent pericardiocentesis of the effusion that was deemed to be malignant after cytologic evaluation. Subsequently, she opted for palliative treatment involving the surgical creation of a right pericardial window and placement of an indwelling pleural catheter. Internists should maintain a high index of suspicion for malignant cardiac tamponade in at-risk patients, especially those with locally aggressive and advanced malignancies.

Shrinking Lung Syndrome in a Young Female: A Rare Pulmonary Manifestation of Systemic Lupus Erythematosus.

Shrinking lung syndrome (SLS) is a rare pulmonary complication of autoimmune conditions. It has been sparsely described in the literature and its pathophysiology remains unclear. SLS is typically reported in patients with a history of systemic lupus erythematosus (SLE) who present with shortness of breath and chest pain associated with breathing. Chest imaging demonstrates no alveolar, interstitial, or pleural abnormalities. Pulmonary function tests (PFTs) are characterized by a restrictive pattern with reduced lung volumes. SLS is a diagnosis of exclusion and there are no validated criteria for the diagnosis. Evaluation requires extensive testing to rule out alternative causes of dyspnea and pleuritic chest pain. In this report, we present a case of SLS in a young African American woman.

Nonuremic Calciphylaxis Precipitated by COVID-19 Infection.

Calciphylaxis is a rare dermatologic condition that is primarily associated with end-stage renal disease (ESRD). Nonuremic calciphylaxis has been reported in patients with autoimmune disorders such as systemic lupus erythematosus and other hypercoagulable states such as anti-phospholipid syndrome. New research throughout the COVID-19 pandemic has shown an increased inflammatory and coagulopathic complication of COVID-19. We present a case of a patient with nonuremic calciphylaxis following treatment for severe COVID-19 and no known cause of hypercoagulability. A 40-year-old Caucasian female with a history of recent COVID-19 infection requiring hospitalization, hypertension, alcohol abuse, anxiety, and one prior spontaneous miscarriage presented to the hospital with bilateral lower extremity wounds. The wounds were seen to have necrosis and eschar formation, as well as blackened mottled skin, and were extremely painful to the patient. The initial lesions were on the anterior thighs bilaterally and spread laterally and to the lower back. Initial autoimmune workup was non-specific, and biopsy confirmed calciphylaxis. Calciphylaxis is a known dermatologic disease that has high mortality and morbidity, but it is usually associated with ESRD. Some cases have been reported for autoimmune or hypercoagulable states. The disease presents with non-healing, painful skin ulcers that are at a high risk of infection and have poor healing. The case presented shows biopsy-confirmed calciphylaxis in the absence of known etiologies, and we hypothesize that it is due to COVID-19 or COVID-19 aggravating an underlying but unidentified hypercoagulable condition.

The role of miR-150 regulates bone cell differentiation and function.

BACKGROUND: mir-RNAs play a role in regulating bone homeostasis. In this study we assessed the functional role of mir-RNA 150 in bone homeostasis. We also assess the effects of miR-150 deficiency on osteoblast and osteoclast differentiation and function using in vivo and in vitro approaches. METHODS: Wild type (WT) (C57BL/6J) and miR-150 KO mice were compared for a variety of parameters. Micro-CT imaging was conducted to quantify trabecular bone mass inferior to the distal growth plate of the femur. Von Kossa staining was performed for osteoblast culture mineralization. RT-qPCR, biochemical analysis and bone histomorphometry were utilized for quantification of relevant genes and serum protein measurements. Differentiation and function of osteoblasts and osteoclasts was performed using primarily cultures and assessed the cell autonomous response of mir-RNA-150 on cell differentiation and function. RESULTS: Mir-150 exhibited expression in a variety of tissues and increases progressively with age. Through micro-CT imaging, we found that KO mice presented reduced bone mass at 4, 8, and 16 weeks of age compared to WT mice. Furthermore, histomorphometric analysis revealed increased trabecular separation, decreased bone thickness, and decreased osteoblast number in KO compared to WT mice. Mir-150 deficiency also correlated with higher bone resorption, accompanied with significant increases in CTX-1 serum levels, and a decrease in cell apoptotic rate ex vivo. Additionally, miR-150 KO mice showed increased osteoblast differentiation and decreased osteoclastogenesis ex vivo. Luciferase assay showed increased Osteoactivin/GPNMB expression in miR-150 KO osteoblasts compared to WT cells. CONCLUSION: Our data suggests that miR-150 influences osteoblast and osteoclast functionality and differentiation; specifically, miR-150 serves as a negative regulator for osteoblasts and a positive regulator for osteoclasts by regulating, at least in part, Osteoactivin/GPNMB expression.