Epithelioid Hemangioendothelioma Presenting as Necrotizing Pneumonia.

Epithelioid hemangioendothelioma (EHE) is a very rare vascular neoplasm that is often asymptomatic. A 40-year-old woman presented to the emergency department for evaluation of a nonproductive cough, chest pain, and dyspnea. A chest computed tomography angiography (CTA) demonstrated necrotizing pneumonia, a loculated left-sided pleural effusion, and an acute pulmonary embolus. She was started on broad-spectrum intravenous antibiotics and heparin infusions, and a chest tube was placed. After minimal improvement in her pleural effusion following instillation of fibrinolytics, she underwent video-assisted thoracoscopic surgery with decortication, and a pleural biopsy was performed. Her presenting symptoms resolved shortly thereafter. Following discharge, surgical pathology resulted in a diagnosis of EHE. She was not a candidate for surgical resection and remained under surveillance. A year later, she was found to have metastatic disease, and radiotherapy was initiated. Our case, which presented as necrotizing pneumonia associated with pulmonary EHE, highlights the challenges in diagnosing this disease given its extreme rarity and discusses its management.

Role of Transbronchial Lung Cryobiopsy in the Diagnosis of Interstitial Lung Disease: A Meta-analysis of 68 Studies and 6300 Patients.

BACKGROUND: Diagnosis of interstitial lung disease (ILD) is based on multidisciplinary team discussion (MDD) with the incorporation of clinical, radiographical, and histopathologic information if available. We aim to evaluate the diagnostic yield and safety outcomes of transbronchial lung cryobiopsy (TBLC) in the diagnosis of ILD. METHODS: We conducted a meta-analysis by comprehensive literature search to include all studies that evaluated the diagnostic yields and/or adverse events with TBLC in patients with ILD. We calculated the pooled event rates and their 95% confidence intervals (CIs) for the diagnostic yield by MDD, histopathologic diagnostic yield, and various clinical adverse events. RESULTS: We included 68 articles (44 full texts and 24 abstracts) totaling 6386 patients with a mean age of 60.7±14.1 years and 56% men. The overall diagnostic yield of TBLC to achieve a definite or high-confidence diagnosis based on MDD was 82.3% (95% CI: 78.9%-85.2%) and histopathologic diagnosis of 72.5% (95% CI: 67.7%-76.9%). The overall rate of pneumothorax was 9.6% (95% CI: 7.9%-11%), while the rate of pneumothorax requiring drainage by a thoracostomy tube was 5.3% (95% CI: 4.1%-6.9%). The rate of moderate bleeding was 11.7% (95% CI: 9.1%-14.9%), while the rate of severe bleeding was 1.9% (95% CI: 1.4%-2.6%). The risk of mortality attributed to the procedure was 0.9% (95% CI: 0.7%-1.3%). CONCLUSION: Among patients with undiagnosed or unclassified ILD requiring tissue biopsy for diagnosis, transbronchial cryobiopsy represents a reliable alternative to surgical lung biopsy with decreased incidence of various clinical adverse events.

A 65-Year-Old Woman With Intractable Cough.

CASE PRESENTATION: A 65-year-old woman was referred for a second opinion regarding a 7-month history of a persistent, progressive, nonproductive cough. Her cough occurred several times a minute, causing a significant impact on her daily activities. She denied fever, chills, weight loss, chest pain, wheezing, symptoms of gastroesophageal reflux, or postnasal drip. She was a never smoker with no history of asthma, allergies, sinus disease, or dermatitis. She had never taken an angiotensin-converting enzyme inhibitor. Her medical history included rheumatoid arthritis, for which she was treated with methotrexate for 3 years. She denied any occupational or environmental exposures. She was previously treated with a short-acting ß-agonist, inhaled corticosteroid/long-acting ß-agonist, montelukast, nasal steroids, a proton pump inhibitor, gabapentin, and azithromycin without relief. She also received codeine, which provided mild relief.

Vitamin C, Thiamine, and Hydrocortisone in the Treatment of Sepsis: A Meta-Analysis and Trial Sequential Analysis of Randomized Controlled Trials.

BACKGROUND: There is a conflicting body of evidence regarding the benefit of vitamin C, thiamine, and hydrocortisone in combination as an adjunctive therapy for sepsis with or without septic shock. We aimed to assess the efficacy of this treatment among predefined populations. METHODS: A literature review of major electronic databases was performed to include randomized controlled trials (RCTs) evaluating vitamin C, thiamine, and hydrocortisone in the treatment of patients with sepsis with or without septic shock in comparison to the control group. RESULTS: Seven studies met our inclusion criteria, and 6 studies were included in the final analysis totaling 839 patients (mean age 64.2 ± 18; SOFA score 8.7 ± 3.3; 46.6% female). There was no significant difference between both groups in long term mortality (Risk Ratio (RR) 1.05; 95% CI 0.85-1.30; P = 0.64), ICU mortality (RR 1.03; 95% CI 0.73-1.44; P = 0.87), or incidence of acute kidney injury (RR 1.05; 95% CI 0.80-1.37; P = 0.75). Furthermore, there was no significant difference in hospital length of stay, ICU length of stay, and ICU free days on day 28 between the intervention and control groups. There was, however, a significant difference in the reduction of SOFA score on day 3 from baseline (MD -0.92; 95% CI -1.43 to -.41; P < 0.05). In a trial sequential analysis for mortality outcomes, our results are inconclusive for excluding lack of benefit of this therapy. CONCLUSION: Among patients with sepsis with or without septic shock, treatment with vitamin C, thiamine, and hydrocortisone was not associated with a significant reduction in mortality, incidence of AKI, hospital and ICU length of stay, or ICU free days on day 28. There was a significant reduction of SOFA score on day 3 post-randomization. Further studies with a larger number of patients are needed to provide further evidence on the efficacy or lack of efficacy of this treatment.

A Rare Twist of the Forgotten Disease: A Case of Fusobacterium necrophorum Sepsis with Portomesenteric Thrombosis and a Review of the Literature.

Abdominal variants of Lemierre's syndrome presenting with pylephlebitis are rare, and the role of anticoagulation in treatment is controversial. We hereby report a case of pylephlebitis secondary to F. necrophorum bacteremia in a 57-year-old female originating from bacterial translocation secondary to colitis, who developed a favorable outcome with prompt treatment with antibiotics and anticoagulation. We also perform a literature review on similar cases in the literature and discuss management options of this rare but potentially fatal complication.

A prospective study to validate pulmonary blood mass changes on non-contrast 4DCT in pulmonary embolism patients.

PURPOSE: Limited diagnostic options exist for patients with suspected pulmonary embolism (PE) who cannot undergo CT-angiogram (CTA). CT-ventilation methods recover respiratory motion-induced lung volume changes as a surrogate for ventilation. We recently demonstrated that pulmonary blood mass change, induced by tidal respiratory motion, is a potential surrogate for pulmonary perfusion. In this study, we examine blood mass and volume change in patients with PE and parenchymal lung abnormalities (PLA). METHODS: A cross-sectional analysis was conducted on a prospective, cohort-study with 129 consecutive PE suspected patients. Patients received 4DCT within 48 h of CTA and were classified as having PLA and/or PE. Global volume change (VC) and percent global pulmonary blood mass change (PBM) were calculated for each patient. Associations with disease type were evaluated using quantile regression. RESULTS: 68 of 129 patients were PE positive on CTA. Median change in PBM for PE-positive patients (0.056; 95% CI: 0.045, 0.068; IQR: 0.051) was smaller than that of PE-negative patients (0.077; 95% CI: 0.064, 0.089; IQR: 0.056), with an estimated difference of 0.021 (95% CI: 0.003, 0.038; p = 0.0190). PLA was detected in 57 (44.2%) patients. Median VC for PLA-positive patients (1.26; 95% CI: 1.22, 1.30; IQR: 0.15) showed no significant difference from PLA-negative VC (1.25; 95% CI: 1.21, 1.28; IQR: 0.15). CONCLUSIONS: We demonstrate that pulmonary blood mass change is significantly lower in PE-positive patients compared to PE-negative patients, indicating that PBM derived from dynamic non-contrast CT is a potentially useful surrogate for pulmonary perfusion.

Hypercalcemia of Malignancy and Acute Pancreatitis.

OBJECTIVES: Hypercalcemia of malignancy confers a poor prognosis. This systematic review evaluated published cases of hypercalcemia of malignancy presenting with acute pancreatitis (AP), in terms of clinical presentation and outcomes. METHODS: A comprehensive review of PubMed and Embase until March 18, 2020, was conducted. Studies were included if they reported on patients with hypercalcemia of malignancy and AP with attempts to exclude other etiologies of hypercalcemia and AP. Two independent reviewers selected and appraised studies using the Murad tool. RESULTS: Thirty-seven cases were identified. Mean (standard deviation) age was 44.8 (2.46) years. Mean (standard deviation) presenting corrected calcium was 14.5 (0.46) mg/dL. Parathyroid carcinoma (21.6%) and multiple myeloma (21.6%) were the most common malignancies. Cases were classified as severe (37.8%), mild (21.6%), and moderately severe (18.9%), whereas 21.6% did not report severity. Necrotizing pancreatitis developed in 21.6% of cases. Most cases were treated with intravenous hydration and bisphosphonates or calcitonin/calcitonin analogues. Mortality was 32.4% during the same presentation of AP. Among mortality cases, 10 of 12 had severe AP, and 5 of 12 had necrotizing pancreatitis. Degree of hypercalcemia did not influence mortality. CONCLUSION: Acute pancreatitis associated with hypercalcemia of malignancy is rare. One in 3 patients with this presentation may not survive AP.

Drug Induced Liver Injury Attributed to a Curcumin Supplement.

More severe reactions, higher acute liver failure rates, and higher recurrence rates on re-challenge occur with supplement-related Drug Induced Liver Injury (DILI) (Medina-Caliz et al., 2018). We report a case of curcumin-induced hepatocellular DILI in a 78-year old female admitted with jaundice, with a one-month latency. Extensive evaluation for alternative etiologies of hepatotoxicity was unremarkable. The Roussel Uclaf Causality Assessment Method (RUCAM) score of 6 for the supplement indicated a probable association (score >8: highly probable association). Peak levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were >20 times upper limit of normal. A 48% decrease in AST and ALT levels was observed 7 days after discontinuation of the supplement, and resolution of transaminitis was observed in 42 days. No re-challenge was performed. In conclusion, this case emphasizes the importance of recognizing curcumin supplements as DILI triggers. Furthermore, it reiterates the need for careful evaluation of herbal and dietary supplements (HDS) consumed by patients to identify potential DILI culprits, and to ultimately prevent DILI reactions with significant morbidity and mortality.

Paced QRS morphology predicts incident left ventricular systolic dysfunction and atrial fibrillation.

BACKGROUND: The prognostic significance of paced QRS complex morphology on surface ECG remains unclear. This study aimed to assess long-term outcomes associated with variations in the paced QRS complex. METHODS: Adult patients who underwent dual-chamber pacemaker implantation with 20% or more ventricular pacing and a 12-lead ECG showing a paced complex were included. The paced QRS was analyzed in leads I and aVL. Long-term clinical and echocardiographic outcomes were compared at 5 years. RESULTS: The study included 844 patients (43.1% female; age 75.0 ±â€¯12.1). Patients with a longer paced QRS (pQRS) duration in lead I had a lower rate of atrial fibrillation (HR 0.80; p = 0.03) and higher rate of systolic dysfunction (HR 1.17; p < 0.001). Total pacing complex (TPC) duration was linked to higher rates of ICD implantation (HR 1.18; p = 0.04) and systolic dysfunction (HR 1.22, p < 0.001). Longer paced intrinsicoid deflection (pID) was associated with less atrial fibrillation (HR 0.75; p = 0.01), more systolic dysfunction (HR 1.17; p < 0.001), ICD implantation (HR 1.23; p = 0.04), and CRT upgrade (HR 1.23; p = 0.03). Exceeding thresholds for TPC, pQRS, and pID of 170, 146, and 112 ms in lead I, respectively, was associated with a substantial increase in systolic dysfunction over 5 years (p < 0.001). CONCLUSIONS: Longer durations of all tested parameters in lead I were associated with increased rates of left ventricular systolic dysfunction. ICD implantation and CRT upgrade were also linked to increased TPC and pID durations. Paradoxically, patients with longer pID and pQRS had less incident atrial fibrillation.

Echocardiographic left ventricular diastolic dysfunction predicts hospital mortality after out-of-hospital cardiac arrest.

PURPOSE: To determine whether systolic or diastolic dysfunction on transthoracic echocardiogram (TTE) predicts mortality after out-of-hospital cardiac arrest (OHCA). METHODS: Retrospective cohort study of 173 OHCA subjects undergoing targeted temperature management who underwent TTE during hospitalization. Univariate analysis and multivariate logistic regression were used to determine associations between TTE measurements of systolic and diastolic function and systemic hemodynamics with all-cause mortality. RESULTS: Mean age was 61.6 ±â€¯12.4 years (72.7% male) and initial rhythm was shockable in 89%. Hospital mortality was 30.6%. Mean LVEF was 40% and was not different in hospital survivors (p = 0.81). TTE parameters reflecting systolic function and systemic hemodynamics did not predict hospital mortality. Medial mitral E/e' ratio was associated with hospital mortality, with an optimal cut-off > 13 (p = 0.002). After multivariate adjustment, medial mitral E/e' ratio remained predictive of hospital mortality (OR 1.11, 95% CI 1.03-1.20, p = 0.004). Subjects with a medial mitral E/e' ratio > 13 had higher mortality during long-term follow-up (p < 0.001 by log-rank). CONCLUSIONS: Diastolic dysfunction (higher medial mitral E/e' ratio) on TTE independently predicted mortality after OHCA; systolic dysfunction and TTE hemodynamic parameters did not. This reflects a novel use of Doppler TTE to predict outcomes after OHCA.