Neuropathological Findings in Susac Syndrome: An Autopsy Report
Cerebral vasculitis; Deafness; Encephalopathy; Susac syndrome; Visual loss
A 24-year-old woman developed encephalopathy, branch retinal artery occlusion, hearing loss, and had "snowball" lesions in the corpus callosum, classic findings of Susac syndrome (SuS). Despite intensive immunosuppressive therapy, she lapsed into a coma, and died 7 months after the onset of her illness. Neuropathological examination, revealed perivascular inflammation and vasculitis involving small vessels, associated with vascular narrowing and occlusion, and numerous microinfarcts diffusely throughout the brain. The findings establish SuS as a neuroinflammatory condition that can include vasculitis. This represents the most comprehensive report of the neuropathological findings in SuS.
Agamanolis Dimitri P; Klonk Collin; Bigley Kim; Rennebohm Robert M
Journal of Neuropathology and Experimental Neurology
2019
2019-06
<a href="http://doi.org/10.1093/jnen/nlz031" target="_blank" rel="noreferrer noopener">10.1093/jnen/nlz031</a>
Brain microvascular pathology in Susac syndrome: an electron microscopic study of five cases
hearing loss; Susac syndrome; electron microscopy; encephalopathy; branch retinal artery occlusion; cerebral vasculitis; corpus callosal lesions
Susac syndrome is a rare, immune-mediated disease characterized by encephalopathy, branch retinal artery occlusion, and hearing loss. Herein, we describe the electron microscopic findings of three brain biopsies and two brain autopsies performed on five patients whose working clinical diagnosis was Susac syndrome. In all five cases, the key findings were basement membrane thickening and collagen deposition in the perivascular space involving small vessels and leading to thickening of vessel walls, narrowing, and vascular occlusion. These findings indicate that Susac syndrome is a microvascular disease. Mononuclear cells were present in the perivascular space, underlining the inflammatory nature of the pathology. Though nonspecific, the changes can be distinguished from genetic and acquired small vessel diseases. The encephalopathy of Susac syndrome overlaps clinically with degenerative and infectious conditions, and brain biopsy may be used for its diagnosis. Its vascular etiology may not be obvious on light microscopy, and electron microscopy is important for its confirmation.
Agamanolis Dimitri P; Prayson Richard A; Asdaghi Negar; Gultekin Sakir H; Bigley Kim; Rennebohm Robert M
Ultrastructural Pathology
2019
2019-11-16
Journal Article
<a href="http://doi.org/10.1080/01913123.2019.1692117" target="_blank" rel="noreferrer noopener">10.1080/01913123.2019.1692117</a>
PMID: 31736417
Hepatitis C Sexual Partner Notification Required, Recommended, or Good Practice?
Alexander TS
Infectious Diseases In Clinical Practice
2021
2021-05
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1097/IPC.0000000000001025" target="_blank" rel="noreferrer noopener">10.1097/IPC.0000000000001025</a>
RE: The WISDOM Personalized Breast Cancer Screening Trial: Simulation Study to Assess Potential Bias and Analytic Approaches.
Carter Kimbroe; Castro Frank; Morcos Roy
JNCI cancer spectrum
2020
2020-04
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1093/jncics/pkaa015" target="_blank" rel="noreferrer noopener">10.1093/jncics/pkaa015</a>
A method for evaluating breast cancer screening strategies using screen-preventable loss of life.
The objective of this study is to describe how screen-preventable loss of life (screen-PLL) can be used to analyze the distribution of life savings with mammographic screening. The determination of screen-PLL with mammography is possible using a natural history model of breast cancer that simulates clinical and pathologic events of this disease. This investigation uses a Monte Carlo Markov model with data from the Surveillance, Epidemiology, and End Results Program; American Cancer Society; and National Vital Statistics System. Populations of one million women per screening strategy are simulated over a lifetime with mammographic screening based on current guidelines of the American Cancer Society (ACS), United States Preventive Services Task Force (USPSTF), triennial screening from age 50-70, and no screening. Screen-PLL curves are generated and show guideline performance over a lifetime. The screen-PLL curve with no screening is determined by tumor discovery through clinical awareness and has the highest values of screen-PLL. The ACS and USPSTF strategies demonstrate screen-PLL curves favoring the elderly. The curve for triennial screening is more uniform than the ACS or USPSTF curves but could be improved by adding screen(s) at either end of the 50-70 age range. This study introduces the use of screen-PLL as a tool to improve the understanding of screening guidelines and allowing a more balanced allocation of life savings across an aging population. The method presented shows how screen-PLL can be used to analyze and potentially improve breast cancer screening guidelines.
Carter KJ; Castro F; Morcos RN
Plos One
2020
1905-07
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1371/journal.pone.0243113" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0243113</a>
Are New beta-Lactam/beta-Lactamase Inhibitors Viable Carbapenem Sparing Options for Treating Serious Infections Caused by Extended-Spectrum beta-Lactamase-Producing Microorganisms?
Infectious Diseases; Immunology; escherichia-coli; susceptibility; cephalosporins
Kallstrom G
Infectious Diseases in Clinical Practice
2019
2019-05
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1097/ipc.0000000000000729" target="_blank" rel="noreferrer noopener">10.1097/ipc.0000000000000729</a>
Decreased Tumoral Expression of Colon-Specific Water Channel Aquaporin 8 Is Associated With Reduced Overall Survival in Colon Adenocarcinoma.
BACKGROUND: Colon cancer survival is dependent upon metastatic potential and treatment. Large RNA-sequencing datasets may assist in identifying colon cancer-specific biomarkers to improve patient outcomes. OBJECTIVE: To identify a highly specific biomarker for overall survival in colon adenocarcinoma using an RNA-sequencing data set. DESIGN: Raw RNA-sequencing and clinical data for patients with colon adenocarcinoma (n=269) were downloaded from The Cancer Genome Atlas. A binomial regression model was used to calculate differential RNA expression between paired colon cancer and normal epithelium samples (n=40). Highly differentially expressed RNAs were examined. SETTINGS: This study was conducted at the University of Louisville using data acquired by The Cancer Genome Atlas. PATIENTS: Patients from United States accredited cancer centers between 1998-2013 were analyzed. MAIN OUTCOME MEASURES: The primary outcome measures were recurrence-free and overall survival. RESULTS: The median age was 66 years (147/269 men, 180/269 Caucasian). Thirty RNAs were differentially expressed in colon adenocarcinoma compared to paired normal epithelium, using a log fold change cutoff of ±6. Using median expression as a cutoff, four RNAs were associated with worse overall survival: decreased ZG16 (log-rank=0.023), aquaporin8 (log-rank=0.023), and SLC26A3 (log-rank=0.098), and increased COL1A1(log-rank=0.105). On multivariable analysis, low aquaporin8 expression (HR=1.748, 95% CI: 1.016-3.008, p=0.044) was a risk factor for worse overall survival. Our final aquaporin8 model had an AUC of 0.85 for overall survival. On subgroup analysis, low aquaporin8 was associated with worse overall survival in MSI-H patients, and in stage II patients. Low aquaporin8 expression was associated with KRAS and BRAF mutations. Aquaporin8 immunohistochemistry was optimized for clinical application. LIMITATIONS: This was a retrospective study. CONCLUSION: Aquaporin8 is a water channel selectively expressed in normal colon tissue. Low AQP8 expression is a risk factor for worse overall survival in colon cancer patients. Aquaporin8 measurement may have a role as a colon-specific prognostic biomarker and help in patient risk stratification for increased surveillance.
O'Brien SJ; Kalbflesich T; Srivastava S; Pan J; Rai S; Petras RE; Ronquillo N; Polk HC; Galandiuk S
Diseases Of The Colon And Rectum
2021
2021-05-13
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1097/DCR.0000000000002071" target="_blank" rel="noreferrer noopener">10.1097/DCR.0000000000002071</a>
Streptococcus bovis Group Bacteremia in the 21st Century Review of 42 Episodes Over a 12-Year Period (2006-2017) at a Large Community Teaching Hospital
association; bacteremia; colonic neoplasia; consultation; endocarditis; identification; Immunology; Infectious Diseases; liver-disease; lutetiensis; mortality; Streptococcus; Streptococcus bovis group; Streptococcus gallolyticus; Streptococcus infantarius; Streptococcus pasteurianus; update
Introduction Advanced phenotypic, genomic, and proteomic laboratory techniques have recently modified Streptococcus bovis group (SBG) nomenclature. We wished to determine if physicians continue to recognize the importance of SBG and its association with gastrointestinal (GI) tract abnormalities and infective endocarditis amid the changes in microbiologic identification and nomenclature of these organisms. Methods We reviewed the medical records of adult patients (>= 18 years of age) with positive blood cultures for SBG organisms admitted to our 510-bed teaching hospital from January 1, 2006, to December 31, 2017. We report the epidemiology, sources of bacteremia, comorbid conditions, courses of treatment, and the mortality for these patients. We also assess the hospital treatment team's (HTT's) knowledge of SBG nomenclature and of the associations of SBG bacteremia and underlying GI disease and infective endocarditis amid the changes in nomenclature of these organisms. Results There were 42 cases of SBG bacteremia during the 12-year study period: 22 in women (52.4%) and 20 in men (47.6%). Patient ages ranged from 51 to 96 years (mean age, 74.3 years; median age, 72.0 years). All but 2 patients had multiple comorbid conditions. Diabetes mellitus was the most common comorbidity. Colonoscopy was performed during hospitalization in 22 (52.5%) of 42 patients. The identifiable sources of bacteremia were as follows: lower GI tract in 19 patients (45.2%), upper GI tract in 5 patients (11.9%), Laennec cirrhosis in 3 patients (7.1%), and pancreatic disorders in 2 patients (4.6%). Eleven patients (26.2%) had primary bacteremia. Two patients with primary bacteremia had prior splenectomy. The historic association between SBG bacteremia and underlying GI tract disease was recognized by 37 (88.1%) of 42 HTTs, but all available provider progress notes mention only "colon carcinoma" as the possibly associated GI tract pathology. The historic association of SBG bacteremia with infective endocarditis was recognized in writing by 32 (76.2%) of 42 HTTs. Endocarditis was diagnosed in 12 patients (28.6%): 9 definite endocarditis and 3 possible endocarditis. The mitral valve was the most commonly involved valve. Four SBG isolates were intermediately susceptible to penicillin G with minimum inhibitory concentrations of 0.125 mu g/mL or greater. Twenty-three (54.8%) of 42 SBG strains were resistant or intermediately susceptible to clindamycin. Twenty-four (57.1%) of 42 strains were resistant or intermediately susceptible to erythromycin. All strains were tested for susceptibility to ceftriaxone and vancomycin and retained susceptibility to both antimicrobial agents throughout the study period. Six of 42 patients died, for a mortality rate of 11.9%. Infectious disease consultation was obtained in 35 (80.0%) of 42 patients. Infectious disease consultation was positively associated with survival (P = 0.0041, Fisher exact test). The new nomenclature schemes for prior members of the SBG were recognized by all HTTs because our microbiology laboratory reported each member of the group, regardless of new name, with "bovis group" added to the identification on all positive culture reports. Conclusions Streptococcus bovis group bacteremia is a disease of older adults with all but 3 patients 60 years or older and a mean age at onset of 73.4 years. Most HTTs considered colon carcinoma as a possible source for and infective endocarditis as a potential complication of SBG bacteremia. However, most HTTs were not aware that SBG bacteremia could be associated with nonmalignant colonic lesions especially polyps, Laennec cirrhosis, or with pancreatic, biliary, and upper GI tract anatomic abnormalities. Of our SBG isolates, 54.8% were not sensitive to clindamycin. Clindamycin should not be used for empiric treatment of SBG bacteremia. The ID service should be consulted on all patients with SBG bacteremia because such consultation had a positive correlation with patient survival (P = 0.0041).
Sidda A; Kallstrom G; Myers J P
Infectious Diseases in Clinical Practice
2019
2019-01
Journal Article
<a href="http://doi.org/10.1097/ipc.0000000000000690" target="_blank" rel="noreferrer noopener">10.1097/ipc.0000000000000690</a>
A case report of mantle cell lymphoma presenting as intussuscepting colon mass.
Case report; Colo-colonic intussusception; Colo-colonic intussusception; follicular lymphoma; gastrointestinal-tract; Mantle cell lymphoma; polyposis; Primary GI lymphoma
INTRODUCTION: Mantle Cell Lymphoma (MCL) is a non-Hodgkin lymphoma accounting for 2.5% of lymphoid neoplasms in the United States. Primary gastrointestinal (GI) lymphomas account for 1-4% of all GI malignancies, with few reports of primary mantle cell lymphoma presenting as a single colonic mass and none to our knowledge with colon-colonic intussusception as the presenting finding. Accurate and timely diagnosis is imperative because MCL has rapid progression and early chemotherapeutic intervention results in improved patient outcomes. This work is reported in line with the SCARE criteria [1] for case report publication. PRESENTATION OF CASE: A 61-year-old male presented with 1 month history of nonspecific right sided abdominal pain. Computed Tomography (CT) of the abdomen identified an intussuscepting mass in the proximal ascending colon and an additional 8 mm hepatic lesion. Colonoscopy identified a large mass in the corresponding area of colon identified on CT. Histology and immunohistochemistry of biopsied specimen diagnosed MCL. DISCUSSION: Planned surgical intervention was deferred and the patient was referred for oncologic treatment. We report the first case to our knowledge of MCL presenting as colon-colonic intussusception and discuss the work-up of this rare lymphoma that clinicians may be required to diagnose and manage. CONCLUSION: This report serves as a reminder to maintain a broad differential inclusive of uncommon diseases and unanticipated pathology. Practicing with a thorough understanding of medical principles and clinical acumen is essential for optimal patient care and, as demonstrated in this case, preventing a potentially unnecessary surgical intervention thus delaying appropriate chemotherapy.
Smith Brandon M; Reilly Kyle; Baker Elena; Deeken Amy; Dan Adrian G
International journal of surgery case reports
2020
1905-07
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.1016/j.ijscr.2020.03.022" target="_blank" rel="noreferrer noopener">10.1016/j.ijscr.2020.03.022</a>