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Therapeutic Area: ASCVD/CVD Risk Assessment Background: Lipoprotein(a) [Lp(a)] is a predictor of atherosclerotic cardiovascular disease (ASCVD); however, there are few algorithms incorporating Lp(a), especially from real-world settings. We developed an electronic health record (EHR)-based risk prediction algorithm including Lp(a). Methods: Utilizing a large EHR database, we categorized Lp(a) cut-points at 25, 50, and 75 mg/dL and constructed 10-year ASCVD risk prediction models incorporating Lp(a) with external validation in a pooled cohort of four US prospective studies. Net reclassification improvement (NRI) was determined among borderline-intermediate risk patients. Results: We included 5,902 patients (mean age 48.7±16.7 years, 51.2% female, and 7.7% Black). Over a mean follow-up of 5.5 years, ASCVD event rates (per 1000 person years) ranged from 8.7 to 16.7 across Lp(a) groups. A 25 mg/dL increment in Lp(a) was associated with 23% higher risk of composite ASCVD. Those with Lp(a) >75 had 88% higher risk of ASCVD (HR = 1.88, 95% CI: 1.30-2.70) and more than double the risk of incident stroke (HR = 2.55, 95% CI: 1.54-4.23). C-statistics for our EHR and EHR+Lp(a) models in our EHR training dataset were 0.7475 and 0.7556, respectively, with external validation of 0.7350 and 0.7368, respectively. Among those at borderline/intermediate risk, the NRI was 21.3%. Conclusions: We show the feasibility of developing an improved ASCVD risk prediction model incorporating Lp(a) based on a real-world adult clinic population. The inclusion of Lp(a) in ASCVD prediction models can reclassify risk in patients who may benefit from more intensified ASCVD prevention efforts.

Torrential bleeding is a rare and life-threatening complication of arteriovenous malformations (AVMs). We report a case of head and neck AVMs present with uncontrollable torrential bleeding, which was treated with embolization and sclerotherapy. Then we explored the potential multidisciplinary handling of the procedure for this kind of case. A 25-year-old female patient was born with right face and head AVMs. The AVMs had grown gradually and ruptured spontaneously with uncontrollable torrential bleeding before admission. Emergent direct hemostasis, nasotracheal intubation, and staged embolization and sclerotherapy were carried out on this patient. Finally, the bleeding stopped and the wound healed successfully. Embolization and sclerotherapy are effective for head and neck AVMs with uncontrollable torrential bleeding. Multidisciplinary collaboration is needed to achieve a good outcome.

Background: Chronic rhinosinusitis (CRS) is a heterogeneous disease characterized by persistent sinonasal inflammation and sinus microbiome dysbiosis. Nasal polyps (NPs) are one of the main manifestations that cause diverse clinical symptoms of CRS. Objective: We sought to conduct a bibliometric and visual analysis of articles on CRS and NPs published between 2003 and 2022 to provide researchers with the current state of research and potential directions. Methods: We searched relevant articles from 2003 to 2022 in the Web of Science database. VOSviewer and the Bibliometrix R package were used to perform the bibliometric analysis. Results: A total of 3907 publications were retrieved. The United States made the highest contributions to global research, followed by China. Northwestern University had the most publications. The most published author was C. Bachert, followed by R. P. Schleimer and R. J. Schlosser. The authors with the most co-citations were C. Bachert, W. J. Fokkens, and P. Gevaert. Moreover, the journal with the most publications was the International Forum of Allergy & Rhinology, and the Journal of Allergy and Clinical Immunology was the most cited. “Covid-19,” “biologics,” and “type 2 inflammation” were the top current research hotspots. Conclusions: The United States and Northwestern University were the leading country and institution in researching CRS and NPs. C. Bachert was the most influential expert. The International Forum of Allergy & Rhinology and the Journal of Allergy and Clinical Immunology were leading journals. “Covid-19,” “biologics,” and “type 2 inflammation” were the trending topics.

Fractures and dislocations of the sternoclavicular joint (SCJ) are uncommon, accounting for

Chronic rhinosinusitis (CRS) is a disease highly associated with abnormal regulation of T and B cells. The underlying pathophysiology of inflammatory pathways has critical implications for the diagnosis and management of CRS. Soluble CD40-ligand (sCD40L) is a cleaved form of CD40L present in plasma which functions the same way as CD40L, which has been observed as an inflammatory biomarker in many diseases. CD40L-positive cells control B-cell maturation, proliferation, apoptosis, and antibody production by binding to its receptor CD40 on B-cells. And our results show for the first time that patients with CRS have lower serum sCD40L levels compared to healthy subjects and that decreased sCD40L levels in patients correlate with increased CD40L-positive cell counts in the sinonasal mucosa. In addition, eosinophilic chronic rhinosinusitis (eCRS) patients tend to exhibit more CD40L-positive cells in the sinonasal mucosa compared to non-eCRS patients. This supports the notion that local blockade of CD40/CD40L may suppress pathogenic T/B cell responses and reduce tissue inflammation. Significantly, sCD40L and CD40L may be involved in the development and progression of CRS by impairing peripheral blood B-cell function and enhancing the local inflammatory response in the sinonasal mucosa.

Background: Central compartment atopic disease (CCAD) is a subtype of chronic rhinosinusitis (CRS). Research focusing on the endoscopic sinus surgery (ESS) outcomes of CCAD is limited. This study aimed to evaluate the outcomes of ESS in CCAD and compared to 2 following subtypes: chronic rhinosinusitis with nasal polyps (CRSwNP) and concomitant polypoid disease in the central compartment (CRSwNP/CC) and CRSwNP not otherwise specified (CRSwNP NOS). Methods: This case-control study enrolled patients with bilateral CRSwNP who underwent ESS and had at least 1 year of follow-up. Patients were classified into CCAD, CRSwNP/CC, and CRSwNP NOS. The demographic data, preoperative disease severity, and surgery outcomes, including CRS control status, endoscopic score, and symptom scores at 1 year postoperatively, were collected. We defined well controlled and partly controlled as appropriate disease control. Results: This study screened 259 patients and enrolled 138 patients with complete medical records and 1-year follow-up (CCAD N = 51, CRSwNP/CC N = 55, CRSwNP NOS N = 32). Among them, appropriate disease control was achieved in 84.3% of patients (43/51) in the CCAD group, 69.1% (38/55) in the CRSwNP/CC group, and 93.7% (30/32) in the CRSwNP NOS group (P = 0.029). Then we performed post-hoc analysis using appropriate disease control and uncontrolled. There was a significant difference between CRSwNP/CC and CRSwNP NOS (P = 0.007), but no significant difference compared CCAD group to CRSwNP/CC group (P = 0.065) and CRSwNP NOS group (P = 0.199). There were significant differences in endoscopic E-score among groups (P

Therapeutic Area: Diabetes Background: Hypertension (HTN) and diabetes mellitus (DM) are both leading contributors to the development of cardiovascular disease (CVD). Furthermore, HTN subtypes may impact CVD outcomes to varying degrees. Data are limited on the association of HTN subtypes comparing those with versus without DM. The aim of this project is to examine the association of DM status with HTN subtypes. Methods: A total of 19,062 adults (projected to 39 million adults) from the National Health and Nutrition Examination Survey years 1999-2016 with untreated HTN, defined as a systolic blood pressure (BP) ≥ 130 or diastolic BP ≥ 80 mm Hg, were included in the analysis. Individuals were further stratified by HTN subtype: isolated systolic hypertension (ISH), isolated diastolic hypertension (IDH), and systolic-diastolic hypertension (SDH). Multiple logistic regression analyses examined the relation of diabetes and glycemic control to the likelihood of ISH. Results: Among the subjects examined, 16.4% had DM and among those a greater proportion (65%) had ISH compared to 36% in those without DM (p

Background: Fluoroscopy is the standard tool for transvenous implantation of traditional and leadless pacemakers (LPs). LPs are used to avoid complications of conventional pacemakers, but there still is a 6.5% risk of major complications. Mid–right ventricular (RV) septal device implantation is suggested to decrease the risk, but helpful cardiac landmarks cannot be visualized under fluoroscopy. Transesophageal echocardiography (TEE) is an alternative intraprocedural imaging method. Objective: The purpose of this study was to explore the spatial relationship of the LP to cardiac landmarks via TEE and their correlations with electrocardiographic (ECG) parameters, and to outline an intraprocedural method to confirm mid-RV nonapical lead positioning. Methods: Fifty-six patients undergoing implantation of LP with TEE guidance were enrolled in the study. Device position was evaluated by fluoroscopy, ECG, and TEE. Distances between the device and cardiac landmarks were measured by TEE and analyzed with ECG parameters with and without RV pacing. Results: Mid-RV septal positioning was achieved in all patients. TEE transgastric view (0°–40°/90°–130°) was the optimal view for visualizing device position. Mean tricuspid valve–LP distance was 4.9 ± 0.9 cm, mean pulmonary valve–LP distance was 4.2 ± 1 cm, and calculated RV apex–LP distance was 2.9 ± 1 cm. Mean LP paced QRS width was 160.8 ± 28 ms and increased from 117.2 ± 34 ms at baseline. LP RV pacing resulted in left bundle branch block pattern on ECG and 37.8% QRS widening by 43.5 ± 29 ms. Conclusion: TEE may guide LP implantation in the nonapical mid-RV position. Further studies are required to establish whether this technique reduces implant complications compared with conventional fluoroscopy.

Background/Objective: Adrenal corticomedullary mixed tumor (CMMT) are extremely rare single adrenal tumor masses containing a mixture of adrenal cortical adenoma and pheochromocytoma cells. Case Report: A 52-year-old woman presented with clinical and biochemical evidence of cortisol and catecholamine excess and was found to have an adrenal CMMT with intermixed chromaffin, cortical adenoma, and ganglioneuroma components. She underwent a successful unilateral adrenalectomy with subsequent improvement in her symptoms. Discussion: We report the first case of a patient with a CMMT that had symptoms of both catecholamine and cortisol excess from her tumor. Typically, patients with similar tumors have signs of cortisol excess; however, the pheochromocytoma portion is clinically silent. Although most CMMT contain 2 distinct cell types, this is the third ever described case of a single adrenal CMMT containing 3 unique cellular components: (1) intermixed chromaffin, (2) cortical adenoma, and (3) ganglioneuroma cells. Conclusion: Our understanding of these rare tumors is limited, and this case serves to broaden our knowledge about their clinical, biochemical, and pathologic features.

Objective: To develop an inflammation-based risk stratification tool for operative mortality in patients with acute type A aortic dissection. Methods: Between January 1, 2016 and December 31, 2021, 3124 patients from Beijing Anzhen Hospital were included for derivation, 571 patients from the same hospital were included for internal validation, and 1319 patients from other 12 hospitals were included for external validation. The primary outcome was operative mortality according to the Society of Thoracic Surgeons criteria. Least absolute shrinkage and selection operator regression were used to identify clinical risk factors. A model was developed using different machine learning algorithms. The performance of the model was determined using the area under the receiver operating characteristic curve (AUC) for discrimination, calibration curves, and Brier score for calibration. The final model (5A score) was tested with respect to the existing clinical scores. Results: Extreme gradient boosting was selected for model training (5A score) using 12 variables for prediction—the ratio of platelet to leukocyte count, creatinine level, age, hemoglobin level, prior cardiac surgery, extent of dissection extension, cerebral perfusion, aortic regurgitation, sex, pericardial effusion, shock, and coronary perfusion—which yields the highest AUC (0.873 [95% confidence interval (CI) 0.845-0.901]). The AUC of 5A score was 0.875 (95% CI 0.814-0.936), 0.845 (95% CI 0.811-0.878), and 0.852 (95% CI 0.821-0.883) in the internal, external, and total cohort, respectively, which outperformed the best existing risk score (German Registry for Acute Type A Aortic Dissection score AUC 0.709 [95% CI 0.669-0.749]). Conclusion: The 5A score is a novel, internally and externally validated inflammation-based tool for risk stratification of patients before surgical repair, potentially advancing individualized treatment. Trial Registration: clinicaltrials.gov Identifier: NCT04918108

Introduction: Savolitinib has been found to have encouraging antitumor activity and a favorable safety profile in Chinese patients with pulmonary sarcomatoid carcinoma and other NSCLCs with MET exon 14 skipping alterations (MET ex14 positive) at the primary analysis of a phase 2 study. Here, we present the long-term efficacy and safety data of savolitinib, including subgroup analyses. Methods: This multicenter, single-arm, open-label, phase 2 study in the People’s Republic of China enrolled MET inhibitor-naive adults with locally advanced or metastatic METex14-positive NSCLC (NCT02897479). Oral savolitinib at a dose of 400 or 600 mg was administered once daily (body weight dependent). The primary objectives of the final analysis were long-term overall survival (OS) and subgroup analyses by previous systemic treatment, NSCLC subtypes, and brain metastases. Results: At the final analysis cutoff date (June 28, 2021), a total of 70 patients were enrolled and receiving savolitinib, and median follow-up was 28.4 (interquartile range: 26.2–36.3) months. The median OS was 12.5 months (95% confidence interval [CI]: 10.5–21.4 [18- and 24-mo OS rates, 42.1% and 31.5%, respectively]). Median OS in pretreated or treatment-naive patients was 19.4 (95% CI: 10.5–31.3) and 10.9 (95% CI: 7.5–14.0) months, respectively; it was 10.6 months (95% CI: 4.6–14.0) in patients with pulmonary sarcomatoid carcinoma, 17.3 months (95% CI: 10.6–23.6) in other NSCLC subtypes, and 17.7 months (95% CI: 10.5–not evaluable) in patients with brain metastases. No new safety signals emerged with prolonged follow-up and exposure. Conclusions: The updated results further confirm the favorable benefit and acceptable safety of savolitinib in Chinese patients with METex14-positive NSCLC.

Introduction: Entrectinib is an approved tyrosine kinase inhibitor (TKI) for ROS1 fusion–positive NSCLC. An updated integrated analysis of entrectinib from the ALKA-372-001, STARTRK-1, and STARTRK-2 trials is presented, with substantially longer follow-up, more patients, and the first description of the median overall survival (OS). An exploratory analysis of entrectinib in ROS1 fusion–positive NSCLC with the central nervous system (CNS)–only progression post-crizotinib is reported. Methods: Adults with ROS1 fusion–positive, locally advanced or metastatic NSCLC who received at least one dose of entrectinib and had 12 months or longer of follow-up were included in the analysis. Co-primary end points were confirmed objective response rate (ORR) and duration of response (DoR) by blinded independent central review. The data cutoff was on August 31, 2020. Results: The efficacy-assessable population comprised 168 ROS1 TKI–naïve patients. The median survival follow-up was 29.1 months (interquartile range, 21.8–35.9). The ORR was 68% (95% confidence interval [CI]: 60.2–74.8); the median DoR was 20.5 months. The median progression-free survival (PFS) was 15.7 months and the median OS was 47.8 months. In the 25 patients with measurable baseline CNS metastases, the intracranial ORR was 80% (95% CI: 59.3–93.2), median intracranial DoR was 12.9 months, and median intracranial PFS was 8.8 months. Among 18 patients with CNS-only progression on previous crizotinib treatment, two achieved a partial response (11%) and four had stable disease (22%). In seven patients with measurable CNS disease from this cohort, the intracranial ORR was 14% (1 partial response). Conclusions: Entrectinib is active and achieves prolonged survival in ROS1 TKI–naïve patients with ROS1 fusion–positive NSCLC. Modest activity is seen in patients with CNS-only progression post-crizotinib.

Background: Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) is used increasingly to support patients who are in cardiogenic shock. Due to the risk of complications, prediction models may aid in identifying patients who would benefit most from VA-ECMO. One such model is the Survival After Veno-Arterial Extracorporeal Membrane Oxygenation (SAVE) score. Therefore, we wanted to validate the utility of the SAVE score in a contemporary cohort of adult patients. Methods: Retrospective data were extracted from electronic health records of 120 patients with cardiogenic shock supported with VA-ECMO between 2011 and 2018. The SAVE score was calculated for each patient to predict survival to hospital discharge. We assessed the SAVE score calibration by comparing predicted vs observed survival at discharge. We assessed discrimination with the area under the receiver operating curve using logistic regression. Results: A total of 45% of patients survived to hospital discharge. Survivors had a significantly higher mean SAVE score (–9.3 ± 4.1 in survivors vs –13.1 ± 4.4, respectively; P = 0.001). SAVE score discrimination was adequate (c = 0.77; 95% confidence interval 0.69-0.86; P < 0.001). SAVE score calibration was limited, as observed survival rates for risk classes II-V were higher in our cohort (II: 67% vs 58%; III: 78% vs 42%; IV: 61% vs 30%; and V: 29% vs 18%). Conclusions: The SAVE score underestimates survival in a contemporary North American cohort of adult patients with cardiogenic shock. Its inaccurate performance could lead to denying ECMO support to patients deemed to be too high risk. Further studies are needed to validate additional predictive models for patients requiring VA-ECMO. Résumé: Contexte: L’oxygénation extracorporelle veino-artérielle (ECMO-VA) est de plus en plus utilisée comme assistance pour les patients qui sont en choc cardiogène. En raison du risque de complications, des modèles de prédiction peuvent aider à déterminer quels patients bénéficieraient le plus d’une ECMO-VA. Le score SAVE (Survival After Veno-Arterial Extracorporeal Membrane Oxygenation) est un modèle de ce genre. Par conséquent, nous voulions valider l’utilité du score SAVE dans une cohorte contemporaine de patients adultes. Méthodologie: Des données rétrospectives ont été extraites de dossiers médicaux électroniques de 120 patients atteints d’un choc cardiogène ayant reçu une ECMO-VA entre 2011 et 2018. Le score SAVE a été calculé pour chaque patient afin de prédire la survie au congé de l’hôpital. Nous avons évalué la calibration du score SAVE en comparant la survie prédite au moment du congé et la survie observée. Nous avons évalué la discrimination par l’aire sous la courbe opérationnelle chez le receveur en faisant appel à la régression logistique. Résultats: Au total, 45 % des patients ont survécu au congé de l’hôpital. Les survivants affichaient un score SAVE moyen considérablement plus élevé (–9,3 ± 4,1 chez les survivants vs –13,1 ± 4,4; p = 0,001). La discrimination par le score SAVE était adéquate (c = 0,77; intervalle de confiance à 95 % : 0,69 à 0,86; p < 0,001). La calibration du score SAVE était limitée, car les taux pour les classes de risque II à V étaient plus élevés dans notre cohorte (II : 67 % vs 58 %; III : 78 % vs 42 %; IV : 61 % vs 30 %; et V : 29 % vs 18 %). Conclusions: Le score SAVE sous-estime la survie dans une cohorte nord-américaine contemporaine de patients adultes atteints d’un choc cardiogène. L’inexactitude de ses résultats pourrait faire en sorte qu’une assistance par ECMO serait refusée à des patients jugés comme présentant un risque élevé. D’autres études sont nécessaires pour valider d’autres modèles de prédiction pour les patients ayant besoin d’une ECMO-VA.

Background: Obesity is associated with a higher incidence of atrial fibrillation (AF). Weight reduction improves outcomes in patients known to have AF. Objective: The purpose of this study was to compare the incidence of heart failure (HF) or first-time AF hospitalization in obese patients undergoing bariatric surgery (BAS) vs other abdominal surgeries. Methods: A retrospective cohort study was conducted using linked hospital discharge records from 1994–2014. Obese patients without known AF or atrial flutter (AFL) who had undergone abdominal hernia or laparoscopic cholecystectomy surgery were identified for each case that underwent BAS (2:1). Clinical outcomes were HF, first-time hospitalization for AF, AFL, gastrointestinal bleeding (GIB), and ischemic or hemorrhagic stroke. Outcomes were analyzed using conditional proportional hazard modeling accounting for the competing risk of death, adjusting for demographics and comorbidities. Results: There were 1581 BAS cases and 3162 controls (48% age

Introduction: Taletrectinib (AB-106/DS-6051b) is an oral, potent selective ROS1 and pan-NTRK tyrosine kinase inhibitor (TKI). Preclinically, taletrectinib has activity against ROS1 G2032R solvent-front mutation. Methods: Patients with ROS1+ NSCLC enrolled into two phase 1 studies conducted in United States (U101, NCT02279433) and Japan (J102, NCT02675491) were analyzed for objective response rate (ORR) by the Response Evaluation Criteria in Solid Tumors version 1.1, progression-free survival, and safety. Results: A total of 22 patients with ROS1+ NSCLC out of the total 61 patients enrolled were analyzed. Taletrectinib was given at the oral dose of 400 mg, 600 mg, 800 mg, and 1200 mg once daily and 400 mg twice daily as part of the dose-escalation schema. Data cutoff was August 19, 2020. Median follow-up time for all 22 patients was 14.9 months (95% confidence interval [CI]: 4.1–33.8). A total of 18 patients with ROS1+ were assessable for response. The confirmed ORR for ROS1 TKI-naive patients (N = 9) was 66.7% (95% CI: 35.4–87.9) with a disease control rate of 100% (70.1–100). The confirmed ORR for crizotinib pretreated patients (N = 6) was 33.3% (95% CI: 9.7–70.0) with a disease control rate of 88.3% (95% CI: 443.6–97.0). The median progression-free survival for ROS1 TKI-naive patients (N = 11) was 29.1 months (95% CI: 2.6–not reached) and 14.2 months (95% CI: 1.5–not reached) for crizotinib-refractory only patients (N = 8). The most common treatment-related adverse events were alanine transaminase elevations (72.7%), aspartate transaminase elevations (72.7%), nausea (50.0%), and diarrhea (50.0%). Grade 3 or higher adverse events were alanine transaminase elevations (18.2%), aspartate transaminase (9.1%), and diarrhea (4.5%). Conclusions: Taletrectinib (AB106/DS6051b) has a meaningful clinical activity in patients with advanced ROS1+ NSCLC who are ROS1 TKI-naive or crizotinib-refractory and a manageable safety profile.

Objectives: To determine the optimal number of examined lymph nodes (ELNs) and examined node stations (ENSs) in patients with radiologically pure-solid NSCLC and to investigate the impact of ELNs and ENSs on accurate staging and long-term survival. Methods: Data from six institutions in the People’s Republic of China on resected c-stage Ⅰ to Ⅱ NSCLCs presenting as pure-solid tumors were analyzed for the impact of ELNs and ENSs on nodal upstaging, stage migration, recurrence-free survival, and overall survival by using multivariate models. The correlations between different end points and ELNs or ENSs were fitted with a smoother (using Locally Weighted Scatterplot Smoothing tool), and the structural break points were determined by the Chow test. Results: Both ELNs and ENSs were identified as prognostic factors for overall survival (ENS: hazard ratio [HR], 0.697; 95% confidence interval [CI]: 0.590–0.824; p < 0.001; ELN: HR, 0.945; 95% CI: 0.909–0.983; p = 0.005) and recurrence-free survival (ENS: HR, 0.863; 95% CI: 0.791–0.941; p = 0.001; ELN: HR, 0.960; 95% CI: 0.938–0.981; p < 0.001). Intraoperative ELNs and ENSs were found to be associated with postoperative nodal upstaging. Cut point analysis revealed an optimal cutoff of 16 LNs and five node stations for patients with c-stage Ⅰ to Ⅱ pure-solid NSCLCs, which were examined in our multi-institutional cohort. Conclusions: Both ELNs and ENSs are associated with more accurate node staging and better long-term survival. We recommend 16 LNs and five stations as the cut point for evaluating the quality of LN examination for c-stage Ⅰ to Ⅱ patients with radiologically pure-solid NSCLCs.

When South Florida became a hot spot for COVID-19 disease in March 2020, we faced an urgent need to develop test capability to detect SARS-CoV-2 infection. We assembled a transdisciplinary team of knowledgeable and dedicated physicians, scientists, technologists, and administrators who rapidly built a multiplatform, polymerase chain reaction- and serology-based detection program, established drive-through facilities, and drafted and implemented guidelines that enabled efficient testing of our patients and employees. This process was extremely complex, due to the limited availability of needed reagents, but outreach to our research scientists and multiple diagnostic laboratory companies, and government officials enabled us to implement both Food and Drug Administration authorized and laboratory-developed testing–based testing protocols. We analyzed our workforce needs and created teams of appropriately skilled and certified workers to safely process patient samples and conduct SARS-CoV-2 testing and contact tracing. We initiated smart test ordering, interfaced all testing platforms with our electronic medical record, and went from zero testing capacity to testing hundreds of health care workers and patients daily, within 3 weeks. We believe our experience can inform the efforts of others when faced with a crisis situation.

ABSTRACT: Objective: Diffuse symmetrical lipomatosis, or Madelung disease, is a condition of benign lipoma formation that occurs around the shoulder girdle and can alter quality of life immensely. As surgery is the mainstay for treatment, recognition and education of this condition is needed in medical literature to expand future treatment modalities.Methods: Case report and review of the literature.Results: A 47-year-old male with primary hypothyroidism, type 2 diabetes mellitus, and peripheral neuropathy presented with a 20-year history of recurrent lipomatous formations. Enlarging lesions were previously on his upper back, posterior left ear, and bilateral shoulders. Pathology showed benign lipomas in all resections. Rapid growth of similar lesions had now occurred throughout his shoulder girdle, upper back, sternum, and submandibular regions. His quality of life had been affected, as the sternal mass caused recumbent pressure. Work-up was negative for Cushing syndrome and acromegaly. No genetic studies were performed. Family history and social history were insignificant. Given his presentation, history of recurrent benign lipomatous formations, and associated endocrinopathies, the diagnosis of Madelung disease was confirmed. As surgery is the mainstay treatment, he is following with plastic surgery for removal of his sternal mass.Conclusion: The uniqueness of this case is his severity of lipomatous enlargement, non-Mediterranean descent, and lack of alcohol abuse. As quality of life can be severely impaired, future medical treatment options should be pursued to assist those patients already through extensive surgeries.

Portal vein thrombosis (PVT) is a potential complication of cirrhosis and can worsen outcomes after liver transplant (LT). Portal vein reconstruction–transjugular intrahepatic portosystemic shunt (PVR-TIPS) can restore flow through the portal vein (PV) and facilitate LT by avoiding complex vascular conduits. We present a case of transsplenic PVR-TIPS in the setting of complete PVT and splenic vein (SV) thrombosis. The patient had a 3-year history of PVT complicated by abdominal pain, ascites, and paraesophageal varices. A SV tributary provided access to the main SV and was punctured percutaneously under ultrasound scan guidance. PV access, PV and SV venoplasty, and TIPS placement were successfully performed without complex techniques. The patient underwent LT with successful end-to-end anastomosis of the PVs. Our case suggests transsplenic PVR-TIPS to be a safe and effective alternative to conventional PVR-TIPS in patients with PVT and SV thrombosis.

Chylous ascites (CA) is the extravasation of lipid-rich lymphatic fluid into the peritoneal space following trauma or obstruction of the lymphatic system. Refractory cases of cirrhosis-related CA may be amendable to transjugular intrahepatic portosystemic shunting (TIPS). We present a case of TIPS in the setting of refractory CA secondary to cirrhosis of a transplanted liver graft. Following TIPS, the patient reported immediate improvement in abdominal pain and no longer requires paracentesis. Our case suggests TIPS to be a safe and effective treatment option for CA in liver transplant patients with cirrhosis.

ABSTRACT: Objective: Our objectives were to describe a rare adult-onset sellar atypical teratoid/rhabdoid tumor (AT/RT) and to identify preoperative diagnostic clues for this aggressive tumor.Methods: A case report and review of the relevant literature.Results: A 36-year-old female presented with headache for 1 month and blurry vision for 6 days. Imaging identified a large sellar mass with suprasellar extension that was indistinguishable from a pituitary adenoma. Hormonal studies revealed hypopituitarism and hyperprolactinemia. A presumptive diagnosis of pituitary macroadenoma was made, but the histologic, immunohistochemical, electron microscopic, and cytogenetic studies of the tumor supported a diagnosis of AT/RT. Despite multiple surgical resections, radiation, and chemotherapy, the tumor recurred and regrew relentlessly. The patient died 2.5 years after presentation.Conclusion: This case highlights the importance of suspecting aggressive sellar masses in management planning and prognosis counseling. Short duration of headache and visual changes associated with a large sellar mass are diagnostic clues for sellar AT/RT or other rapidly expanding sellar masses. Endocrinologists should pay attention to symptom duration in patients with large sellar masses.Abbreviations: AT/RT atypical teratoid/rhabdoid tumor INI1 integrase interactor 1 MRI magnetic resonance imaging

Purpose: Intimate partner violence (IPV) is prevalent among adolescents. Civil protection orders (CPOs) are the most common legal remedy used by individuals experiencing IPV to prevent recurrence. However, laws on whether adolescents can access CPOs vary by state. We assessed the landscape of state legislation regarding adolescents' access to CPOs for IPV., Methods: We systematically assessed statutes across all 50 U.S. states and the District of Columbia for 5 indicators: whether minors can be granted CPOs, whether minors can file on their own behalf, whether dating relationships are eligible, definitions of abuse qualifying for CPOs, and whether a CPO prohibits firearm possession and requires surrender of firearms., Results: A total of 48 states (94%) explicitly allow minors to obtain CPOs. Fourteen states (27%) explicitly allow minors to file for a CPO on their own behalf. Forty-four states (86%) recognize dating relationships as qualifying relationships for a CPO. A CPO may be issued for physical violence in all 51 states (100%), sexual violence in 46 states (90%), threats of physical or sexual violence in 47 states (92%), stalking in 44 states (86%), and psychological/emotional violence in 28 states (55%). In 22 states (43%), an individual subject to a CPO is prohibited from possessing firearms and is required to surrender firearms they already have., Conclusions: There is a significant variation in states' CPO statutes related to adolescent IPV. Improvements in legislation that reduce the barriers to legal relief for those experiencing violence can be an important part of a comprehensive solution to reducing IPV among adolescents., (Copyright © 2019 Society for Adolescent Health and Medicine. Published by Elsevier Inc. All rights reserved.)