Clinical Course Of Patients Research Articles

ATrial arrhythmias in inhEriTed aRrhythmIa Syndromes: results from the TETRIS study

Abstract Background Little is known about the distribution and clinical course of patients with inherited arrhythmia syndrome (IAS) and concomitant atrial arrhythmias (AAs). Aim 1) to characterize the distribution of AAs in patients with IAS and 2) evaluate the long-term clinical course of these patients. Methods An international multicenter study was performed and involved 28 centers in 16 countries. Inclusion criteria were: 1) IAS and 2) ECG documentation of AAs. The primary endpoint was a composite of sudden cardiac death, sustained VAs or appropriate ICD interventions. Strokes, inappropriate ICD shocks due to AAs, and the occurrence of sinus node dysfunction were assessed. Results A total of 522 patients with IAS and AAs were included. Most patients were diagnosed with Brugada syndrome (n=355, 68%) and long-QT syndrome (n=93, 18%). The remaining patients (n=71, 14%) presented with short-QT syndrome, early repolarization syndrome (ERS), catecholaminergic polymorphic ventricular tachycardia (CPVT), progressive cardiac conduction diseases, or idiopathic ventricular fibrillation. Atrial fibrillation (AF) was the most prevalent AA (82%), followed by atrial flutter (9%) and atrial tachycardia (9%). AA was the first clinical manifestation of IAS in 52% of patients. More than one type of AAs was documented in 23% of patients. Nine patients (3%) experienced VA before the diagnosis of IAS, due the use of anti-arrhythmic medications taken for the AA. The incidence of the primary endpoint was 1.4% per year, with a twofold increase observed in patients who experienced their first AA before the age of 20 (OR 2.2, p=0.043). This was consistent across the different forms of IAS. Inappropriate ICD shock due to AAs were reported in 2.8% of patients, strokes in 4.4% and sinus node dysfunction in 9.6%. Conclusions Among patients with IAS and AAs, AA is the first clinical manifestation in about half of the cases, with more than one form of AAs present in one-fourth of the patients. The occurrence of AA earlier in life may be associated with a higher risk of ventricular arrhythmias. The occurrence of stroke and sinus node dysfunction is not-infrequently in this cohort.

Patterns and factors associated with electrolyte abnormalities among patients with heart failure in Uganda.

Electrolyte abnormalities (EAs) worsen the clinical course of patients with heart failure (HF). The patterns of EAs vary among patients with HF. This study investigated patterns and factors associated with EAs among patients with HF admitted to Hoima Regional Referral Hospital (HRRH) in western Uganda. This hospital-based cross-sectional study used quantitative data of 384 HF patients admitted at HRRH between 21st February and 15th May 2023. Data on sociodemographic, lifestyle, and medical characteristics were collected and presented as descriptive statistics. EAs were considered electrolyte values below or above the reference normal ranges. Bivariate and multiple logistic regression analyses were conducted to establish associations. An association with a p < 0.05 is considered statistically significant. Of 384 HF patients, 342 (89.1%) had EAs. Hypocalcemia was the most common EA, 165 (43.0%). Among the patients, 69 (18.0%) were on diuretics, 185 (48.2%) were on angiotensin-converting enzyme inhibitors (ACEIs)/angiotensin receptor blockers (ARBs), and 105 (27.3%) were on calcium channel blockers (CCBs). Additionally, 264 (68.8%) had a history of hypertension, and 20 (5.2%) demonstrated good drug adherence. Patients with good drug adherence had lower odds of EAs (Adjusted Prevalence Odds Ratio [Adjusted POR] = 0.2, 95% CI: 0.1-0.7, p = 0.009). Those on diuretics had higher odds of EAs compared to those on ACEIs/ARBs and CCBs, with an Adjusted POR of 5.7 (95% CI: 1.3-15.0, p = 0.019). A history of hypertension also increased the odds of EAs (Adjusted POR = 4.0, 95% CI: 1.9-8.4, p < 0.001). The prevalence of EAs in patients with HF at HRRH was high, with hypocalcemia being the most common. Patients with good drug adherence had lower odds of EAs. On the other hand, diuretic use and a history of hypertension were associated with increased odds of EAs.

Mechanism and significance of diffusion restriction followed by calcification in high-grade glioma treated with bevacizumab

In this study, we focused on calcification and diffusion restriction, which sometimes appear around the resection cavity or periventricular white matter in patients with high-grade glioma (HGG) treated with bevacizumab (BVZ), as candidate imaging biomarkers for BVZ treatment efficacy. We investigated the timing of the appearance of diffusion restriction and calcification using magnetic resonance imaging and computed tomography in 35 patients with newly diagnosed or recurrent HGG treated with BVZ. In 17 (48.6%) patients, calcification was identified around the resection cavity or periventricular white matter at a median of 12 months after the initiation of BVZ treatment. Patients with calcification had significantly longer progression-free survival (16 vs. 7 months; p = 0.0023) and overall survival (36 vs. 12 months; p = 0.0006) than those without calcification. Histopathological examination revealed the presence of scattered microcalcifications within areas of necrosis, which suggested dystrophic calcification induced by BVZ. Diffusion-restricted lesions that appeared in patients with calcification had significantly lower apparent diffusion coefficients than those in patients without calcifications, indicating the presence of treatment-related necrosis but not hypercellularity. In conclusion, the radiological finding of diffusion restriction followed by calcification could be a potential imaging biomarker for favorable clinical course in patients with HGG treated with BVZ.

Clinical Course of Patients Relisted for Orthotopic Heart Transplantation: A National Study

Clinical Course of Patients Relisted for Orthotopic Heart Transplantation: A National Study

Risk of death, thromboembolic events, and bleeding in elderly patients with Atrial Fibrillation and Clinical Complexity who are not taking oral anticoagulation

Abstract Background Patients with Atrial Fibrillation (AF) often exhibit Clinical Complexity (CC), defined as ≥2 features, i.e. patients aged ≥75 years plus a body mass index (BMI) &amp;lt; 23/kg/m2 and/or bleeding history and/or chronic kidney disease. No data are available about the clinical course of patients with Atrial Fibrillation (AF) and CC who are not taking oral anticoagulation (OAC). Purpose To assess the risk of thrombotic and hemorrhagic events in patients with Atrial Fibrillation (AF) and CC who are not taking OAC. Methods Retrospective study with a health care research network (TriNetX). Based on the ICD-10-CM codes entered between 2011-2022 from 85 health care organizations mainly located in the United States, AF patients ≥75 years with CC were categorized into two groups based on the use of OAC during the last year before the study period. The primary outcomes were the one-year risk of death, major cardiovascular events ([MACE]: heart failure, stroke, and myocardial infarction), and major bleeding (central nervous system, gastrointestinal, internal bleeding, and hypovolemic shock). Secondary outcomes were each component of the primary outcome and catheter ablation. Cox regression analyses were used to calculate hazard ratios (HRs) and 95% CIs following propensity score matching 1:1. Results Overall, we identified 271,402 AF CC patients not on OAC (80.1±6.8 years, 46.5% females) and 87,101 AF CC patients on OAC (79.8±6.5 years, 47.6% females). Before PSM, AF CC patients not on OAC showed no significant differences in cardiovascular and bleeding risk factors but were less treated with pharmacological treatment than AF CC patients on OAC. After PSM, no significant differences were found between the two groups, but AF CC not on OAC were at higher risk of all-cause death (HR 1.54, 95%CI 1.50-1.57), MACE (HR 1.13, 95%CI 1.11-1.16), and major bleeding (HR 1.34, 95%CI 1.31-1.38), when compared to AF CC on OAC. AF CC patients not on OAC were associated with a higher risk of each component of the primary outcome and with a lower use of catheter ablation procedures (HR 0.48, 95%CI 0.43-0.54) (Table 1). The risk of the composite outcomes was the highest in those with all the three CC components. A progressive increase in OAC prescription was observed during the follow-up period, with increasing of NOACs and lower use of warfarin being observed. There was a clear temporal relationship between increasing OAC prescription and incidence of bleeding in CC patients (Figure 1) Conclusions AF CC patients not on OAC have a high risk of all-cause death, thrombotic and hemorrhagic events. Over time, increasing NOAC use was associated with more bleeding in CC patients. New antithrombotic approaches aimed at reducing thrombotic risk without increasing bleeding risk are needed in these patients.Figure 1 OAC new prescriptions

Novel risk scoring model to assess the need for venoarterial-extracorporeal membrane oxygenation in patients with acute myocarditis

Abstract Background Some patients with acute myocarditis unpredictably deteriorate into a fulminant form and their prognosis is poor. However, it is uncertain which acute myocarditis patients are likely to exacerbate and even require venoarterial-extracorporeal membrane oxygenation (VA-ECMO). Objectives This study aimed to identify the risk factors associated with the need for VA-ECMO in patients with acute myocarditis and to develop a predictive model. Methods This retrospective study included 841 patients from seven hospitals in Korea with biopsy-proven or clinically suspected acute myocarditis. Logistic regression was performed to identify clinical characteristics of patients who required VA-ECMO and to construct a scoring system that can predict the need for VA-ECMO. Results Among the study population, 217 (25.8%) patients underwent VA-ECMO. The study population was divided into training (N=621) and testing (N=220) cohorts according to participating center. The final predictive model of VA-ECMO insertion derived from the training cohort included the following: initial mean blood pressure &amp;lt;65mmHg, cardiac arrest, Glasgow Coma Scale ≤12, platelet &amp;lt;100×103/mL, pulmonary congestion on chest X-ray, QRS interval ≥120ms, left or right bundle branch block, and left ventricular ejection fraction &amp;lt;40%. Using this predictive model, a β-coefficient-weighted Korean Acute Myocarditis (KAM) score was developed. The VA-ECMO insertion rate increased with increasing KAM score (odds ratio 1.683, 95% confidence interval 1.529-1.853, P&amp;lt;0.001). External validation of the predictive model and KAM score using the testing cohort showed excellent discriminant ability (areas under the curve of 0.945 and 0.921, respectively). Furthermore, at a KAM score of 8 or higher, which was the cut-off KAM score to predict the need for VA-ECMO, patients without VA-ECMO tended to have higher in-hospital mortality than patients who needed VA-ECMO. Conclusions A risk scoring system based on simple clinical and laboratory parameters at initial presentation could predict the need for VA-ECMO and clinical course in patients with acute myocarditis.Graphic Abstract

Adverse events following transcatheter aortic valve implantation (TAVI) in patients with amyloidosis: observations from a global federated network

Abstract Background A high proportion of patients with aortic stenosis are affected by cardiac amyloidosis, but there is a paucity of data describing the effect of amyloidosis on the clinical course of patients who underwent transcatheter aortic valve implantation (TAVI). Purpose To assess the one-year risk of adverse events following TAVI in patients with amyloidosis. Methods Retrospective study using data from a large multinational electronic health record network (TriNetX). Patients aged 18 years or older who underwent TAVI between 2005 and 2022 were included in the analysis. The patients were categorised in two groups based on the presence of amyloidosis (ICD-10-cm code E85). Primary outcome was the one-year risk of the composite of emergency department (ED) admission, acute heart failure (HF), stroke, conduction system disease, pacemaker (PM) implantation, acute kidney injury, and all-cause death. Secondary outcomes were the risks for each component of the composite outcome. We used Cox regression analysis to calculate hazard ratio (HR) and 95% confidence interval (CI) after propensity score matching (PSM) 1:1. Results We identified 477 TAVI recipients with a diagnosis of amyloidosis (age 78.6±8.1 years, 31.4% females) and 43,379 TAVI patients without a diagnosis of amyloidosis (78.1±8.8, 40.2% females). Before PSM, patients affected by amyloidosis were more likely to be Black or African American and had a higher prevalence of cardiovascular risk factors compared to TAVI patients without amyloidosis. After PSM, patients with a diagnosis of amyloidosis showed a higher one-year risk of composite outcome (HR 1.28, 95%CI 1.10-1.49) compared to those who underwent TAVI without amyloidosis. Regarding secondary outcomes, amyloidosis patients had a higher risk of ED admission (HR 1.41, 95%CI 1.13-1.75), acute HF (HR 1.56, 1.23-1.99), stroke (HR 1.55, 1.04-2.32), conduction system disease (HR 1.27, 1.04-1.55), PM implantation (HR 1.55, 1.06-2.28), but not acute kidney injury (HR 1.11, 0.84-1.48) and all-cause death (HR 0.98, 0.66-1.45). Conclusion Among patients undergoing TAVI, those affected by amyloidosis are at higher risk of adverse events in the first year after TAVI. More studies are needed to define the exact role of amyloid fibrils in structural valve deterioration and to elucidate whether subtypes of amyloidosis have a different impact on patients’ outcomes following TAVI.Risk of composite outcome following TAVI

Left ventricular myocardial work predicts clinical course in patients with heart failure with mildly reduced ejection fraction

Abstract Heart failure with mildly reduced ejection fraction (HFmrEF) often represents a movable patients subset that changes its HF category by increasing or reducing EF. Course prediction is challenging due to insufficient evidence to date. We studied multiple newly established parameters in order to find predictors of clinical course of HFmrEF. Methods 135 patients (61 women) 62±5 years with HFmrEF NYHA II-III on GDMT were enrolled. Longitudinal (GLS), circumferential (GCS) and radial strain (GRS), peak LV twist (LVTR) were measured by speckle tracking from apical and short axis views. Left ventricular (LV) global myocardial work (GW) index (GWI) was obtained from pressure-strain loops derived from LV GLS and supine systemic blood pressure measured right before the image acquisition. Then constructive GW (GCW) as the sum of positive work due to myocardial shortening during systole and negative work due to lengthening during isovolumic relaxation, wasted GW (GWW) as energy loss by myocardial lengthening in systole and shortening in isovolumic relaxation and GW efficiency (GWE) as the percentage ratio of constructive work to the sum of constructive work and wasted work were obtained by dedicated software. All parameters were analyzed offline by one experienced specialist. Patients were followed up for 1 year. Results 38 (28%) patients improved EF to the value ≥50%, 31 (23%) reduced EF to ≤40%. Patients with improved EF had significantly higher absolute values of GLS (17.3±2.2 vs 15.8±3.1%, p&amp;lt;0.03), GCS (18.9±2.6 vs 16.1±1.9%, p&amp;lt;0.02), GRS (42.5±7.2% vs 31.3±4.7%, p&amp;lt;0.02), LVT (6.2±2.5° vs 5.3±2.3°, p&amp;lt;0.03), GCW (2253±121 vs 1872±132 mmHg%, p&amp;lt;0.001) and lower GWW (108±7 vs 128±12 mmHg%) compared with patients with later reduced EF. Multivariate analysis showed that lower GCW was associated with EF reduction (HR 0.982, 95% CI 0.969–0.996, p = 0.012). GCW was the powerful predictor of CV events (AUC 0.94) with cut off value of 1495 mmHg% that had 98% sensitivity and 86% specificity. Conclusion LV GCW predicts EF changes in patients with HFmrEF.

One-year risk of all-cause death and major cardiovascular events in patients presenting with hypertensive crisis. A report from a global federated research health network

Abstract Background Few data are available about the clinical course of patients presenting with hypertensive crisis who are discharged alive from hospital. Purpose To assess the long-term risk of adverse events in patients with hypertensive crises. Methods Retrospective study utilizing a research health network. Based on the ICD-10-CM codes recorded between 2000 and 2022, from 85 health care associations mainly located in the United States, patients with hypertensive crisis were subdivided into hypertensive urgencies (I16.0) and hypertensive emergencies (I16.1). In those with hypertensive emergencies, the type of target organ damage was reported, ie. central nervous system (ischemic stroke, hypertensive encephalopathy, intracerebral hemorrhages), cardiovascular (CVS: myocardial infarction (MI), heart failure (HF), aorta dissection (AD)), or renal (acute kidney failure). Primary outcomes were the one-year risks of all-cause death, and major cardiovascular events (MACE: MI, stroke, cardiac arrest, AD, and HF). Secondary outcomes were the risks for each type of MACE. Cox regression analysis after propensity score matching (PSM) 1:1 was used to produce hazard ratios (HRs) and 95% Confidence Intervals (95%CIs). Results Overall, we identified 25 294 patients with hypertensive emergency (age 62.4±15.7, 46.1% females) and 21,737 patients with hypertensive urgency (age 63.5±17.2, 55.5% females). Patients with hypertensive emergency were more likely males, Black or African American origin and showed a higher prevalence of chronic hypertensive-related complications, vs. those with hypertensive urgency. After PSM, patients with hypertensive emergency showed a higher risk of all-cause death (HR, 1.34, 95%CI 1.25-1.44) and MACE (HR 4.09, 95%CI 3.87-4.33), vs. those with hypertensive urgency. Of the secondary outcomes, patients with hypertensive emergency had increased risks of MI (HR 3.33, 95%CI 3.03-3.67), stroke (HR 4.73, 95%CI 4.33-5.17), cardiac arrest (HR 1.30, 95%CI 1.07-1.58), AD (HR 5.49, 95%CI 3.90-7.73), and HF (HR 3.01, 95%CI 2.74-3.14). All the different types of organ involvement during the hypertensive emergency were each associated with similar long-term risks of adverse events (Figure 1). Conclusion Patients with hypertensive emergency have a high long-term risk of MACE and all-cause death. Preventing the onset of target organ damage in patients with hypertensive crisis is crucial to mitigate their long-term risk of adverse events.

The characteristics and clinical course of patients with melioidosis and cancer.

Patients with an active cancer are more likely to develop melioidosis, but the characteristics and clinical course of melioidosis in patients with cancer have not been examined in detail. Trimethoprim/sulfamethoxazole (TMP-SMX) prophylaxis is prescribed to prevent melioidosis in patients receiving immune suppressing anti-cancer therapy in some jurisdictions-and is recommended in national Australian guidelines-however the risks and benefits of this strategy are incompletely defined. The study took place in Far North Queensland (FNQ) in tropical Australia. The characteristics and clinical course of patients with melioidosis diagnosed in the FNQ region between January 1, 1998, and June 1, 2023, who had-and did not have-an active cancer were compared. We also determined the subsequent incidence of melioidosis in patients receiving immune suppressing anti-cancer therapy in the FNQ region between January 1, 2008, and June 1, 2023, who did-and did not-receive TMP-SMX chemoprophylaxis for Pneumocystis jirovecii infection. An active cancer was present in 47/446 (11%) cases of melioidosis diagnosed between January 1, 1998, and June 1, 2023; there was no association between melioidosis and any cancer type. Patients with melioidosis and cancer were more likely to be older (odds ratio (OR) (95% confidence interval (CI): 1.05 (1.03-1.08) P<0.0001) and immunosuppressed (OR (95% CI): 11.54 (5.41-24.6), p<0.0001) than patients without cancer. Immune suppressing anti-cancer therapy had been prescribed to 17/47 (36%) in the 12 months prior to their diagnosis of melioidosis. Only 10/47 (21%) with cancer and melioidosis in the cohort had received no immune suppressing anti-cancer therapy and had no other risk factors for melioidosis. Twelve months after the diagnosis of melioidosis, 25/47 (53%) were still alive; 9/22 (41%) deaths were due to melioidosis and 13/22 (59%) were due to the underlying cancer. Between 2008 and June 2023, there were 4400 individuals who received myelosuppressive anti-cancer therapy in the FNQ region. There was no significant difference in the incidence of melioidosis between patients who did-and did not-receive TMP-SMX chemoprophylaxis with their myelosuppressive anti-cancer therapy (1/737 (0.15%) versus 16/3663 (0.44%); relative risk (95% confidence interval): 0.31 (0.04-2.34), p = 0.20) and no significant difference in the incidence of fatal melioidosis (0/737 versus 3/3663 (0.08%), p = 0.58). Patients with cancer are predisposed to developing melioidosis and immune suppressing anti-cancer therapy increases this risk further. However, in this region of Australia, there was no significant difference in the subsequent development of melioidosis in patients who did-and did not-receive TMP-SMX chemoprophylaxis during their myelosuppressive anti-cancer therapy.

Foscarnet eyedrops for the treatment of refractory herpetic keratitis

PurposeThe purpose of this case series is to describe the clinical course of patients receiving foscarnet eyedrops for the treatment of refractory herpetic keratitis.ObservationsSix patients diagnosed with herpetic keratitis were treated with foscarnet 24 mg/mL (2.4%) eyedrops with resulting improvement in keratitis.ConclusionTopical foscarnet may be a safe and effective treatment for herpetic keratitis in conjunction with, or as an alternative to, conventional antiviral therapy. This is an off-label use of foscarnet.

Incidence and Risk Factors for Toxic Shock Syndrome After Endoscopic Sinus Surgery: A Systematic Review.

Toxic shock syndrome (TSS) is a rare but serious complication after sinonasal surgery and a commonly cited consideration for prescribing antibiotics when nasal packing or stents are placed. Most reports are limited to case reports or small series. The aim of this systematic review was to describe the incidence, risk factors, and clinical course of patients who developed TSS as a complication of endoscopic sinus surgery (ESS) and/or septoplasty. A systematic review was conducted using MEDLINE, EMBASE, CINAHL, Web of Science, and Cochrane Library databases. Eligibility criteria were patients of any age who developed TSS as a complication of ESS and/or septoplasty. Twenty-five studies were included in the final review. Seventy cases were identified over 38 years (1982-2020). The pooled incidence of TSS after sinonasal surgery was 0.0003 (0.0002-0.0004, 95% confidence interval, CI) or 30 per 100,000 persons. Among 33 patients with single-subject data, mean age was 33.8 years (29.73-37.87, 95% CI). Seventeen (51.5%) patients had nasal packing, 3 (9.1%) had stents, 5 (15.2%) had both, 7 (21.2%) had none. Fourteen (42.4%) patients had nonabsorbable packing and 5 (15.2%) had absorbable packing. Sixteen (48%) patients received peri-operative antibiotics. Thirty-two (97%) patients had full recovery. The incidence of TSS after sinonasal surgery is very rare. TSS can occur with and without nasal packing and prophylactic antibiotics were not protective. Outcomes were favorable with appropriate recognition and management. Further study is needed to determine which patients are most at risk for TSS.

OPTIC PATHWAY GLIOMAS AFTER TRANSITIONING INTO ADULTHOOD – AN INSTITUTIONAL EXPERIENCE

Abstract AIMS Optic pathway gliomas (OPG) are rare tumors affecting the optic nerve and the chiasm that occur predominantly during childhood and are generally managed by pediatric neurosurgeons/ pediatric oncologists. Prognosis is quite good for these benign tumors, and many patients survive into adulthood. While clinical care for these patients in childhood is well-standardized, the clinical course of patients with OPG after transitioning into adulthood is not well studied.We herein aimed to characterize patients with OPG diagnosed in childhood after transitioning into adult neurosurgical care in order in order to ellucidate the clinical course of OPG in adulthood and to identify potential problems with clinical care of these special patients in adult neurosurgery or neuro-oncology services. METHOD Clinical and radiographical data from adult patients (&amp;gt; 16 years of age) with OPG diagnosed in childhood and treated in our institution were collected from patient charts. Descriptive statistics and outcome analysis were performed using commercial statistical software. RESULTS Of 124 patients with OPG diagnosed in childhood, 35 patients transitioned into adulthood. The population included equal distribution of male (18, 51.4%) vs female (17, 48.6%) patients. Median age at diagnosis was 8.0 years (range 0.5 – 15.0) and median age at last follow up was 20.8 years (range 17.1 – 38.6). 12 patients were positive for NF (34.3%). 9 patients had undergone surgery for the OPG (25.7%), 11 had chemotherapy (31.4%) and 15 (42.8%) had received radiotherapy. 8 patients showed an endocrine deficiency (22.9%), 4 hypothalamic dysfunction (11.4%) and 8 cognitive/behavioral difficulties (22.9%). Visual acuity impairment was detected in 12 patients (34.3%). 9 patients experienced recurrence (25.7%). CONCLUSION Patients with OPG transition into adulthood frequently and experience a wide range of clinical problems. Close neurosurgical/neuro-oncology specialist follow-up is recommendable.

Rapidly Progressive Primary Cutaneous Gamma Delta T-Cell Lymphoma With FYN Gene Alteration.

Primary cutaneous gamma delta T-cell lymphoma (PCGDTCL) is a rare type of non-Hodgkin lymphoma accounting for <1% of primary cutaneous T-cell lymphomas. The exact cause of PCGDTCL is not known, however, it is thought that chronic antigen exposure in the skin may lead to immune dysregulation at the site, resulting in abnormal proliferation of mature, post-thymic cytotoxic gamma delta T cells. Mutations are the most common genetic alteration seen in PCGDTCL, while structural abnormalities such as gene fusions are not common. We report a case of PCGDTCL with atypical immunophenotypic features, including expression of CD5 with lack of cytotoxic marker expression, and a structural alteration leading to FYN deletion at exon 8. Recently, it was described that a deletion of the area between FYN exon 8 and TRAF3IP2 intron 2 results in a novel FYN::TRAF3IP2 fusion in peripheral T-cell lymphoma, not otherwise specified. We describe our patient's clinical course, differential diagnosis, and potential implications of FYN deletion on disease pathogenesis. To our knowledge, this is the first report of an FYN structural alteration to be described in PCGDTCL.

Persistent Increase in the Blood Urea Nitrogen/Creatinine Index (PI-BUN/Cr) Phenotypes the Clinical Course of Patients with Severe COVID-19

Persistent Increase in the Blood Urea Nitrogen/Creatinine Index (PI-BUN/Cr) Phenotypes the Clinical Course of Patients with Severe COVID-19

Impact of SARS-CoV-2 Vaccination and/or COVID-19 in the Clinical Course of Patients with IgA Nephropathy

Impact of SARS-CoV-2 Vaccination and/or COVID-19 in the Clinical Course of Patients with IgA Nephropathy

Outcomes with initiation of inpatient immunotherapy.

7 Background: Immune checkpoint inhibitors (ICIs) have transformed cancer care; however, studies establishing their benefit have been primarily conducted in robust patients (pts) with good performance status. Administration of ICI to hospitalized pts remains a subject of debate. While there is often a desire to reverse clinical course in patients who are acutely ill, previous studies show minimal benefit from anticancer therapy near the end of life with the potential for toxicity. While ICI are generally better tolerated than traditional cancer therapies, the median time to response ranges from 2-6 months and pts with a poor PS may not derive benefit. ICIs also bring significant financial burdens to pts and healthcare systems. The role of ICIs in the inpatient setting remains unclear. Methods: This study retrospectively examined pts who received PD-(L)1 inhibitors while IP between 2016 and 2023 at MedStar Georgetown University Hospital. Data were collected manually from institutional EMR. Descriptive statistics were used to depict the pt population and show their outcomes. The Kaplan-Meier method estimated survival probabilities from treatment initiation to follow-up or death, with 95% confidence intervals (α = 0.05). The Log rank non-parametric test compared survival distributions among ICIs. Results: Of the 38 pts who initiated ICI as an inpatient, 16 were male (42.1%) and 22 were female (57.9%). The median age was 60 years (31–88 years). Gastrointestinal malignancies were most common indication (31.6%), followed by thoracic/head and neck (21.1%) and hematologic (18.4%) cancers. Among the cohort, 26.3% received nivolumab, 68.4% received pembrolizumab, and 5.3% received a combination of nivolumab and ipilimumab. PD-L1 status was unknown for the majority (79.0%) of pts at time of ICI; 15.8 % were known to be PD-L1 high. Only 14 of 35 (40.0%) patients went on to receive any additional immunotherapy after discharge. The median days to death after discharge was 16 days (0-376 days), with a mean of 36.4 days. The median overall survival was 137 days from IP treatment initiation. The survival probability at 91 days was 65.5% (95% CI: 45.4-79.7) and 31.0% (95% CI: 15.6-47.9) at 182 days. No significant differences were observed between the survival distributions by ICI type (p=0.966) or primary malignancy (p=0.488). Conclusions: Our findings underscore the complexities of initiating inpatient ICI treatment and question its appropriateness. Delaying ICI to outpatient settings may enhance pt tolerance, optimize outcomes, and potentially mitigate unnecessary costs. This approach may allow thorough consideration of performance status outside the hospital, possibly improving treatment effectiveness while reducing futile acute care costs.

Metastatic Malignant Pseudomyogenic Hemangioendothelioma: An Exceedingly Rare Entity That Challenges Conventional Paradigms.

Pseudomyogenic hemangioendothelioma (PMHE), a rare soft tissue tumor predominantly affecting young adults, often presents as multiple nodules in various tissue planes of a limb. Malignant transformation and metastatic disease are unusual and pose diagnostic and therapeutic challenges. A 17-year-old patient from Western India, with a history of recurrent excisions for a toe swelling presented to our center for evaluation and management. A below-knee amputation was performed, and histopathology revealed PMHE. Adjuvant therapy was deemed unnecessary given the borderline nature of the tumor. Shortly thereafter, he developed features of local recurrence and underwent above-knee amputation. An expert histopathological review confirmed the diagnosis and noted features of malignant transformation-progression to a higher grade with greater cytological atypia, confluent growth, and increased mitotic activity over time. Upon further distant progression in the lung, he was started on a palliative regimen of weekly paclitaxel, vinblastine, and propranolol but eventually succumbed to his illness. In contrast to conventional descriptions of low mitotic activity, minimal nuclear atypia, and absence of necrosis, our patient exhibited increased mitotic rates, nuclear atypia, and evolving necrosis in serial histopathological evaluations. The fulminant clinical progression within a short interval was also atypical. Our patient's clinical course underscores the need for meticulous histopathological and molecular characterization and vigilant clinical surveillance after resection in patients with PMHE. Providing the standard of care for malignant disease in the adjuvant setting is challenging owing to the rarity and the lack of treatment guidelines.

Endoscopic features of rectal mucosal prolapse syndrome (RMPS): differentiation with malignant rectal tumor

Background: Rectal mucosal prolapse syndrome (RMPS) usually manifests as rectal bleeding, and tenesmus. Endoscopically it could be easily misdiagnosed with malignant rectal tumor (MRT). This study aims to investigate factors to distinguish RMPS and MRT, and explore endoscopic features of RMPS. Methods: Patients endoscopically diagnosed as rectal lesions, mass, or tumors, were retrospectively collected. Clinical information, endoscopic images, histologic reports were reviewed. Patients endoscopically and histologically diagnosed as RMPS were included for phenotype classification. Results: 826 patients were enrolled, among them 755 (91.4%), 22 (2.7%), 10 (1.2%) and 39 (4.7%) were respectively diagnosed with MRT, RMPS, endometriosis, and neuroendocrine tumor. Compared with MRT, patients with RMPS were significantly younger (33.5 vs. 62, P&lt; 0.001), and lesions were significantly smaller (2cm vs. 3cm, P=0.007). Moreover, clinical course of patients with RMPS would be significantly longer than those with MRT (12 months vs. 3 months, P&lt;0.001). Morphologically, we classified lesions of RMPS into five phenotypes, that is, lesions with circumferential stenosis (19.4%), protrusions (41.7%), both ulcers and protrusions (11.1%), ulcers (11.1%), flat manifestations (16.7%). Protruding lesions were more frequently observed in females (P=0.039), while ulcerative lesions were found involving a smaller proportion of the rectal circumference (P=0.028). Lesions with only ulcers were found with a shorter distance compared with those with only protrusions (5cm vs. 10cm, P=0.034). Conclusions: Age, clinical course, and size of the lesion, could be applied to distinguish MRT and RMPS. Five phenotypes are introduced and features of ulcers/protrusions are further explored.

Short-term Tezepelumab effectiveness in patients with severe asthma: a multicenter study

Objective Severe asthma presents significant management challenges, often requiring advanced treatments to control symptoms and reduce exacerbations. The use of monoclonal antibodies has revolutionized the clinical course of patients with severe asthma, showing a significant impact on exacerbations reduction, oral corticosteroids (OCS) cessation and on the improvement of lung function and quality of life. Tezepelumab, an anti-thymic stromal lymphopoietin (TSLP) monoclonal antibody, has emerged as a potential therapeutic option for these patients. Methods We conducted an observational, prospective, multicenter study including 20 patients with confirmed severe asthma according to ERS guidelines and GINA recommendations. Patients received Tezepelumab 210 mg every 4 wk due to uncontrolled asthma despite maximal inhalation treatment with ICS/LABA. Data were collected before treatment initiation (T0) and after three months from the first administration (T3). Results After three months of Tezepelumab treatment, we reported significant improvements in asthma symptoms and quality of life, as well as a consistent reduction in exacerbations and OCS use. We found no statistically meaningful differences among main clinical and functional outcomes according to inflammatory biomarkers, while lung function improved significantly in patients with less allergic sensitization. No serious adverse event was reported during the follow up, while the rates of mild adverse effects were comparable to those from registration trials. Conclusion Tezepelumab demonstrated short-term efficacy in improving asthma control and quality of life, showing a favorable safety profile. Further studies with larger sample sizes and longer follow-up would confirm these findings and identify predictors of response to Tezepelumab.