Publications by authors named "Elizabeth C Burton"

28 Publications

  • Page 1 of 1

Evaluation of a shared decision-making intervention for pediatric patients with asthma in the emergency department.

Implement Sci Commun 2020 4;1:22. Epub 2020 Mar 4.

Department of Family Medicine Research, Atrium Health, 2001 Vail Avenue, Suite 400B Mercy Medical Plaza, Charlotte, NC 28207 USA.

Background: Asthma is a difficult-to-manage chronic disease marked with associated outcome disparities including an increase rate of emergency department (ED) visits for uncontrolled asthma among patients who are most at-risk. Shared decision making (SDM) is a process by which the patient and provider jointly make a healthcare choice. SDM improves patient outcomes; however, implementation barriers of time constraints and staff availability are limitations. The use of health IT solutions may increase the adoption of SDM, but best practices for implementation are not well understood. The Consolidated Framework for Implementation Research (CFIR) is a flexible comprehensive model used to identify barriers and facilitators influencing implementation. The goal of this study is to implement an innovative web-based pediatric SDM tool in the real-world setting of two large healthcare system EDs through the following aims: (1) convene a patient, research, and ED stakeholder advisory board to oversee review of protocol and study materials prior to implementation, (2) implement the SDM intervention where providers and staff will be trained to incorporate use of this SDM intervention, (3) conduct on-going evaluation of barriers, facilitators, and implementation outcomes to tailor implementation in the EDs, (4) evaluate patient-centered outcomes of primary care utilization and changes in ED visits and hospitalizations before and after the SDM intervention, and (5) understand and document best practices for ED implementation.

Methods: The CFIR model will guide the implementation evaluation. Researchers will administer surveys to the clinical team and patients at baseline, 3, 6, and 12 months to inform implementation design, determine barriers and facilitators, and resource-needs to allow for real-time process adjustments within the EDs. Focus group or key-informant interviews and analysis will provide additional feedback to the stakeholder team to iterate the implementation process. Researchers will track patient-centered outcomes including increased primary care, ED, and inpatient utilization over the duration of the study.

Discussion: To advance asthma care and the field of implementation science, further research is needed to assess best practices for incorporating SDM into high-need healthcare settings such as the ED. This knowledge will facilitate improved outcomes and appropriate policy changes towards further use of SDM interventions in local and national acute care settings.
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http://dx.doi.org/10.1186/s43058-020-00010-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7427946PMC
March 2020

Fatal spontaneous Clostridium septicum gas gangrene: a possible association with iatrogenic gastric acid suppression.

Arch Pathol Lab Med 2014 Jun;138(6):837-41

From Johns Hopkins School of Medicine, Baltimore, Maryland (Ms Wu); and the Department of Pathology (Drs Baras, Cornish, Riedel, and Burton), Johns Hopkins Medicine, Baltimore, Maryland.

The long-term use of proton pump inhibitors has been linked to an increased risk for the development of gastric polyps, hip fractures, pneumonia, and Clostridium difficile colitis. There is evidence that chronic acid suppression from long-term use of proton pump inhibitors poses some risk for the development of C difficile-associated diarrhea by decreasing the elimination of pathogenic microbes before reaching the lower gastrointestinal tract. Here we present a case of a 51-year-old woman with a recent history of abdominal pain and fever who presented to the emergency department with rapidly progressive spontaneous necrotizing fasciitis and gas gangrene and died within hours of presentation. Postmortem examination confirmed spreading tissue gas gangrene and myonecrosis. In addition, multiple intestinal ulcers containing Clostridium septicum were present at autopsy. This case illustrates a possible association between proton pump inhibitor therapy and fatal C septicum infection.
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http://dx.doi.org/10.5858/arpa.2013-0104-CRDOI Listing
June 2014

Defects in laterality with emphasis on heterotaxy syndromes with asplenia and polysplenia: an autopsy case series at a single institution.

Pediatr Dev Pathol 2014 Jul-Aug;17(4):250-64. Epub 2014 Apr 15.

1  Johns Hopkins Medicine, Department of Pathology, Autopsy Division, Pathology building, Room B106, 600 N. Wolfe Street, Baltimore, MD 21287-6417.

Heterotaxy is a rare disease with high morbidity and mortality. Controversy exists over how to classify these syndromes with most cases stratified into asplenia/polysplenia syndromes or right/left isomerism. In an effort to review comprehensively specific pheonotypes associated with heterotaxy syndromes, we reviewed published cases series, adopted a classification scheme based on spleen status, and evaluated autopsy cases retrospectively with abnormal laterality at our institution. We categorized 116 cases as situs inversus totalis, polysplenia, asplenia, and single right-sided spleen. Cardiovascular abnormalities occurred in 87.1% of polysplenia, 90.5% of asplenia, and all cases of single right-sided spleen. For polysplenia, 48.9% had bilateral bilobed lungs, 87% had right-sided stomach, 58.1% had midline symmetric liver, and 60.4% had malrotated intestines. For asplenia, 51.9% had bilateral trilobed lungs, 86.7% had right-sided stomach, 45.8% had symmetric liver, and 65.5% had malrotated intestines. Atrioventricular septal defects occurred in 91.2% of asplenia compared to 56.8% of polysplenia cases. Eight percent had pulmonary/aortic stenosis or atresia. Double outlet right ventricle was more common in polysplenia (32.6%) compared to asplenia (21.4%). Total anomalous systemic venous return was described in 55.6% of polysplenia and total anomalous pulmonary venous connections in 81% of asplenia cases. Greater than half of the cases had no heterotaxy diagnosis. Although, we found similar heterotaxy-associated characteristics, the frequencies differed from previous studies. We found great variation in how heterotaxy-associated defects were described, diagnosed, and reported. Although there are known associated characteristics with the polysplenia/asplenia syndromes, correct identification requires a standardized approach for diagnosis and reporting.
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http://dx.doi.org/10.2350/13-11-1406-OA.1DOI Listing
October 2014

Fetal death resulting from an isolated congenital partial pericardial defect.

Obstet Gynecol 2014 Feb;123(2 Pt 2 Suppl 2):443-446

Department of Pathology, Autopsy Division, Johns Hopkins Hospital, and the Department of Gynecology and Obstetrics, Maternal Fetal Medicine Division and the Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Background: Congenital pericardial defects occur from a defect in the formation of the pleuropericardial membrane during embryonic development. This defect may be asymptomatic but can be fatal if complicated by herniation of any portion of the heart.

Cases: We report two cases in which herniation of a portion of the heart occurred through a partial left pericardial defect and resulted in fetal death. In case one, there were no fetal symptoms, and in case two, an irregular heartbeat was detected prompting a fetal echocardiogram that was negative for heart abnormalities.

Conclusion: Although isolated congenital pericardial defects are rare, they can result in fetal death. Awareness may help to refine ultrasonography or other diagnostic modalities to evaluate possible congenital pericardial defects in utero.
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http://dx.doi.org/10.1097/AOG.0000000000000000DOI Listing
February 2014

AIRP best cases in radiologic-pathologic correlation: cerebral fat embolism syndrome in sickle cell β-thalassemia.

Radiographics 2012 Sep-Oct;32(5):1301-6

Department of Radiology, Baylor University Medical Center, Dallas, TX 75246, USA.

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http://dx.doi.org/10.1148/rg.325115055DOI Listing
March 2013

To image or to autopsy?

Ann Intern Med 2012 Jan;156(2):158-9

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http://dx.doi.org/10.7326/0003-4819-156-2-201201170-00014DOI Listing
January 2012

Thymic stromal lymphopoietin fosters human breast tumor growth by promoting type 2 inflammation.

J Exp Med 2011 Mar 21;208(3):479-90. Epub 2011 Feb 21.

Baylor Institute for Immunology Research, Baylor Research Institute, Dallas, TX 75204, USA.

The human breast tumor microenvironment can display features of T helper type 2 (Th2) inflammation, and Th2 inflammation can promote tumor development. However, the molecular and cellular mechanisms contributing to Th2 inflammation in breast tumors remain unclear. Here, we show that human breast cancer cells produce thymic stromal lymphopoietin (TSLP). Breast tumor supernatants, in a TSLP-dependent manner, induce expression of OX40L on dendritic cells (DCs). OX40L(+) DCs are found in primary breast tumor infiltrates. OX40L(+) DCs drive development of inflammatory Th2 cells producing interleukin-13 and tumor necrosis factor in vitro. Antibodies neutralizing TSLP or OX40L inhibit breast tumor growth and interleukin-13 production in a xenograft model. Thus, breast cancer cell-derived TSLP contributes to the inflammatory Th2 microenvironment conducive to breast tumor development by inducing OX40L expression on DCs.
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http://dx.doi.org/10.1084/jem.20102131DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3058586PMC
March 2011

Carcinoid heart disease without the carcinoid syndrome but with quadrivalvular regurgitation and unsuccessful operative intervention.

Am J Cardiol 2011 Mar;107(5):788-92

Department of Pathology, Baylor University Medical Center, Dallas, TX, USA.

A 53-year-old woman is described who underwent mitral and aortic valve replacement and tricuspid valve annuloplasty for pure regurgitation at all 3 valve sites for unrecognized carcinoid heart disease without the carcinoid syndrome 22 days before death. Metastatic carcinoid was not recognized until necropsy, which disclosed a probable ovarian primary but with large hepatic metastases and left-sided cardiac involvement either greater than or equal to the right-sided involvement. Pulmonary hypertension, very unusual in carcinoid heart disease, persisted postoperatively and probably played a role in the patient's early death. Hepatic metastasis with ovarian primary is most unusual in this circumstance.
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http://dx.doi.org/10.1016/j.amjcard.2010.10.064DOI Listing
March 2011

Embedding measurement within existing computerized data systems: scaling clinical laboratory and medical records heart failure data to predict ICU admission.

J Appl Meas 2010 ;11(3):271-87

LivingCapitalMetrics.com, Sausalito, CA 94965, USA.

This study employs existing data sources to develop a new measure of intensive care unit (ICU) admission risk for heart failure patients. Outcome measures were constructed from laboratory, accounting, and medical record data for 973 adult inpatients with primary or secondary heart failure. Several scoring interpretations of the laboratory indicators were evaluated relative to their measurement and predictive properties. Cases were restricted to tests within first lab draw that included at least 15 indicators. After optimizing the original clinical observations, a satisfactory heart failure severity scale was calibrated on a 0-1000 continuum. Patients with unadjusted CHF severity measures of 550 or less were 2.7 times more likely to be admitted to the ICU than those with higher measures. Patients with low HF severity measures (550 or less) adjusted for demographic and diagnostic risk factors are about six times more likely to be admitted to the ICU than those with higher adjusted measures. A nomogram facilitates routine clinical application. Existing computerized data systems could be programmed to automatically structure clinical laboratory reports using the results of studies like this one to reduce data volume with no loss of information, make laboratory results more meaningful to clinical end users, improve the quality of care, reduce errors and unneeded tests, prevent unnecessary ICU admissions, lower costs, and improve patient satisfaction. Existing data typically examined piecemeal form a coherent scale measuring heart failure severity sensitive to increased likelihood of ICU admission. Marked improvements in ROC curves were found for the aggregate measures relative to individual clinical indicators.
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October 2010

Full blown cardiovascular syphilis with aneurysm of the innominate artery.

Am J Cardiol 2009 Dec 16;104(11):1595-600. Epub 2009 Sep 16.

Department of Internal Medicine, Division of Cardiology, Baylor University Medical Center, Dallas, Texas, USA.

The investigators report the case of a 44-year-old man who presented acutely and was found to have saccular aneurysm of the innominate artery, narrowed or totally occluded aortic arch arteries, and marked thickening of the thoracic aorta except for the wall behind the sinuses of Valsalva. The abdominal aorta was entirely normal. Results of the serologic test for syphilis were strongly positive. Because cardiovascular syphilis appears to be a disease that affects the vasa vasora and because these channels are limited to the thoracic aorta, the abdominal aorta is uninvolved, as demonstrated so nicely in the patient described in this case report. Because most patients with cardiovascular syphilis are much older than the patient described, it is unusual to see a perfectly normal abdominal aorta, as in the present patient. In conclusion, syphilis producing aneurysm of the innominate artery is unusual but is always associated with syphilitic involvement of the thoracic aorta.
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http://dx.doi.org/10.1016/j.amjcard.2009.06.070DOI Listing
December 2009

Diagnosing cardiac sarcoidosis clinically without tissue confirmation.

Proc (Bayl Univ Med Cent) 2009 Jul;22(3):236-8

Department of Internal Medicine, Baylor University Medical Center, Dallas, Texas, USA.

A 52-year-old woman is described in whom cardiac sarcoidosis was diagnosed clinically on the basis of high-grade heart block, recurring ventricular tachyarrhythmias, and acute cardiac decompensation. Confirmation of sarcoidosis was not determined until necropsy. When it causes cardiac dysfunction, sarcoidosis rarely causes dysfunction of another body organ, although small sarcoid granulomas may also be present in noncardiac organs or tissues. In the present patient, neurosarcoidosis accompanied the cardiac sarcoidosis, but it was the latter that produced the life-threatening symptoms and was fatal.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2709088PMC
http://dx.doi.org/10.1080/08998280.2009.11928525DOI Listing
July 2009

Pathologic findings in larval and juvenile anurans inhabiting farm ponds in Tennessee, USA.

J Wildl Dis 2009 Apr;45(2):314-24

University of Georgia, College of Veterinary Medicine, Tifton, Georgia 31793, USA.

Amphibian populations are declining globally, yet general pathologic surveys for free-ranging amphibians are uncommon. Pathologic surveys are necessary to provide insight into the impacts of humans on emergence of pathogens in amphibian populations. During 2005, 104 American bullfrog (Rana catesbeiana) and 80 green frog (Rana clamitans) larvae and 40 green frog juveniles were collected from farm ponds in Tennessee, and complete necropsies were performed. Diagnostic testing included bacterial culture, virus testing, fecal parasite analysis, and histologic examination. Gross and histologic examination revealed that all individuals, except one bullfrog tadpole, could be classified as clinically normal. The clinically abnormal tadpole had swollen erythemic legs, and was positive for Aeromonas hydrophila but negative for Ranavirus. Parasites were common (43%) among specimens, with myxosporidium and trematodes most often noted. Commensal and opportunistic microorganisms were cultured from the tissues. Ranavirus was detected in 29% of individuals but generally not associated with significant histopathologic changes. Myxosporidia and Ranavirus coinfections occurred in 7 and 26% of green and bullfrog tadpoles, respectively, with the highest coinfection rate (83%) in bullfrog tadpoles during winter. Protozoans were most common in fecal examination. These data can serve as a baseline to evaluate the presence of clinical disease in larval and juvenile amphibians.
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http://dx.doi.org/10.7589/0090-3558-45.2.314DOI Listing
April 2009

Disseminated toxoplasmosis in a patient with undiagnosed AIDS.

Proc (Bayl Univ Med Cent) 2009 Jan;22(1):20-2

Department of Pathology, Baylor University Medical Center, Dallas, Texas, USA.

Approximately 60% of deaths in patients with AIDS are a direct result of infection other than HIV. The more severe and life-threatening complications of HIV infection occur in patients with a CD4(+) T cell count <200 cells/μL. In the absence of effective retroviral therapy, these infections are associated with a high mortality. We describe a case of disseminated toxoplasmosis discovered at autopsy in a patient with undiagnosed AIDS.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2626354PMC
http://dx.doi.org/10.1080/08998280.2009.11928463DOI Listing
January 2009

Pulmonary invasive mucormycosis in a patient with secondary iron overload following deferoxamine therapy.

Proc (Bayl Univ Med Cent) 2008 Oct;21(4):378-81

Departments of Internal Medicine, Baylor University Medical Center, Dallas, Texas, USA.

Mucormycosis (zygomycosis) is an acute and often fatal opportunistic fungal infection. Predisposing factors in the development of mucormycosis are nonspecific and include hyperglycemia, hematologic malignancies, neutropenia, pharmacologic immunosuppression, solid organ or bone marrow/stem cell transplantation, burns, trauma, malnutrition, and intravenous drug use. Mucormycosis has also been described in patients with iron and aluminum overload, patients on dialysis, and patients receiving iron chelating therapy. We describe a 75-year-old man with myelodysplastic syndrome and iron overload secondary to multiple red blood cell transfusions who had been treated with deferoxamine chelation therapy. He was admitted to the hospital for atrial fibrillation, developed multiple organ failure, and died. Pulmonary invasive mucormycosis was demonstrated at autopsy. This case further documents an association between invasive mucormycosis, iron overload, and deferoxamine therapy.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2566908PMC
http://dx.doi.org/10.1080/08998280.2008.11928431DOI Listing
October 2008

The vanishing nonforensic autopsy.

N Engl J Med 2008 Feb;358(9):873-5

University of Ottawa and Ottawa Health Research Institute, Ottawa.

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http://dx.doi.org/10.1056/NEJMp0707996DOI Listing
February 2008

Amphibian ocular malformation associated with frog virus 3.

Vet J 2008 Sep 2;177(3):442-4. Epub 2007 Jul 2.

The University of Tennessee, Department of Forestry, Wildlife and Fisheries, Center for Wildlife Health, Knoxville, TN 37996, USA.

During an on-going amphibian ecology study, a free-ranging American bullfrog (Rana catesbeiana) metamorph was captured in a pitfall trap adjacent to a constructed farm pond at the Plateau Research and Education Center (PREC) on the Cumberland Plateau near Crossville, Tennessee, USA. Grossly, the right eye was approximately 50% the size of the left. Stereo and light microscopic examination revealed two granulomas within the orbit. Electron microscopic examination revealed virus particles scattered throughout one structure but mostly aggregated toward the center. Subsequent PCR and sequencing (GenBank accession Number EF175670) confirmed frog virus 3 (FV3). This represents the first report of a malformation in an anuran associated with FV3.
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http://dx.doi.org/10.1016/j.tvjl.2007.05.006DOI Listing
September 2008

Preliminary evidence that American bullfrogs (Rana catesbeiana) are suitable hosts for Escherichia coli O157:H7.

Appl Environ Microbiol 2007 Jun 20;73(12):4066-8. Epub 2007 Apr 20.

The University of Tennessee, Department of Forestry, Wildlife and Fisheries, Knoxville 37996, USA.

We orally inoculated Rana catesbeiana tadpoles (n=23) and metamorphs (n=24) to test their suitability as hosts for Escherichia coli O157:H7. Tadpoles were housed in flowthrough aquaria and did not become infected. Metamorphs were housed in stagnant aquaria, and 54% tested positive through 14 days postinoculation, suggesting that they are suitable hosts for E. coli O157:H7.
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http://dx.doi.org/10.1128/AEM.02905-06DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1932734PMC
June 2007

Breast cancer instructs dendritic cells to prime interleukin 13-secreting CD4+ T cells that facilitate tumor development.

J Exp Med 2007 May 16;204(5):1037-47. Epub 2007 Apr 16.

Baylor Institute for Immunology Research and Baylor National Institute of Allergy and Infectious Diseases Cooperative Center for Translational Research on Human Immunology and Biodefense, Baylor University Medical Center, Dallas, TX 75204, USA.

We previously reported (Bell, D., P. Chomarat, D. Broyles, G. Netto, G.M. Harb, S. Lebecque, J. Valladeau, J. Davoust, K.A. Palucka, and J. Banchereau. 1999. J. Exp. Med. 190: 1417-1426) that breast cancer tumors are infiltrated with mature dendritic cells (DCs), which cluster with CD4(+) T cells. We now show that CD4(+) T cells infiltrating breast cancer tumors secrete type 1 (interferon gamma) as well as high levels of type 2 (interleukin [IL] 4 and IL-13) cytokines. Immunofluorescence staining of tissue sections revealed intense IL-13 staining on breast cancer cells. The expression of phosphorylated signal transducer and activator of transcription 6 in breast cancer cells suggests that IL-13 actually delivers signals to cancer cells. To determine the link between breast cancer, DCs, and CD4(+) T cells, we implanted human breast cancer cell lines in nonobese diabetic/LtSz-scid/scid beta2 microglobulin-deficient mice engrafted with human CD34(+) hematopoietic progenitor cells and autologous T cells. There, CD4(+) T cells promote early tumor development. This is dependent on DCs and can be partially prevented by administration of IL-13 antagonists. Thus, breast cancer targets DCs to facilitate its development.
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http://dx.doi.org/10.1084/jem.20061120DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2118566PMC
May 2007

Attitudes toward the autopsy--an 8-state survey.

MedGenMed 2006 Sep 21;8(3):80. Epub 2006 Sep 21.

Strategy and Business Economics Division, Sauder School of Business, University of British Columbia, Vancouver, British Columbia, Canada.

Context: National autopsy rates have declined for several decades, and the reasons for such decline remain contentious.

Objective: To elicit the opinions of one group of crucial decision makers as to the reasons for this decline and possible modes of reversal.

Design: A 2-part survey, composed of multiple choice questions and questions requesting specific data on autopsy rates and costs.

Setting: Illinois, Iowa, Louisiana, Minnesota, Nebraska, North Dakota, South Dakota, and Wisconsin.

Participants: Hospital administrators within the 8 states.

Main Outcome Measures: Six-point survey scale relating to reasons for autopsy decline and possible remedial measures, as well as estimates of autopsy rates and costs.

Results: The response rate was 43% and the median autopsy rate was 2.4% (mean 6.1%). The median cost of autopsy was estimated at $852 (mean $1275). Larger hospitals were associated with higher autopsy rates than smaller hospitals (9.6% vs 4.0%), and teaching hospitals had a significantly higher autopsy rate than nonteaching institutions (11.4% vs 3.8%). Autopsy rates also varied by type of hospital control, with federal government hospitals having the highest autopsy rate at 15.1%. Sixty-six percent of all respondents agreed that current autopsy rates were adequate. Of the respondents, the highest percent (86%) agreed that improved diagnostics contributed to the decline in autopsies, and the highest percent (78%) agreed that direct payment to pathologists for autopsies under the physician fee schedule might lead to an increase in autopsies.

Conclusions: Our data support the conclusion that the decline in autopsy performance is multifactorial, although the variable that dominates in this analysis is the contentious perception that improved diagnostic technology renders the autopsy redundant. The rate of autopsy is conditional, at least in part, on individual hospital characteristics such as large hospital size, teaching status, and federal ownership. Three underlying factors may explain these associations: resources, mission, and case mix. An important factor in declining autopsy rates appears to be the changing economic landscape, with its increased focus on cost control within both the public and private healthcare sectors.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1781284PMC
September 2006

Disseminated aspergillosis following infliximab therapy in an immunosuppressed patient with Crohn's disease and chronic hepatitis C: a case study and review of the literature.

MedGenMed 2005 Sep 21;7(3). Epub 2005 Sep 21.

Department of Pathology, Baylor University Medical Center, Dallas, Texas, USA.

A 55-year-old white woman with a greater than 25-year history of Crohn's disease developed disseminated aspergillosis following combination therapy with Methylprednisolone, azathioprine, and infliximab. The patient was hospitalized 11 days after initiation of infliximab for respiratory symptoms and developed respiratory failure, coma, and died. Postmortem examination revealed disseminated Aspergillus fumigatus involving multiple organs. This case demonstrates that combined treatment with infliximab, methylprednisone, and azathioprine may induce severe immunosuppression and depressed cellular immunity, leading to severe opportunistic infections. Given the increasing use of antitumor necrosis factor agents, physicians should be aware of the risk of opportunistic infections and be vigilant about diagnosing and aggressively treating these infections to reduce the risk of disseminated disease.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1681636PMC
September 2005

Rabies encephalomyelitis: clinical, neuroradiological, and pathological findings in 4 transplant recipients.

Arch Neurol 2005 Jun;62(6):873-82

Department of Pathology and Laboratory Medicine, Baylor University Medical Center, Dallas, Tex 75246, USA.

Background: Three patients received solid organ transplants from a common donor and were subsequently discharged from the hospital following an uneventful hospital course. Within 30 days, all 3 organ recipients returned to the hospital with varying symptoms that progressed to rapid neurological deterioration, coma, and death.

Objective: To describe the clinical, neuroradiological, and pathological findings of rabies virus infection in organ transplant recipients infected from a common donor.

Design: Case series involving a common donor and 3 organ recipients ascertained through review of clinical course and autopsy findings. A fourth case was determined by review of pending autopsy cases in which death occurred within the same time interval. Portions of postmortem central nervous system and organ tissues were frozen and formalin-fixed. Fluids and tissues were also collected for cultures, serology, and molecular studies. Postmortem fluids and tissues and antemortem fluids and tissues from all 4 transplant recipients and serum and banked lymphocyte or spleen cells from the donors were sent to the Centers for Disease Control and Prevention for further evaluation.

Setting: Transplant unit of an urban teaching hospital.

Results: Antemortem cerebrospinal fluid analysis for 3 of the 4 recipients was consistent with a viral etiology. Neuroimaging and electroencephalogram studies were suggestive of an infectious encephalitis or a toxic encephalopathy. Initial laboratory testing did not demonstrate an infectious etiology. Postmortem histologic analysis, immunohistochemistry, electron microscopy, and direct fluorescence antibody testing revealed rabies virus infection. Serological testing done postmortem confirmed rabies virus infection in the common donor.

Conclusions: These cases demonstrate a risk for transmitting rabies virus infection through solid organ and tissue transplantation, and this diagnosis should be considered in any rapidly progressing neurological disease.
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http://dx.doi.org/10.1001/archneur.62.6.873DOI Listing
June 2005

Transmission of rabies virus from an organ donor to four transplant recipients.

N Engl J Med 2005 Mar;352(11):1103-11

Division of Healthcare Quality Promotion, National Center for Infectious Diseases, and the Epidemic Intelligence Service Branch, Centers for Disease Control and Prevention, Atlanta, USA.

Background: In 2004, four recipients of kidneys, a liver, and an arterial segment from a common organ donor died of encephalitis of an unknown cause.

Methods: We reviewed the medical records of the organ donor and the recipients. Blood, cerebrospinal fluid, and tissues from the recipients were tested with a variety of assays and pathological stains for numerous causes of encephalitis. Samples from the recipients were also inoculated into mice.

Results: The organ donor had been healthy before having a subarachnoid hemorrhage that led to his death. Encephalitis developed in all four recipients within 30 days after transplantation and was accompanied by rapid neurologic deterioration characterized by agitated delirium, seizures, respiratory failure, and coma. They died an average of 13 days after the onset of neurologic symptoms. Mice inoculated with samples from the affected patients became ill seven to eight days later, and electron microscopy of central nervous system (CNS) tissue demonstrated rhabdovirus particles. Rabies-specific immunohistochemical and direct fluorescence antibody staining demonstrated rabies virus in multiple tissues from all recipients. Cytoplasmic inclusions consistent with Negri bodies were seen in CNS tissue from all recipients. Antibodies against rabies virus were present in three of the four recipients and the donor. The donor had told others of being bitten by a bat.

Conclusions: This report documenting the transmission of rabies virus from an organ donor to multiple recipients underscores the challenges of preventing and detecting transmission of unusual pathogens through transplantation.
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http://dx.doi.org/10.1056/NEJMoa043018DOI Listing
March 2005

The relation of autopsy rate to physicians' beliefs and recommendations regarding autopsy.

Am J Med 2004 Aug;117(4):255-61

Department of Pathology, Baylor University Medical Center, Dallas, Texas 75246, USA.

Purpose: Multiple factors have affected the decline in autopsy rates. Our goal was to determine the relation of physicians' recommendations regarding autopsy, as well as patient and surrogate decision-maker characteristics, to autopsy performance.

Methods: We assessed measures related to autopsy performance using data from two teaching institutions in the Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatments. We included patients who had died within 6 months of their index hospitalization and for whom information was available on autopsy performance, physicians' response to questions about autopsy, and interviews with surrogate decision makers about autopsy performance. We assessed the association between autopsy performance and the strength of a physician's recommendation for autopsy, adjusting for patient, surrogate, and physician characteristics.

Results: Of the 680 patients who died, 59% (n = 402) met our inclusion criteria. Based on physician and surrogate responses, the expected autopsy rate was 42% while the actual autopsy rate was 23%. The autopsy rate was higher when the physician's recommendation for autopsy was strong or very strong at the time of death compared with when autopsy was not recommended strongly or not at all (P <0.001). The strength of the physician's postmortem recommendation was independently associated with autopsy performance after adjusting for patient, surrogate, and physician characteristics (P <0.001).

Conclusion: Autopsies are less likely to be performed when not recommended strongly or not at all. Training physicians (or others) how to recommend autopsies may increase autopsy rates.
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http://dx.doi.org/10.1016/j.amjmed.2004.01.028DOI Listing
August 2004

The persistent value of the autopsy.

Am Fam Physician 2004 Jun;69(11):2540-2

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June 2004

Fatal mesenteric fibromuscular dysplasia: a case report and review of the literature.

Arch Intern Med 2004 May;164(10):1148-53

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, TX, USA.

Fibromuscular dysplasia is a rare nonatherosclerotic, noninflammatory angiopathy of uncertain etiology and high morbidity. Because of its propensity to affect medium-sized vessels in a variety of locations, presenting symptoms may vary substantially, resulting in a delayed or missed diagnosis. We describe a 57-year-old woman who, on multiple occasions, presented with progressive gastrointestinal symptoms and eventually underwent surgical revascularization for celiac and superior mesenteric artery stenosis of uncertain etiology. Her postoperative course was complicated by bowel ischemia, multiple organ failure, and death. Autopsy findings proved useful in determining the underlying disease process and cause of death. This case report and a review of the literature illustrate the high morbidity and mortality that are caused by mesenteric fibromuscular dysplasia, the challenge in establishing a correct diagnosis, and the importance of early detection and treatment.
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http://dx.doi.org/10.1001/archinte.164.10.1148DOI Listing
May 2004

Intravascular thrombosis and thromboembolism during liver transplantation: antifibrinolytic therapy implicated?

Liver Transpl 2004 Feb;10(2):310-4

Department of Anesthesiology and Pain Management, Baylor Regional Transplant Institute, Baylor University Medical Center, Dallas, TX 75246, USA.

This case report describes a patient who underwent orthotopic liver transplantation and developed extensive hyperacute venous and arterial intravascular thromboses and thromboemboli intraoperatively. The patient was receiving antifibrinolytic therapy with aprotinin. The safety of routine aprotinin therapy in liver transplantation is examined. The value of the thrombelastograph (TEG) as a qualitative assessment of the coagulation system is emphasized.
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http://dx.doi.org/10.1002/lt.20064DOI Listing
February 2004

Changes in rates of autopsy-detected diagnostic errors over time: a systematic review.

JAMA 2003 Jun;289(21):2849-56

Department of Medicine, University of California, San Francisco, CA 94143, USA.

Context: Substantial discrepanies exist between clinical diagnoses and findings at autopsy. Autopsy may be used as a tool for quality management to analyze diagnostic discrepanies.

Objective: To determine the rate at which autopsies detect important, clinically missed diagnoses, and the extent to which this rate has changed over time.

Data Sources: A systematic literature search for English-language articles available on MEDLINE from 1966 to April 2002, using the search terms autopsy, postmortem changes, post-mortem, postmortem, necropsy, and posthumous, identified 45 studies reporting 53 distinct autopsy series meeting prospectively defined criteria. Reference lists were reviewed to identify additional studies, and the final bibliography was distributed to experts in the field to identify missing or unpublished studies.

Study Selection: Included studies reported clinically missed diagnoses involving a primary cause of death (major errors), with the most serious being those likely to have affected patient outcome (class I errors).

Data Extraction: Logistic regression was performed using data from 53 distinct autopsy series over a 40-year period and adjusting for the effects of changes in autopsy rates, country, case mix (general autopsies; adult medical; adult intensive care; adult or pediatric surgery; general pediatrics or pediatric inpatients; neonatal or pediatric intensive care; and other autopsy), and important methodological features of the primary studies.

Data Synthesis: Of 53 autopsy series identified, 42 reported major errors and 37 reported class I errors. Twenty-six autopsy series reported both major and class I error rates. The median error rate was 23.5% (range, 4.1%-49.8%) for major errors and 9.0% (range, 0%-20.7%) for class I errors. Analyses of diagnostic error rates adjusting for the effects of case mix, country, and autopsy rate yielded relative decreases per decade of 19.4% (95% confidence interval [CI], 1.8%-33.8%) for major errors and 33.4% (95% [CI], 8.4%-51.6%) for class I errors. Despite these decreases, we estimated that a contemporary US institution (based on autopsy rates ranging from 100% [the extrapolated extreme at which clinical selection is eliminated] to 5% [roughly the national average]), could observe a major error rate from 8.4% to 24.4% and a class I error rate from 4.1% to 6.7%.

Conclusion: The possibility that a given autopsy will reveal important unsuspected diagnoses has decreased over time, but remains sufficiently high that encouraging ongoing use of the autopsy appears warranted.
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http://dx.doi.org/10.1001/jama.289.21.2849DOI Listing
June 2003

The autopsy: a professional responsibility in assuring quality of care.

Am J Med Qual 2002 Mar-Apr;17(2):56-60

Louisiana State University Health Sciences Center, Department of Pathology, New Orleans, LA, USA.

Forty years ago, the value of autopsies was widely recognized as new diseases were discovered or clarified and scientific technology advanced greatly. Despite the autopsy's strong foundation, its value is not currently being properly conveyed to physicians or patients. Although autopsy-related policy exists, these policies have had little effect on increasing or even maintaining adequate autopsy rates. More recently, the autopsy has fallen on hard times, with US hospital rates now below 5%. The reasons for the decline in rates are multifaceted and include a lack of direct reimbursement for the procedure, lack of defined minimum rate standards, overconfidence in diagnostic technology, and the fear of litigation. Regardless of the reasons for the declining rates, the ethical and professional reasons for increasing the number of autopsies are far more important.
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http://dx.doi.org/10.1177/106286060201700203DOI Listing
April 2002
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